A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of IgPro20 (Subcutaneous Immunoglobulin, Hizentra®) in Adults With Dermatomyositis (DM) - The RECLAIIM Study
Phase: PHASE3
Conditions: Dermatomyositis
Sponsor: CSL Behring

Protocol version: Amendment 4 
 
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CLINICAL STUDY PROTOCOL 
 
A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of IgPro20 
(Subcutaneous Immunoglobulin, Hizentra®) in Adults with Dermatomyositis (DM) – 
The RECLAIIM Study 
Study Number: 
IgPro20_3007 
Study Product: 
IgPro20 (Hizentra®) 
Development Phase: 
Phase 3 
Sponsor: 
CSL Behring, LLC 
1020 First Avenue 
King of Prussia, PA 
United States of America 
Protocol Version: 
Amendment 4  
EudraCT Number: 
2018-003171-35 
IND Number: 
18942 
Protocol Date: 
20 October 2022 
Compliance: 
This study will be conducted in accordance with standards of 
Good Clinical Practice (as defined by the International Council 
for Harmonisation), ethical principles that have their origin in 
the Declaration of Helsinki, and all applicable national and local 
regulations. 
Commercial in Confidence. This document and the information contained herein are proprietary and 
confidential. This document and the contained information are intended for disclosure to and use by those 
personnel who are under an obligation of confidentiality by a signed agreement with Sponsor (“CSL Behring”). 
This document and the contained information may be disclosed and used only to the extent necessary to conduct 
the clinical study. Reproduction or disclosure of this document or its contained information is forbidden unless 
at the express request or with the written consent of CSL Behring. 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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LIST OF PERSONNEL AND ORGANIZATIONS RESPONSIBLE FOR CONDUCT 
OF THE STUDY 
A list of personnel and organizations responsible for the conduct of the study will be supplied 
to study sites as part of the Investigator’s Study File. This list will be updated by 
CSL Behring (or delegate) and provided to the study sites as needed. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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REVISION HISTORY 
Date 
Version  
Summary of Changes 
07 March 2019 
Original 
Not applicable 
07 June 2019 
Amendment 1 
• Modification of study title 
• Addition of text regarding identified risk of 
TEE in treatment population, including 
new exclusion criteria, monitoring, 
Adverse Events of Special Interest, and 
study stopping rules 
• Addition of details regarding oral 
corticosteroid treatment, rescue, taper, and 
monitoring 
• Addition of key secondary endpoint for 
reduction of concomitant corticosteroid  
• Addition and clarification of objective 
disease measures for eligibility and 
stratification  
• Definition of clinically relevant 
improvement 
• Addition of monitoring for hemolysis 
• Addition of rationale of PK timing and 
addition of PK sample 
• Minor corrections, clarifications, and 
administrative changes including the 
addition of a Weight-based Dosing 
Regimen table 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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05 February 2020 
Amendment 2 
• Clarification of Exclusion Criteria 1 and 2 
• Modification of definition of clinically 
relevant improvement 
• Deletion of permitted training on the 
infusion device at home 
• Removal of Definition of Worsening 
(DOW) assessments from Week 29 to End 
of Study (EOS) 
• Modification of list of autoantibody testing 
• Addition of allowance for drawing PK 
samples at home  
• Addition of specific reasons for which 
investigational medicinal product (IMP) 
must be discontinued 
• Clarification to the DOW and oral rescue 
corticosteroid treatment 
• Clarification to the concomitant oral 
corticosteroid tapering guidelines 
• Modification of acceptable methods of 
contraception 
• Modification to monitoring for 
thromboembolic events (TEEs) 
• Addition of detail about hemolysis grading 
• Update to the approximate volume of blood 
drawn from each subject 
• Addition of analysis sets 
• Minor corrections and clarifications, 
including word modifications and 
administrative changes 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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21 July 2020 
Amendment 3 
• Addition of Study Period 3 to assess the 
safety and efficacy of long-term treatment 
with IgPro20 for DM. 
• Addition of safety, efficacy, and 
exploratory endpoints associated with the 
addition of Study Period 3  
• Modified stopping rules and monitoring 
related to TEEs 
• Adjusted language in blinding procedures 
to account for Period 3 
• Added detail to contraindications 
• Clarified lifestyle restrictions with relation 
to physiotherapy 
• Adjusted the Study Procedures and Visit 
Schedule for Study Period 3 
• Adjusted language for blood and PK 
sample collection 
• Minor corrections and clarifications, 
including word modifications and 
administrative changes 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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Clinical Study Protocol Synopsis 
 
Title 
A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics 
of IgPro20 (Subcutaneous Immunoglobulin, Hizentra®) in Adults 
with Dermatomyositis (DM) – The RECLAIIM Study 
Study Number 
IgPro20_3007 
Sponsor 
CSL Behring (CSLB) 
Development Phase 
Phase 3 
Study Product 
IgPro20 (Hizentra®) 
Indication 
Dermatomyositis 
Study Summary and 
Overview 
This is a phase 3, multicenter, randomized, placebo-controlled, 
double-blind study of IgPro20 (subcutaneous immunoglobulin G 
[SCIG]) treatment in adult subjects with dermatomyositis (DM) 
with or without muscle weakness. 
After Screening, subjects will be randomized to 1 of 2 treatment 
sequences: 
Sequence A: 0.5 g/kg IgPro20 for 24 weeks (Study Period 1) 
followed by 0.5 g/kg IgPro20 for 28 weeks (Study Period 2) 
 - OR - 
Sequence B: placebo for 24 weeks (Study Period 1) followed by 
0.5 g/kg IgPro20 for 28 weeks (Study Period 2) 
Subjects with demonstrated treatment benefit at the End of Study 
Period 2 (EOP2) (Total Improvement Score [TIS] ≥ 20 points at 
Week 49) will be eligible to continue long-term treatment with 
open-label IgPro20 until the end of the study in Study Period 3.  
Primary 
Objective 
The primary objective of this study is to assess the efficacy of 
IgPro20 0.5 g/kg weekly subcutaneous (SC) doses in comparison 
to placebo in adult subjects with DM, as measured by responder 
status based on the TIS assessments at Weeks 17, 21, and 25. 
Primary 
Endpoint 
The primary endpoint is the responder status based on the TIS 
assessments at Weeks 17, 21, and 25. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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Secondary Objectives 
The secondary objectives of the study are: 
• To assess the efficacy, with additional clinical outcome 
measures, of IgPro20 in comparison to placebo 
• To assess the safety of IgPro20 in comparison to placebo 
• To assess the safety and efficacy of IgPro20 at Week 53 
• To assess the safety of IgPro20 after Week 53 to end of 
study participation 
Key Secondary 
Endpoints 
Key secondary endpoints of the study are: 
• TIS at Week 25 
• Change from Baseline in Manual Muscle Testing (MMT-8) 
at Week 25 
• Change from Baseline in Cutaneous Dermatomyositis 
Disease Area and Severity Index (CDASI) total activity 
score at Week 25 
• Reduction of oral corticosteroid dose at Week 25 
Secondary Endpoints 
Additional secondary endpoints of the study are: 
• TIS from Week 5 to Week 53 
• Individual core set measures (CSMs; except muscle 
enzyme), and CDASI from Baseline to Week 53 
o Physician Global VAS 
o Patient Global VAS  
o MMT-8 
o Health Assessment Questionnaire - Disability Index 
(HAQ-DI) 
o Extramuscular Global Assessment 
• Definition of Worsening from Baseline to Week 53 
• Reduction of oral concomitant corticosteroid dose from 
Baseline to Week 53 
• Use of rescue corticosteroid treatment from Baseline to 
Week 25 
• Mobility, Self-care, and Usual Activities domains of 
EuroQoL 5-Dimension Questionnaire (EQ-5D-5L) from 
Baseline to Week 53 
• Treatment-emergent adverse events (TEAEs) 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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Study Duration 
After up to 2 months of Screening, the duration for an individual 
subject is expected to be 24 weeks in Study Period 1 and 
28 weeks in Study Period 2. The maximum study duration in the 
open-label Study Period 3 will be approximately 7 years; 
however, subject participation will end if the drug becomes 
commercially available (see Section 3.7) or if there is no longer 
benefit from the study drug. For subjects not eligible to 
participate in Study Period 3, study duration will be up to 
56 weeks (including the Follow-up Telephone Call).  
Number of Subjects 
A sufficient number of subjects will be screened in order to 
randomize approximately 126 subjects into the study. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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Study Population and 
Criteria for Eligibility 
Inclusion criteria: 
• Capable of providing written informed consent by signing an 
informed consent form and willing and able to adhere to all 
protocol requirements 
• Age ≥ 18 years 
• Diagnosis of at least probable idiopathic inflammatory 
myopathies per European League Against 
Rheumatism/American College of Rheumatology 
(EULAR/ACR) Classification Criteria: minimum aggregate 
score of 5.5 without muscle biopsy and 6.7 with muscle 
biopsy (historical muscle biopsy is acceptable) which 
includes confirmation of DM rash/skin manifestation 
(present or by history [historical skin biopsy is required for 
amyopathic DM subjects]) 
• Disease activity defined by: 
o Presence of DM rash/skin manifestation (eg, Gottron's 
papules/sign, heliotrope rash, periorbital edema, V sign, 
Shawl sign) at Screening Visit - OR - 
o One objective disease activity measure within 3 months 
before Baseline: 
i. Magnetic resonance imaging (MRI) scan showing 
active inflammation (edema) of a proximal skeletal 
muscle - OR - 
ii. Electromyogram showing acute changes such as 
spontaneous activity not explained by other 
disease - OR - 
iii. Muscle biopsy with perivascular or perimysial 
inflammation - OR - 
iv. Creatine kinase (CK) > 4 times the upper limit of 
normal (4 × ULN) 
• Disease severity at Screening and Baseline defined by a 
minimum value of 2 cm on a 10-cm Physician Global 
Disease Activity Visual Analog Scale and: 
o MMT-8 ≤ 142 - OR - 
o CDASI total activity score ≥ 14 
• Subject has failed previous DM treatment or is on DM 
treatment such as immunosuppressants and/or antimalarials 
on a stable dose ≥ 3 months before Baseline; and/or oral 
corticosteroids (≤ 20 mg/day prednisolone equivalent and/or 
topical) on a stable dose ≥ 1 month before Baseline 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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Exclusion criteria: 
• Cancer-associated myositis, defined as the diagnosis of 
myositis within 2 years of the diagnosis of cancer  
• Evidence of malignancies diagnosed within the previous 
5 years 
Note: Subjects with a history of carcinoma in situ of the 
cervix that has been excised and cured with ≥ 5 years 
since excision or subjects with documented history of 
treated basal or squamous cell skin cancer may be 
enrolled into the study.  
• Physician Global Damage Assessment ≥ 3 on a 5-point 
Likert scale where a score of 3 represents severe damage 
• Clinically relevant improvement between Screening Visit and 
Baseline, defined by ≥ 2 cm improvement on a 10-cm 
Physician Global Disease Activity Assessment Visual Analog 
Scale 
• Known or suspected hypersensitivity or other severe 
reactions to IgPro20 or to any of its excipients, or other 
immunoglobulins (Igs) or severe reactions to blood products 
• Other significant medical conditions that could increase the 
risk to the subject, eg: 
o History of allogeneic bone marrow/stem cell 
transplant/solid organ transplant 
o Cardiac insufficiency (New York Heart Association 
Class III or IV) or unstable ischemic heart disease 
o Chronic kidney disease stage IV or V 
o Recent surgery requiring general anesthesia within the 
previous 4 weeks before Screening 
o Known hyperprolinemia type I or type II 
o Documented thrombophilic abnormalities including 
blood hyperviscosity, protein C or protein S deficiency, 
antithrombin-III deficiency, plasminogen deficiency, 
antiphospholipid antibodies, Factor V Leiden mutation, 
dysfibrinogenemia, or prothrombin G20210A mutation 
o History of documented thrombotic episode, 
eg, pulmonary embolism, deep vein thrombosis, 
myocardial infarction, or thromboembolic stroke at any 
time 
o More than 3 of the following specified risk factors for 
thromboembolic events (documented and current 
conditions) occurring concurrently: atrial fibrillation, 
coronary disease, diabetes mellitus, dyslipidemia, 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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hypertension, obesity (body mass index ≥ 30 kg/m2), 
recent significant trauma and immobility 
(wheelchair-bound or bedridden) 
o Uncontrolled, severe, or rapidly progressive interstitial 
lung disease which will prevent the subject from 
successful participation in the study 
o Severe skin disease at planned infusion sites that would 
make SC infusions infeasible 
o Medical conditions whose symptoms and effects could 
alter protein catabolism and or IgG utilization 
(eg, protein-losing enteropathies, nephrotic syndrome, 
known immunoglobulin A [IgA] deficiency with 
antibodies to IgA) 
• Other conditions which would prevent correct assessment or 
lead to impaired muscle strength (eg, other neurological 
disorders including, but not limited to, Parkinson’s disease or 
severe musculoskeletal conditions like severe osteoarthritis 
or deformities) 
• Laboratory exclusions at Screening: 
o Positive result for any of the following: human 
immunodeficiency virus (HIV), hepatitis B virus (HBV), 
hepatitis C virus (HCV)  
o Creatinine > 1.5 × ULN or blood urea nitrogen (BUN) 
> 3 × ULN 
• Any of the following therapies: 
o Within 1 month before Baseline: intramuscular, 
intravenous, or intra-articular corticosteroids including 
adrenocorticotropic hormone (any dose), doses 
> 20 mg/day prednisolone equivalent (any route), or any 
change to physiotherapy 
o Within 2 months before Baseline: IgG (Note: Subject 
may enroll < 2 months after stopping IgG therapy if 
clinical deterioration is experienced after withdrawal) 
o Within 3 months before Baseline: plasma exchange or 
plasmapheresis 
o Within 6 months before Baseline: cyclophosphamide or 
alkylating agents 
o Within 6 months or 5 half-lives of the drug, whichever 
is longer, before Baseline: other biologic therapies 
including investigational agents 
o Within 9 months before Baseline: rituximab or evidence 
of persistent B cell depletion after stopping therapy 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
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• Male subject or female subject of childbearing potential 
either not using or not willing to use a medically reliable 
method of contraception (see Section 7.4.2), not sexually 
abstinent during the study, or not surgically sterile before 
study enrollment 
• Pregnant or breastfeeding 
• Alcohol, drug, or medication abuse within 1 year of 
providing informed consent 
• Previously received investigational medicinal product (IMP) 
in this study or failed Screening more than 1 time in this 
study 
• Involved in the planning and/or conduct of the study (applies 
to CSLB staff, staff at the study site, and third-party vendors) 
Any issue that, in the opinion of the investigator, would render 
the subject unsuitable for participation in the study or unable to 
comply with study procedures, eg, inability to self-administer 
IMP or by aid through a caregiver 
IMP Dose, Dosing 
Regimen and 
Administration  
IgPro20 will comprise a liquid, ready-to-use 20% IgG solution 
stabilized with 250 mmol/L L-proline and 20 mg/L 
polysorbate 80. 
All IgPro20 will be administered by SC infusions as weekly 
doses. The total dose/volume of IgPro20 will be calculated on 
the basis of the body weight. The weekly SC infusions can be 
administered on 1, 2, 3, or 4 days per week. 
Comparator Product, 
Dose, Dosing Regimen 
and Administration 
The placebo product will comprise a 2% human albumin 
solution in 250 mmol/L L-proline and 20 mg/L polysorbate 80 
manufactured from the licensed, pasteurized CSLB human 
albumin 5% final product. 
All placebo will be administered by SC infusions as weekly 
doses. The total volume of placebo will be calculated on the 
basis of the body weight and volume-matched to the 0.5 g/kg 
IgPro20 dose. The weekly SC infusions can be administered on 
1, 2, 3, or 4 days per week. 
Efficacy Assessments 
Efficacy will be assessed using the 6 CSMs used to calculate TIS 
(Patient Global Activity Assessment, MMT-8, Health 
Assessment Questionnaire, Muscle Enzymes, Physician Global 
Disease Activity, and Extramuscular Global Assessment), 
CDASI, and Timed Up and Go. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
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Safety Assessments 
Safety will be assessed through TEAE documentation, laboratory 
safety parameters, vital signs, and physical examination. Specific 
monitoring for corticosteroid treatment, thromboembolic events 
(including implementation of study stopping rules for treatment 
imbalance), and hemolysis will also be performed. 
Pharmacokinetics 
Serum samples for IgG level determination will be collected at 
Screening and specified study visits. Additional blood samples for 
rich pharmacokinetic sampling of IgG levels will be collected at 
Week 37 on up to 10 Japanese subjects and 30 non-Japanese 
subjects. 
Pharmacodynamics 
Not applicable. 
Other Assessments 
Patient reported outcomes including 5-Dimension Itch Score, 
EQ-5D-5L, Work Productivity and Activity Impairment 
Questionnaire: General Health, and Treatment Satisfaction 
Questionnaire for Medication-9 will be assessed. 
Statistical Analyses 
The study is designed as a superiority study. The sample size 
calculation is based on the hypothesis that IgPro20 will have a 
higher responder rate than placebo based on the TIS assessments 
at Weeks 17, 21, and 25. 
A responder is defined as a subject with a TIS ≥ 20 points at 
Week 25 and at least 1 of the previous scheduled visits (Week 17 
or Week 21), who completes 24 weeks of randomized IMP 
treatment without the use of rescue corticosteroid treatment.  
Note: for subjects who discontinue from IMP or the study for 
reasons that are related to the Ukraine war before Week 25, a 
multiple imputation approach will be used for dealing with 
missing TIS values.  
For power simulation, it is assumed that 65% of subjects 
randomized to IgPro20 and 30% of subjects randomized to 
placebo have a TIS ≥ 20 points at Week 17. Further, it is 
assumed that for each subject, the response at Week 21 is 
conditional on the response at Week 17, and the response at 
Week 25 is conditional on the response at Week 21. 
Simulation shows a total of 126 subjects (63 subjects per arm) 
will be required for a power of 90% in 1-sided Fisher’s exact test 
at a significance level of 0.025. Fisher’s exact test is used in the 
power simulation as a close approximation to the exact logistic 
regression model in the primary endpoint analysis. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
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Notes for the Schedule of Assessments for Screening: 
A. Screening may be up to 2 months. Re-screening once per subject will be permitted for failed eligibility other than DM 
diagnosis or disease activity. 
B. DM treatment history including IgG, corticosteroid, and other treatment. 
C. Vital sign assessments include supine systolic and diastolic blood pressure, pulse rate, respiratory rate, and body 
temperature. 
D. Serum hCG pregnancy test is to be completed for all premenopausal female subjects capable of becoming pregnant. 
This includes women on oral, injectable, or mechanical contraception and women whose partners have been 
vasectomized or have received or are utilizing mechanical contraceptive devices. 
E. Hematology: hemoglobin, hematocrit, platelets, erythrocytes, leukocytes with differential counts (neutrophils, 
basophils, eosinophils, lymphocytes, monocytes). 
F. Muscle enzymes: CK, LDH, AST, ALT, aldolase. 
G. Other biochemistry: sodium, potassium, chloride, bicarbonate, BUN, glucose, GGT, alkaline phosphatase, creatinine. 
H. Thrombophilic Abnormality Screen: protein C, protein S, antithrombin-III, lupus anticoagulant, cardiolipin antibodies, 
prothrombin G20210A mutation, Factor V Leiden mutation. 
I. 
Coagulation: D-dimer. 
J. 
MSA and MAA: anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-SRP, anti-Mi-2, anti-p155/140, anti-TIF-1γ, 
anti-MDA5, anti-NXP-2, anti-PM/Scl, anti-Ro-SSA, anti-Ku, anti-U1 RNP, anti-U3 RNP, anti-HMGCR, 
anti-SAE-1Ab. 
K. HBV, HCV, and HIV. 
L. The 6 CSMs used in the TIS calculation ([1] Physician Global Disease Activity, [2] Patient Global Activity 
Assessment, [3] MMT-8, [4] HAQ, [5] Muscle Enzyme, and [6] Extramuscular Global Assessment) will be assessed 
for eligibility, along with Physician Global Damage Assessment. 
 

CSL Behring 
Study Number:  IgPro20_3007 
 
Study Product: 
IgPro20  
 
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O. Hemolysis: DAT, total bilirubin, direct bilirubin, indirect bilirubin, haptoglobin, reticulocytes, and peripheral blood smear to check for spherocytosis. Peripheral blood smear 
will be performed at Week 2 and Week 5 only. 
P. Other biochemistry: sodium, potassium, chloride, bicarbonate, BUN, glucose, GGT, alkaline phosphatase, creatinine. 
Q. Coagulation: D-dimer complex. 
R. MSA and MAA: anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, anti-SRP, anti-Mi-2, anti-p155/140, anti-TIF-1γ, anti-MDA5, anti-NXP-2, anti-PM/Scl, anti-Ro-SSA, 
anti-Ku, anti-U1 RNP, anti-U3 RNP, anti-HMGCR, anti-SAE-1Ab. 
S. Retention samples at Baseline and Week 53/end of study participation. A virology retention sample should be obtained for all subjects who discontinue at any time during the 
study. 
T. IMP SC infusions at Week 1 and Week 2 are to be administered only after the visit-specific assessments (except SC infusion diary review) are completed. 
U. Training and supervised IMP SC infusions at the site on Week 1 and Week 2. If additional infusion training at the site is requested by the subject for Week 3 and/or Week 4, 
no other UNS Visit assessments are required to be performed. Week 3 and Week 4 additional infusion training will be documented in the eCRF. After training is completed, 
IMP will not be administered during subsequent study visits. For the remainder of their participation in the study, subjects will self-administer IMP at home. 
V. Physiotherapy regimen may be modified starting at Week 17 if clinically relevant improvement is achieved. 
W. Only if subject discontinues IMP treatment before Week 25. 
X. IMP will only be administered at the site for unscheduled visits which occur at Week 3 or Week 4. 
Y. See Section 5.3.1 for DOW criteria and corticosteroid rescue details (Week 9 to Week 25). DOW assessment at UNS Visit only if before Week 25. 
Z. ≥ 2 cm improvement from Baseline on 10-cm Physician Global Disease Activity VAS. 
AA. Six individual CSMs will be assessed during the study for TIS calculation: (1) Physician Global Disease Activity, (2) Patient Global Activity Assessment, (3) Manual Muscle 
Testing of 8 muscle groups (MMT-8), (4) HAQ, (5) Muscle Enzyme, and (6) Extramuscular Global Assessment. 
BB. TSQM-9 is not performed at Baseline. 
CC. The Week 56 Safety Follow-up Telephone Call will not be performed for subjects who discontinue IMP treatment before Week 53 but remain in the study for at least 
1 subsequent study visit 4 weeks after IMP discontinuation or subjects who are eligible to continue treatment in Study Period 3. 
 

CSL Behring 
Study Number:  IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
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D. Hematology: hemoglobin, hematocrit, platelets, erythrocytes, leukocytes with differential counts (neutrophils, basophils, eosinophils, lymphocytes, monocytes). 
E. 
Biochemistry: sodium, potassium, chloride, bicarbonate, BUN, glucose, GGT, alkaline phosphatase, creatinine. 
F. 
Four individual CSMs will be assessed in Study Period 3: Physician Global Disease Activity and Patient Global Activity Assessment, MMT-8, and HAQ. 
G. Additional IMP will be assigned at UNS Visit if the subject’s weight has increased (> 2 kg), which will require a dose volume increase (see Appendix 15). 
H.  IMP will be dispensed at this visit only if the subject is continuing in Study Period 3. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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Table of Contents 
Clinical Study Protocol Synopsis .............................................................................................. 7 
Schedules of Assessments ........................................................................................................ 15 
Table of Contents ..................................................................................................................... 24 
List of Abbreviations ............................................................................................................... 31 
Glossary of Terms .................................................................................................................... 34 
1 
Introduction .......................................................................................................... 36 
1.1 
Background ...................................................................................................... 36 
1.2 
Information on IgPro20 .................................................................................... 37 
1.2.1 
Overview ...................................................................................................... 37 
1.2.2 
Nonclinical Evaluation ................................................................................. 38 
1.2.3 
Clinical Experience ...................................................................................... 38 
1.3 
Study Overview ................................................................................................ 38 
1.4 
Potential Risks and Benefits ............................................................................. 39 
2 
Study Objectives and Endpoints ........................................................................... 40 
2.1 
Primary Objective and Endpoint ...................................................................... 40 
2.1.1 
Primary Objective ......................................................................................... 40 
2.1.2 
Primary Endpoint ......................................................................................... 41 
2.2 
Secondary Objectives and Endpoints ............................................................... 41 
2.2.1 
Secondary Objectives ................................................................................... 41 
2.2.2 
Key Secondary Efficacy Endpoints .............................................................. 41 
2.2.3 
Secondary Efficacy Endpoints ..................................................................... 42 
2.2.4 
Safety Endpoints ........................................................................................... 44 
2.3 
Exploratory Objectives and Endpoints ............................................................. 44 
2.3.1 
Exploratory Objectives ................................................................................. 44 
3 
Study Design and Oversight ................................................................................. 45 
3.1 
Overall Design .................................................................................................. 45 
3.2 
Dose and Dosing Regimen ............................................................................... 49 
3.3 
Scientific Rationale .......................................................................................... 49 
3.3.1 
Study Design Rationale ................................................................................ 49 
3.3.2 
Dose Rationale ............................................................................................. 53 
3.4 
Planned Study Duration ................................................................................... 54 
3.5 
Planned Number of Subjects ............................................................................ 54 

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Study Number: 
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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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3.6 
Definition of Start of the Clinical Study .......................................................... 54 
3.7 
Definition of End of the Clinical Study ........................................................... 54 
3.8 
Study Oversight ................................................................................................ 54 
3.8.1 
Steering Committee ...................................................................................... 54 
3.8.2 
Independent Data Monitoring Committee .................................................... 55 
3.9 
Thromboembolic Event Stopping Criteria ....................................................... 55 
4 
Selection and Withdrawal of Subjects .................................................................. 56 
4.1 
Eligibility Criteria ............................................................................................ 56 
4.1.1 
Inclusion Criteria .......................................................................................... 56 
4.1.2 
Exclusion Criteria ......................................................................................... 57 
4.1.3 
Eligibility Criteria for Study Period 3 .......................................................... 60 
4.2 
Screen Failures ................................................................................................. 60 
4.3 
Discontinuation of IMP and Subject Study Withdrawal .................................. 60 
4.3.1 
Discontinuation of IMP ................................................................................ 60 
4.3.2 
Subject Withdrawal from Study ................................................................... 61 
4.3.2.1 
Procedures for Handling Withdrawals ...................................................... 61 
4.3.3 
Subjects Lost to Follow-up ........................................................................... 62 
4.3.4 
Replacement Policy ...................................................................................... 62 
4.3.5 
Consequences of Subject Withdrawal or IMP Discontinuation ................... 62 
5 
Study Interventions ............................................................................................... 63 
5.1 
Investigational Products ................................................................................... 63 
5.1.1 
Description of IgPro20 ................................................................................. 63 
5.1.2 
Description of Placebo ................................................................................. 63 
5.1.3 
Dosing and Administration of IgPro20 and Placebo .................................... 64 
5.1.3.1 
Dosing Modification ................................................................................. 66 
5.1.3.2 
Treatment Compliance .............................................................................. 66 
5.1.3.3 
Overdose ................................................................................................... 66 
5.1.4 
Packaging, Labeling, Supply and Storage .................................................... 66 
5.1.4.1 
Packaging and Labeling ............................................................................ 66 
5.1.4.2 
Supply and Storage ................................................................................... 67 
5.2 
Accountability and Destruction ........................................................................ 67 
5.3 
Other Interventions ........................................................................................... 67 
5.3.1 
Oral Rescue Corticosteroid Treatment ......................................................... 67 
5.3.2 
Concomitant Oral Corticosteroid Tapering .................................................. 69 
5.3.3 
Access to IMP after the End of Study Participation ..................................... 69 

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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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6 
Allocation to Treatment ........................................................................................ 69 
6.1 
Subject Assignment .......................................................................................... 69 
6.2 
Randomization Procedures ............................................................................... 70 
6.3 
Blinding Procedures ......................................................................................... 70 
6.3.1 
Blinding Method ........................................................................................... 70 
6.3.2 
Breaking the Blind for an Emergency .......................................................... 70 
6.3.3 
Planned Unblinding Procedures ................................................................... 71 
6.3.4 
Ad hoc Safety Unblinding ............................................................................ 71 
7 
Contraindications, Permitted Therapies and Prohibited Therapies ...................... 71 
7.1 
Contraindications and Precautions to Further Dosing ...................................... 71 
7.2 
Permitted Therapies .......................................................................................... 72 
7.3 
Prohibited Therapies......................................................................................... 73 
7.4 
Lifestyle Restrictions ........................................................................................ 73 
7.4.1 
Physical Activity ........................................................................................... 73 
7.4.2 
Contraception ............................................................................................... 74 
8 
Study Procedures and Visit Schedule ................................................................... 74 
8.1 
Clinical Procedures .......................................................................................... 74 
8.1.1 
Handling of Study Assessments ................................................................... 74 
8.1.2 
Demographics and Safety Assessments ....................................................... 77 
8.1.2.1 
Monitoring for Corticosteroid Treatment ................................................. 78 
8.1.2.2 
Monitoring for Thromboembolic Events (TEEs) ..................................... 78 
8.1.2.3 
Monitoring for Hemolysis ........................................................................ 81 
8.1.3 
ANA, MSA, and MAA Assessments ............................................................ 82 
8.1.4 
Pharmacokinetic Assessments ...................................................................... 83 
8.1.5 
Efficacy Assessments ................................................................................... 83 
8.1.6 
Other Assessments ........................................................................................ 85 
8.1.6.1 
Disease Specific ........................................................................................ 85 
8.1.6.2 
Non-disease Specific ................................................................................ 85 
8.1.6.3 
Exploratory Biomarkers ........................................................................... 86 
8.2 
Blood Samples .................................................................................................. 87 
8.3 
Retention of Samples ....................................................................................... 87 
8.4 
Prior and Concomitant Therapies ..................................................................... 88 
8.5 
Visit Schedule ................................................................................................... 89 
8.5.1 
Assessment Time Windows .......................................................................... 89 
8.5.2 
Screening ...................................................................................................... 90 

CSL Behring 
Study Number: 
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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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8.5.3 
Study Period 1 .............................................................................................. 92 
8.5.3.1 
Baseline/Week 1 ....................................................................................... 92 
8.5.3.2 
Week 2 ...................................................................................................... 94 
8.5.3.3 
Week 5 ...................................................................................................... 94 
8.5.3.4 
Week 9 ...................................................................................................... 95 
8.5.3.5 
Week 13 .................................................................................................... 96 
8.5.3.6 
Week 17 .................................................................................................... 97 
8.5.3.7 
Week 21 .................................................................................................... 98 
8.5.3.8 
Week 25 .................................................................................................... 99 
8.5.4 
Study Period 2 ............................................................................................ 100 
8.5.4.1 
Week 29 .................................................................................................. 100 
8.5.4.2 
Week 33 .................................................................................................. 101 
8.5.4.3 
Week 37 .................................................................................................. 102 
8.5.4.4 
Week 41 .................................................................................................. 103 
8.5.4.5 
Week 45 .................................................................................................. 104 
8.5.4.6 
Week 49 .................................................................................................. 104 
8.5.4.7 
Week 53 .................................................................................................. 105 
8.5.4.8 
Week 56 (Safety Follow-up Telephone Call) ......................................... 107 
8.5.4.9 
Unscheduled Study Visit for Study Period 1 or Study Period 2 ............. 107 
8.5.5 
Study Period 3 ............................................................................................ 108 
8.5.5.1 
Monthly (every 4 weeks) Study Visits.................................................... 108 
8.5.5.2 
Study Visits Every 6 Months (24 weeks) ............................................... 109 
8.5.5.3 
End of Year Visit ..................................................................................... 110 
8.5.5.4 
End of Period 3 Visit............................................................................... 111 
8.5.5.5 
Unscheduled Study Visit for Study Period 3 .......................................... 112 
8.5.6 
Discontinuation of IMP .............................................................................. 113 
9 
Adverse Events ................................................................................................... 113 
9.1 
Definitions ...................................................................................................... 113 
9.1.1 
Adverse Event ............................................................................................ 113 
9.1.2 
Serious Adverse Event ................................................................................ 115 
9.1.3 
Adverse Event of Special Interest .............................................................. 116 
9.2 
Severity of Adverse Events ............................................................................ 116 
9.3 
Causality of Adverse Events .......................................................................... 116 
9.4 
Observation Period for Adverse Events ......................................................... 118 
9.5 
Follow-up of Adverse Events ......................................................................... 118 
9.6 
Adverse Event Reporting ............................................................................... 118 

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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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9.6.1 
Adverse Events ........................................................................................... 118 
9.6.2 
Adverse Events of Special Interest ............................................................. 118 
9.6.3 
Serious Adverse Events .............................................................................. 119 
9.6.4 
Other Significant Events ............................................................................ 120 
9.6.5 
Overdose ..................................................................................................... 120 
9.6.6 
Pregnancy and Breastfeeding ..................................................................... 120 
9.7 
IRB/IEC Reporting Requirements ................................................................. 121 
10 
Statistics .............................................................................................................. 121 
10.1 
Sample Size Estimation .................................................................................. 121 
10.2 
Definition of Periods and Reference Visits .................................................... 122 
10.3 
Description of Study Analysis Sets ................................................................ 123 
10.4 
Statistical Analyses and Methods for Study Period 1 and Study Period 2 ..... 125 
10.4.1 
Subject Disposition and Characteristics ..................................................... 125 
10.4.1.1 
Subject Disposition ................................................................................. 125 
10.4.1.2 
Subject Characteristics ........................................................................... 125 
10.4.2 
Efficacy Analyses ....................................................................................... 125 
10.4.2.1 
Study Hypotheses ................................................................................... 125 
10.4.2.2 
Primary Estimand ................................................................................... 126 
10.4.2.3 
Primary Efficacy Endpoint ..................................................................... 127 
10.4.2.4 
Secondary Efficacy Endpoints ................................................................ 131 
10.4.2.5 
Subgroup Analyses ................................................................................. 137 
10.4.3 
Safety Analyses .......................................................................................... 137 
10.4.4 
Exploratory Analyses ................................................................................. 139 
10.4.4.1 
Pharmacokinetics Analyses .................................................................... 139 
10.4.5 
Interim Analysis ......................................................................................... 140 
10.5 
Statistical Analyses and Methods for Study Period 3 .................................... 140 
11 
Quality Assurance ............................................................................................... 140 
12 
Regulatory and Ethics Considerations ................................................................ 141 
12.1 
Regulatory Considerations ............................................................................. 141 
12.2 
Institutional Review Board/Independent Ethics Committee .......................... 141 
12.3 
Subject Information and Informed Consent ................................................... 141 
12.4 
Subject Confidentiality ................................................................................... 142 
12.5 
Indemnity and Compensation ......................................................................... 142 
13 
Administrative Considerations ........................................................................... 142 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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13.1 
Clinical Trial Research Agreement ................................................................ 142 
13.2 
Clinical Study Registration and Results Disclosure ....................................... 143 
13.3 
Implementation of the Clinical Study Protocol and Amendments ................. 143 
13.4 
Protocol Deviations ........................................................................................ 143 
13.5 
Documentation and Record Keeping ............................................................. 144 
13.5.1 
Data Collection ........................................................................................... 144 
13.5.2 
Data Quality Assurance .............................................................................. 145 
13.5.3 
Record Retention ........................................................................................ 146 
13.6 
Study and Site Closure ................................................................................... 146 
13.7 
Clinical Study Report ..................................................................................... 147 
13.8 
Use of Data and Publications ......................................................................... 147 
14 
References .......................................................................................................... 148 
15 
Appendices ......................................................................................................... 155 
 
Signatures ....................................................................................................... 156 
 
EULAR/ACR Classification Criteria for Idiopathic Inflammatory Myopathies
 ........................................................................................................................ 158 
 
Physician Global Damage Assessment .......................................................... 160 
 
Myositis Disease Activity Assessment Tool (MDAAT) ................................. 161 
 
Patient Global Activity Assessment ............................................................... 166 
 
Manual Muscle Testing Scoring Sheet (MMT-8) ........................................... 167 
 
Health Assessment Questionnaire (HAQ) ...................................................... 168 
 
Serum Levels of Muscle Enzymes ................................................................. 171 
 
Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) ver02
 ........................................................................................................................ 172 
  5-D Pruritus Scale .......................................................................................... 173 
  Timed Up and Go ........................................................................................... 174 
  EuroQol 5-Dimension Questionnaire (EQ-5D-5L) ........................................ 176 
  Work Productivity and Activity Impairment Questionnaire: General Health 
v2.0 (WPAI-GH) ............................................................................................ 179 
  Abbreviated Treatment Satisfaction Questionnaire for Medication (TSQM-9)
 ........................................................................................................................ 181 
  Weight-based Dosing Regimen ...................................................................... 184 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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List of Tables 
Table 1 
Schedule of Assessments for Screening........................................................... 15 
Table 2 
Schedule of Assessments for Study Period 1 and Study Period 2 ................... 17 
Table 3 
Schedule of Assessments for Open-label Study Period 3 ................................ 21 
Table 4 
Schedule of Assessments for Rich Pharmacokinetic Sampling ....................... 23 
Table 5 
Investigational Medicinal Product Dosing Characteristics .............................. 64 
Table 6 
Corticosteroid Dose Equivalency .................................................................... 72 
Table 7 
Role Responsibilities for Study Assessments .................................................. 76 
Table 8 
Study Procedures: Demographics and Safety Assessments ............................. 77 
Table 9 
Wells’ Criteria Scoring for Suspicion of DVT ................................................. 80 
Table 10 
Wells’ Criteria Scoring for Suspicion of PE .................................................... 80 
Table 11 
Clinical Procedures: ANA, MSA, and MAA Assessments .............................. 82 
Table 12 
Clinical Procedures: Pharmacokinetic Assessments ........................................ 83 
Table 13 
Clinical Procedures: Exploratory Biomarkers ................................................. 87 
Table 14 
Time Windows for Assessments ...................................................................... 89 
Table 15 
Assumptions for Power Analysis ................................................................... 122 
Table 16 
Study Periods and Reference Visits ............................................................... 123 
Table 17 
Total Improvement Score (TIS) Using Core Set Measures ........................... 129 
 
List of Figures 
Figure 1 
Study Design .................................................................................................... 48 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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List of Abbreviations 
Abbreviation 
Term 
5-D 
5-Dimension 
AE 
Adverse event 
AESI 
Adverse event of special interest 
ALT 
Alanine aminotransferase  
ANA 
Antinuclear antibody 
AST 
Aspartate aminotransferase 
AUC 
Area under the concentration-time curve 
BDRM 
Blinded Data Review Meeting 
BUN 
Blood urea nitrogen 
Cmax 
Maximum concentration 
Ctrough 
Trough concentration 
CDASI 
Cutaneous Dermatomyositis Disease Area and Severity Index 
CI 
Confidence interval 
CIDP 
Chronic inflammatory demyelinating polyneuropathy 
CK 
Creatine kinase 
CRF 
Case report form 
CSLB 
CSL Behring 
CSM 
Core set measure 
CTA 
Clinical trial application 
CTN 
Clinical trial notification 
DAT 
Direct antiglobulin test 
DM 
Dermatomyositis 
DOW 
Definition of Worsening 
DVT 
Deep vein thrombosis 
eCOA 
Electronic clinical outcomes assessment 
ELISA 
Enzyme-linked immunosorbent assay 
EOP 
End of Study Period  
EOS 
End of Study 
EOT 
End of Treatment 
EQ-5D-5L 
EuroQoL 5-Dimension Questionnaire 
EULAR / ACR 
European League Against Rheumatism/American College of 
Rheumatology 
FDA 
Food and Drug Administration 
GCP 
Good Clinical Practice 

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Study Number: 
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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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Abbreviation 
Term 
GMP 
Good Manufacturing Practice 
HAQ 
Health Assessment Questionnaire 
HBV 
Hepatitis B virus 
HCV 
Hepatitis C virus 
HIV 
Human immunodeficiency virus 
IB 
Investigator’s brochure 
ICF 
Informed consent form 
ICH 
International Council for Harmonisation of Technical 
Requirements for Pharmaceuticals for Human Use 
IDMC 
Independent data monitoring committee 
IEC 
Independent Ethics Committee 
IgA 
Immunoglobulin A 
IgG 
Immunoglobulin G 
IIM 
Idiopathic inflammatory myopathies 
IMACS 
International Myositis Assessment and Clinical Studies 
IM 
Intramuscular 
IMP 
Investigational medicinal product 
IND 
Investigational new drug 
IRB 
Institutional Review Board 
IRT 
Interactive response technology 
IV 
Intravenous 
IVIG 
Intravenous immunoglobulin G 
LDH 
Lactate dehydrogenase 
MAA 
Myositis-associated autoantibodies 
MDAAT 
Myositis Disease Activity Assessment Tool 
MedDRA 
Medical Dictionary for Regulatory Activities 
mITT 
modified Intent-to-Treat analysis set 
mITT-Ex 
modified Intent-to-Treat analysis set extended 
MMRM 
Mixed Model Repeated Measures 
MMT-8 
Manual Muscle Testing of 8 muscle groups 
MRI 
Magnetic resonance imaging 
MSA 
Myositis-specific autoantibodies 
PE 
Pulmonary embolism 
PK 
Pharmacokinetic analysis set 
PM 
Polymyositis 

CSL Behring 
Study Number: 
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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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Abbreviation 
Term 
PP 
Per Protocol analysis set 
PT 
Preferred term 
SAE 
Serious adverse event 
SAF 
Safety analysis set 
SAF-Ex 
Safety analysis set extended 
SAP 
Statistical analysis plan 
SC 
Subcutaneous 
SCIG 
Subcutaneous immunoglobulin G 
SCR 
Screened analysis set 
SOC 
System organ class 
TEAE 
Treatment-emergent Adverse Event 
TEE 
Thromboembolic event 
TIS 
Total Improvement Score 
TSQM-9 
Abbreviated Treatment Satisfaction Questionnaire for 
Medication 
ULN 
Upper limit of normal 
VAS 
Visual analog scale 
WPAI-GH 
Work Productivity and Activity Impairment Questionnaire for 
General Health 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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Glossary of Terms 
Term 
Definition 
Baseline 
Week 1, before infusion of investigational medicinal product 
(IMP) 
DOW 
The DOW consists of meeting 1 of the following 3 criteria on 
2 consecutive study visits at least 2 weeks apart: 
• Physician Global Disease Activity Assessment VAS 
worsening ≥ 2 cm* and MMT-8 worsening ≥ absolute 
10%, - OR - 
• Extramuscular Global Assessment VAS worsening 
≥ 2 cm, - OR - 
• Any 3 of 6 CSMs (see Section 3.3.1) worsening by ≥ absolute 
20% 
* If baseline Physician Global VAS is 8 to 10, then any 
worsening on Physician Global VAS is acceptable as long 
as MMT-8 criterion is met. 
EOP 
End of Study Period distinguishes periods 1, 2 and 3 of the study.  
g/kg 
IMP dose throughout the study will be calculated by gram/kg of 
body weight. For all dosing references in the protocol, the dose 
description is shortened to g/kg. 
Primary data analysis 
The primary data analysis (ie, EOP1 analysis) includes all data 
collected during Study Period 1. 
Primary endpoint 
analysis 
The primary endpoint analysis refers to the analysis of the 
primary objective, ie, the responder status based on the TIS 
assessments at Weeks 17, 21, and 25. 
Responder 
A subject with a TIS ≥ 20 points at Week 25 and at least 1 of the 
previous scheduled visits (Week 17 or Week 21), who completes 
24 weeks of randomized IMP treatment (ie, Study Period 1) 
without the use of rescue corticosteroid treatment. 
Note: for subjects who discontinue from IMP or the study for 
reasons that are related to the Ukraine war before Week 25, a 
multiple imputation approach will be used for dealing with 
missing TIS values (see Section 10.4.2). 
Study Treatment 
Refers to IMP 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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Term 
Definition 
Subject eligible for 
continuing treatment 
in Study Period 3 
At the Week 53 Visit, subjects may continue to the Study Period 3 
if eligible. The basis for eligibility is a TIS ≥ 20 points at the 
Week 49 Visit. Subjects who continue the study will receive 
open-label IgPro20 0.5 g/kg SC infusions weekly in 
Study Period 3. 
Abbreviations: See List of Abbreviations 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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1 Introduction 
1.1 Background 
Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired muscle 
disorders characterized by muscle weakness, elevated creatine kinase (CK) levels, myopathic 
electromyographic findings, characteristic histopathological findings [Malik et al, 2016; 
Anh-Tu Hoa and Hudson, 2017; Mandel et al, 2017; Milone, 2017; Schmidt, 2018], and in 
certain cases, an association with autoantibodies [Tieu et al, 2016; Schmidt, 2018]. IIM are 
best classified into 4 main subtypes: dermatomyositis (DM), polymyositis (PM), necrotizing 
myopathy, and inclusion body myositis [Malik et al, 2016; Milone, 2017]. The identification 
of the correct subtype and the distinction of these diseases from other disorders which have 
characteristics that mimic them is fundamental to treatment, because each subtype has a 
different prognosis and response to therapies [Dalakas, 2015]. 
Incidence and prevalence of DM varies greatly across studies. In a nationwide patient register 
and rheumatology quality register, the average incidence was 11 per 1,000,000 (13 for 
women and 9.7 for men) while prevalence rate was 14 per 100,000 (17 for women and 11 for 
men). Both incidence and prevalence rates increased with age with a peak in the 70 to 79 year 
range [Svensson et al, 2017]. 
Diagnosis of DM is achieved by the evaluation of a combination of clinical and pathological 
features. Dermatomyositis, with its characteristic skin rash and relatively greater 
responsiveness to corticosteroids and other immunomodulators than the other IIM subgroups, 
is quite distinguishable [Findlay et al, 2015; Oldroyd et al, 2017; Lahouti, 2015] and carries 
significant morbidity and mortality [Mandel et al, 2017]. If left untreated, muscle weakness 
leads to ambulation difficulties (restriction to wheelchair or confinement to bed). Within the 
patients with DM, a subgroup of patients with amyopathic/hypomyopathic DM has been 
identified. These patients present with characteristic rash but without obvious muscle 
weakness [Dalakas and Hohlfeld, 2003] or elevations in muscle enzyme [Ghazi et al, 2013]. 
This DM subgroup represents about 20% of all DM cases [Patel et al, 2018]. These patients 
are at increased risk of breast, lung, and ovarian cancer and may also have systemic 
involvement such as interstitial lung disease and cardiac disease [Udkoff and Cohen, 2016]. 
Primary treatment modalities include corticosteroids, intravenous immunoglobulin G (IVIG), 
plasmapheresis, and other immunomodulators (such as methotrexate, azathioprine, and 
cyclosporine A). In clinical practice, corticosteroids are usually the first choice medication in 
DM; however, long-term treatment with corticosteroids has significant side effects and is not 
well tolerated in patients [Yasir and Sonthalia, 2019]. One study estimated the 10-year 

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Study Product: 
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Protocol version: Amendment 4 
 
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survival rate of 160 DM/PM patients treated with immunomodulators such as corticosteroids, 
methotrexate, and azathioprine to be 62% [Schiopu et al, 2012; Findlay et al, 2015], 
highlighting the continued unmet need in this disease. 
The trigger that initiates DM or other IIM subtypes or causes flares is not clearly understood. 
However, it is known that environmental factors in genetically susceptible individuals can 
trigger myositis with involvement of several cellular and humoral mechanisms [Carstens and 
Schmidt, 2014]. In DM, inflammatory cells (predominantly CD4+ cells) and perifascicular 
atrophy, in addition to immune attacks targeting capillaries leading to capillary loss and 
subsequent ischemia are observed. More recently, the importance of B lymphocytes, 
macrophages, and dendritic cells [Blank et al, 2014] and CD4+ CD25+ T-regulatory cells 
[Kessel et al, 2007] have been described as having a different role and weight in DM versus 
PM (where inflammatory cells [predominantly CD8+ cells] are noted in the perimysium and 
in perivascular areas). 
Because of the heterogeneity of IIM as mentioned above, and the possibility of different 
clinical outcomes among subtypes, this study will enroll only subjects with DM, including 
(clinically) amyopathic DM and adults first diagnosed with DM as juveniles (< 18 years old), 
to evaluate the safety and efficacy of IgPro20. 
1.2 Information on IgPro20 
1.2.1 Overview 
IgPro20 is a ready-to-use 20% liquid formulation of human immunoglobulin G (IgG) with 
greater than 98% IgG purity for subcutaneous (SC) administration, and is manufactured by 
CSL Behring (CSLB). IgPro20 is approved in the United States of America, the European 
Union, Japan, and other countries under the brand name Hizentra® for SC administration in 
primary immune deficiency syndromes, chronic inflammatory demyelinating polyneuropathy 
(CIDP), and other indications. 
The precise mechanism of action underlying immunoglobulin immunomodulatory treatment 
in autoimmune/immune-mediated and inflammatory disease is not fully understood. 
However, several mutually nonexclusive mechanisms have been proposed to explain the 
broad spectrum of action of immunoglobulin. These include anti-idiotype regulation, 
modifications in cytokine production, the inhibition of complement activation, the 
neutralization of autoantibodies, killing of target cells by antibody-dependent cytotoxicity, 
and the blockade of cell-cell interaction [Danieli et al, 2014]. 

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Study Number: 
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Study Product: 
IgPro20 
 
Protocol version: Amendment 4 
 
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A detailed description of the chemistry, pharmacology, efficacy, and safety of IgPro20 is 
provided in the IgPro20 Investigator’s Brochure (IB). 
1.2.2 Nonclinical Evaluation 
Based on the overall assessment of the available nonclinical information from the different 
studies performed with IgPro20 or its stabilizers L-proline and polysorbate 80, there are no 
safety concerns with the administration of IgPro20. A detailed description of nonclinical 
studies of IgPro20 is provided in the IgPro20 IB. 
1.2.3 Clinical Experience 
To date, SC infusions of IgPro20 have been administered to humans in clinical studies in both 
immunodeficiency and immunomodulatory indications. This will be the first study in subjects 
with DM. 
A detailed description of clinical studies of IgPro20 is provided in the IgPro20 IB. 
1.3 Study Overview 
This is a phase 3, multicenter, randomized, placebo-controlled, double-blind study of IgPro20 
(subcutaneous immunoglobulin G [SCIG]) treatment in adult subjects with DM with or 
without muscle weakness. This study consists of a Screening Period (≤ 2 months), 
Study Period 1 (24 weeks of either IgPro20 or placebo administration), Study Period 2 
(28 weeks of IgPro20 administration), and Study Period 3 (open-label IgPro20 administration 
until the end of the study) for subjects who have shown a treatment benefit at the End of 
Study Period 2 (EOP2). Study Period 3 will provide additional long-term safety and efficacy 
data for this patient population. 
Subjects will be randomized to 1 of 2 treatment sequences: 
• Sequence A: 0.5 g/kg IgPro20 for 24 weeks (Study Period 1) followed by 0.5 g/kg 
IgPro20 for 28 weeks (Study Period 2) 
- OR -  
• Sequence B: placebo for 24 weeks (Study Period 1) followed by 0.5 g/kg IgPro20 
for 28 weeks (Study Period 2) 
After completing Study Period 2, eligible subjects may continue treatment with SC infusions 
of open-label IgPro20 0.5 g/kg in Study Period 3.  

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The primary objective of this study is to assess the efficacy of IgPro20 0.5 g/kg weekly SC 
doses in comparison to placebo in adult subjects with DM, as measured by responder status 
based on Total Improvement Score (TIS) assessments at Weeks 17, 21, and 25. 
1.4 Potential Risks and Benefits  
Identified and potential risks pertaining to IgPro20 
Although the safety profile of IgPro20 has not been evaluated in subjects with DM, IgPro20 
has been demonstrated in general to be a safe product based on safety data obtained from 
previous CSLB-sponsored clinical studies in various indications including primary immune 
deficiency and CIDP, and post-marketing experience collected over the past 9 years. 
IgPro20 safety data has been accumulated in weekly doses up to 0.4 g/kg body weight. In a 
recently completed global CIDP program (one 24-week pivotal study IgPro20_3003 
[172 subjects] and one 48-week extension study IgPro20_3004 [82 subjects]), 0.4 and 
0.2 g/kg weekly doses were evaluated. In both CIDP studies, the systemic adverse event (AE) 
rate was low and local reactions were mild to moderate and decreased in frequency over time. 
For these reasons, no increased risk with 0.5 g/kg weekly is expected in this study. 
Thrombotic and thromboembolic event (TEE) is an identified risk in class labeling for IVIG 
and SCIG. TEE is also an identified risk in the DM population [Nowak et al, 2016]. Any TEE 
in this study will be classified as an adverse event of special interest (AESI) 
(see Section 9.1.3 for details). Study stopping rules based on the observation of a prespecified 
increase in IgPro20 TEEs over placebo will be employed for this identified risk 
(see Section 3.9 for details). 
Further details on identified and potential risks associated with IgPro20 are provided in the 
IgPro20 IB. 
Potential risks pertaining to placebo 
In this study, a 2% human albumin formulation will be used as placebo. The formulation will 
be developed by dilution of the commercially available liquid albumin preparation 
manufactured by the sponsor under Good Manufacturing Practice (GMP) conditions. The 
tolerability of SC administration of human albumin was tested in a pivotal CIDP study that 
included 57 subjects on placebo, and was found to be similar to that experienced with other 
plasma proteins, eg, IgG, as described above. No harm is to be expected from the use of 
albumin as placebo in DM subjects. The potential risk of disease flare in the placebo arm 

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during double-blind treatment has been mitigated by the provision of oral corticosteroid 
rescue treatment while continuing double-blind treatment until Week 25. 
There is a high permeability for albumin between the SC compartment and the vascular 
system. In view of the SC data available, the absence of immunogenicity after intramuscular 
(IM) administration, and the high abundance of albumin in the body (> 25 mg/mL) [Poulsen, 
1974], local reactions and immunogenicity are considered highly improbable at the dose 
administered in the present study. 
Potential risks of failure of IgPro20 
The efficacy of IgPro20 in DM is unknown. The potential risk of disease flare is mitigated by 
the provision of oral corticosteroid rescue treatment while continuing double-blind treatment 
until Week 25. 
Potential benefit of IgPro20 
A potential benefit of the study is the improvement of the symptoms of DM (eg, muscle 
strength, rash) as assessed by the outcome measures. Improvement in muscle strength and 
other measures has been noted in small preliminary studies using SCIG, based on literature 
[Cherin et al, 2016]. 
In addition, a low rate of systemic AEs as compared to those commonly seen with IVIG, and 
an increase in subject autonomy and quality of life through self-treatment may be observed. 
IgG preparations in general have a well-known safety and efficacy profile documented in the 
literature [Eibl, 2003; Wittstock et al, 2003; Wittstock and Zettl, 2006] and as recently 
described in a completed CIDP study [van Schaik et al, 2018]. 
Thus, the associated benefit-risk is acceptable for DM subjects enrolled in the present study. 
2 Study Objectives and Endpoints 
2.1 Primary Objective and Endpoint 
2.1.1 Primary Objective 
The primary objective of this study is to assess the efficacy of IgPro20 0.5 g/kg weekly SC 
doses in comparison to placebo in adult subjects with DM, as measured by responder status 
based on the TIS assessments at Weeks 17, 21, and 25. 

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2.1.2 Primary Endpoint 
Endpoint 
Summary Measure 
Responder status based on the TIS 
assessments at Weeks 17, 21, and 25 
• Point estimate and 95% confidence interval 
(CI) for the responder rate by treatment 
sequence 
• Point estimate and 95% CI for the odds ratio 
(IgPro20:placebo) 
A responder is defined as a subject with a TIS ≥ 20 points [Aggarwal et al, 2017] at Week 25 
and at least 1 of the previous scheduled visits (Week 17 or Week 21), who completes 
24 weeks of randomized IMP treatment (Study Period 1) without the use of rescue 
corticosteroid treatment. Note: for subjects who discontinue from IMP or the study for 
reasons which are related to the Ukraine war before Week 25, a multiple imputation approach 
will be used for dealing with missing TIS values (see Section 10.4.2.3). 
2.2 Secondary Objectives and Endpoints 
2.2.1 Secondary Objectives 
The secondary objectives of the study are: 
1. To assess the efficacy, with additional clinical outcome measures, of IgPro20 in 
comparison to placebo 
2. To assess the safety of IgPro20 in comparison to placebo 
3. To assess the safety and efficacy of IgPro20 at Week 53 
4. To assess the safety of IgPro20 from after completion of Week 53 to the end of 
study participation 
2.2.2 Key Secondary Efficacy Endpoints 
Secondary 
Objective 
Endpoint 
Summary Measures 
1 
TIS at Week 25 
• Mean TIS values at Week 25 by treatment 
sequence 
• Point estimates and 95% CI for mean 
difference (IgPro20 – placebo) at Week 25 

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Secondary 
Objective 
Endpoint 
Summary Measures 
1 
Change from Baseline 
in MMT-8 (Manual 
Muscle Testing of 
8 muscle groups) at 
Week 25 
• Mean changes from Baseline in MMT-8 at 
Week 25 by treatment sequence 
• Point estimates and 95% CI for mean change 
difference (IgPro20 – placebo) at Week 25 
1 
Change from Baseline 
in Cutaneous 
Dermatomyositis 
Disease Area and 
Severity Index 
(CDASI) total activity 
score at Week 25 
• Mean changes from Baseline in CDASI total 
activity score at Week 25 by treatment 
sequence 
• Point estimates and 95% CI for mean change 
difference (IgPro20 – placebo) at Week 25 
1 
Reduction of oral 
corticosteroid dose at 
Week 25 
• Number and percentage and 95% CI of subjects 
who are able to reduce the oral corticosteroid 
dose by ≥ 25% at Week 25 by treatment 
sequence 
• Point estimates and 95% CI for the odds ratio 
(IgPro20:placebo) at Week 25 
 
2.2.3 Secondary Efficacy Endpoints 
Secondary 
Objectives 
Endpoints 
Summary Measures 
1, 3 
TIS from Week 5 to 
Week 53 
• Mean TIS by treatment sequence at each visit 
• Percentage of subjects achieving TIS ≥ 20, 
≥ 40, and ≥ 60 points by treatment sequence at 
each visit 
• Time to first achieving TIS ≥ 20, ≥ 40, and 
≥ 60 points by treatment sequence 
• Percentage of subjects achieving 
TIS ≥ 20 points at the end of the Study Period 2 

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Secondary 
Objectives 
Endpoints 
Summary Measures 
1, 3 
Individual core set 
measures (CSMs; 
except muscle 
enzyme) and CDASI 
from Baseline to 
Week 53 
• Mean changes from Baseline by treatment 
sequence between Week 5 and Week 25 
• Mean changes from Week 25 by treatment 
sequence between Week 29 and Week 53 
Individual CSMs include: 
• Physician Global Disease Activity 
• Patient Global Activity Assessment 
• MMT-8 
• Health Assessment Questionnaire – Disability 
Index 
• Extramuscular Global Assessment 
1, 3 
Definition of 
Worsening (DOW) 
from Baseline to 
Week 53 
• Number and percentage of subjects meeting 
DOW at least once, twice, or > twice by 
treatment sequence 
• Time to meeting DOW for the first time by 
treatment sequence 
• Number and percentage of subjects meeting 
DOW and receiving rescue corticosteroid 
treatment by treatment sequence 
1 
Reduction of oral 
concomitant 
corticosteroid dose 
from Baseline to 
Week 53 
• Number and percentage of subjects who start 
oral concomitant corticosteroid dose taper 
before Week 25 by treatment sequence 
• Number and percentage of subjects who are 
able to reduce their oral concomitant 
corticosteroid dose by ≥ 25%, ≥ 50%, and 
≥ 75% by Week 25 or by Week 53 by treatment 
sequence 
3 
Use of rescue 
corticosteroid 
treatment from 
Baseline to Week 25 
• Percentage of subjects receiving rescue 
corticosteroid treatment by treatment sequence 
and visit 
• Percentage of subjects whose rescue 
corticosteroid treatment is tapered by treatment 
sequence and visit 
• Time to first administration of rescue 
corticosteroid treatment 
1, 3 
Mobility, Self-care, 
and Usual Activities 
domains of EuroQoL 
5-Dimension 
• Number and percentage of subjects having at 
least 1 level, 2 levels, and > 2 levels of 
improvement from Baseline by treatment 
sequence at Week 13 and Week 25 

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Secondary 
Objectives 
Endpoints 
Summary Measures 
Questionnaire 
(EQ-5D-5L) to 
Week 53  
• Number and percentage of subjects having no 
reduction in levels, at least 1 level, 2 levels, and 
> 2 levels of improvement from Week 25 by 
treatment sequence at Week 41 and Week 53 
 
2.2.4 Safety Endpoints 
Secondary 
Objective 
Endpoint 
Summary Measures 
2,3,4 
Treatment-emergent 
adverse events 
(TEAEs) 
• Percentage of subjects with TEAEs 
• Percentage of subjects with related TEAEs 
• Percentage of subjects with serious TEAEs 
• Rate of TEAEs per time at risk 
• Rate of mild, moderate, and severe TEAEs per 
time at risk 
• Rate of related TEAEs per time at risk 
• Rate of serious TEAEs per time at risk 
 
In addition to TEAEs for IMP and rescue corticosteroid treatment, safety will be assessed 
throughout the study based on laboratory assessment, physical examination, and vital signs. 
Specific monitoring for corticosteroid treatment, TEEs (including assessment whether study 
stopping rules were met [Section 3.9]), and hemolysis will also be performed 
(Sections 8.1.2.1, 8.1.2.2, and 8.1.2.3, respectively). 
2.3 Exploratory Objectives and Endpoints 
2.3.1 Exploratory Objectives 
The exploratory objectives of this study are: 
• Study Period 1 and Study Period 2 (From Baseline to Week 53): 
o To assess pain / discomfort and anxiety / depression domains of EQ-5D-5L 
o To assess Work Productivity and Activity Impairment Questionnaire for General 
Health (WPAI-GH) 
o To assess Abbreviated Treatment Satisfaction Questionnaire for Medication 
(TSQM-9) 

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o To assess Timed Up and Go result for functional ability to rise from a chair and 
ambulate 
o To assess itch (pruritus) 
o To assess muscle enzymes 
o To assess antinuclear antibody (ANA), myositis-specific autoantibodies (MSA), and 
myositis-associated autoantibodies (MAA) 
o To assess the pharmacokinetics (PK) of IgG in Week 37 
• Study Period 3 (after Week 53): 
o To assess the efficacy of IgPro20 from after completion of Week 53 to the end of 
study participation  
o To assess quality of life of IgPro20 from after completion of Week 53 to the end of 
study participation 
o Use of corticosteroid treatment from Week 53 to the end of study participation 
3 Study Design and Oversight 
3.1 Overall Design 
This is a phase 3, multicenter, randomized, placebo-controlled, double-blind study of IgPro20 
(SCIG) treatment in adult subjects with DM, with or without muscle weakness. 
This study consists of Screening and 3 treatment periods: 
• Screening Period: Up to 2 months during which screening assessments must be 
completed. 
• Study Period 1: 24 weeks of either IgPro20 or placebo administration. 
• Study Period 2: 28 weeks of IgPro20 administration. 
• Study Period 3: additional treatment with open-label IgPro20 for subjects who have 
shown treatment benefit (see Section 4.1.3 for details) at Week 53. See Section 3.7 
for the definition of end of study. 
After Screening, subjects will be randomly assigned (1:1) to 1 of 2 sequences of double-blind 
weekly IMP treatment (0.5 g/kg IgPro20 and placebo). Subjects assigned to Sequence A will 
receive 0.5 g/kg IgPro20 for 24 weeks (Study Period 1) followed by 0.5 g/kg IgPro20 for 
28 weeks (Study Period 2). Subjects assigned to Sequence B will receive placebo for 
24 weeks (Study Period 1) followed by 0.5 g/kg IgPro20 for 28 weeks (Study Period 2). 

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The study data will be unblinded for the primary data analysis (ie, EOP1 analysis) after all 
subjects have completed all assessments for Study Period 1 and the data has been cleaned for 
primary data analysis. Subjects and caregivers, as well as site personnel involved in the 
conduct of the study, will remain blinded to treatment allocation until all subjects have 
completed all assessments for Study Period 2. 
At the Week 53 Visit, subjects may be eligible to continue to Study Period 3 if their TIS 
was ≥ 20 points at the Week 49 Visit. In Study Period 3 subjects will receive open-label 
IgPro20 0.5 g/kg SC infusions weekly until the end of the study (Section 3.7). 
The study population consists of subjects with the diagnosis of IIM according to European 
League Against Rheumatism/American College of Rheumatology (EULAR / ACR) criteria 
[Lundberg et al, 2017] and the presence of an active specific DM rash or other objective 
evidence. In addition, subjects must present with a defined degree of disease severity, 
manifested by a decrease in strength (MMT-8) or affected skin (CDASI) and a global 
physician impression (on a visual analog scale [VAS]). 
At Baseline (Week 1, before infusion of IMP), the site physicians must confirm that no 
clinically relevant improvement has occurred since the Screening Visit. Clinically relevant 
improvement is defined as ≥ 2 cm improvement on a 10-cm Physician Global Disease 
Activity Assessment VAS. A sufficient number of subjects will be screened in order to 
randomize approximately 126 subjects into the study. Subjects will be randomized at a 
1:1 ratio to receive either Sequence A or Sequence B during Study Period 1 and 
Study Period 2. 
During the first 24 weeks of IMP treatment, subjects will receive either IgPro20 0.5 g/kg SC 
or placebo (Study Period 1); during the subsequent 28 weeks all subjects will receive IgPro20 
at a dose of 0.5 g/kg (Study Period 2); and additionally, subjects will be treated with open-
label IgPro20 0.5 g/kg SC on a weekly basis until the end of the study (Study Period 3). 
If clinically relevant improvement is achieved at Week 17, the subject’s concomitant oral 
corticosteroid dose (≤ 20 mg/day prednisolone equivalent at Baseline) must be tapered 
downward according to the guidance provided (Section 5.3.2) and physiotherapy regimen 
may be modified. Prior to Week 17, the dose of corticosteroids should remain stable unless 
intolerance or side effects require dose reduction. 
In the event of clinical worsening at or after Week 9, site physicians may provide rescue 
corticosteroid treatment to subjects. Clinical worsening in this study is defined by meeting 
the criteria for DOW. If an unscheduled visit is used for DOW assessment, the 2 assessments 

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must be done at study visits at least 2 weeks apart. The oral rescue corticosteroid 
(prednisolone equivalent) treatment should be provided as per the Treating Physician’s 
standard of care (see Section 5.3.1). Any oral corticosteroid rescue treatment given during the 
first 24 weeks of randomized IMP treatment should be tapered by Week 25 as much as 
clinically possible to the corticosteroid dose at Baseline. Subjects who have received rescue 
corticosteroid treatment before Week 25 will be considered non-responders for the primary 
endpoint (see Section 2.1.2) and will continue study participation and IMP treatment. All 
subjects continuing in the study after Week 25 will receive IgPro20 treatment until Week 53. 
At Week 53, eligibility for continuation of open-label IgPro20 treatment in Study Period 3 
will be confirmed based on the TIS conducted in Week 49 (see Section 4.1.3 for details). If 
subjects do not continue treatment after Week 53, an EOP2 Safety Follow-up Telephone Call 
will be conducted to assess the subject’s safety status at Week 56 (or 4 weeks after the final 
infusion). If the subject continues treatment in Study Period 3, the telephone call will not be 
performed. 
See Section 3.7 for the definition of study end.

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Figure 1 
Study Design 
 
Abbreviations: DM = dermatomyositis; DOW = Definition of Worsening; EOP = End of Study Period; TIS = Total Improvement Score. 

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3.2 Dose and Dosing Regimen 
Subjects will be randomized 1:1 into 1 of 2 weekly treatment sequences: 
Sequence A: 0.5 g/kg IgPro20 for 24 weeks (Study Period 1) followed by 0.5 g/kg IgPro20 
for 28 weeks (Study Period 2) 
OR 
Sequence B: placebo for 24 weeks (Study Period 1) followed by 0.5 g/kg IgPro20 for 
28 weeks (Study Period 2) 
In Study Period 3, subjects who show benefit (TIS ≥ 20 points) at Week 49 will receive 
open-label 0.5 g/kg IgPro20 until the end of the study (Section 3.7). 
3.3 Scientific Rationale 
3.3.1 Study Design Rationale 
In this study, the EULAR/ACR classification criteria will be used to confirm the DM 
diagnosis. In short, the EULAR/ACR classification criteria assigns a numeric score to 
subjects with various clinical features such as skin and other clinical manifestations, muscle 
weakness patterns, laboratory evaluations, and muscle biopsy features. The aggregate score 
obtained will allow the subject to be classified with probable or definite IIM, and then 
presence of specific rash or other objective evidence (eg, muscle biopsy) will be used to 
identify the subjects with DM [Lundberg et al, 2017]. 
Evidence for the efficacy of immunoglobulin (IVIG or SCIG) treatment in patients with DM 
is limited. IVIG was shown to be efficacious in 2 randomized controlled studies of DM. The 
first study reported in 1993 by Dalakas et al, randomized 15 subjects with refractory disease 
to either corticosteroids and placebo or corticosteroids and high dose IVIG 
(1 gram/kg/day × 2 days monthly for 3 months). The authors reported that subjects treated 
with IVIG had a significant improvement in scores of muscle strength (p < 0.018) and 
neuromuscular symptoms (p < 0.035) compared to subjects treated with placebo who had no 
change. The second study reported in 2008 by Tian et al, randomized 60 elderly (> 60 years 
of age) subjects with DM to either corticosteroids or corticosteroids and low dose IVIG 
(0.4 gram/kg/day × 3 days monthly for 3 months). The authors reported greater clinical and 
biochemical improvements in the group treated with IVIG compared to those who were not 
treated with IVIG and minor side effects. In addition to these 2 existing randomized 
controlled studies of IVIG therapy in DM, several prospective and retrospective open-label 

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studies have been conducted and are summarized in a systematic review [Wang et al, 2012]. 
Clinical improvement was reported in up to 93% of subjects in early and therapy-refractory 
DM/PM; the typical dose was 2 g/kg IVIG given over 3 to 6 months. It has been reported that 
long-term use of IgG therapy is safe and effective, but this has not yet been confirmed in 
clinical studies. In a retrospective long-term study, 42 subjects with DM were followed; 
24 received IVIG therapy regularly. After 6 months of treatment, most IVIG-treated subjects 
(91.7%) presented with muscular disease remission, compared to 55.6% of non-IVIG-treated 
subjects (p = 0.007). During long-term IVIG treatment (up to 48 months), 16.7% of subjects 
presented with muscular relapses, which is significantly less than those who received no or 
only a few IVIG courses. The overall relapse-free period from any DM symptom was 
38.5 ± 24.3 months [Kampylafka, 2012]. 
SCIG has also been shown to be efficacious in treating subjects with DM/PM in smaller 
uncontrolled studies [Schleinitz et al, 2008; Danieli et al, 2011; Danieli et al, 2014; Gelardi 
et al, 2014; Cherin et al, 2016]. Long-term use of SCIG therapy has been described by Cherin 
[Cherin et al, 2016]. In doses of 1.9 g/kg monthly, 19 subjects were treated for a mean of 
18.8 months. Median doses infused were 180 mL/week using 2 infusion pumps; similar to the 
infusion parameters in this study. Although these smaller studies have shown promising 
results, no large-scale or randomized controlled studies are available to corroborate the 
efficacy of SCIG against placebo in subjects with DM, which is a reason for the initiation of 
this study. 
This study will build on previous evidence that has shown efficacy in the first 3 to 6 months 
of IgG treatment and will investigate whether the initial improvement can be sustained for 
6 months to up to 7 years. 
The primary endpoint of this study is based on the TIS [Aggarwal et al, 2017]. The TIS is a 
sum response criterion which incorporates 6 weighted International Myositis Assessment and 
Clinical Studies (IMACS) CSMs, previously validated for the assessment of DM and PM 
[Rider et al, 2003; Rider et al, 2002]. They include physician and patient global activity on a 
10-cm VAS, muscle strength measured by manual muscle testing (MMT-8), physical function 
measured by the Health Assessment Questionnaire, extramuscular global activity measured 
by the physician on a 10-cm VAS, and the most abnormal serum muscle enzyme. The TIS 
was evaluated on patient profiles using expert consensus (gold standard) and was validated 
using data from a clinical study [Oddis et al, 2013], the largest study done in DM and PM so 
far. A total improvement score (range: 0 to 100), determined by summing scores for each 
CSM, was based on improvement in and relative weight of each CSM. Thresholds for 
minimal, moderate, and major improvement were ≥ 20, ≥ 40, and ≥ 60 points on the TIS. 

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The TIS was chosen as the basis for the primary endpoint of this study given the fact that no 
single CSM adequately covers all the domains in DM. For example, muscle enzyme levels 
can be normal in active DM, and active muscle weakness in DM can occur without active 
rash. The previously developed IMACS response criteria were based on at least 20% 
improvement in 3 of 6 CSMs, with no more than 2 CSMs worsening by at least 25% (which 
cannot be muscle strength). The limitations of these criteria included that equal weights had 
been applied to each CSM and a quantitative or continuous outcome was lacking. 
Given that the TIS is focused on improvement, one of the limitations is that it cannot 
differentiate between no change and worsening and, therefore, these criteria might not be 
applicable in studies of worsening disease activity (ie, disease flare designs) in DM. 
However, this study will only include subjects with active disease with background 
immunomodulatory therapy and IgPro20 will be used as add-on therapy. It is, therefore, not 
expected that a relevant number of subjects will experience disease flares that meet the 
criteria for DOW. Any subject who meets DOW criterion will be offered standard-of-care 
rescue corticosteroid treatment until recovery. By definition of the primary endpoint, these 
rescued subjects will be classified as treatment failures (non-responders). 
With the abovementioned limitations of TIS in identifying deterioration, key secondary 
endpoints have been defined that allow for identification of both improvement and 
deterioration and that are relevant in the assessment of DM. MMT-8 is a set of 8 designated 
muscles which will be tested bilaterally (potential score 0 to 150): 7 biaxial muscles with 
potential score 0 to 140 and 1 axial (neck flexors) with potential score 0 to 10. Validation of 
MMT-8 has demonstrated internal reliability and consistency, rater reliability, convergent 
construct validity, and responsiveness in assessing strength in patients with adult DM (also in 
PM and juvenile IIM) [Rider et al, 2010]. The MMT-8 is 1 of the CSMs and is the most 
heavily weighted CSM in the TIS. 
The CDASI in its modified version (version 2) is a validated tool of skin disease activity 
(3 items) and damage (3 items) assessment. It has been developed as an instrument for 
clinical studies and longitudinal patient assessment [Klein et al, 2008; Goreshi et al, 2012; 
Yassaee et al, 2010]. 
These 2 validated tools (MMT-8 and CDASI) will enhance knowledge on the outcome data 
as key secondary endpoints, as these 2 domains (muscle strength and skin disease activity) 
are the most relevant manifestations of DM. They will provide evidence of treatment effect to 
supplement the composite improvement scale — TIS. 

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The primary endpoint will be assessed at Weeks 17, 21, and 25. In Study Period 2, all 
subjects will be on IgPro20 treatment; subjects who were previously allocated to placebo will 
have the opportunity to respond to IgPro20 treatment, and subjects who were already treated 
with IgPro20 in Study Period 1 will have the opportunity to maintain or improve their 
response. In addition, starting at Week 17, stable background concomitant corticosteroid 
treatment must be tapered, if the subject has demonstrated clinically relevant improvement, 
ie, ≥ 2 cm improvement from Baseline on a 10-cm Physician Global Disease Activity 
Assessment VAS (see Section 5.3.2). Tapering concomitant corticosteroid treatment will 
reduce the risk for steroid-related side effects, eg, uncontrolled hypertension, hyperglycemia, 
and weight gain. 
Study Period 3 of the study will provide data on the long-term safety and efficacy of 
treatment with IgPro20. Eligible subjects may continue treatment with open-label IgPro20 in 
Study Period 3 starting at Week 53. The study will end 
: 
 months 
 
 
 for the indication Dermatomyositis, 
unless the decision has been made by CSLB that 
 
) is not reasonably possible, in which case the study will end 6 months after notice is 
provided to the applicable regulatory authorities of that decision. 
CCI
CCI
CCI
CCI
CCI
CCI

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3.3.2 Dose Rationale 
The few small studies that have utilized SCIG for treatment in patients with DM/PM relied 
on precedence from IVIG dosing to determine optimal SCIG dosing. From the first study in 
1987 in a single subject with PM, to the Dalakas 1993 study and 2 Japanese studies, a 2 g/kg 
body weight per month IVIG dose was used as a standard dose in treatment of subjects with 
DM/PM [Dalakas, 1991; Dalakas et al, 1993; Saito et al, 2008; Miyasaka et al, 2012], and 
has been commonly adopted in clinical practice and standard of care. In smaller, open-label 
studies, similar SCIG monthly doses were used (2 g/kg body weight [Danieli et al, 2011; 
Gelardi et al, 2014], 1.9 g/kg body weight [Cherin et al, 2016], and 1.7 g/kg body weight 
[Schleinitz et al, 2008]) in subjects with DM/PM. In these studies, the standard IVIG dose for 
subjects with DM/PM was divided into weekly SCIG doses approximately equivalent to 
0.5 g/kg body weight per week. Positive responses were noted as early as 3 months (the 
earliest tested time points for improvement). In addition, CSLB performed a simulation using 
a previously developed population PK model based on data from subjects with CIDP 
(mechanism of action postulated to be immunomodulatory) to assess in subjects with DM, 
predicted IgG exposure after treatment with a 0.5 g/kg SC dose of IgPro20. These analyses 
predicted that the median average concentration estimated as area under the 
concentration-time curve for a specific time interval (AUC0-time)/dosing interval was 
comparable following 2 g/kg monthly IVIG (22.20 g/L) and 0.5 g/kg weekly SCIG 
(20.85 g/L), after 8 to 10 weeks. The analysis also predicted trough concentration (Ctrough) at 
steady state, which appears to be higher for 0.5 g/kg weekly SCIG (17.12 g/L) compared to 
2 g/kg monthly IVIG (13.15 g/L). The higher Ctrough following 0.5 g/kg SCIG dose is likely 
due to a shorter dosing interval following weekly dosing. Overall, this analysis provides 
evidence of comparable steady-state IgG exposure after SC dosing of 0.5 g/kg body weight 
weekly and IV dosing of 2 g/kg body weight monthly. Based on the clinical evidence and 
outcome of the simulations, we have chosen 0.5 g/kg body weight as the weekly IgPro20 
dose in this study. 
A weekly flexible SC infusion dosing regimen will be employed during the study. This means 
that the subject and study site can select the most appropriate and convenient regimen based 
on body weight and subject preference (see Appendix 15 for examples). The maximum 
volume per infusion will be 50 mL which can be divided into up to 3 infusion sites per 
infusion. The infusion rate can be increased gradually to 50 mL/h per infusion site, as 
tolerated during the study. 
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3.4 Planned Study Duration 
After up to 2 months of Screening, the duration for an individual subject is expected to be 
24 weeks in Study Period 1 and 28 weeks in Study Period 2. The maximum study duration in 
the open-label Study Period 3 will be approximately 7 years; however, subject participation 
will end if the drug becomes commercially available or if there is no longer benefit from the 
study drug. For subjects not eligible to participate in Study Period 3, study duration will be 
up to 56 weeks (including the Follow-up Telephone Call).  
See Section 3.7 for the definition of end of study. 
3.5 Planned Number of Subjects 
A sufficient number of subjects will be screened in order to randomize approximately 
126 subjects into the study (63 per treatment sequence) at approximately 80 to 100 study sites 
globally. At least 10 Japanese subjects will be randomized at study sites in Japan. 
3.6 Definition of Start of the Clinical Study 
The start of the clinical study is defined as the date of the first signed informed consent of a 
potential subject. 
3.7 Definition of End of the Clinical Study 
The study will end 
: 
 months 
 
 
 for the indication Dermatomyositis, 
unless the decision has been made by CSLB that 
 
 is not reasonably possible, in which case the study will end 6 months after notice is 
provided to the applicable regulatory authorities of that decision. 
3.8 Study Oversight 
3.8.1 Steering Committee 
The steering committee will provide scientific support, scientific expertise for substantial 
amendments to the protocol, advice to the investigators on all aspects of the study, and 
support recruitment/feasibility of the study (ie, educational training or lectures) and 
preparation of publications. The steering committee will consist of external experts in the 
CCI
CCI
CCI
CCI
CCI
CCI

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fields of dermatology, rheumatology, neurology, and statistics who have experience treating 
patients with DM and/or experience in clinical studies. 
3.8.2 Independent Data Monitoring Committee 
The independent data monitoring committee (IDMC) will monitor the efficacy and safety 
data generated during Study Period 1 and Study Period 2 (through Week 53 / Week 56) in 
order to conduct assessments of the benefit-risk profile. The IDMC will consist of external 
experts in the fields of dermatology, rheumatology, neurology, hematology/coagulation, and 
statistics who have experience treating patients with DM and/or experience in clinical 
studies. The IDMC will receive notification of all serious adverse events (SAEs), AESIs, and 
deaths reported throughout the study. In addition, the IDMC will meet at regularly scheduled 
intervals to evaluate compiled efficacy and safety data to assess overall benefit-risk. After 
each meeting, recommendations will be issued to CSLB regarding the continuation of the 
study, potential need for protocol modifications, or study termination. No success or futility 
thresholds will be set for the IDMC reviews; CSLB will not stop the study based on these 
data unless a major safety issue has been identified (see Section 3.9). The composition, 
activities, and responsibilities of the IDMC will be described in the IDMC Charter. 
3.9 Thromboembolic Event Stopping Criteria 
The elevated risk of TEEs associated with IgPro20 treatment as compared with placebo will 
be evaluated by the IDMC on an ongoing basis and at the defined data cut points described 
below. 
Data Cut Point – percentage of subjects having reached the end of the primary endpoint 
analysis period (Week 25): 
• 25% 
• 50% 
• 75% 
• 100% 
TEE rates will be calculated for each treatment group, along with relative risk (IgPro20 over 
placebo) and associated 80% credibility intervals based on Bayesian technique [Barker et al, 
2008] which accounts for the consideration of differential exposure times. The analysis will 
include data from Study Period 1 and Study Period 2, as available. Further details and 
calculations will be provided in the IDMC statistical analysis plan. 

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An excess of TEEs in IgPro20 over placebo will be considered if the lower limit of the 80% 
credibility interval for the relative risk is > 1.1 (presumably an IgPro20 rate greater than 10% 
[relative] over placebo). In this case the IDMC may recommend stopping the study. 
Further details on how the IDMC will review the TEE data and provide recommendations is 
included in the IDMC Charter. 
Monitoring of TEE risk is described in Section 8.1.2.2. 
4 Selection and Withdrawal of Subjects 
4.1 Eligibility Criteria 
The study population will be selected on the basis of the inclusion and exclusion criteria 
described in the sections below. Each subject should meet all of the inclusion criteria and 
none of the exclusion criteria for this study. Subject eligibility should be reviewed and 
documented by an appropriately medically qualified member of the investigator’s study team 
before subjects are included in the study. Full eligibility for the study will be determined at 
Baseline. 
4.1.1 Inclusion Criteria 
To be enrolled and randomized into the study, subjects must meet all of the following 
inclusion criteria: 
1 Capable of providing written informed consent by signing an informed consent form 
(ICF) and willing and able to adhere to all protocol requirements 
2 Age ≥ 18 years 
3 Diagnosis of at least probable IIM per EULAR/ACR Classification Criteria (Appendix 
2): minimum aggregate score of 5.5 without muscle biopsy and 6.7 with muscle biopsy 
(historical muscle biopsy is acceptable) which includes confirmation of DM rash/skin 
manifestation (present or by history [historical skin biopsy is required for amyopathic 
DM subjects]) 
4 Disease activity defined by: 
• Presence of DM rash/skin manifestation (eg, Gottron's papules/sign, heliotrope rash, 
periorbital edema, V sign, Shawl sign) at Screening Visit - OR - 

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• One objective disease activity measure within 3 months before Baseline: 
a. Magnetic resonance imaging (MRI) scan showing active inflammation 
(edema) of a proximal skeletal muscle - OR - 
b. Electromyogram showing acute changes such as spontaneous activity not 
explained by other disease - OR - 
c. Muscle biopsy with perivascular or perimysial inflammation - OR - 
d. CK > 4 times the upper limit of normal (4 × ULN) 
5 Disease severity at Screening and Baseline defined by a minimum value of 2 cm on a 
10-cm Physician Global Disease Activity VAS* and: 
• MMT-8 ≤ 142 – OR - 
• CDASI total activity score ≥ 14 
 
* On the VAS, 0 cm = absence of disease activity and 10 cm = extremely active/severe disease activity. 
6 Subject has failed previous DM treatment or is on DM treatment such as 
immunosuppressants and/or antimalarials on a stable dose ≥ 3 months before Baseline; 
and/or oral corticosteroids (≤ 20 mg/day prednisolone equivalent) on a stable dose 
≥ 1 month before Baseline (also see Section 7.2 for details) 
4.1.2 Exclusion Criteria 
Subjects must not be enrolled or into the study or randomly assigned to treatment if they meet 
any of the following exclusion criteria: 
1 
Cancer-associated myositis, defined as the diagnosis of myositis within 2 years of the 
diagnosis of cancer 
2 
Evidence of malignancies diagnosed within the previous 5 years 
Note: Subjects with a history of carcinoma in situ of the cervix that has been excised 
and cured with ≥ 5 years since excision or subjects with documented history of treated 
basal or squamous cell skin cancer may be enrolled into the study. 
3 
Physician Global Damage Assessment ≥ 3 on a 5-point Likert scale where a score of 
3 represents severe damage 
4 
Clinically relevant improvement between Screening and Baseline, defined by ≥ 2 cm 
improvement on a 10-cm Physician Global Disease Activity Assessment VAS 
5 
Known or suspected hypersensitivity or other severe reactions to IgPro20 or to any of 
its excipients, or other immunoglobulins or severe reactions to blood products 

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6 
Other significant medical conditions that could increase the risk to the subject, eg: 
• History of allogeneic bone marrow/stem cell transplant/solid organ transplant 
• Cardiac insufficiency (New York Heart Association Class III or IV) or unstable 
ischemic heart disease 
• Chronic kidney disease stage IV or V 
• Recent surgery requiring general anesthesia within the previous 4 weeks before 
Screening 
• Known hyperprolinemia type I or type II 
• Thrombophilic abnormalities including blood hyperviscosity, protein C or 
protein S deficiency, antithrombin-III deficiency, plasminogen deficiency, 
antiphospholipid antibodies, Factor V Leiden mutation, dysfibrinogenemia, or 
prothrombin G20210A mutation 
• History of documented thrombotic episode, eg, pulmonary embolism (PE), deep 
vein thrombosis (DVT), myocardial infarction, thromboembolic stroke at any time 
• More than 3 of the following specified risk factors for TEEs (documented and 
current conditions) occurring concurrently: atrial fibrillation, coronary disease, 
diabetes mellitus, dyslipidemia, hypertension, obesity (body mass 
index ≥ 30 kg/m2), recent significant trauma and immobility (wheelchair-bound or 
bedridden) 
• Uncontrolled, severe, or rapidly progressive interstitial lung disease which will 
prevent the subject from successful participation in the study 
• Severe skin disease at planned infusion sites that would make SC infusions 
infeasible 
• Medical conditions whose symptoms and effects could alter protein catabolism and 
or IgG utilization (eg, protein-losing enteropathies, nephrotic syndrome, known 
immunoglobulin A [IgA] deficiency with antibodies to IgA) 
7 Other conditions which would prevent correct assessment or lead to impaired muscle 
strength (eg, other neurological disorders including, but not limited to, Parkinson’s 
disease or severe musculoskeletal conditions like severe osteoarthritis or deformities) 

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8 Laboratory exclusions at screening: 
• Positive result at for any of the following: human immunodeficiency virus (HIV), 
hepatitis B virus (HBV), or hepatitis C virus (HCV) 
• Creatinine > 1.5 × ULN or blood urea nitrogen (BUN) > 3 × ULN 
9 Any of the following therapies: 
• Within 1 month before Baseline: IM, IV, or intra-articular corticosteroids including 
adrenocorticotropic hormone (any dose), doses > 20 mg/day prednisolone 
equivalent (any route), or any change to physiotherapy 
• Within 2 months before Baseline: IgG (Note: Subject may enroll < 2 months after 
stopping IgG therapy if clinical deterioration is experienced after withdrawal.) 
• Within 3 months before Baseline: plasma exchange or plasmapheresis 
• Within 6 months before Baseline: cyclophosphamide or alkylating agents 
• Within 6 months or 5 half-lives of the drug, whichever is longer, before Baseline: 
other biologic therapies including investigational agents 
• Within 9 months before Baseline: rituximab or evidence of persistent B cell 
depletion after stopping therapy 
10 Male subject or female subject of childbearing potential either not using or not willing 
to use a medically reliable method of contraception (see Section 7.4.2), not sexually 
abstinent during the study, or not surgically sterile before study enrollment 
11 Pregnant or breastfeeding 
12 Alcohol, drug, or medication abuse within 1 year of providing informed consent 
13 Previously received IMP in this study or failed Screening more than 1 time in this 
study 
14 Involved in the planning and/or conduct of this study (applies to CSLB staff, staff at 
the study site, and third-party vendors) 
15 Any issue that, in the opinion of the investigator, would render the subject unsuitable 
for participation in the study or unable to comply with study procedures, eg, inability 
to self-administer IMP or by aid through a caregiver 

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4.1.3 Eligibility Criteria for Study Period 3 
At the Week 53 Visit, subjects may continue to Study Period 3 if they have a TIS ≥ 20 points 
at the Week 49 Visit. Subjects who participate in Study Period 3 will continue to receive 
open-label IgPro20 0.5 g/kg SC infusions weekly until the end of the study (Section 3.7). 
4.2 Screen Failures 
Screen failures are defined as individuals who consent to participate in the clinical study but 
who do not meet the eligibility criteria for participation (see Section 4.1) by Baseline. 
A minimal set of information including demography, eligibility criteria, screen failure details, 
and any SAE should be recorded for all individuals considered screen failures. 
Individuals who do not meet the eligibility criteria by Baseline (ie, screen failure) other than 
actual DM diagnosis and disease activity may be rescreened once for failed eligibility. 
4.3 Discontinuation of IMP and Subject Study Withdrawal 
4.3.1 Discontinuation of IMP 
If any of the following apply, the subject must discontinue IMP: 
• Subject wishes to discontinue IMP 
• Subject withdraws consent for study participation 
• Investigator advises that the subject’s safety or well-being could be compromised by 
further treatment 
• Subject or caregiver is not able to administer the SC infusions at home by Week 4 
• Subject has confirmed diagnosis of DVT or PE 
• Subject has Grade 3 hemolysis (see Section 8.1.2.3) 
• Subject becomes pregnant 
• Relevant treatment noncompliance 
During participation in Study Period 3, if the investigator determines that the subject is no 
longer benefiting from IgPro20 and/or is showing signs of clinical deterioration in DM status, 
the subject must be discontinued from the study. 
The investigator should record in the electronic case report form (eCRF) and in the subject's 
medical records the reason and date of IMP discontinuation. 

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Subjects who discontinue IMP will be encouraged to remain in the study until Week 25 if 
discontinuation occurs during Study Period 1, or until Week 53 if discontinuation occurs 
during Study Period 2, in order to collect study assessments. The Safety Follow-up Telephone 
Call will not be performed for subjects who discontinue IMP treatment but remain in the 
study for at least 1 subsequent study visit 4 weeks after IMP discontinuation. See Schedule of 
Assessments for details on assessments no longer collected after IMP discontinuation if the 
subject remains in the study, and Section 4.3.2 for details on handling subject withdrawal 
from study. 
Subjects who discontinue IMP during Study Period 3 will be withdrawn from the study. 
Withdrawal from IMP Study Period 1 or Study Period 2 renders a subject ineligible for 
participation in Study Period 3 of this study. 
4.3.2 Subject Withdrawal from Study 
Subjects may withdraw from the study at any time at their own request or at the discretion of 
the investigator or CSLB. The investigator should record in the eCRF and in the subject's 
medical records the reason for study withdrawal, if provided by the subject in the case of 
withdrawn consent, and date of subject withdrawal. 
In accordance with the International Council for Harmonisation of Technical Requirements 
for Pharmaceuticals for Human Use (ICH) principles of Good Clinical Practice (GCP), the 
investigator always has the option to advise a subject to withdraw from treatment or the study 
if the subject’s safety or well-being is compromised by his or her further participation in the 
study. Concern for the interests of the subject must always prevail over the interests of the 
study. 
4.3.2.1 Procedures for Handling Withdrawals 
If a subject is withdrawn from the study during Study Period 1 or Study Period 2, attempts 
will be made to complete and document the Week 53 assessments. If the subject is withdrawn 
from the study after receiving IMP, every effort will be made to ensure that the relevant 
safety assessments, including the 4-week Safety Follow-up Telephone Call (only in 
Study Period 1 and Study Period 2), are completed. 

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If the subject is withdrawn from the study during Study Period 3, the EOP3 Visit assessments 
must be performed. The subject may also be asked by the investigator to complete additional 
study assessments. If, during Study Period 3, the investigator determines that the subject is no 
longer benefiting from IgPro20 and/or is showing signs of clinical deterioration in the status 
of DM, the subject must be discontinued from the study. If the subject withdraws from the 
study, and also withdraws consent for disclosure of future information, CSLB may retain and 
continue to use any data and samples collected before withdrawal of consent. 
4.3.3 Subjects Lost to Follow-up 
If a subject repeatedly fails to return for scheduled visits, the site must attempt to contact the 
subject and counsel the subject on the importance of maintaining the assigned study visit 
schedule and ascertain whether or not the subject wishes to and/or should continue in the 
study. All attempts to contact the subject should be documented in the subject’s medical 
record. 
A subject will be considered lost to follow-up if he or she repeatedly fails to return for 
scheduled visits and is unable to be contacted by the study site. Subjects lost to follow-up 
will be considered to have withdrawn from the study. 
4.3.4 Replacement Policy 
Subjects withdrawn from the study will not be replaced. 
4.3.5 Consequences of Subject Withdrawal or IMP Discontinuation 
Subjects who have been withdrawn from the study or who have discontinued IMP before 
Week 25 will be considered non-responders for the primary endpoint analysis, except if 
withdrawal or discontinuation was due to the Ukraine war (see Section 10.4.2) Withdrawal 
from IMP during Study Period 1 or Study Period 2 renders a subject ineligible for 
participation in Study Period 3 of this study. 

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5 Study Interventions 
5.1 Investigational Products 
5.1.1 Description of IgPro20 
Substance name 
IgPro20 
Active substance 
Human normal IgG 
Trade name 
Hizentra® 
Storage 
Temperature-controlled and monitored conditions at 2 to 8°C in a 
secure storage area at the site 
Dosage form 
Liquid, ready-to-use 20% IgG solution stabilized with 250 mmol/L 
L-proline and 20 mg/L polysorbate 80 
IgG = immunoglobulin G. 
IgPro20 for SC administration will be provided as a 20% IgG solution in a vial containing 
10 g IgG in a 50 mL volume. IgPro20 will be manufactured by CSLB, in accordance with 
ICH GMP guidelines and local regulatory requirements. 
Information on the preparation and SC administration of IgPro20 is provided in the IMP 
manual. 
5.1.2 Description of Placebo 
Substance number Not applicable 
Substance 
Placebo 
Trade name 
Not applicable 
Storage 
Temperature-controlled and monitored conditions 2 to 8°C in a 
secure storage area at the site  
Dosage form 
2% human albumin solution in 250 mmol/L L-proline and 20 mg/L 
polysorbate 80 manufactured from the licensed, pasteurized CSLB 
human albumin 5% final product 
CSLB = CSL Behring. 
Placebo for SC administration will be manufactured by CSLB, and provided in vials of 
50 mL volume. 

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5.1.3 Dosing and Administration of IgPro20 and Placebo 
The investigator (or delegate) will dispense or administer IMP only to subjects included in 
this study following the procedures set out in this study protocol. Information on the dosing 
characteristics of IMP is provided in Table 5. 
Table 5 
Investigational Medicinal Product Dosing Characteristics 
SC Infusion Parameter 
IgPro20 
Placebo 
Route 
SC 
SC 
Anatomical location 
Abdomen, thigh, upper arm, 
and/or lateral hip 
Abdomen, thigh, upper arm, 
and/or lateral hip 
Total infusion volume 
Dependent on body weight 
(eg, 80 kg subject, 200 mL) 
Dependent on body weight 
(eg, 80 kg subject, 200 mL) 
Infusion duration 
Volume and number of 
infusion sites dependent 
Volume and number of 
infusion sites dependent 
SC = subcutaneous. 
All IMP in the study will be administered by SC infusions as weekly doses of 0.5 g/kg. At 
least the first SC infusion for Week 1 and Week 2 must be administered during the study visit 
at the investigational site to allow for training and confirmation of appropriate SC infusion 
technique. After Week 1 and Week 2, all weekly SC infusions will be self-administered at 
home. The SC infusions can be performed on 1, 2, 3, or 4 days each week and more than 
1 infusion can be performed on a single day. It is preferred that the SC infusions be 
performed in a similar way throughout the study, ie, the same number of infusions and the 
same number of days each week after Week 2; however, the dosing regimen can be adjusted 
during the study in case of intolerability or inconvenience. All SC infusions for the previous 
week must be performed before the following study visit, eg, all Week 4 SC infusions 
performed before the day of the Week 5 Visit. Body weight will be measured at every study 
visit and changes in body weight > 2 kg will require a dose volume adjustment. The total 
dosing volume of IMP will be managed by the interactive response technology (IRT) system. 
Preparation instructions are provided in the IMP manual. 

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Subcutaneous Infusion Dosing Regimen Example: 
Total dose volume per week needed for 80 kg subject = 200 mL. See Appendix 15 for weekly 
weight-based dosing regimen table. 
Infusion dosing options may include but are not limited to the following: 
• Total volume can be delivered on 1 day using 4 infusions of 50 mL 
• Total volume can be delivered on 2 days using 2 infusions of 50 mL each day 
• Total volume can be delivered on 3 days using 1 infusion of 50 mL each for 2 days 
and 2 infusions of 50 mL on the third day 
• Total volume can be delivered on 4 days using 1 infusion of 50 mL each day 
The SC infusions should be performed at appropriate infusion sites (eg, on the abdomen, 
thighs, upper arm, and/or lateral hip). The number of infusion sites is dependent upon the 
total volume administered. The maximum rate and the maximum volume per infusion site 
should not be exceeded and increases up to the maximum should be done gradually as 
tolerated. 
Subcutaneous Infusion Volume and Rate of Infusion: 
• Volume (mL/Site): ≤ 20 for Week 1 and ≤ 50 as tolerated for all subsequent study 
weeks 
• Rate of infusion (mL/hour/site): ≤ 20 for Week 1 and ≤ 50 as tolerated for all 
subsequent study weeks 
Subcutaneous Treatment Training 
Every randomized subject (and their caregiver, if applicable) will receive instructions on the 
SC infusion pump and SC infusion technique. Training on SC infusion through the use of 
video and/or verbal instruction should first be discussed with the subject during Screening to 
familiarize the subject with the SC infusion procedure before randomization. Site staff should 
recommend the weekly dosing regimen based on the subject’s weight, ie, number of SC 
infusions needed to deliver the total volume in 1 week. The Crono Pump should be 
programmed by the site staff with the correct volume based on body weight before the study 
visit ends. The programmed pump volume should be confirmed at every study visit. 
The subject will be trained at the study site at the Week 1 Visit and Week 2 Visit by the site 
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(Week 3 and Week 4), only if the subject or caregiver experiences difficulty with the SC 
infusion technique. Additional trainings needed beyond Week 2 will be documented in the 
eCRF. No other study procedures are required to be performed at these additional SC infusion 
trainings. If the subject or caregiver still is not able to administer the SC infusions at home by 
Week 4, the subject will be withdrawn from treatment and encouraged to continue study 
assessments to Week 25 (EOP1). 
Detailed information on the home storage, preparation, and SC infusion of IMP will be 
provided in the IMP manual. 
The CRONO S-PID 50 Pump will be provided to deliver the SC infusions to the subject. 
Refer to the IMP manual and the CRONO Pump user guide for further details on the device. 
5.1.3.1 Dosing Modification 
Modifications of IMP dose are not permitted. 
5.1.3.2 Treatment Compliance 
Subjects will record details of their infusion in the diary including whether the caregiver 
assisted in the SC infusion. In addition, subjects will bring all of their used/partially used 
vials of IMP to the study site at every study visit. Treatment compliance will be monitored 
based on review of the diary data and the number of returned vials, the results of which will 
be documented. 
5.1.3.3 Overdose 
Overdose is defined as the accidental or intentional infusion or ingestion of any dose of a 
product that is considered excessive. The effects of any potential overdose with IgPro20 have 
not been studied. See Section 9.6.5 for IMP overdose reporting requirements. 
5.1.4 Packaging, Labeling, Supply and Storage 
5.1.4.1 Packaging and Labeling 
IMP will be packaged and labeled according to current ICH GMP and GCP guidelines and 
national legal requirements. Specific details regarding packaging of IMP are provided in the 
IMP manual. 

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5.1.4.2 Supply and Storage 
IMP will be supplied to the study sites by CSLB or delegate. 
Refer to Section 5.1.1 and Section 5.1.2 for IMP site storage instructions. Subjects will be 
required to place IMP in home refrigerators for storage. Storage details will also be included 
in the IMP manual provided to the sites and the subjects. 
5.2 Accountability and Destruction 
IMP must be used only as directed in the clinical study protocol. 
The investigator (or delegate) will confirm receipt of all shipments of IMP (IgPro20 and 
placebo) in the IRT system. All supplies of IMP must be accounted for throughout the study. 
Records for the delivery of IMP to the study site, the inventory at the study site, the use by 
each subject, and the destruction or return of IMP to CSLB/designee must be maintained by 
the investigator (or delegate) using the appropriate form or IRT system. The investigator 
(or delegate) must provide reasons for any discrepancies in drug accountability. 
For Japan sites only, drug inventory and accountability logs/reports must be dated and signed 
by the head of the medical institute or the study drug storage manager (if assigned by the 
head of medical institute). The study drug storage manager may delegate the tasks to the 
responsible hospital pharmacists. 
Further details regarding accountability and destruction of IMP are provided in the IMP 
manual. 
5.3 Other Interventions 
5.3.1 Oral Rescue Corticosteroid Treatment 
In the event of clinical worsening (eg, subject meets DOW criteria) between Week 9 and 
Week 25, the investigator may decide that the subject requires oral rescue corticosteroid 
treatment for recovery. Intravenous pulse rescue corticosteroid treatment is not permitted. 
In this case, the following guidance applies: 
1 Oral rescue corticosteroid treatment should be given only if DOW criteria are met. 
2 The earliest that oral rescue corticosteroid treatment can be given is Week 9, to allow 
for IMP steady state to be achieved. The DOW consists of meeting 1 of the following 
3 criteria on 2 consecutive study visits at least 2 weeks apart, up to Week 25: 

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a) Physician Global Disease Activity Assessment VAS worsening ≥ 2 cm* and 
MMT-8 worsening ≥ absolute 10%, - OR - 
b) Extramuscular Global Assessment VAS worsening ≥ 2 cm, - OR - 
c) Any 3 of 6 CSMs (see Section 3.3.1) worsening by ≥ absolute 20% 
* If baseline Physician Global VAS is 8 to 10, then any worsening on Physician 
Global VAS is acceptable as long as MMT-8 criterion is met. 
3 The rescue oral corticosteroid (prednisolone equivalent) treatment should be provided 
as per the Treating Physician’s (see Table 7) standard of care but should not exceed 
1 mg/kg/day for up to 5 days. 
4 It is recommended that after oral rescue corticosteroid treatment, the oral 
corticosteroid dose be returned to Baseline dose by Week 25, if clinically possible. 
Clinical worsening according to DOW is finished at the first subsequent study visit where 
none of the criteria are met. A potential next DOW and possible rescue oral corticosteroid 
treatment can start only after the end of the preceding DOW. 
Oral rescue corticosteroid treatment is not permitted in Study Period 2. During 
Study Period 3, the use of additional oral corticosteroid treatment is permitted. Prior to 
increasing corticosteroids, investigators should verify that subjects are still benefiting from 
treatment with IgPro20. 
Consequence of oral rescue corticosteroid treatment during Study Period 1 
The subject will be classified as a non-responder at Week 25 but will continue the study as 
long as the subject remains on IMP treatment. 

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5.3.2 Concomitant Oral Corticosteroid Tapering 
The permitted ≤ 20 mg/day concomitant oral corticosteroid (prednisolone equivalent) dose 
must be tapered starting at Week 17 if the subject has demonstrated clinically relevant 
improvement, ie, ≥ 2 cm improvement on the 10-cm Physician Global Disease Activity 
Assessment VAS. The taper regimen to be followed depends on the subject’s previous stable 
corticosteroid dose: 
a) If stable dose is 11 to 20 mg/day oral prednisolone equivalent, decrease dose by 
2.5 mg/day every 2 weeks, as tolerated, to 10 mg, then by 1 mg/day every 2 weeks as 
tolerated 
b) If stable dose is ≤ 10 mg/day, taper by 1 mg/day every 2 weeks as tolerated 
If lowering the dose of corticosteroids results in an increase in symptoms that does not meet 
criteria for oral corticosteroid rescue treatment but in the judgement of the Treating Physician 
requires response, it is permissible to increase the dose of concomitant oral corticosteroid to 
up to 125% of the Baseline corticosteroid dose; only an increase to doses greater than 125% 
of the Baseline corticosteroid dose following corticosteroid taper starting at Week 17 will be 
considered a “rescue” corticosteroid dose. Once clinical stabilization has occurred, the taper 
schedule will resume (ie, taper will continue to a dose in which the subject remains stable). 
5.3.3 Access to IMP after the End of Study Participation  
Subjects will not be provided with IMP by CSLB after completion or discontinuation from 
the study.  
6 Allocation to Treatment 
6.1 Subject Assignment 
After providing written informed consent, the subject will be issued with a study-level unique 
subject identification number via an IRT system. The subject identification number will be 
used to identify the subject for the duration of the study. Subject identification numbers will 
not be reassigned. If a subject is rescreened, the original subject identification number will be 
used. 

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6.2 Randomization Procedures 
Eligible subjects will be randomized to 1 of the 2 double-blind treatment sequences by means 
of IRT. The IRT will assign the IMP to each subject and the correct dose volume based on the 
subject’s body weight. Randomization will be done centrally and CSLB will supply the 
investigator with a user guide for the IRT. 
Randomization will be stratified by region (Japan vs. non-Japan) and by MMT-8 assessment 
(≤ 142 points vs. > 142 points) to ensure even distribution of subjects to treatment sequences 
within stratum. For subjects with MMT-8 score at Baseline of ≤ 142 points and a muscle 
group not able to be assessed because of a non-DM related injury or an amputation, the 
Evaluating Physician will need to determine the appropriate stratification according to DM 
with muscle weakness (classic DM, ≤ 142 points) or DM with little or no muscle weakness 
(hypo/amyopathic DM, > 142 points) and assign appropriate stratum for this subject. 
The randomization list will be generated by the IRT external service provider according to 
the approved randomization specifications. The IRT external service provider will keep the 
randomization code on file. 
6.3 Blinding Procedures 
6.3.1 Blinding Method 
Throughout the study, the IMP will be dispensed in 50-mL vials in blinded packaging that 
ensures IgPro20 will not be distinguishable from the placebo.  
Adequate procedures are in place to ensure the integrity of the blinded data within CSLB. 
Study data will be provided to the IDMC as unblinded data, as requested. These procedures 
are outlined in the IDMC Charter and a separate Study Blinding/Unblinding Procedures 
document. 
Serum IgG levels will not be disclosed to the investigative site or any blinded study 
personnel before unblinding the study. 
6.3.2 Breaking the Blind for an Emergency 
The randomization code for individual subjects may be unblinded to a site during the study in 
emergency situations for reasons of subject safety, if knowing treatment assignment will 
change subject management. In case of an emergency situation for the reason of subject 
safety, the investigator should use the IRT to identify the treatment allocation for a subject. 
Whenever possible, the investigator should consult with CSLB before unblinding the 

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randomization code. The reason for unblinding the randomization code must be fully 
recorded in the subject’s source documents, and the investigator must follow the defined 
procedures provided in the study reference manuals. The subject’s treatment allocation 
should not be recorded in the subject’s source document. 
6.3.3 Planned Unblinding Procedures 
CSLB will authorize that the study be unblinded for analysis after all subjects have 
completed all assessments for Study Period 1 and the data has been cleaned for primary data 
analysis: 
• CSLB employees will be blinded to treatment allocation until all subjects have 
completed all assessments for Study Period 1 and the data has been cleaned for 
primary data analysis. Site monitors will remain blinded to treatment allocation until 
all subjects have completed all assessments for Study Period 2. 
• Subjects and caregivers, as well as site personnel involved in the conduct of the study, 
will be blinded to treatment allocation until all subjects have completed all 
assessments for Study Period 2. 
6.3.4 Ad hoc Safety Unblinding 
CSLB’s Global Clinical Safety and Pharmacovigilance personnel may, on an ad hoc basis, 
unblind the randomization code directly in the IRT at any time during the study because of a 
safety concern. The purpose of the unblinded data review is to determine if there is a risk to 
subject safety that would require further action either for the individual management of a 
study subject or for the ongoing conduct of the study. The need to unblind a subject or group 
of subjects may not necessarily arise because of an SAE. The need to unblind on an ad hoc 
basis will be determined by CSLB’s Global Clinical Safety and Pharmacovigilance. 
7 Contraindications, Permitted Therapies and Prohibited Therapies 
7.1 Contraindications and Precautions to Further Dosing 
IgPro20 is contraindicated in IgA-deficient patients with antibodies against IgA and with a 
history of severe systemic hypersensitivity or anaphylactic reactions/anaphylaxis to the active 
substance of IgPro20 or to any of its excipients (L-proline stabilizer and polysorbate 80). 
IgPro20 must not be administered if hyperprolinemia type I or II condition exists 
[IgPro20 IB]. 

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7.2 Permitted Therapies 
The following therapies are PERMITTED during the study (note: this list is not intended to 
be all inclusive) provided a stable dose/regimen has been achieved according to the 
designated time period (in parentheses) before Baseline: 
• 
Prednisolone equivalent (≤ 20 mg/day, except for oral rescue corticosteroid treatment 
[Table 6]), topical corticosteroids (low to medium potency), inhaled corticosteroids, 
topical immunosuppressants, physiotherapy (1 month). Taper of concomitant oral 
corticosteroid dose is required starting at Week 17 if the subject has demonstrated 
clinically relevant improvement (see Section 5.3.2). Monitoring for corticosteroid side 
effects should continue (see Section 8.1.2.1). 
Table 6 
Corticosteroid Dose Equivalency 
Corticosteroid 
Equivalent Dose Corticosteroid 
Equivalent Dose 
Prednisolone 
20 mg 
Betamethasone 
2.4 mg 
Prednisone 
20 mg 
Dexamethasone 
3.0 mg 
Methylprednisolone 
16 mg 
Hydrocortisone 
80 mg 
Triamcinolone 
16 mg 
Cortisone 
100 mg 
Source: Farinde, 2019; values in source were multiplied by 2 to equal 20 mg prednisolone dose. 
• Antimalarial agents, eg, hydroxychloroquine, chloroquine (3 months) 
• Systemic immunosuppressives or disease-modifying antirheumatic drugs, 
eg, azathioprine, methotrexate, mycophenolate, cyclosporine, tacrolimus (3 months) 
Doses of stable permitted therapy (including corticosteroids) may be reduced or discontinued 
after enrollment because of side effects, abnormal laboratory values, or concurrent illness. No 
other change to permitted concomitant therapy, with the exception of taper of corticosteroid 
dose starting at Week 17 (see Section 5.3.2), is allowed during Study Periods 1 and 2. 
In Study Period 3, doses of stable concomitant medication may be decreased based on subject 
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7.3 Prohibited Therapies 
The following therapies are NOT PERMITTED during the study and require a minimum 
washout period (in parentheses) before Baseline if discontinued: 
• IM, IV, or intra-articular corticosteroids including adrenocorticotropic hormone 
(1 month) 
• IgG (up to 2 months [Note: Subject may enroll before the end of the 2 month washout 
period if clinical deterioration is experienced after withdrawal of IgG.]) 
• Plasma exchange or plasmapheresis (3 months) 
• Cyclophosphamide or alkylating agents (6 months) 
• Other biologic therapies including investigational agents (6 months or 5 half-lives, 
whichever is longer) 
• Rituximab or other B cell depleting therapies (9 months or evidence of persistent 
B cell depletion after stopping therapy) 
Subjects will not be enrolled into the study if they receive any prohibited therapy or any 
therapy in a prohibited dosage that cannot be discontinued or reduced to a permitted dose 
before Baseline. 
7.4 Lifestyle Restrictions 
7.4.1 Physical Activity 
During study participation, there are no restrictions on the subject’s normal physical activity, 
but subjects should not change their physiotherapy regimen from at least 1 month before 
Baseline to Week 17. After completing Week 17, the subject’s physiotherapy regimen can be 
modified if clinically relevant improvement is achieved; however, any changes should be 
documented in the subject’s eCRF. During Study Period 3, there are no restrictions with 
regard to physiotherapy. 

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7.4.2 Contraception 
Female subjects of childbearing potential or male subjects must use a medically reliable form 
of contraception for the study duration and for 30 days after the last SC infusion of IMP. 
Acceptable methods of contraception are: 
• Abstinence where local law permits abstinence as a contraception method and where 
abstinence is the preferred and usual lifestyle of the subject, including refraining from 
heterosexual intercourse during the entire period of risk associated with the IMP 
• Hormonal methods associated with inhibition of ovulation. Acceptable hormonal methods 
include: oral contraceptives, contraceptive medication patch, contraceptive medication 
injection, estrogen/progestin vaginal ring, or contraceptive medication implant 
• At least 2 barrier methods. For example, female or male condoms with spermicidal foam 
or spermicidal jelly, or diaphragm with spermicidal foam or spermicidal jelly. The female 
condom and male condom should not be used together 
• Use of intrauterine device (placed more than 3 months before providing informed consent) 
• Bilateral tubal occlusion of female subjects (3 months before providing informed consent) 
• Vasectomy of male subjects or male partners/spouses of monogamous female subjects 
(3 months before providing informed consent) 
Periodic abstinence (calendar, symptothermal, postovulation methods), withdrawal (coitus 
interruptus), spermicides only, and lactational amenorrhea method are NOT acceptable 
definitions of contraception. 
8 Study Procedures and Visit Schedule 
8.1 Clinical Procedures 
The timing and frequency of the clinical procedures described in the following sections are 
detailed in the Schedules of Assessments. More frequent assessments may be performed at 
unscheduled visits if clinically indicated, at the discretion of the investigator. Refer to the 
provided study manuals for detailed instructions on how and when the scheduled and 
unscheduled assessments should be performed. 
8.1.1 Handling of Study Assessments 
During Study Period 1 and Study Period 2, sites should provide at least 2 physicians for study 
related assessments: a “Treating Physician” and an “Evaluating Physician.” The Treating 
Physician will be the primary contact for the subject and will be responsible for all safety and 

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treatment-related tasks (Table 7). It is recommended that the Treating Physician be the 
Principal Investigator for the study. Tasks assigned to the Treating Physician may be 
delegated to other qualified site staff according to site general practice. The Evaluating 
Physician will be responsible for efficacy assessments and the review of all subject-entered 
data (Table 7); these tasks may not be delegated. At Baseline before randomization, the 
Treating and Evaluating Physicians should review and confirm the subject’s DM status, to 
confirm that no clinically relevant improvement has occurred since Screening 
(see Section 8.5.3.1), and final eligibility. 
In Study Period 3, it is recommended that the Evaluating Physician continues the 
assessments, if at all possible. 
Training for all CSMs and other efficacy assessments will be provided to ensure that the 
assessments made are attributable to DM and not to other disease. The training will also 
include detailed instructions on how to complete the assessments. The Treating and 
Evaluating Physicians should remain the same for each subject through all study visits. 
Training will be provided to ensure consistency in safety and efficacy assessments in the 
event that 1 of the physicians is not available for a study visit. The Treating Physician will be 
notified when the subject has met the criteria for DOW, so that appropriate steps regarding 
the subject’s status per protocol can be implemented. The Treating Physician will have the 
responsibility to provide the final eCRF signature for each subject. 

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Table 7 
Role Responsibilities for Study Assessments 
Treating Physician 
Evaluating Physician 
Subject 
• Informed consent 
• Timed Up and Go 
• EQ-5D-5L 
• EULAR/ACR criteria 
• CDASI 
• TSQM-9 
• Physician Global Damage Assessment 
• MMT-8 
• WPAI-PH 
• Eligibility 
• MDAATA 
• HAQ 
• Medical/Surgical history 
 
• 5-D Itch (Pruritus) 
• DM treatment history 
 
• Patient Global Activity 
• General physical exam  
 
• SC Infusion Diary 
• Height and body weight 
 
 
• 12-lead ECG 
 
 
• Vital signs 
 
 
• All laboratory collections 
 
 
• AEs 
 
 
• Concomitant therapies 
 
 
• Physiotherapy documentation 
 
 
• SC infusion training 
 
 
• Randomization/IRT 
 
 
• SC Infusion Diary review 
 
 
• IMP dispensing/accountability 
 
 
• Rescue corticosteroid treatmentB 
 
 
• Withdrawal decision 
 
 
• Safety assessmentsC 
 
 
5-D = 5-Dimension; AE = adverse event; CDASI = Cutaneous Dermatomyositis Disease Area and Severity Index; 
CSMs = Core Set Measures; DM = dermatomyositis; DOW = Definition of Worsening; DVT = deep vein thrombosis; 
ECG = electrocardiogram; eCOA = Electronic Clinical Outcomes Assessment; EQ-5D-5L = EuroQoL 5-Dimension 
Questionnaire; EULAR/ACR = European League Against Rheumatism/American College of Rheumatology; HAQ = Health 
Assessment Questionnaire; IMP = investigational medicinal product; IRT = Interactive response technology; 
MDAAT = Myositis Disease Activity Assessment Tool; MMT-8 = manual muscle testing of 8 muscle groups; 
PE = pulmonary embolism; SC = subcutaneous; TEE = thromboembolic event; TIS = Total Improvement Score; 
TSQM-9 = Abbreviated Treatment Satisfaction Questionnaire for Medication; WPAI-PH = Work Productivity and Activity 
Impairment Questionnaire for General Health. 
A MDAAT includes Extramuscular Global Assessment and Physician Global Disease Activity components, 2 of the 
CSMs used for TIS and DOW calculations. The DOW calculation is performed by the eCOA solution and available for 
Treating Physician assessment of subject’s need for rescue corticosteroid treatment. 
B Rescue corticosteroid treatment can only be administered if DOW has been met. 
C Safety assessments include monitoring for corticosteroid treatment, monitoring for TEEs (including Wells’ Criteria for 
DVT and/or Wells’ Criteria for PE), monitoring for hemolysis at Week 2 and Week 5. 

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8.1.2 Demographics and Safety Assessments 
Subject demographics and safety assessments to be conducted during this study are provided 
in Table 8. Clinical laboratory assessments are to be performed at time points as detailed in 
the Schedule of Assessments. The time windows for each type of assessment are detailed in 
Table 14. 
Table 8 
Study Procedures: Demographics and Safety Assessments 
Assessment 
Description 
Medical/surgical 
history 
• Diagnosis and disease status 
• Relevant medical/surgical history, including DM history details 
• DM treatment history 
• Current/concomitant therapies 
• Contraception method (if relevant) 
Demographics 
Age, sex, race, and ethnicity (where permitted) 
Physical 
examination 
As per the site’s standard procedure, including height and Physician Global Damage 
Assessment only at Screening 
12-lead ECG 
As per the site’s standard procedure 
Safety assessments 
Refer to Section 8.1.2.1 (Monitoring for Corticosteroid Treatment), Section 8.1.2.2 
(Monitoring for Thromboembolic Events), and Section 8.1.2.3 (Monitoring for 
Hemolysis Risk Assessment) 
Adverse events 
Refer to Section 9 (Adverse Events) 
Body weight 
As per the site’s standard procedure 
Waist circumference As per the site’s standard procedure 
Vital signs 
• Blood pressure 
(systolic and diastolic) 
• Pulse rate (per minute) 
• Respiratory rate 
• Body temperature 
Pregnancy testA 
Serum test at Screening and urine test at all other assessments for hCG, as indicated for 
premenopausal females capable of becoming pregnant  
Thrombophilic 
Abnormality ScreenB 
• Protein C 
• Antithrombin III 
• Protein S 
• Cardiolipin antibodies 
• Lupus anticoagulant 
• Factor V Leiden mutation 
• Prothrombin G20210A mutation 
 
Hematology 
• ABO blood group 
• Rh factor 
• HbA1c 
• Hemoglobin 
• Hematocrit 
 
• Blood cell counts: erythrocytes, leukocytes with differential counts (neutrophils, 
basophils, eosinophils, lymphocytes, monocytes), platelets 
Muscle enzymes 
• CK 
• AST 
• Aldolase 
• LDH 
• ALT 
 
Hemolysis 
• DAT 
• Total bilirubin 
• Direct bilirubin 
• Indirect bilirubin 
• Haptoglobin 
• Reticulocytes 
• Peripheral blood smearC 
 
 
Other biochemistry 
• Alkaline phosphatase 
• Bicarbonate 
• BUN 
• Chloride 
• GGT 
• Sodium 
• Glucose 
• Potassium 
• Creatinine 

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Assessment 
Description 
Coagulation 
• D-dimer 
 
 
Virus serology 
Blood samples are to be tested for HBsAg, HCV antibodies, and HIV-1, HIV-2 
antibodies 
ABO = blood group type A, type B, type AB, or type O; ALT = alanine aminotransferase; AST = aspartate 
aminotransferase; BUN = blood urea nitrogen; CK = creatine kinase; DAT = direct antiglobulin test; 
DM = dermatomyositis; ECG = electrocardiogram; GGT = gamma-glutamyl transferase; HbA1c = glycated hemoglobin; 
HBsAg = hepatitis B virus surface antigen; hCG = human chorionic gonadotropin; HCV = hepatitis C virus; HIV = human 
immunodeficiency virus; LDH = lactate dehydrogenase; Rh = Rhesus. 
A Serum/urine pregnancy test is to be completed for all females of reproductive potential. A female of reproductive potential 
is defined as a premenopausal female capable of becoming pregnant. This includes women on oral, injectable, or 
mechanical contraception and women whose partners have been vasectomized or have received or are utilizing 
mechanical contraceptive devices. 
B Known deficiencies in proteins C, protein S, and antithrombin-III levels and presence of lupus anticoagulant/cardiolipin 
antibodies (antiphospholipid antibodies) and/or the 2 gene mutations (Prothrombin G20210A and Factor V Leiden) will 
exclude a subject from the study (Section 4.1.2, Exclusion Criterion 6). 
C Peripheral blood smear will only be analyzed for a drop in hemoglobin from the previous study visit ≥ 1 g/dL. 
Results of laboratory tests should be signed and dated in a timely manner and retained at the 
study site as source data. Refer to Section 9 for information on how AEs based on laboratory 
tests should be assessed and reported. 
8.1.2.1 Monitoring for Corticosteroid Treatment 
For subjects receiving concomitant corticosteroids, the Treating Physician should continue 
the monitoring and management of corticosteroid treatment at every study visit according to 
current standard of care and as recommended by the protocol. It is anticipated that subjects in 
the study were previously on corticosteroids that have been reduced to prednisolone 
equivalent 20 mg/day or less at least 1 month before Baseline. Further dose reduction (taper) 
must be started at Week 17 if clinically relevant improvement (see Section 3.1) has been 
achieved. Subjects with clinical worsening in Study Period 1 (eg, subject meets DOW 
criteria) will be eligible for oral corticosteroid rescue treatment of up to prednisolone 
equivalent 1 mg/kg daily for up to 5 days to be given at the discretion of the Treating 
Physician. Corticosteroids are known to be associated with a variety of side effects in a dose- 
and duration-of-treatment-dependent manner [Oray et al, 2016]. Please refer to Section 5.3.1 
for more information on rescue treatment. Reporting of AEs related to corticosteroid 
treatment should be handled according to Section 9.2. 
8.1.2.2 Monitoring for Thromboembolic Events (TEEs)  
To reduce the risk of potential TEEs, this study excludes specific patient groups 
(Section 4.1.2, exclusion criterion 6 items pertaining to TEE risk). Nevertheless, TEE risk 
will be continuously monitored during the study. 

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The Treating Physician will assess the subject for the suspicion of a TEE per standard of care 
at every study visit during all Study Periods. 
If there is suspicion of DVT or PE during Study Periods 1 or 2, the Wells’ Criteria for DVT 
(Table 9) or Wells’ Criteria for PE (Table 10) will be assessed and entered in the eCRF [Modi 
et al, 2016; Wells’ Criteria for DVT; Doherty, 2017; Wells’ Criteria for Pulmonary 
Embolism]. 
A score of > 1 on the Wells’ Criteria for DVT requires diagnostic imaging (lower extremity 
ultrasound) to rule out DVT. A score of > 1 on the Wells’ Criteria for PE requires additional 
imaging, (eg, computerized tomography scan, ventilation/perfusion scan, pulmonary 
angiography, or other modalities) be performed as per the site’s standard of care. 
When a diagnosis of DVT or PE is confirmed, IMP treatment must be discontinued; however, 
the subject may continue in the study for other study assessments up until Week 53. During 
Study Period 3, there will be no formal assessment of Wells’ Criteria. TEEs will be monitored 
as part of standard assessment for AEs at each monthly site visit. If a TEE occurs during 
participation in Study Period 3, the subject must be discontinued from the study. 

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Table 9 
Wells’ Criteria Scoring for Suspicion of DVT 
Assessment 
Score 
Active cancer (Treatment or palliation within 6 months)A 
+1 
Bedridden recently > 3 days or major surgery within 12 weeksA 
+1 
Calf swelling > 3 cm compared to the other leg (Measured 10 cm below tibial 
tuberosity) 
+1 
Collateral (nonvaricose) superficial veins present 
+1 
Entire leg swollen 
+1 
Localized tenderness along the deep venous system 
+1 
Pitting edema, confined to symptomatic leg 
+1 
Paralysis, paresis, or recent plaster immobilization of the lower extremity 
+1 
Previously documented DVTA 
+1 
Alternative diagnosis to DVT as likely or more likely 
-2 
Total Score 
 
DVT = deep vein thrombosis. 
A 
Exclusion Criterion (see Section 4.1.2) 
Table 10 
Wells’ Criteria Scoring for Suspicion of PE 
Assessment 
Score 
Clinical signs and symptoms of DVT 
+3 
PE is #1 diagnosis OR equally likely 
+3 
Heart rate > 100 bpm 
+1.5 
Immobilization at least 3 days OR surgery in the previous 4 weeksA 
+1.5 
Previous, objectively diagnosed PE or DVTA 
+1.5 
Hemoptysis 
+1 
Malignancy with treatment within 6 months or palliativeA 
+1 
Total Score 
 
DVT = deep vein thrombosis; PE = pulmonary embolism. 
A 
Exclusion Criterion (see Section 4.1.2) 
All TEEs, including but not limited to DVT and PE, will be classified as AESIs 
(see Sections 9.1.3 and 9.6.2). Follow-up monitoring and treatment (eg, anticoagulation) will 
be performed according to the site’s standard of care using established guidelines 
(eg, American Society of Hematology 2018). 

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8.1.2.3 Monitoring for Hemolysis 
For determination of hemolysis, an assessment of clinical signs of hemolysis will be 
performed at Week 2 and Week 5 and compared to Baseline. In combination with laboratory 
results confirmed or suspected hemolysis cases will be defined as follows: 
Confirmed hemolysis: 
Subjects fulfilling criterion A and at least 2 of criteria B, where 1 of the minor criteria must 
be direct antiglobulin test (DAT) positive after infusion and within 28 days of the first IMP 
infusion, will be considered to have “confirmed hemolysis.” Confirmed hemolysis will be 
reported as an AE. 
Suspected hemolysis: 
Subjects fulfilling criterion A and at least 1 of criteria B will be considered to have 
“suspected hemolysis.” 
• Criterion A 
Drop in hemoglobin of > 1 g/dL since the time of first administration of IMP 
without clinical evidence of blood loss from gastrointestinal bleeding, menorrhagia, 
hemoptysis, major hematoma, or injury and not explained by repeated phlebotomy 
• Criteria B 
Presence of minor criteria documented since the time of first administration of IMP, 
consisting of: 
o DAT positive 
o Haptoglobin < lower limit of normal 
o LDH > ULN 
o Total or indirect (unconjugated) bilirubin > ULN or jaundiced 
o Hemoglobinuria or red/dark urine 
o Hemoglobinemia 
o Spherocytosis 
o Hepatosplenomegaly 

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If AE of hemolysis is reported, it must be graded by the investigator: 
• Grade 1 – Laboratory evidence of hemolysis only (eg, DAT positive, schistocytes, 
decreased haptoglobin) 
• Grade 2 – Clinical evidence of hemolysis and ≥ 2 g/dL decrease in hemoglobin 
• Grade 3 –Transfusion or medical intervention indicated (eg, steroids) 
• Grade 4 – Life-threatening consequences; urgent intervention indicated 
• Grade 5 – Death 
8.1.3 ANA, MSA, and MAA Assessments 
Additional blood samples for presence of ANA, MSA, and MAA will be collected at 
Screening, Week 25, and Week 53 (Table 11). 
The following MSA will be analyzed: anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ, anti-OJ, 
anti-SRP, anti-Mi-2, anti-p155/140, anti-TIF-1γ, anti-MDA5, anti-NXP-2, anti-HMGCR, 
anti-SAE-1Ab. 
The following MAA will be analyzed: anti-PM/Scl, anti-Ro-SSA, anti-Ku, anti-U1 RNP, 
anti-U3 RNP. 
The samples for ANA, MSA, and MAA will be analyzed by the central laboratory (ANA) 
and a specialized reference laboratory (MSA and MAA). 
Table 11 
Clinical Procedures: ANA, MSA, and MAA Assessments 
Procedure 
Description 
ANA, MSA, and MAA 
assessments 
Blood samples will be collected for ANA, MSA, and MAA 
assessments 
ANA = antinuclear antibody; MAA = myositis-associated autoantibodies; MSA = myositis-specific 
autoantibodies. 

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8.1.4 Pharmacokinetic Assessments 
Trough serum samples for IgG level determination will be collected at Screening and at 
specified study visits (Table 12). 
Additional blood samples for rich PK sampling of IgG levels will be collected at Week 37 
(Study Period 2). Rich PK samples will be collected from up to 10 Japanese subjects and 
30 non-Japanese subjects. The Week 37, 168-hour rich PK blood sample must be drawn 
before start of Week 38 dose infusion. The additional samples will be collected at the times 
listed in Table 14. Blood draws for rich PK samples may be drawn by a phlebotomist at the 
subject’s home where available and/or allowed by local law. 
The samples for serum IgG concentrations will be analyzed by the central laboratory by 
immunoturbidimetry. Serum IgG levels will not be disclosed to the investigative site or any 
blinded study personnel prior to unblinding the study. See Schedule of Assessments for Rich 
Pharmacokinetic Sampling for details. 
Table 12 
Clinical Procedures: Pharmacokinetic Assessments 
Procedure 
Description 
Pharmacokinetics 
evaluations 
Blood samples will be collected for IgG level determination 
IgG = immunoglobulin G. 
8.1.5 Efficacy Assessments 
Efficacy assessments are to be performed by the Evaluating Physician and the subject at time 
points as detailed in the Schedule of Assessments. The time windows for each type of 
assessment are detailed in Section 8.5.1. 
IMACS 6 CSMs: 
Patient Global Activity Assessment: The subject will assess his/her global DM disease 
activity at the study visit by selecting a point on a 10-cm line with “No evidence of disease 
activity” on the left and “Extremely active or severe disease activity” on the right. For details, 
see Appendix 5. 

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Manual Muscle Testing of 8 Muscle Groups (MMT-8): The Evaluating Physician will 
assess the MMT-8. The 8 muscles to be tested are 1 axial muscle group - neck flexors; and 
7 bilateral muscles - proximal: deltoid middle, biceps brachii, gluteus maximus, gluteus 
medius, and quadriceps; and distal: wrist extensors and ankle dorsiflexors. DM subjects with 
muscle weakness (classic DM) will have MMT-8 scores ≤ 142 points and DM subjects with 
little or no muscle weakness (hypo/amyopathic DM) will have MMT-8 scores > 142 points. 
For subjects with a muscle group not able to be assessed because of a non-DM related injury 
or an amputation at Baseline, the Evaluating Physician will determine the appropriate muscle 
weakness stratum for this subject. For details, see Appendix 6. 
Health Assessment Questionnaire (HAQ): The subject will answer a series of questions 
which assess how DM is affecting 8 activities of daily living: dressing/grooming, arising 
(position changes), eating, walking, hygiene, reach, grip, and activities, eg, running errands, 
chores. For details, see Appendix 7. 
Muscle Enzyme: The most abnormal muscle enzyme (CK, LDH, AST, ALT, or aldolase) at 
Baseline will be assessed for changes during Study Period 1 and Study Period 2. If all muscle 
enzymes are within normal range at Baseline, the most abnormal enzyme will be determined 
by considering the value in relation to the ULN range. If more than one muscle enzyme has 
the same highest abnormality score at Baseline, the most abnormal muscle enzyme will be 
chosen according to the following priority list: CK, LDH, AST, ALT, aldolase. For details, 
see Appendix 8. 
Myositis Disease Activity Assessment Tool (MDAAT) for Physician Global Disease 
Activity and Extramuscular Global Assessment: The MDAAT is a combined tool which 
will capture the Evaluating Physician’s assessment of disease activity of various organ 
systems using a 0 to 4 point scale as well as a VAS for each disease activity component. 
There are 7 extramuscular disease activity components (Constitutional, Cutaneous, Skeletal, 
Gastrointestinal, Pulmonary, Cardiovascular, Other [specify]) and a muscle disease activity 
component, each with an accompanying VAS. Additionally, an Extramuscular Global 
Assessment VAS and a Physician Global Disease Activity VAS will be assessed. 
Extramuscular Global Assessment and Physician Global Disease Activity components are 
the 2 CSMs used for TIS and DOW calculations. In Study Period 3, only the Physician 
Global Disease Activity VAS will be collected. For details, see Appendix 4. 

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Other Measures: 
Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI): The Evaluating 
Physician will assess 4 disease areas (Extent [anatomical locations], Gottron’s Hands, 
Periungual, and Alopecia) for activity and damage and provide a total score for each. For 
details, see Appendix 9. 
Timed Up and Go: The Evaluating Physician will measure how quickly the subject can rise 
from a sitting position, walk 3 meters, and return to same sitting position in Study Period 1 
and Study Period 2. For details, see Appendix 11. 
8.1.6 Other Assessments 
8.1.6.1 Disease Specific 
Physician Global Damage Assessment: At Screening only, the Treating Physician will rate 
the global (overall) assessment of current DM disease damage by using a 5-point Likert scale 
(0 to 4). This global assessment is to be performed using all information available to the 
Treating Physician at Screening, including the subject’s appearance, history, physical 
examination, diagnostic laboratory testing, and the resultant medical therapy. For details, see 
Appendix 3. 
8.1.6.2 Non-disease Specific 
5-D Itch (Pruritus) Score: Subjects will assess 5 dimensions of itch history in 
Study Period 1 and Study Period 2: Duration, Degree (intensity), Direction (better or worse 
from previous month), Disability of 4 activities of daily living, and Distribution (location[s] 
on body). For details, see Appendix 10. 
EuroQoL 5-Dimension Questionnaire (EQ-5D-5L): The subject will select which best 
describes his own health state at the study visit in the following 5 dimensions: Mobility, 
Self-care, Usual Activities, Pain/discomfort, and Anxiety/depression. The subject will also 
select a point on a scale drawn like a thermometer to indicate how good or bad the health 
state is, with best state as “100” and worst state as “0.” For details, see Appendix 12. 
Work Productivity and Activity Impairment Questionnaire: General Health 
(WPAI-GH): The subject will answer questions about the effect of his/her health on the 
ability to work or perform regular activities in Study Period 1 and Study Period 2. General 
health is defined as any symptoms relating to physical or emotional well-being. For details, 
see Appendix 13. 

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Abbreviated Treatment Satisfaction Questionnaire for Medication (TSQM-9): The 
TSQM-9 is a 9-item general instrument that measures the major dimensions of satisfaction 
with a medication [Bharmal et al, 2009]. Scores on the TSQM-9 range from 0 (indicating 
poor satisfaction) to 100 (indicating perfect satisfaction). This instrument usually requires 
less than 5 minutes for subject completion and was developed to be used with any medication 
and across cultural and different language settings. Scores on the Effectiveness (3 items), 
Convenience (3 items), and Global Satisfaction (3 items) scales will be assessed in a 
cross-sectional manner in Study Period 1 and Study Period 2. For details, see Appendix 14. 
8.1.6.3 Exploratory Biomarkers 
Blood samples will be collected and stored to assess exploratory biomarkers in DM for a 
separate, future study (Table 13). These exploratory assessments may include but will not be 
limited to the following: chemokines, cytokines, complement, and protein expressions, DNA, 
RNA, and messenger RNA. Sample collection for exploratory evaluation of biomarkers will 
be clearly mentioned in the ICF for this study. Sample collection for DNA testing is optional 
for all subjects and will require a separate consent. These potential DM biomarkers may 
allow for better understanding of prognosis, disease course, and correlation to treatment in 
DM subjects and, therefore, might allow for individualized treatment. The exploratory nature 
of these analyses precludes the use of the results to predict future risk or to inform health 
care. None of the 59 genes of potential high medical importance which appear on the 
American College of Medical Genetics list of published recommendations for reporting of 
incidental findings will be examined. Therefore, neither the investigator nor the subject will 
be informed of these results (DNA, RNA, or serum proteins). 
In order to understand how the biomarker results might inform on these aspects of DM, they 
will need to be correlated with the subject’s treatment in the study, gender, ethnicity, age, 
hematology, serum chemistry, serum IgG, efficacy and quality of life assessments. Assays 
will be performed in-house at CSL laboratories and by external service providers. The CSL 
laboratory is located at 30 Flemington Road, Parkville, Victoria 3052, Australia. DNA 
samples require anonymization by the central laboratory before transfer to CSL Australia for 
analysis at the end of the study. All biomarker samples will be retained for a maximum of 
10 years. 

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Table 13 
Clinical Procedures: Exploratory Biomarkers 
Procedure 
Description 
Biomarker samples 
Blood samples will be collected for exploratory biomarker 
assessments (serum and DNA and RNA analyses) 
 
8.2 Blood Samples 
During the study, blood will be taken from each subject for laboratory safety assessments, PK 
evaluations, ANA, MSA and MAA, exploratory biomarkers, and virology (HBV, HCV, and 
HIV) samples. Approximately 360.5 mL of blood will be drawn from each subject during 
Study Period 1 and Study Period 2. An additional 8 mL of blood per year of study 
participation will be collected during Study Period 3, with an additional 7.5 mL collected for 
virology retention at the EOP3 Visit, leading to a maximum of 392 mL collected over all 
3 study periods. Detailed information on the volume of blood to be sampled for each 
assessment will be available in the ICF and laboratory manual. 
Repeat or unscheduled blood samples may be taken for safety reasons or for technical issues 
with the samples and will be in addition to the estimated 392 mL blood volume for scheduled 
assessments. 
Refer to the study manual for details about the collection, storage, handling and processing of 
blood samples. 
8.3 Retention of Samples 
Retention samples of serum for virology will be obtained as specified in the Schedule of 
Assessments. Virology retention samples will be stored at the central laboratory. In case of 
any evidence for a possible treatment-emergent virus infection within 1 year after study 
completion, the retention sample will be analyzed in the central laboratory. 
All exploratory biomarker samples collected during the study will be retained and destroyed 
within 10 years after completion of the study. Biomarker retention samples will be stored at 
CSLB (30 Flemington Road, Parkville, Victoria 3052, Australia). Refer to the study manual 
for further details about the storage and destruction of retention samples. 

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8.4 Prior and Concomitant Therapies 
All medications taken by a subject within 3 months before Baseline are considered prior 
therapy and must be documented in the eCRF. 
Previous medications and therapies for DM that the subject has received within 1 year before 
Baseline must be documented in the eCRF. If possible, all available data regarding the 
subject’s treatment with IgG therapy since being diagnosed with DM should be recorded in 
the subject’s eCRF. 
All drugs and/or procedures currently being administered to a subject at the time of signing 
informed consent, and which continue to be taken in addition to IgPro20 or placebo during 
the study, as well as drugs and/or procedures initiated after the first infusion of study product, 
are regarded as concomitant therapies and must be documented as such in the eCRF. 
Any prohibited dose adjustments in permitted concomitant therapies (see Section 7.2) will 
lead to withdrawal from treatment. 

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8.5 Visit Schedule 
8.5.1 Assessment Time Windows 
The timing and frequency of the study visits are described in the Schedule of Assessments. 
Time windows for all assessments are detailed in Table 14. 
Table 14 
Time Windows for Assessments 
Visit/Procedure 
Time window 
(relative to scheduled visit/procedure) 
Screening 
Up to 2 months before Baseline 
Week 2 and all subsequent study 
visits in Study Period 1 and 
Study Period 2 
± 3 days 
Monthly study visits in 
Study Period 3 
± 7 days 
Physical examination, to include 
Physician Global Damage 
Assessment only at Screening 
Before first SC infusion on Week 1A 
Vital signs, including body 
weight 
Before first SC infusion on Week 1 and Week 2A 
Urine collection for pregnancy 
testB 
Before first SC infusion on Week 1A 
Blood collection for laboratory 
tests 
Before first SC infusion on Week 1 and Week 2A 
Blood collection for rich PK 
(serum IgG and exploratory 
biomarkers)  
Week 37, trough sample collection at study visit 
Week 37, 24 ± 3 h relative to the start of first infusion 
Week 37, 48 ± 3 h relative to the start of first infusion 
Week 37, 72 ± 3 h relative to the start of first infusion 
Week 37, 120 ± 3 h relative to the start of first infusion 
Week 37, 168 ± 3 h relative to the start of first infusion 
Subject SC Infusion Diary review 
At the end of SC infusions on Week 1 and Week 2A 
Adverse event documentation 
Before first SC infusion on Week 1 and Week 2A 
Safety assessment documentation 
Before first SC infusion on Week 1 and Week 2A 

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Visit/Procedure 
Time window 
(relative to scheduled visit/procedure) 
Concomitant therapy 
documentation 
Before first SC infusion on Week 1 and Week 2A 
Physiotherapy documentation 
Before first SC infusion on Week 1 and Week 2A 
Efficacy assessments 
Before first SC infusion on Week 1 and Week 2A 
h = hour; IgG = immunoglobulin G; PK = pharmacokinetic; SC = subcutaneous. 
A 
For all other study visits, SC infusions are not performed at the study visit. 
B 
Urine pregnancy test is to be completed for all females of reproductive potential. 
8.5.2 Screening 
All subjects must provide written informed consent before any study-specific assessments or 
procedures are performed (see Section 12.3).  
The Screening examination must be performed no more than 2 months before the intended 
Baseline date. Note: A duration of 2 months may be required from last IgG dose to Baseline 
for subjects on IgG therapy before Screening (see Section 7.3). For subjects enrolling without 
active DM rash, the other objective evidence to confirm disease activity must be reviewed by 
CSLB medical monitoring staff (or delegate) before final eligibility is determined and the 
subject is randomized. The other objective evidence to confirm disease activity may include 
any of the following: MRI scan, electromyogram, muscle biopsy, or CK > 4 × ULN within 
3 months before Baseline. If a potential subject fails eligibility for a reason other than DM 
diagnosis or disease activity, the potential subject may be rescreened 1 time for the study and 
all Screening assessments must be repeated. 
The following procedures will be conducted and documented at the Screening Visit as 
specified in the Schedule of Assessments for Screening (Table 1): 
• Obtain written informed consent and register the subject via IRT 
• Relevant medical and surgical history to include DM history details (including age at 
first diagnosis) from time of diagnosis (available surgical history for the past 5 years), 
and prior treatments for DM 
o Available DM treatment history (eg, corticosteroids, other immunosuppressants, 
antimalarials, IgG [to include IgG details including type of therapy {eg, IV or 
SC}, method of administration {eg, central venous port, peripherally inserted 
central catheter}, dosage and frequency, and types of AEs experienced associated 
with the method of administration, since diagnosis]) 

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• Documentation of previous age-appropriate cancer screening  
• Demographics 
• Review of inclusion and exclusion criteria including the EULAR/ACR classification 
criteria for DM 
• CDASI 
• General physical exam, including height 
• 12-lead electrocardiogram (ECG) 
• Body weight 
• Vital signs 
• Wells’ Criteria 
• Collection of blood sample for serum hCG pregnancy test, if applicable 
• Collection of blood samples to assess the following: 
o Serum IgG level 
o Hematology 
o ABO blood group (blood group type A, type B, type AB, or type O) and Rhesus 
(Rh) factor 
o Muscle enzymes 
o Other biochemistry 
o Thrombophilic abnormality screen 
o Coagulation 
o ANA, MSA, and MAA 
o Virology 
• Document AEs 
• Document concomitant therapies 
• Document physiotherapy 
• Individual CSMs 
• Physician Global Damage Assessment 

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If the subject is not eligible for the study, the primary reason for screen failure must be 
entered in the IRT. 
8.5.3 Study Period 1 
Study Period 1 is a 24-week, double-blind, placebo-controlled treatment period during which 
participating subjects receive either IgPro20 or placebo at 0.5 g/kg SC on a weekly basis. 
Study Period 1 starts with Baseline/Week 1 and continues through the Week 25 Visit 
(24 weeks ± 3 days after the first SC infusion of IMP). Study assessments will be performed 
as specified in the Schedule of Assessments for Study Period 1 (Table 2). 
8.5.3.1 Baseline/Week 1 
The Baseline and Week 1 visits occur at the same study visit; however, all Baseline 
procedures must be completed before the Week 1 procedures begin. The final eligibility 
criterion to be met is no clinically relevant improvement between Screening and Baseline 
(Section 4.1.2, Exclusion criterion 4). This determination will be made based on the Treating 
and Evaluating Physicians’ clinical judgement following review of the efficacy assessments 
performed at Screening and Baseline. 
Subjects who complete all Screening and Baseline CSM assessments and who fulfill all 
eligibility criteria (ie, eligible subjects) will be randomized. At the Baseline Visit, the 
following procedures are to be conducted and documented: 
Baseline 
• Confirm eligibility  
• Randomization into double-blind treatment (see Section 6.2) 
• General physical examination 
• Body weight 
• Waist circumference 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 

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• Collection of blood samples for virology retention and exploratory biomarkers (serum 
and DNA/RNA analyses) 
• Collection of blood samples to assess the following: 
o Hematology 
o Glycated hemoglobin (HbA1c) 
o Muscle enzymes 
o Hemolysis 
o Other biochemistry 
o Coagulation 
o Serum IgG 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• Assess for clinically relevant improvement 
• Individual CSMs 
• CDASI 
• 5-D Itch Score 
• Timed Up and Go 
• EQ-5D-5L, WPAI-GH 
Week 1 
• Document AEs 
• Document concomitant therapies 
• Training and supervision of first IMP SC infusion 
• Supervision of subject SC Infusion Diary entry 

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8.5.3.2 Week 2 
The following procedures will be conducted at 1 week ± 3 days after the first SC infusion of 
IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of blood samples to assess the following: 
o Hematology with peripheral blood smear 
o Hemolysis 
o Coagulation 
o Serum IgG 
• Training and supervision of IMP SC infusion for Week 2 
• Supervision of subject SC Infusion Diary entry 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
Starting with Week 3, the subject will adjust their first dosing day so that no SC infusions 
occur on the day of a study visit. Site instruction for this transition should be provided. 
8.5.3.3 Week 5 
The following procedures will be conducted at 4 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 

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• Collection of blood samples for exploratory biomarkers (serum and RNA analyses) 
• Collection of blood samples to assess the following: 
o Hematology with peripheral blood smear 
o Muscle enzymes 
o Hemolysis 
o Coagulation 
o Serum IgG 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• DOW assessment 
• Individual CSMs  
• CDASI 
8.5.3.4 Week 9 
The following procedures will be conducted at 8 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (serum analysis) 
• Collection of blood samples to assess the following: 
o Hematology 
o Muscle enzymes 
o Other biochemistry 

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o Coagulation 
o Serum IgG 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• DOW assessment 
• Individual CSMs 
• CDASI 
• 5-D Itch Score 
• Timed Up and Go 
8.5.3.5 Week 13 
The following procedures will be conducted at 12 weeks ± 3 days after the first infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples to assess the following: 
o Muscle enzymes 
o Coagulation 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• DOW assessment 

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• Individual CSMs 
• CDASI 
• EQ-5D-5L, WPAI-GH, TSQM-9 
8.5.3.6 Week 17 
The following procedures will be conducted at 16 weeks ± 3 days after the first SC 
infusion of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (serum analysis) 
• Collection of blood samples to assess the following: 
o Hematology 
o Muscle enzymes 
o Other biochemistry 
o Coagulation 
o Serum IgG 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Assess if subject has clinically relevant improvement, and if so, subjects must initiate 
oral corticosteroid taper (see Section 5.3.2) 
• Document any change to physiotherapy since the previous study visit 
• DOW assessment 
• Individual CSMs 
• CDASI 

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• 5-D Itch Score 
• Timed Up and Go 
8.5.3.7 Week 21 
The following procedures will be conducted at 20 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (serum analysis) 
• Collection of blood samples to assess the following: 
o Muscle enzymes 
o Coagulation 
o Serum IgG 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies  
• Document any change to physiotherapy since the previous study visit 
• DOW assessment 
• Assess if subject has clinically relevant improvement, and if so, subjects must initiate 
oral corticosteroid taper (see Section 5.3.2) 
• Individual CSMs 
• CDASI 

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8.5.3.8 Week 25 
The following procedures will be conducted at 24 weeks ± 3 days after the first SC infusion 
of IMP: 
• General physical examination 
• Body weight 
• Waist circumference 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (RNA and serum analyses) 
• Collection of blood samples to assess the following: 
o Hematology 
o HbA1c 
o Muscle enzymes 
o Other biochemistry 
o Coagulation 
o Serum IgG 
o ANA, MSA, and MAA 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• DOW assessment 
• Assess if subject has clinically relevant improvement, and if so, subjects must initiate 
oral corticosteroid taper (see Section 5.3.2) 
• Individual CSMs 

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• CDASI 
• 5-D Itch Score 
• Timed Up and Go 
• EQ-5D-5L, WPAI-GH, TSQM-9 
8.5.4 Study Period 2 
During Study Period 2, all participating subjects will receive 28 weeks of active treatment 
with IgPro20 at 0.5 g/kg SC on a weekly basis. Study Period 2 starts with administration of 
IgPro20 at Week 25, after all Week 25 assessments (as specified in Section 8.5.3.8) have been 
completed. Study Period 2 continues through the Week 53 Visit when the subject’s eligibility 
for continuation of IgPro20 treatment in Study Period 3 will be confirmed (Section 8.5.4.7). 
Subjects not eligible for participation in Study Period 3 must complete the Week 56 Safety 
Follow-up Telephone Call (Section 8.5.4.8).  
Study assessments will be performed as specified in the Schedule of Assessments for 
Study Period 2 (Table 2). 
8.5.4.1 Week 29 
The following procedures will be conducted at 28 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples to assess the following: 
o Muscle enzymes 
o Coagulation 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies  

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• Document any change to physiotherapy since the previous study visit 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 
8.5.4.2 Week 33 
The following procedures will be conducted at 32 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples to assess the following: 
o Hematology 
o Muscle enzymes 
o Other biochemistry 
o Coagulation 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 
• 5-D Itch Score 

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• EQ-5D-5L, WPAI-GH, TSQM-9 
8.5.4.3 Week 37 
The following procedures will be conducted at 36 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (serum analysis) 
• Collection of blood samples to assess the following: 
o Muscle enzymes 
o Coagulation 
o Serum IgG 
• Additional sampling for rich PK analysis, if applicable 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 

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8.5.4.4 Week 41 
The following procedures will be conducted at 40 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples to assess the following: 
o Muscle enzymes 
o Coagulation 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies  
• Document any change to physiotherapy since the previous study visit 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 
• 5-D Itch Score 
• Timed Up and Go 
• EQ-5D-5L, WPAI-GH, TSQM-9 

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8.5.4.5 Week 45 
The following procedures will be conducted at 44 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples to assess the following: 
o Hematology 
o Muscle enzymes 
o Other biochemistry 
o Coagulation 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 
8.5.4.6 Week 49 
The following procedures will be conducted at 48 weeks ± 3 days after the first SC infusion 
of IMP: 
• Body weight 
• IMP assignment 
• Vital signs 

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• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (serum analysis) 
• Collection of blood samples to assess the following: 
o Muscle enzymes 
o Coagulation 
o Serum IgG 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 
8.5.4.7 Week 53 
Eligibility for continuation of treatment in Study Period 3 is based on a TIS of ≥ 20 points at 
Week 49 and is to be confirmed at the Week 53 Visit. If the subject is not eligible to continue 
treatment with IgPro20, study participation will end with the Week 56 Safety Follow-up 
Telephone Call (see Section 8.5.4.8). If a subject is withdrawn from the study before the 
Week 53 Visit, the investigator should make every effort to perform the assessments 
scheduled for the Week 53 Visit: 
• General physical examination 
• 12-lead ECG 
• Body weight 
• Waist circumference 
• IMP assignment, if subject is eligible to continue treatment with IgPro20 in 
Study Period 3 

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• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for virology retention and exploratory biomarkers (RNA 
and serum analyses) 
• Collection of blood samples to assess the following: 
o Hematology 
o HbA1c 
o Muscle enzymes 
o Other biochemistry 
o Coagulation 
o Serum IgG 
o ANA, MSA, and MAA 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• DOW (only applicable to subjects who discontinue IMP treatment before Week 25) 
• Individual CSMs 
• CDASI 
• 5-D Itch Score 
• Timed Up and Go 
• EQ-5D-5L, WPAI-GH, TSQM-9 

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• Document reason for withdrawal, if applicable 
• Assess the subject’s eligibility for treatment continuation in Study Period 3, if 
applicable 
8.5.4.8 Week 56 (Safety Follow-up Telephone Call) 
If a subject is not eligible to continue treatment with IgPro20 in Study Period 3, a telephone 
call to assess AEs and concomitant therapies will occur 4 weeks after last IMP infusion, after 
which no further study-related procedures will be performed. In this case, the Safety 
Follow-up Telephone Call constitutes the EOP2 Visit. The Safety Follow-up Telephone Call 
will not be performed for subjects who discontinue IMP treatment but remain in the study for 
at least 1 subsequent study visit 4 weeks after IMP discontinuation, or for subjects who 
continue treatment with IgPro20 in Study Period 3. 
8.5.4.9 Unscheduled Study Visit for Study Period 1 or Study Period 2 
The following procedures may be conducted at any Unscheduled Study Visit that occurs 
during Study Period 1 or Study Period 2: 
• Body weight 
• IMP assignment, if weight change (> 2 kg) requires dose volume adjustment 
• Vital signs 
• Safety assessments 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Collection of blood samples for exploratory biomarkers (serum analysis) 
• Collection of blood samples to assess the following: 
o Hematology 
o Muscle enzymes 
o Other biochemistry 
o Coagulation 
o Serum IgG 
• Subject SC Infusion Diary review 
• Document AEs 

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• Document concomitant therapies 
• Document any change to physiotherapy since the previous study visit 
• DOW assessment if before Week 25 (Note: 2 consecutive assessments must be at least 
2 weeks apart to meet the criteria for DOW.) 
• Assess if subject has clinically relevant improvement, and if so, initiate oral 
corticosteroid taper 
• Individual CSMs 
• CDASI 
• SC infusion, if applicable, at Week 3 or Week 4 only 
8.5.5 Study Period 3 
During Study Period 3, subjects will be treated with open-label IgPro20 at 0.5 g/kg SC on a 
weekly basis and subjects will be asked to return to the study site to complete safety 
assessments and measurement of body weight on a monthly basis as specified in the Schedule 
of Assessments for Study Period 3 (Table 3). Additionally, efficacy assessments will be 
performed at 6-month and 12-month intervals during the subject’s continued participation in 
Study Period 3. Adverse events and concomitant medications will be assessed throughout the 
subject’s participation in the study. 
If a subject is withdrawn from the study before completing the EOP3 Visit, all EOP3 Visit 
assessments should be performed at the last study visit. 
8.5.5.1 Monthly (every 4 weeks) Study Visits 
The following procedures will be conducted at each monthly study visit (± 7 days) during 
Study Period 3: 
• Body weight 
• IMP assignment (weight change of >2kg requires dose volume adjustment)  
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 

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8.5.5.2 Study Visits Every 6 Months (24 weeks) 
In addition to assessments collected at monthly visits, the following will be conducted every 
6 months (24 weeks): 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Vital signs 
• Body weight 
• Efficacy Assessment (4 Individual CSMs): 
o Physician Global VAS 
o Patient Global VAS 
o MMT-8  
o HAQ 
• CDASI 
• EQ-5D-5L  
• IMP assignment 
• IMP SC weekly infusions 
• Subject SC infusion diary review 

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8.5.5.3 End of Year Visit 
During participation in Study Period 3, subjects may continue treatment with open-label 
IgPro20 0.5 g/kg SC weekly until the end of the study (see Section 3.7). At scheduled study 
visits at the end of each year (± 7 days) during the subject’s participation in Study Period 3, 
the following assessments will be performed in addition to the assessments specified for the 
study visits every month (see Section 8.5.5.2): 
• Collection of urine sample for hCG pregnancy test, if applicable 
• General physical examination 
• Vital signs 
• Collection of blood samples to assess the following: 
o Hematology 
o Biochemistry 
• Efficacy Assessment (4 Individual CSMs): 
o Physician Global VAS 
o Patient Global VAS 
o MMT-8  
o HAQ 
• CDASI 
• EQ-5D-5L  
• IMP Assignment for subjects continuing in Study Period 3 
• IMP SC weekly infusions for subjects continuing in Study Period 3 
• Document reason for withdrawal, if applicable 

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8.5.5.4 End of Period 3 Visit 
If a subject is withdrawn from the study at any time during Study Period 3, the investigator 
should make every effort to perform the assessments scheduled for the EOP3 Visit: 
• Collection of urine sample for hCG pregnancy test, if applicable 
• General physical examination 
• Vital signs 
• Body weight 
• Collection of blood samples for virology retention 
• Collection of blood samples to assess the following: 
o Hematology 
o Biochemistry 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Efficacy Assessment (4 Individual CSMs): 
o Physician Global VAS 
o Patient Global VAS 
o MMT-8 
o HAQ 
• CDASI 
• EQ-5D-5L  
• Document reason for withdrawal, if applicable 

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8.5.5.5 Unscheduled Study Visit for Study Period 3 
The following procedures may be performed at any Unscheduled Study Visit that occurs 
during Study Period 3: 
• Collection of urine sample for hCG pregnancy test, if applicable 
• Vital signs 
• Body weight 
• IMP assignment, if weight change (> 2 kg) requires dose volume adjustment 
• Safety assessments 
• Collection of blood samples to assess the following: 
o Hematology 
o Biochemistry 
• Subject SC Infusion Diary review 
• Document AEs 
• Document concomitant therapies 
• Efficacy Assessments (4 Individual CSMs): 
o Physician Global VAS  
o  Patient Global VAS 
o MMT-8 
o HAQ 
• CDASI 
• EQ-5D-5L 
• Document reason for withdrawal, if applicable 

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8.5.6 Discontinuation of IMP 
Subjects may discontinue IMP at any time at their own request or at the discretion of the 
investigator. The investigator should record in the eCRF and in the subject's medical records 
the reason and date of IMP discontinuation. 
Subjects who discontinue IMP will be encouraged to remain in the study until Week 25 if 
discontinuation occurs before Week 25, or until Week 53, if discontinuation occurs after 
Week 25, in order to collect study assessments. 
If the subject discontinues IMP during Study Period 1 or Study Period 2, all Week 53 
assessments (see Section 8.5.4.7) will be performed at the study visit where IMP treatment is 
discontinued, instead of the assessments scheduled for that particular study visit. If subjects 
discontinue IMP during Study Period 3, subjects will be withdrawn from the study after they 
have completed the EOP3 Visit assessments.  
See the Schedule of Assessments for details on assessments no longer collected after IMP 
discontinuation if the subject remains in the study. 
9 Adverse Events 
9.1 Definitions 
9.1.1 Adverse Event 
As per the ICH Topic E2A (Clinical Safety Data Management: Definitions and Standards for 
Expedited Reporting), an AE is any untoward medical occurrence in a patient or clinical 
investigation subject administered a pharmaceutical product and which does not necessarily 
have a causal relationship with this treatment. An AE can, therefore, be any unfavorable and 
unintended sign (including an abnormal, clinically significant laboratory finding), symptom, 
or disease temporally associated with the use of a medicinal (investigational) product, 
whether or not considered related to the medicinal (investigational) product. 
The period of observation for AEs extends from the time the subject gives informed consent 
until the end of study participation (see Section 9.4 for further details). 

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Adverse events may include: 
Exacerbation (ie, an increase in the frequency or severity) of a pre-existing condition, 
with the exception of DM 
o Illness present before study entry should be recorded in the medical history 
section of the eCRF and only be reported as an AE if there is an increase in the 
frequency or severity of the condition during the study 
A clinical event occurring after consent but before IMP administration 
Intercurrent illnesses with an onset after administration of IMP 
Adverse events do not include: 
Any worsening of DM including worsening of DM that meets serious criteria 
(eg, hospitalization) 
Events identified at Screening that meet exclusion criteria 
Medical or surgical procedures (the condition that leads to the procedure is the AE) 
Situations where an untoward medical occurrence has not taken place, eg: 
o Planned hospitalizations due to pre-existing conditions, which have not worsened 
o Hospitalizations that occur for procedures not due to an AE (eg, cosmetic surgery) 
o Hospitalizations for a diagnostic procedure where the hospital stay is less than 
24 hours in duration or for normal management procedures (eg, chemotherapy) 
o Overdose of IMP or any concomitant therapy that does not result in any adverse 
signs or symptoms 
For laboratory safety parameters, any instances of absolute values being outside the reference 
range or changes at any study visit after the start of the study that are considered by the 
investigator as clinically significant must recorded in the eCRF as AEs. In addition, at the 
investigator’s discretion, any changes or trends over time in laboratory parameters can be 
recorded in the eCRF as AEs if such changes or trends are considered to be clinically 
relevant, even if the absolute values are within the reference range. 

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Laboratory findings do not need to be reported as AEs in the following cases: 
Laboratory parameters already beyond the reference range at Screening, unless a further 
increase/decrease can be considered an exacerbation of a pre-existing condition 
Abnormal laboratory parameters caused by mechanical or physical influences on the blood 
sample (eg, in vitro hemolysis) and flagged as such by the laboratory in the laboratory report 
Abnormal parameters that are obviously biologically implausible (eg, values that are 
incompatible with life or outside the measuring range) 
An abnormal laboratory value that cannot be confirmed after repeat analysis, preferably 
in the same laboratory (ie, the previous result could be marked as not valid and should 
not necessarily be reported as an AE) 
9.1.2 Serious Adverse Event 
An SAE is defined as any untoward medical occurrence that at any dose: 
Results in death – The event must be the cause of death for the SAE to meet this serious 
criterion 
Is life-threatening – The term “life-threatening” refers to an event in which the subject was 
at risk of death at the time of the event; it does not refer to an event that hypothetically might 
have caused death if it had been more severe 
Requires in-patient hospitalization or prolongation of existing hospitalization – CSLB 
considers “hospitalization or prolongation of existing hospitalization” for at least 24 hours as 
the defining criterion for an SAE. Hospital admissions for planned surgery or for normal 
disease management procedures (eg, chemotherapy) are not considered as defining criteria 
for SAEs 
Results in persistent or significant disability or incapacity 
Is a congenital anomaly or birth defect 
Is medically significant – A medically significant event is defined as an event that does 
not necessarily meet any of the SAE criteria, but which is judged by a physician to 
potentially jeopardize the subject or require medical or surgical intervention to 
prevent one of the above outcomes listed as an SAE criterion 
Adverse events that do not fall into the above categories are defined as non-serious AEs. 

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9.1.3 Adverse Event of Special Interest 
An AESI is an AE of scientific and medical concern specific to the sponsor’s product or 
program, for which ongoing monitoring and rapid communication by the investigator to the 
sponsor is appropriate. Such an event might require further investigation in order to 
characterize and understand it. Depending on the nature of the event, rapid communication 
by the sponsor to other parties (eg, IDMC, regulators) will be warranted. 
In this study, TEEs will be treated as AESIs. The following 3 narrow standardized Medical 
Dictionary for Regulatory Activities (MedDRA) queries will be utilized for TEE evaluation: 
• Embolic and thrombotic events, arterial 
• Embolic and thrombotic events, venous 
• Embolic and thrombotic events, vessel type unspecified and mixed arterial and 
venous 
9.2 Severity of Adverse Events 
The severity of each AE (ie, nonserious and SAEs) is to be assessed by the investigator as 
follows: 
Severity 
Definition 
Mild 
A type of AE that is usually transient and may require only minimal 
treatment or therapeutic intervention. The event does not generally interfere 
with usual activities of daily living. 
Moderate 
A type of AE that is usually alleviated with additional specific therapeutic 
intervention. The event interferes with usual activities of daily living, 
causing discomfort but poses no significant or permanent risk of harm to the 
subject. 
Severe 
A type of AE that interrupts usual activities of daily living, or significantly 
affects clinical status, or may require intensive therapeutic intervention. 
 
9.3 Causality of Adverse Events 
The causal relationship of an AE to either IMP or corticosteroid treatment must always be 
assessed by the investigator (Treating Physician). All AEs will be classified as either related 
or not related to either IMP or corticosteroid treatment. The AE eCRF will provide the 

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option to select either IMP or corticosteroid treatment relatedness (see Section 8.1.2.1 for 
Monitoring for Corticosteroid Treatment). 
Adverse events attributable to corticosteroid administration include but are not limited to the 
following [Oray et al, 2016]: 
• Uncontrolled hypertension defined as average systolic blood pressure ≥ 140 mmHg 
and/or average diastolic blood pressure ≥ 90 mmHg 
• Hypokalemia 
• Hyperglycemia 
• Weight gain ≥ 5%, increase in waist circumference 
• Osteopenia or osteoporosis 
• Depression, mania, or other relevant psychiatric diagnoses 
• Evidence of peptic ulcer disease or erosive gastritis 
The degree of certainty with which an AE is attributed to IMP or an alternative cause 
(eg, corticosteroid treatment, natural history of the underlying disease, other concomitant 
therapy) will be determined by how well the event can be understood in terms of: 
• Known pharmacology of IMP 
• Clinically and/or pathophysiologically plausible context 
• Reaction of a similar nature previously observed with similar products, or reported in 
the literature for similar products as being product related (eg, headache, facial 
flushing, pallor) 
• Plausibility supported by the temporal relationship (eg, the event being related by 
time to administration or termination of treatment with IMP or corticosteroid 
treatment, drug withdrawal or reproduced on rechallenge) 
When there is uncertainty whether an AE is related to IMP or corticosteroid treatment, the AE 
should be considered related to IMP. If a causality assessment is not provided for an AE 
(including an SAE), that AE will be considered related to IMP. 

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9.4 Observation Period for Adverse Events 
The observation period for AE and SAE reporting for an individual subject will start at the 
time of giving written informed consent for participation in the current study and finish at the 
EOP3 Visit (or last completed study visit). 
If the investigator becomes aware of an SAE that has started after the observation period has 
finished, and there is at least a possible causal relationship with the IMP, the event must be 
reported to CSLB (see Section 9.6.3). 
9.5 Follow-up of Adverse Events 
Every effort should be made to follow AEs until resolution or stabilization. Ongoing, 
non-serious AEs that have not resolved or stabilized will be followed until resolution, 
stabilization, or the subject is lost to follow-up. Serious adverse events will be followed until 
the SAE resolves, stabilizes, or the subject is lost to follow-up. 
9.6 Adverse Event Reporting 
9.6.1 Adverse Events 
At each clinical evaluation, the investigator (or delegate) will determine whether any AEs 
have occurred. All AEs are to be recorded in the eCRF. If known, the medical diagnosis of an 
AE should be recorded in preference to the listing of individual signs, laboratory findings, 
and/or symptoms. The investigator must follow up on the course of an AE until resolution or 
stabilization. If an AE is ongoing after the end of study participation, the AE will continue to 
be followed up until resolution, stabilization, or the subject is lost to follow-up. 
If, during the study period, a subject presents with a preexisting condition that was not noted 
at the time of study entry, the condition should be retrospectively recorded in the Medical 
History eCRF. 
9.6.2 Adverse Events of Special Interest 
Adverse events of special interest should be reported following expedited reporting 
procedures, as described for SAEs (Section 9.6.3). 

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9.6.3 Serious Adverse Events 
This study will comply with all applicable regulatory requirements and adhere to the full 
requirements of ICH Topic E2A (Clinical Safety Data Management: Definitions and 
Standards for Expedited Reporting). 
For SAEs occurring during the study, the investigator (or delegate) will enter all relevant 
information in the eCRF. 
All SAEs that occur during the course of the study, whether or not causally related to 
IMP, must be entered into the eCRF and must be reported immediately (within 
24 hours of the investigator becoming aware of the event) to CSLB. For Japan sites only, 
the investigator must also inform the head of the medical institution of the SAE and related 
information in accordance with the Japanese regulatory requirements and ICH GCP. 
Adverse events occurring in the period between the time the subject gave written informed 
consent and the first exposure to IMP, and meeting 1 or more of the seriousness criteria, must 
be entered into the eCRF in the same manner as other SAEs. 
Any SAE that occurs after the end of study participation that is considered to be causally 
related to IMP must be reported immediately (ie, within 24 hours of the investigator 
becoming aware of the event) to CSLB. Such events are not entered into the eCRF. For 
Japan sites only, the investigator must also inform the head of the medical institution of the 
SAE and related information in accordance with the Japanese regulatory requirements and 
ICH GCP. 
The minimum reporting requirements for reporting of SAEs include: 
• Subject identification number 
• Suspected medicinal product and/or procedure 
• Event term 
• Reporting source identification 
• If the minimum requirements for reporting are fulfilled, the investigator should not 
wait to receive additional information to fully document the event. 
• In addition, the investigator must: 

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• If required by local regulations, report all SAEs to the relevant Institutional Review 
Board (IRB)/Independent Ethics Committee (IEC) within the timeframe specified 
by the IRB/IEC 
• If the subject is an active participant in the study: 
o Enter follow-up information in the eCRF until the SAE has resolved, or, in the 
case of permanent impairment, until stabilized 
o Ensure that the causality assessment for all SAEs is entered in the eCRF 
• If the subject is no longer participating in the study, report the follow-up 
information to CSLB 
In cases of death, the investigator should supply CSLB and the IEC/IRB (as applicable) with 
any additional information as it becomes available (eg, autopsy reports and detailed medical 
reports). 
9.6.4 Other Significant Events 
Not applicable. 
9.6.5 Overdose 
Any IMP overdose that occurs in association with an adverse sign or symptom must be 
entered into the eCRF as an AE; if the AE meets any seriousness criteria, the event must be 
reported as an SAE (see Section 9.6.3). 
Details of overdose of IMP (defined in Section 5.1.3.3) must be recorded in the subject SC 
Infusion Diary. Details of overdose of any concomitant therapy must be recorded in the 
Concomitant Medication eCRF. 
9.6.6 Pregnancy and Breastfeeding  
A female subject who becomes pregnant while participating in the study must notify the 
investigator immediately. 
If a female subject becomes pregnant, she must discontinue treatment with IMP, but will be 
encouraged to remain in the study for the remainder of the planned duration in order to 
collect safety information. 

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CSLB must be notified via the CSLB Pregnancy Outcome Form as well as completion of the 
AE/SAE eCRF identifying the pregnancy, within 5 days of the investigator becoming aware 
of the pregnancy. 
Whenever possible, a pregnancy in a subject exposed to IMP should be followed to term so 
as to assess any potential occurrence of congenital anomalies or birth defects. Any follow-up 
information, including premature termination and the status of the mother and child after 
delivery, should be reported by the investigator to CSLB using a Pregnancy 
Reporting/Outcome Form. 
9.7 IRB/IEC Reporting Requirements 
The timeframe within which an IRB/IEC must be notified of deaths and investigational 
product-related unexpected SAEs is stipulated by each IRB/IEC. It is the investigator’s (for 
Japan sites only: the head of the medical institution’s and the investigator’s) responsibility to 
comply with the requirements for IRB/IEC notification. CSLB will provide investigators with 
all details of all SAEs reported to health authorities. For Japan sites, CSLB will follow local 
regulations and provide the head of the medical institutions and the investigators with all 
details of all SAEs excluding the known SAEs that originated outside of Japan. For Japan 
sites, CSLB will list the sponsor’s opinions based on the accumulated evaluations for SAEs 
that originated outside of Japan in the periodic safety update reports. 
10 Statistics 
10.1 Sample Size Estimation 
The study is designed as a superiority study. The sample size calculation is based on the 
hypothesis that IgPro20 will have a higher responder rate than placebo based on the TIS 
assessments at Weeks 17, 21, and 25. 
A responder is defined as a subject with a TIS ≥ 20 points at Week 25 and at least 1 of the 
previous scheduled visits (Week 17 or Week 21), who completes 24 weeks of randomized 
IMP treatment without the use of rescue corticosteroid treatment. 
Two recent studies [Aggarwal et al, 2018; Tjarnlund et al, 2018] of subjects with DM or PM 
reported the responder rates by the IMACS definition of improvement. However, there are 
currently no data in the literature on the responder rates in the TIS for subjects with DM. For 
power simulation, it is assumed that 65% of subjects randomized to IgPro20 and 30% of 
subjects randomized to placebo have a TIS ≥ 20 points at Week 17. Further, it is assumed that 
for each subject, the response at Week 21 is conditional on the response at Week 17, and the 

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response at Week 25 is conditional on the response at Week 21. Assumptions used are 
detailed in Table 15. 
Table 15 
Assumptions for Power Analysis 
Treatment 
SequenceA 
Probability of 
having a 
TIS ≥ 20 points 
at Week 17 
Probability of having a 
TIS ≥ 20 points at 
Week 21 
Probability of having a 
TIS ≥ 20 points at 
Week 25 
A 
65% 
90%, if TIS ≥ 20 points at 
Week 17 
90%, if TIS ≥ 20 points at 
Week 21 
20%, if TIS < 20 points at 
Week 17, or withdraw, or 
receive rescue corticosteroid 
treatment before Week 17 
20%, if TIS < 20 points at 
Week 21, or withdraw, or 
receive rescue corticosteroid 
treatment before Week 21 
B 
30% 
90%, if TIS ≥ 20 points at 
Week 17 
90%, if TIS ≥ 20 points at 
Week 21 
10%, if TIS < 20 points at 
Week 17, or withdraw, or 
receive rescue corticosteroid 
treatment before Week 17 
10%, if TIS < 20 points at 
Week 21, or withdraw, or 
receive rescue corticosteroid 
treatment before Week 21 
TIS = Total Improvement Score. 
A 
Treatment sequence A = 0.5 g/kg IgPro20 for 24 weeks followed by 0.5 g/kg IgPro20 for 28 weeks. 
Treatment sequence B = placebo for 24 weeks followed by 0.5 g/kg IgPro20 for 28 weeks. 
Simulation shows a total of 126 subjects (63 subjects per treatment sequence) will be 
required for a power of 90% in 1-sided Fisher’s exact test at a significance level of 0.025. 
Fisher’s exact test is used in the power simulation as a close approximation to the exact 
logistic regression model in the primary endpoint analysis. 
10.2 Definition of Periods and Reference Visits 
The TIS calculation will use Baseline assessments throughout Study Period 1 and 
Study Period 2 and DOW assessment will use Baseline as the reference visit for 
Study Period 1 and Study Period 2. Definitions of the start and end of periods and reference 
visits for all other endpoints are presented in Table 16. 

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Table 16 
Study Periods and Reference Visits 
Study 
Period 
Weeks 
Start of Study 
Period 
Reference Visit of 
Period 
End of PeriodA 
1 
Week 1 to 
Week 25 
Start date and time 
of the first IMP 
infusion 
Baseline (before 
infusion of IMP) 
Week 25 (before 
infusion of IMP) 
2 
Week 25 to 
Week 53/Week 56 
Start date and time 
of the first IMP 
infusion at 
Week 25 
Baseline (before 
infusion of IMP) 
 
Week 25 (before 
infusion of IMP) 
Week 53 (before 
infusion of 
IMP) / Week 56 
(for subjects not 
entering SP3) 
3 
Week 53 to EOP3 Start date and time 
of the first IMP 
infusion at 
Week 53 
Week 53 (before 
infusion of IMP) 
EOP3 
IMP = investigational medicinal product; EOP3 = End of Study Period 3; SP3 = Study Period 3. 
A  
The end of the period is the listed study visit or the last study visit before/at withdrawal. 
10.3 Description of Study Analysis Sets 
Screened Analysis Set – SCR 
The SCR analysis set comprises all subjects who provide written informed consent and who 
undergo study Screening procedures. 
Modified Intent-to-Treat Analysis Set – mITT 
The mITT analysis set is as complete and as close as possible to the Intent-to-Treat (ITT) 
principle. It comprises all subjects in the SCR who are randomized, meet the major 
inclusion/exclusion criteria and receive any amount of randomized IMP. The documented 
failure to take any amount of randomized IMP or the lack of any post randomization efficacy 
data in Study Period 1 [ICH E9, 1998] will lead to the exclusion of the subject from the 
mITT. Documentation and final assessment will be done in the Blinded Data Review Meeting 
(BDRM). The mITT analysis set will be based on the treatment sequence to which subjects is 
randomized. 
Modified Intent-to-Treat Analysis Set Extended – mITT-Ex 
The mITT-Ex analysis set comprises all subjects in the mITT who complete the first 
24 weeks of double-blind treatment (Study Period 1) and receive any amount of the IMP 
between Week 25 and Week 53 (Study Period 2). The documented failure to take any amount 
of randomized IMP between Week 25 and Week 53 (Study Period 2) will lead to the 

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exclusion of the subject from the mITT-Ex. The mITT-Ex analysis will be based on the 
treatment sequence to which subjects were randomized. 
Safety Analysis Set – SAF 
The SAF analysis set comprises all subjects who are randomized and receive any amount of 
randomized IMP. The safety analysis will be based on the actual treatment sequence received. 
Per Protocol Analysis Set – PP 
The PP analysis set comprises all subjects in the mITT without protocol violations. Protocol 
violations will be defined in detail in the BDRM before unblinding for the primary data 
analysis (ie, when all subjects have completed all assessments for Study Period 1 and the data 
has been cleaned for primary data analysis). The PP analysis will be based on the treatment 
sequence to which subjects were randomized. 
Safety Analysis Set Extended – SAF-Ex 
The SAF-Ex analysis set comprises all subjects in the SAF who complete the first 24 weeks 
of double-blind treatment (Study Period 1) and receive any amount of the IMP between 
Week 25 and Week 53 (Study Period 2). The SAF-Ex analysis will be based on the actual 
treatment sequence received. 
Safety Analysis Set IgPro20 – SAF-IgPro20 
The SAF-IgPro20 analysis set comprises all subjects in the SAF who are randomized to 
Sequence A, and all subjects in SAF-Ex who are randomized to Sequence B. 
Pharmacokinetic Analysis Set – PK 
The PK analysis set comprises all subjects, without any protocol violations that may impact 
PK analysis, who provide rich PK samples for noncompartmental PK parameter estimation. 
Protocol violations that may impact PK analysis will be defined in detail in the BDRM before 
database lock. 
Safety Analysis Set for Study Period 3 – SAF-P3 
The SAF-P3 Analysis Set comprises all subjects in the SAF-Ex who complete the first 
52 weeks of treatment in Study Period 1 and Study Period 2 and receive any amount of the 
IMP after Week 52 (ie, in Study Period 3). 

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10.4 Statistical Analyses and Methods for Study Period 1 and 
Study Period 2 
A complete description of the statistical analyses and methods will be available in the SAP, 
which will be finalized before unblinding for the primary data analysis (ie, when all subjects 
have completed all assessments for Study Period 1 and the data has been cleaned for primary 
data analysis). 
10.4.1 Subject Disposition and Characteristics 
10.4.1.1 Subject Disposition 
The number of subjects who are screened, randomized, completed the study, discontinued 
IMP (including the reason), and withdraw consent from the study (including the reason) will 
be summarized by treatment sequence, period, and total subjects. Screen failure rates will be 
presented by reason. The reason for discontinuing IMP or withdrawing of consent from the 
study will be listed by subject. 
10.4.1.2 Subject Characteristics 
All demographic and Baseline characteristics summaries will be based on the mITT analysis 
set and the SAF-P3 analysis set for Study Period 3. They will be presented in summary tables 
by treatment sequence. Continuous data will be summarized by descriptive statistics and 
categorical data will be summarized by frequency distributions. Age will be described as both 
a continuous and a discrete variable. By-subject listings will be provided for demographic 
and Baseline characteristic data. 
10.4.2 Efficacy Analyses 
10.4.2.1 Study Hypotheses 
The study is designed with the objective of testing the null and the alternative hypotheses for 
the responder rates (see Section 2.1.2) as defined below: 
H0: πIgPro20 ≤ πPbo 
 
vs. 
 
H1: πIgPro20 > πPbo, 
where πIgPro20 and πPbo represent the responder rates with IgPro20 and placebo, respectively. 
Under the null hypothesis, the assumption is that no beneficial effect is afforded by IgPro20 
while the alternative hypothesis states that IgPro20 is effective in increasing the responder 
rate compared to the placebo arm. 

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10.4.2.2 Primary Estimand 
The primary interest is to quantify the treatment effect of IgPro20  
• where the potential confounding effect of rescue corticosteroid treatment is excluded  
• where the potential confounding of subjects who withdraw from treatment or 
withdraw from the study before Week 25 and cannot provide sufficient data to 
demonstrate a benefit of treatment is excluded, and 
• in the absence of premature withdrawal from IMP or the study before Week 25 due to 
the Ukraine war [EMA Biostatistics Working Party, 2022]. 
The primary estimand in line with the primary interest of the study is described as follows: 
• Population: the target patient population defined by eligibility criteria and who 
received any amount of randomized IMP treatment and have post baseline efficacy 
results in Study Period 1 (mITT). See Section 10.3 for details. 
• Variable: responder status based on TIS assessments at Weeks 17, 21, and 25 
• Intercurrent events:  
o withdrawal from randomized IMP treatment or the study, which is not related 
to the Ukraine war, or receiving rescue corticosteroid treatment before 
Week 25 is considered treatment failure 
o withdrawal from IMP or the study that is related to the Ukraine war will be 
addressed using the hypothetical strategy [Meyer et al, 2020; Collins et al, 
2020] 
• Population-level summary: responder rate by treatment sequence 

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10.4.2.3 Primary Efficacy Endpoint 
The primary endpoint is the responder status in each treatment sequence based on TIS 
assessments at Weeks 17, 21, and 25. Following the estimand described in Section 10.4.2.2, a 
responder is defined as a subject with a TIS ≥ 20 points at Week 25 and at least 1 of the 
previous scheduled visits (Week 17 or Week 21), who completes 24 weeks of randomized 
IMP treatment without the use of rescue corticosteroid treatment. Therefore, for subjects who 
do not withdraw from IMP or the study for reasons that are related to the Ukraine war before 
Week 25, non-responders will include subjects who meet any 1 of the following: 
• TIS < 20 points at Week 25 
• TIS < 20 points at both Weeks 17 and 21 
• Withdraw from treatment or the study or receive rescue corticosteroid treatment 
before Week 25 
To ensure that the treatment effect is not confounded by the intercurrent event of premature 
withdrawal from IMP or the study due to the Ukraine war, the hypothetical strategy is applied 
for this intercurrent event to quantify the treatment effect in the absence of the Ukraine war 
[Meyer et al, 2020; Collins et al, 2020]. For subjects who do withdraw from IMP or the study 
for reasons which are related to the Ukraine war before Week 25, scheduled TIS assessments 
after withdrawal are missing. These missing values can be considered missing at random and 
multiple imputation will be used to derive the hypothetical post-intercurrent event TIS values 
and the corresponding response variables assuming that the subjects with missing TIS values 
continue in the same way as similar subjects in the study who did not withdraw prematurely. 
Multiple imputation is a simulation based approach where missing values are replaced by 
multiple Bayesian draws from the conditional distribution of missing data given the observed 
data and covariates, creating multiple completed data sets. The completed data sets can then be 
analyzed using standard methods (see Section 10.4.2.3.1). Note: If no TIS values are available 
for the subject for any post-randomization visit during Study Period 1, the subject will be 
excluded from the mITT analysis set (see Section 10.3). 
Details of the multiple imputation process for TIS values and derivation of the responder 
status are provided below: 
• The multiple imputation process will be done for subjects who withdraw from IMP or 
the study for reasons related to the Ukraine war before Week 25 including all subjects 
who complete Study Period 1 without receiving rescue medication. TIS values in 
subjects who withdraw from IMP for reasons related to the Ukraine war before Week 

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25 that are assessed more than 6 weeks after the last IMP administration will be set to 
missing. Replacement of missing TIS values after discontinuation of IMP or the study 
(ie, monotone missings) for subjects who withdraw from IMP or the study for reasons 
related to the Ukraine war before Week 25 will be done using multiple imputations 
based on monotone regression with the covariates treatment, country, and Baseline 
MMT-8 (≤ 142 points vs. >142 points) and TIS values from previous visits (50 
imputations) by SAS® PROC MI. The seed of the pseudo random number generator 
to be used to generate the imputations for missing values will be 30071234. 
  
Note: If intermittent values are missing for any of the data included in the multiple 
imputation analysis the intermittent values will be imputed first using the MCMC 
option in SAS® PROC MI 50 times to create 50 data sets with only monotone 
missings. The corresponding data sets will be used as input data sets for replacement 
of the trailing missing values with SAS® PROC MI using monotone regression, as the 
preceding step led already to 50 data sets, the number of imputations in this step is to 
be set to 1. 
• In each of the data sets subjects’ responses will be derived according to the definition 
provided above. 
• In order to get data sets including all mITT subjects with complete response 
information, data of the following subjects will be added to each of the 50 data sets 
o Subjects who withdraw from IMP or the study for reasons not related to the 
Ukraine war before Week 25 or 
o Subjects who receive rescue medication in Study Period 1 
According to the definition above these subjects are all non-responders.  
The TIS is a validated assessment for DM and PM [Aggarwal et al, 2017]. It is the sum of all 
improvement scores (derived from 6 CSMs) associated with the percent change in each CSM 
as in Table 17. All 6 CSMs have been established and validated as measurement of myositis 
disease activity for adult DM/PM clinical studies by the IMACS [Rider et al, 2011; 
Miller et al, 2001]. 
According to the Web calculator for 2016 ACR/EULAR Criteria the TIS calculation requires 
at minimum CSM1 (Physician Global Disease Activity), CSM3 (MMT-8) and at least 2 other 
CSMs. Missing CSMs are considered as not changed (improvement score = 0). 

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Table 17 
Total Improvement Score (TIS) Using Core Set Measures 
Core set 
measures  
Level of improvement 
based on absolute percent change 
Improvement 
score 
Physician 
Global 
Disease 
Activity 
Worsening to ≤ 5% 
0 
> 5% up to ≤ 15% 
7.5 
> 15% up to ≤ 25% 
15 
> 25% up to ≤ 40% 
17.5 
> 40% 
20 
Patient 
Global 
Activity 
Worsening to ≤ 5% 
0 
> 5% up to ≤ 15% 
2.5 
> 15% up to ≤ 25% 
5 
> 25% up to ≤ 40% 
7.5 
> 40% 
10 
Manual 
Muscle 
Testing-8 
Worsening to ≤ 2% 
0 
> 2% up to ≤ 10% 
10 
> 10% up to ≤ 20% 
20 
> 20% up to ≤ 30% 
27.5 
> 30% 
32.5 
Health 
Assessment 
Questionnaire 
Worsening to ≤ 5% 
0 
> 5% up to ≤ 15% 
5 
> 15% up to ≤ 25% 
7.5 
> 25% up to ≤ 40% 
7.5 
> 40% 
10 
Muscle 
Enzyme  
Worsening to ≤ 5% 
0 
> 5% up to ≤ 15% 
2.5 
> 15% up to ≤ 25% 
5 
> 25% up to ≤ 40% 
7.5 
> 40% 
7.5 
Extramuscular 
Global 
Activity 
Worsening to ≤ 5% 
0 
> 5% up to ≤ 15% 
7.5 
> 15% up to ≤ 25% 
12.5 
> 25% up to ≤ 40% 
15 
> 40% 
20 
Total Improvement Score in the patient (scale: 0-100) 
 

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10.4.2.3.1 Primary Efficacy Analysis 
For each of the 50 data sets resulting from the multiple imputation process described in 
Section 10.4.2.3 the primary endpoint analysis of responder status will be based on an exact 
logistic regression model including fixed effects for treatment, region (Japan vs. non-Japan), 
and Baseline MMT-8 assessment (≤ 142 points vs. > 142 points). (Note: if a subject is 
erroneously assigned to the wrong baseline randomization stratum [eg, actual Baseline 
MMT-8 ≤ 142 points, but randomized in the stratum > 142 points] the analysis will be done 
according to the actual stratum and not according to the stratum used for randomization: This 
applies to all analyses.) The corresponding results across the data sets are combined for 
overall inference using SAS® PROC MIANALYZE according to Rubin’s rule to give the 
1-sided p-value for hypothesis testing, the odds ratio (IgPro20:placebo), and the 
corresponding 2-sided 95% CI. The primary endpoint analysis will utilize the mITT analysis 
set. Statistical significance will be assessed using a 1-sided alpha level of 0.025. The 
responder rate and corresponding 2-sided 95% CI will be estimated by treatment sequence. 
The primary endpoint analysis will be repeated on the PP as a supportive analysis. 
10.4.2.3.2 Sensitivity and Supplementary Analyses of the Primary Efficacy 
Endpoint 
Sensitivity analyses of the primary efficacy endpoint will be performed to examine the 
robustness of the conclusion from the planned primary endpoint analysis to deviations from 
its underlying modelling assumptions and limitations in the data. Consistency of findings 
from the primary and sensitivity or supplementary analyses will be investigated and clinical 
plausibility of findings will be examined. 
The following sensitivity analyses will be done based on the imputed TIS data used for the 
primary efficacy analysis and combined using Rubin’s rule. Sensitivity analyses will address 
the following aspects: 
• Regression model: standard logistic regression model will be fitted instead of exact 
logistic regression model. In case of non-convergence, the model will run without the 
fixed effects for region and MMT-8 stratification variable. 

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• Reasons for withdrawal: sensitivity to the assumption that all early dropouts are 
non-responders will be assessed in this analysis. The responder status of subjects 
withdrawn from the study before Week 25 will be set to “responder” if: 
o TIS is ≥ 20 points at the last study visit and at least at either of the previous 
2 scheduled visits, and 
o reason for withdrawal is not due to lack of efficacy, or IMP related AEs 
An additional sensitivity analysis will be done excluding subjects who drop out for reasons 
related to the Ukraine war using an exact logistic regression model including fixed effects for 
treatment, region (Japan vs. non-Japan), and Baseline MMT-8 assessment (≤ 142 points vs. 
> 142 points). 
 A supplementary analyses will address the following: 
• 
TIS with restrictions: individual CSMs are considered in addition to the TIS in the 
evaluation of responses at Week 17, Week 21, and Week 25. A subject should meet all of 
the following 3 criteria to be considered as showing response at each of the 3 study 
visits: TIS ≥ 20 points; no deterioration > absolute 10% from Baseline in MMT-8; and 
no deterioration > absolute 20% from Baseline in any other 2 CSMs. This analysis will 
exclude subjects who drop out for reasons related to the Ukraine war before Week 25 
and thus will be based on an exact logistic regression model including fixed effects for 
treatment, region (Japan vs. non-Japan), and Baseline MMT-8 assessment (≤ 142 points 
vs. > 142 points). 
The analytical approach in the sensitivity or supplementary analyses will be the same as 
described in Section 10.4.2.3.1, unless otherwise stated. The sensitivity analyses will be 
based on the mITT and PP. 
10.4.2.4 Secondary Efficacy Endpoints 
10.4.2.4.1 Key Secondary Estimand 
The key secondary interest is to quantify the treatment effect of IgPro20 under the 
hypothetical situation where no subjects receive rescue corticosteroid treatment or withdraw 
from treatment or the study before Week 25. 

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The key secondary estimands in line with the key secondary interest of the study are 
described as follows for the continuous key secondary endpoints: 
• Population: the target patient population defined by eligibility criteria and who 
received any amount of randomized IMP treatment and have post baseline efficacy 
results in Study Period 1 (mITT). See Section 10.3 for details. 
• Variables: TIS, changes from Baseline in MMT-8, and changes from Baseline in 
CDASI total activity score at Week 25  
• Intercurrent event: when a subject withdraws from randomized IMP treatment or 
receives rescue corticosteroid treatment before Week 25, data after withdrawal or 
rescue will be considered missing for the continuous endpoints, even if data are 
collected in the study  
• Population-level summary: mean of the variables by treatment sequence 
The key secondary estimand for the binary key secondary endpoint reduction of oral 
concomitant corticosteroid dose by at least 25% at Week 25 is described as follows: 
• Population: the target patient population defined by eligibility criteria and who 
received any amount of randomized IMP treatment and have post baseline efficacy 
results in Study Period 1 (mITT). For details see Section 10.3. 
• Variables: reduction of oral concomitant corticosteroid dose at Week 25 
• Intercurrent events: 
o withdrawal from randomized IMP treatment or the study which is not related 
to the Ukraine war or receives oral rescue corticosteroid treatment before 
Week 25, data after withdrawal or oral rescue will be considered no reduction 
of oral concomitant corticosteroid even if data are collected in the study 
o withdrawal from IMP or the study which are related to the Ukraine war will be 
addressed using the hypothetical strategy. 
• Population-level summary: percentage of subjects who are able to reduce the oral 
concomitant corticosteroid dose by at least 25% by treatment sequence 

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10.4.2.4.2 Key Secondary Endpoint 
The following key secondary efficacy endpoints are defined as follows: 
• TIS at Week 25 
• Changes from Baseline in MMT-8 at Week 25 
• Changes from Baseline in CDASI total activity score at Week 25 
• Reduction of oral concomitant corticosteroid dose at Week 25 
10.4.2.4.3 Key Secondary Efficacy Analyses 
Each of the following 3 key secondary efficacy endpoints will be analyzed separately using 
the MMRM model. The terms to be included in the model for each key secondary endpoint 
are as follows: 
• TIS at Week 25: treatment, study visit, the interaction between treatment and study 
visit, region, and Baseline MMT-8 assessment (≤ 142 points vs. > 142 points), as 
fixed effects, subjects as a random effect 
• Changes from Baseline MMT-8 at Week 25: treatment, study visit, the interaction 
between treatment and study visit, and region as fixed effects, Baseline MMT-8 as a 
continuous covariate, subjects as a random effect 
• Changes from Baseline CDASI total activity score at Week 25: treatment, study 
visit, the interaction between treatment and study visit, region, and Baseline MMT-8 
assessment (≤ 142 points vs. > 142 points) as fixed effects, Baseline CDASI total 
activity score as a continuous covariate, subjects as a random effect 
Analyses will start with the unstructured covariance matrix; compound symmetry will be 
adopted in case of convergence difficulties. The differences between treatment sequences, the 
corresponding 95% CIs, and 1-sided p-values from the models will be provided. Point 
estimates for the average values in each treatment sequence will be presented along with their 
standard errors at all visits. 
For subjects who receive rescue corticosteroid treatment or who withdraw from treatment or 
the study, data after the first administration of rescue corticosteroid treatment or withdrawal 
from randomized IMP treatment will be considered missing and thus not included in the 
MMRM analysis. 

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The following sensitivity analyses are planned for the first 3 key secondary endpoints: 
• A tipping point analysis as described in Ouyang, 2017 will be performed as a 
sensitivity analysis. The base case for the tipping point analysis is to include all 
available data as collected in the MMRM analysis. 
• The tipping point analysis will be repeated under the abovementioned hypothetical 
estimand, ie, where data obtained after rescue corticosteroid treatment or 
withdrawal from randomized IMP treatment for any reason are excluded from the 
MMRM analysis. 
The analysis of the fourth key secondary endpoint, ie, the proportion of subjects who are able 
to reduce the oral concomitant corticosteroid dose by at least 25% at Week 25 will be done in 
analogy to the analysis of the primary endpoint (see Section 10.3).  
For subjects who do withdraw from IMP or the study for reasons which are related to the 
Ukraine war before Week 25, the resulting missing values can be considered missing at 
random and multiple imputation will be used to derive the hypothetical post-intercurrent 
event reduction of oral concomitant corticosteroid dose in percent (%reduction). 
Replacement of trailing missing %reduction values for Week 17 to Week 25 (ie, monotone 
missings) will be done if the subject received oral corticosteroid medication at baseline using 
multiple imputations based on monotone regression with the covariates treatment, country, 
and Baseline MMT-8 (≤ 142 points vs. > 142 points), baseline oral corticosteroid dose and 
%reduction values from previous weeks starting at Week 17 (50 imputations) by SAS® 
PROC MI. Note: If the subject did not receive any oral corticosteroid medication at baseline 
%reduction will be set to 0. The multiple imputation process will correspond to the one for 
the primary endpoint described in Section 10.4.2.3. A subject is considered a responder 
regarding this endpoint, if he/she is able to reduce the oral concomitant corticosteroid dose by 
at least 25% at Week 25, ie if %reduction is ≥ 25. 
For each of the 50 data sets resulting from the multiple imputation process the analysis of the 
4th key secondary endpoint will be based on an exact logistic regression model including 
fixed effects for treatment, region (Japan vs. non-Japan), and Baseline MMT-8 assessment 
(≤ 142 points vs. > 142 points). The corresponding results across the data sets are combined 
for overall inference using SAS® PROC MIANALYZE according to Rubin’s rule. 
The 4 key secondary endpoints will be analyzed with an overall 1-sided alpha level of 0.025. 
The hypotheses associated with the key secondary endpoints will be formally tested and, 

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therefore, adjusted for multiplicity as described in Section 10.4.2.4.4. The key secondary 
analyses will be performed based on the mITT and repeated for the PP. 
10.4.2.4.4 Multiplicity 
Issues related to multiplicity arising from testing the primary endpoint and key secondary 
endpoints will be addressed using the serial gatekeeping procedure [Dmitrienko et al, 2013] 
to control the overall family error rate at a 1-sided significance level of 0.025. Two families 
of null hypotheses are defined with family 1 consisting of the primary endpoint (H01) and 
family 2 consisting of the 4 key secondary endpoints in the Treatment Phase (H02, H03, H04, 
and H05). The null hypothesis associated with each of these endpoints is given below: 
• H01: Responder rate in IgPro20 0.5 g/kg is ≤ placebo 
• H02: Mean TIS in IgPro20 0.5 g/kg is ≤ placebo at Week 25 
• H03: Mean change from Baseline in MMT-8 in IgPro20 0.5 g/kg is ≤ placebo at 
Week 25 
• H04: Mean change from Baseline in CDASI total activity score in IgPro20 0.5 g/kg 
is ≥ placebo at Week 25 
• H05: Proportion of subjects who are able to reduce the oral corticosteroid dose by at 
least 25% in IgPro20 0.5 g/kg ≤ placebo at Week 25 
The general process flow of the hypothesis testing is as follows: the null hypothesis 
associated with the primary endpoint H01 will be tested first. If and only if the resulting 
1-sided p-value is ≤ 0.025, testing of H02, H03, H04, and H05 will be undertaken; otherwise, 
further hypothesis testing will cease and H02 through H05 will be retained. 
After H01 is rejected, the hypothesis tests associated with key secondary endpoints in the 
Treatment Phase will be performed using the Hommel step-up procedure at a 1-sided 
significance level of 0.025. The resulting p-values of the second family are ordered from the 
smallest to the largest, P(1), P(2), P(3), P(4), with the associated ordered null hypotheses H(1), 
H(2), H(3), and H(4). The Hommel procedure is described below: 
• Step 1: start with the largest p-value P(4). If P(4) ≤ 0.025, reject all null hypotheses in 
the family, H(1), H(2), H(3), and H(4). Otherwise, fail to reject H(4) and proceed to 
Step 2. 
• Step 2: test the null hypothesis H(3) associated with the p-value P(3). If P(3) ≤ 0.0125, 
reject H(1), H(2), and H(3). Otherwise, fail to reject H(3) and proceed to Step 3. 

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• Step 3: test the null hypothesis H(2) associated with the p-value P(2). If P(2) ≤ 0.00833 
(or P(3) ≤ 0.01667 and P(2) ≤ 0.0125), reject H(1) and H(2). Otherwise, fail to reject H(2) 
and proceed to the last step, Step 4. 
• Step 4: test the null hypothesis H(1) associated with the smallest p-value P(1). 
If P(1) ≤ 0.00625, or P(1) ≤ 0.00833 and P(2) ≤ 0.0125, or P(1) ≤ 0.00833 and 
P(3) ≤ 0.01875, reject the null hypothesis H(1). Otherwise, fail to reject H(1). 
10.4.2.4.5 Other Secondary Efficacy Endpoints and Analyses 
All other secondary efficacy endpoints will be summarized descriptively by treatment 
sequence and visit. Raw data and changes from the reference visit will be summarized at all 
visits as appropriate. Comparison between treatment sequences will be performed using 
statistical models as described below. No imputation is planned for missing data in secondary 
endpoints. P-values from the statistical models are intended to be descriptive. 
TIS from Week 5 to Week 53: The TIS values between Week 5 and Week 25 will be 
analyzed in MMRM as described in Section 10.4.2.3. The TIS values between Week 29 and 
Week 53 will be analyzed in a similar MMRM model with TIS at Week 25 as a covariate. 
The percentage of subjects achieving TIS ≥ 20, ≥ 40, and ≥ 60 points will be compared 
between treatment sequences with a logistic regression model. Kaplan-Meier estimates will 
be presented for time to first achieving TIS ≥ 20, ≥ 40, and ≥ 60 points for both treatment 
sequences. Time to first achieving TIS ≥ 20, ≥ 40, and ≥ 60 points during study period 1 will 
be compared between treatment sequences with a Cox regression model. 
Individual CSMs except muscle enzyme and CDASI from Baseline to Week 53: The 
individual CSMs will be analyzed in the same way as MMT-8 and CDASI described in 
Section 10.4.2.4.3 by period. 
DOW from Baseline to Week 53: The percentage of subjects meeting DOW once, twice, or 
more than twice, and the percentage of subjects meeting DOW and receiving rescue 
corticosteroid treatment will be compared between treatment sequences with a logistics 
regression. Kaplan-Meier estimates will be presented for the time to meeting DOW for the 
first time from baseline to Week 53 for both treatment sequences. In addition, time to meeting 
DOW for the first time during Study Period 1 will be compared between treatment sequences 
with a Cox regression model. 
Reduction of oral corticosteroid dose from Baseline to Week 53: The percentage of 
subjects who start oral corticosteroid dose taper before Week 25 by treatment sequence will 

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be compared between treatment sequences with an exact logistic regression. Additionally, the 
percentage of subjects who are able to reduce the oral corticosteroid dose by ≥ 25%, ≥ 50%, 
≥ 75% by Week 25 or by Week 53 will be compared between treatment sequences with an 
exact logistic regression. 
Use of rescue corticosteroid treatment from Baseline to Week 25: The percentage of 
subjects receiving rescue corticosteroid treatment will be compared by treatment sequence 
with an exact logistic regression by visit. 
Mobility, Self-care, and Usual Activities domains of EQ-5D-5L from Baseline to 
Week 53: The number of subjects having at least 1, 2, or > 2 levels up from Baseline at 
Week 25 and Week 53 will be compared by treatment sequence using the cumulative 
distribution function. The cumulative distribution function will also be derived to describe the 
changes in levels from Week 25 by treatment sequence at Week 41 and Week 53. 
10.4.2.5 Subgroup Analyses 
Internal consistency of observed treatment effect across major subgroups will be investigated 
as part of exploratory analyses. Subject groups may include but are not limited to, age, 
gender, region, MMT-8 (≤ 142 points or > 142 points). Further details will be provided in the 
SAP. 
10.4.3 Safety Analyses 
All Safety analyses will be performed separately by treatment sequence based on the SAF, 
SAF-Ex and overall. Adverse events will be coded using MedDRA version 22.0 or higher. 
TEAEs are defined as AEs reported at or after the start of study treatment. All AEs regardless 
of when they were reported will be listed. 
• An overview summary of TEAEs, including frequency counts and percentages of 
subjects and the number of events and TEAE rate per time at risk, including the 
following: 
o Any TEAE 
o TEAEs related to IMP 
o TEAEs related to corticosteroid treatment 
o Temporally associated TEAEs (defined as an AE with an onset between the start 
of the IMP infusion and up to 72 hours after the end of IMP infusion) 

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o Related TEAEs or temporally associated TEAEs to IMP 
o TEAEs leading to permanent discontinuation of IMP 
o TEAEs leading to withdrawal from study 
o TEAEs leading to IMP dose interruptions 
o AESIs 
o AESIs related to the IMP 
o Temporally associated AESIs (defined as a AESI with an onset between the start 
of the IMP infusion and up to 72 hours after the end of IMP infusion) 
o Serious TEAEs 
o Serious TEAEs related to the IMP 
o Serious TEAEs related to corticosteroid treatment 
o Temporally associated serious TEAEs (defined as a serious TEAE with an onset 
between the start of the IMP infusion and up to 72 hours after the end of IMP 
infusion) 
o Fatal serious TEAEs 
• TEAEs will be summarized by severity. In addition, the frequency counts and 
percentage of AEs will be summarized in descending order of total incidence by 
preferred term (PT) only and by system organ class (SOC) and PT. The following 
descriptive tables will be generated for TEAEs, including frequency counts and 
percentages of subjects and the number of events and TEAE rate per time at risk: 
o TEAEs by SOC and PT 
o TEAEs by SOC, PT, and maximum severity 
o Causally related TEAEs by SOC and PT 
o Temporally associated TEAEs by SOC and PT 
o Causally related TEAEs or temporally associated TEAEs by SOC and PT 
The following safety parameters will be analyzed descriptively: 
• Hemolysis, suspected or confirmed 
• Wells’ Criteria score for DVT 

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• Wells’ Criteria score for PE 
• Laboratory safety parameters 
• Vital signs 
• Physical examination 
10.4.4 Exploratory Analyses 
Pain/discomfort and Anxiety/depression domains of EQ-5D-5L, WPAI-GH, TSQM-9, 
Timed Up and Go, 5-D Itch Score, Muscle Enzyme, concomitant corticosteroid tapering, and 
ANA, MSA, and MAA will be described similarly to secondary efficacy endpoints as 
described in Section 10.4.2.4.5. The observed values and changes from reference visits of 
these endpoints will be summarized by visit, and treatment sequence as appropriate. 
10.4.4.1 Pharmacokinetics Analyses 
Serum IgG levels will be summarized by visit, treatment sequence, and period. 
Rich PK sampling includes a sufficient number of sampling PK time points (from up to 
10 Japanese subjects and 30 non-Japanese subjects) during Week 37 to enable a complete 
concentration-time IgG profile. IgG concentrations from rich PK sampling will be listed and 
summarized by nominal (planned) time points. Individual concentration-time profiles and 
mean (± standard deviation) profiles will be plotted using actual time points for individual 
plots and nominal (planned) time points for mean profiles. 
PK parameters Cmax, Ctrough, and AUC will be derived using standard noncompartmental 
analysis using Phoenix WinNonlin, version 6.3 or higher. Cmax and Ctrough will be assessed 
based on the observed values and the method of AUC estimation will be by linear/log trapezoidal 
rule. Each of Cmax, Ctrough, and AUC will be described using individual listings and summary 
tables based on the PK analysis set. 
Additional information on the planned analyses of PK concentration and PK parameters will 
be provided in the SAP. A separate PK Report will be appended to the clinical study report. 

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10.4.5 Interim Analysis 
No interim analysis for sample size re-estimation, futility stopping, or efficacy stopping is 
planned.  
The primary data analysis (EOP1 analysis) will be done after all subjects have completed all 
assessments for Study Period 1. At the time of the EOP1 analysis additional AE, efficacy, and 
pharmacokinetic data of Study Period 2 will be analyzed. Efficacy analysis of the Study 
Period 2 data will be restricted to all subjects completing or discontinuing prematurely Study 
Period 2 at the time of data cut-off for the EOP1 analysis; the adverse events analysis will 
include all subjects treated in any study period at the time of data cut-off for EOP1 analysis; 
the pharmacokinetic analysis will include all subjects who have completed the Rich PK 
sampling at Week 37. 
Two additional analyses will be done after all subjects have completed all assessments for 
Study Period 2 (EOP2 analysis) and one after all subjects have completed all assessments for 
Study Period 3. 
10.5 Statistical Analyses and Methods for Study Period 3 
All analyses for Study Period 3 will be based on the SAF-P3. 
All secondary efficacy endpoints and all exploratory endpoints referring to Study Period 3 
will be analyzed descriptively. TEAEs occurring during Study Period 3 will be summarized 
descriptively including frequency counts and percentages of subjects and the number of 
events and TEAE rate per time at risk in analogy to Section 10.4.3. 
A complete description of the statistical analyses and methods will be available in the SAP. 
11 Quality Assurance 
The study may be subject to an audit by CSLB, an authorized representatives of CSLB and/or 
inspections by an authorized health authority (eg, United States Food and Drug 
Administration [FDA]). Health authorities may request access to all study documentation, 
including source documents for inspection and copying, in keeping with local regulations. 
CSLB will notify the investigator of any upcoming audit/inspection. 
In the event of an audit, all pertinent study-related documentation must be made available to 
the auditors. If an audit or inspection occurs, the investigator at each study site will permit the 

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auditor/inspector direct access to all relevant documents and allocate their time as well as the 
time of relevant staff to discuss the findings and any relevant issues. 
12 Regulatory and Ethics Considerations 
12.1 Regulatory Considerations 
CSLB or its agents will submit the appropriate documents to the appropriate global 
regulatory agencies and will await investigational new drug (IND)/clinical trial application 
(CTA)/clinical trial notification (CTN) approval/notification before study start. 
The procedures set out in this clinical study protocol are designed to ensure that CSLB and 
the investigator abide by the principles of the current ICH GCP guideline on the conduct, 
evaluation and documentation of this study, as described in ICH Topic E6 (Guideline for 
GCP). The study will also be carried out according to all applicable international and national 
regulatory requirements. 
12.2 Institutional Review Board/Independent Ethics Committee 
The investigator must submit the clinical study protocol and ICFs for review by an authorized 
and properly constituted (according to local guidelines) IRB/IEC. Written approval must be 
received from the IRB/IEC before commencement of the study. 
For Japan sites only, the head of the study site should submit a written report to the IRB 
providing the details of all safety-related information reported by CSLB. In addition, the 
study continuation is to be reapproved by the IRB annually. 
12.3 Subject Information and Informed Consent 
Informed consent of subjects according to the standards of GCP and the principles in the 
Declaration of Helsinki must be implemented in this clinical study before protocol-specified 
procedures are carried out. Written informed consent is not required for assessments or 
procedures performed according to standard of care (eg, for diagnosis or treatment); results 
from such assessments may be used in the determination of study eligibility. Information 
should be given in both oral and written form and should be deemed appropriate by the 
IRB/IEC. Subjects, their relatives (or if necessary, legally acceptable representatives) must be 
given ample opportunity to inquire about details of the study. 
The subject (or if necessary, legally acceptable representatives) must be provided with a copy 
of the signed informed consent form. 

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Should there be any amendments to the clinical study protocol that would directly affect the 
subject’s participation in the study (eg, a change in any procedure), the ICF must be amended 
to incorporate this modification. Subjects must be informed of the change and they must sign 
the amended ICF indicating that they re-consent to participate in the study. 
12.4 Subject Confidentiality 
All subject names and contact details will be kept confidential. Subjects will be identified 
throughout documentation and evaluation by the number allotted to them during the study. 
Each subject will be told that all study findings will be handled in the strictest confidence. 
The investigator at the study site will be responsible for retaining sufficient information about 
each subject (eg, name, address, phone number and identity in the study) so that regulatory 
agencies or CSLB may access this information should the need arise. These records should be 
retained in a confidential manner as long as legally mandated according to local 
requirements. 
Subject medical records pertaining to the study may be inspected/audited at any time by 
CSLB employees or their duly authorized representatives, a health authority or the IRB/IEC. 
All records accessed will be strictly confidential. Consent to participate in the study includes 
consent to these inspections/audits. 
12.5 Indemnity and Compensation 
CSLB has secured insurance to cover its obligations under both the Indemnity and the 
Compensation guidelines for injury to subjects involved in the study. 
Other details regarding compensation and the obligations of the investigator/CSLB are 
provided in the Clinical Trial Research Agreement for the study (see Section 13.1). 
13Administrative Considerations 
13.1 Clinical Trial Research Agreement 
This study will be conducted under a Clinical Trial Research Agreement between CSLB 
(“Sponsor”) and the institutions representing the investigational study sites (“Authority”). 
Financial support to the investigational sites will be detailed in the Clinical Trial Research 
Agreement. The Clinical Trial Research Agreement must be signed before the 
commencement of the study and will clearly delineate the responsibilities and obligations of 
investigator and CSLB, and will form the contractual basis under which the clinical study 
will be conducted. Clinical Trial Research Agreements may be executed by electronic 

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signature (current provider DocuSign) in compliance with 21 Code of Federal Regulations 
(CFR) Part 11 and simple or advanced electronic signature according to European Union 
Regulation No 910/2014 – eIDAS. 
13.2 Clinical Study Registration and Results Disclosure 
CSLB will provide the relevant clinical study protocol information in public databases before 
or at commencement of the study. CSLB may also provide study information for inclusion in 
national registries according to local regulatory requirements. 
Results of this study will be disclosed according to the relevant regulatory requirements. All 
publications in peer-reviewed medical journals resulting from this study will be listed in the 
original clinical study protocol registration record. 
13.3 Implementation of the Clinical Study Protocol and Amendments 
With the exception of medical emergencies, no changes or deviations in the conduct of the 
signed clinical study protocol will be permitted without documented approval of the CSLB 
Medical Monitor or designee and the IRB/IEC. In the event of a medical emergency, the 
investigator at the study site will institute any medical procedures deemed appropriate. 
However, all such procedures must be promptly reported to the CSLB Medical Monitor and 
the IRB/IEC. 
Modifications to the clinical study protocol that may affect subject safety or the way the 
study is to be conducted will be documented in a protocol amendment, which must be 
approved by the IRB/IEC. 
Administrative changes to the clinical study protocol, defined as minor corrections and/or 
clarifications that have no effect on the way the study is to be conducted, will not require 
IRB/IEC approval, but will be submitted to the IRB/IEC for their information. 
13.4 Protocol Deviations 
All instances where the requirements of the clinical study protocol are not complied with will 
be tracked. Corresponding subjects may be withdrawn from the study at the discretion of the 
investigator and/or CSLB. Clinical study protocol deviations arise when either subjects who 
have been entered in the study and/or the study sites deviate from the IEC/IRB-approved 
study protocol. 

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If a major protocol deviation (ie, a deviation that could have a significant effect on the 
subject’s safety, rights, or welfare and/or on the integrity of the study data) occurs, the 
investigator must notify CSLB and the appropriate IRB/IEC as soon as possible or as per 
local requirements. 
13.5 Documentation and Record Keeping 
13.5.1 Data Collection 
The physicians (or delegates) will maintain individual records for each subject. These records 
should include dates when a subject visited the study site, records of vital signs, medical 
history, or physical examinations, administration of IMP or concomitant therapy, any AEs 
experienced, efficacy status (improvement or deterioration) and other notes as appropriate. 
These records (electronic or paper) constitute source data. 
Electronic CRF entries will be considered source data if the eCRF is the site of the original 
recordings (ie, there is no other written or electronic record of the data). 
An eCRF will be provided by CSLB for each subject enrolled into the study. The Treating 
Physician (or delegate) is responsible for ensuring accurate and proper completion of the 
eCRF in a timely manner so that it always reflects the latest observations on the subjects 
enrolled in the study. All entries on the eCRF must be supported by source data unless the 
eCRF is considered source data. All source data will be kept according to all applicable 
regulatory requirements. Source data must be completed legibly for each subject enrolled into 
the study and signed by the investigator (or delegate). 
The subject SC Infusion Diary will be completed by the subject at home. At each site visit, 
the Treating Physician (or delegate) will review entries with the subject before recording 
them in the eCRF. 
An electronic clinical outcomes assessment (eCOA) solution will be used by the subjects 
and physicians. 
The eCOA solution is provided as a means to capture electronic source data in a controlled 
and consistent way, and to provide access to these source data for Treating Physician and 
Evaluating Physician. The system also allows the subject's health status to be remotely 
monitored during the study. The data residing in the eCOA system provider’s database are 
considered the source, and are under the control of the Treating Physician and Evaluating 
Physician at all times. 

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The eCOA system provider will transfer a copy of the data across to CSLB’s clinical data 
warehouse at a predefined frequency via a secure data channel for systematic review by the 
CSL clinical team. The eCOA vendor engaged for this study is responsible for providing a 
solution that conforms to all pertinent regulations. The solution is not in any way intended as 
a substitute for normal medical care of the subject. The vendor provides the service of 
hosting of the eCOA data on behalf of the site, until such a time as the site is in receipt of a 
certified archive copy of all eCOA data relating to subjects at that site and has confirmed it is 
readable. 
13.5.2 Data Quality Assurance 
Data generated throughout the study will be monitored and the eCRFs checked against the 
subject/source (eCOA, IRT, eCRF, etc) for completeness and accuracy. The Treating 
Physician/Evaluating Physician (or delegate) must provide direct access to source data 
documents. CSLB’s study monitor (or delegate) will perform this function. 
The following data are considered source data with regard to Clinician Reported Outcomes: 
• Entries recorded directly on a tablet (eCOA) at site (eg, EULAR/ACR 
Classification Criteria, Physician Global Damage Assessment, MMT-8, MDAAT, 
CDASI, Timed Up and Go) 
• Entries recorded directly on paper (eg, medical history, IgG treatment history, 
demographics, physical examination, AEs, body weight, vital signs, urine 
pregnancy test, efficacy status notes) 
The site personnel can request, approve and/or execute a data correction to all aspects of the 
Clinician Reported Outcomes data pursuant to the applicable processes. Changes will be 
processed as per the data management procedures/plan created for the database where the 
information is first stored electronically. 
The following data are considered source data with regard to Patient Reported Outcomes: 
• Entries recorded by the subject directly on the SC Infusion Diary 
• Entries recorded by the subject directly on a tablet (eCOA) at site (eg, Patient 
Global Activity Assessment, HAQ, 5-D Itch [Pruritus] Score, EQ-5D-5L, 
WPAI-GH, TSQM-9) 

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Site personnel can request, approve, and/or execute a correction or deletion of subject 
metadata (any data point not directly entered in the device by the subject) pursuant to the 
applicable processes. No other modification of the subjects’ directly entered data will be 
allowed. 
It is the expectation that, for all executed and/or requested changes to eCOA data, appropriate 
supplemental source documentation will be maintained to support said changes and that this 
documentation can be made available for review upon request. 
Following completion of eCRF pages and entry of the data into a database, the data will be 
checked electronically and manually for consistency and plausibility. Queries will be 
generated for questionable data and clarification sought from the investigator. When possible, 
queries will be raised within the appropriate systems. These data queries must be resolved in 
a timely manner by the investigator (or delegate). 
13.5.3 Record Retention 
The investigator must follow the principles for record retention outlined in the Clinical Trial 
Research Agreement. An investigator study file prepared by CSLB (or delegate), containing 
all applicable documents for use at the study site, will be made available to the investigator 
before the start of the study. All study documentation and materials maintained in the 
investigator study file must be kept in conformance with applicable national laws and 
regulations. 
All study documentation and materials maintained in the investigator study file at the study 
site must be available for inspection by CSLB’s study monitor (or delegate) to determine that 
all required documentation is present and correct. 
The study may be audited or inspected by qualified delegates from CSLB or a competent 
health authority. 
Following completion of the study, the investigator is responsible for archiving the 
investigator’s study file, the subject’s records and the source data according to applicable 
regulatory requirements. 
13.6 Study and Site Closure 
CSLB reserves the right to prematurely discontinue or suspend the study either at a particular 
site or at all study sites at any time and for any reason. If such action is taken, the CSLB 
Study Monitor (or delegate) will discuss this with the investigator at each study site at that 

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time and notify the investigators (for Japan sites only: the heads of the medical institutes by 
the investigators) in writing. If the study is suspended or terminated for safety reasons, all 
investigators (for Japan sites only: the heads of the medical institutes as well as the 
investigators) and the relevant regulatory agencies will be immediately notified of the action 
as well as the reason for the suspension/termination. The investigator (for Japan sites only: 
the head of the medical institute) at each study site will advise their IRB/IEC overseeing the 
study of the suspension/termination. 
13.7 Clinical Study Report 
Clinical study report(s) will be written after the completion of the study. CSLB or its agent 
will write the report(s) in consultation with the investigator or, if applicable, a nominated 
coordinating investigator (or delegate). CSLB requires that the coordinating investigator will 
sign the clinical study report(s). 
Progress reports may be provided to the relevant regulatory bodies in accordance with their 
requirements. 
13.8 Use of Data and Publications 
The rights and obligations of investigators and CSLB concerning any formal presentation or 
publication of data collected as a direct or indirect result of this study will be addressed 
specifically in the Clinical Trial Research Agreement for the study. 

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14 References 
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Anh-Tu Hoa S, Hudson M. Critical review of the role of intravenous immunoglobulins in 
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CSL Behring. Human Immunoglobulin G, 20% Liquid (IgPro20, Hizentra®) Investigator’s 
Brochure, version 11. 04 December 2019. 

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Dermatol. 2010;162(3):669-673. 
 

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15 
Appendices 
Instructions for assessments included in the Appendices will be provided in a manual for use 
at the study site. 

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Signature of Principal Investigator 
 
Study Title: 
A Study to Evaluate the Efficacy, Safety, and Pharmacokinetics of 
IgPro20 (Subcutaneous Immunoglobulin, Hizentra®) in Adults 
with Dermatomyositis (DM) – The RECLAIIM Study 
Protocol 
Number: 
IgPro20_3007 
Site Number: 
 
I have read the Clinical Study Protocol, dated 20 October 2022, titled A Study to Evaluate 
the Efficacy, Safety, and Pharmacokinetics of IgPro20 (Subcutaneous Immunoglobulin, 
Hizentra®) in Adults with Dermatomyositis (DM) – The RECLAIIM Study. 
By signing this Clinical Study Protocol, I agree to conduct the clinical study, after approval 
by an Institutional Review Board or Independent Ethics Committee (as appropriate), in 
accordance with the Clinical Study Protocol, the standards of Good Clinical Practice (as 
defined by the International Conference on Harmonisation) and applicable regulatory 
requirements. 
Changes to the Clinical Study Protocol will only be implemented after written approval is 
received from CSL Behring and the Institutional Review Board or Independent Ethics 
Committee (as appropriate) with the exception of medical emergencies. 
I will ensure that study staff fully understand and follow the Clinical Study Protocol. 
 
 
 
(Signature) 
 
 
Date (DD MMM YYYY) 
(Printed name) 
 
 
 
(Title) 
 
 
 

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EULAR/ACR Classification Criteria for Idiopathic 
Inflammatory Myopathies 
 
To be enrolled subject must have a minimum aggregate score of 5.5 without and 6.7 with muscle biopsy 

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ACR = American College of Rheumatology; ADM = amyopathic dermatomyositis, DM = dermatomyositis; 
EULAR = European League Against Rheumatism; IBM = inclusion body myositis, IIM = idiopathic 
inflammatory myopathies; IMNM = immune-mediated necrotizing myopathy; JDM = juvenile dermatomyositis; 
PM = polymyositis. 
Source: Lundberg et al, 2017. 

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Physician Global Damage Assessment 
Physician Global Disease Damage Assessment 
 
Disease Damage is defined as persistent changes in anatomy, pathology, physiology, or 
function, such as fibrosis, scarring, or atrophy, resulting from any cause (including prior 
treatment) since the onset of the myositis. Features of damage are ascertained by clinical 
assessment and must be present for at least 6 months (or the pathology that led to the 
feature must have been present for at least 6 months) despite immunosuppressive or other 
therapy, including exercise and rehabilitation. The global assessment of disease damage is 
to be judged from all the information available to you today including the patient’s 
appearance, history, physical examination, diagnostic laboratory testing and your resultant 
medical therapy. 
 
Please rate your global (overall) assessment of the current myositis disease damage by 
drawing a vertical mark on the 10-cm. line according to the following scale: left end of 
line = no evidence of disease activity, midpoint of line = moderate disease activity, and 
right end of line = extreme or maximum disease. 
 
 
 
 
 
 
No evidence of  
Extremely severe 
disease damage 
 disease damage 
 
 
 
 
 
Also rate global disease damage on a 5-point Likert scale: 
  
0 = none 
  
1 = mild damage 
  
2 = moderate damage 
  
3 = severe damage 
  
4 = extremely severe damage 
 
 
 
 
 
 
 
 
IMACs form 09: Physician Global Damage Assessment 
 
 
Assessment is only used for eligibility, exclusion criterion # 3 
 

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Myositis Disease Activity Assessment Tool (MDAAT) 
 

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Extramuscular global and global disease activity will be 2 of 6 CSMs used for TIS and DOW calculations  
IMACS FORM 7a: Myositis Disease Activity Assessment Tool (MDAAT) 
 

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Patient Global Activity Assessment 
 
Your myositis is the result of the combined effects of many disease processes. One of 
these is disease activity, which is active inflammation in your muscles, skin, joints, 
intestines, heart, lungs or other parts of your body, which can improve when treated with 
medicines. 
 
 
 
1. Considering all the ways that myositis affects you, please rate the overall activity of 
your disease today by placing a mark on the line below. 
 
 
 
  
 
 
 
No evidence of 
disease activity 
Extremely active or  
severe disease activity 
 
 
 
 
 
 
 
 
 
 
IMACS FORM 03: Patient Global Activity Assessment 
 

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Manual Muscle Testing Scoring Sheet (MMT-8) 
 
Muscle Groups 
Right (0 – 10) 
Left (0 – 10) 
Axial (0 – 10) 
 
 
 
 
Sitting 
Deltoid middle 
0-10 
0-10 
X 
Biceps brachii 
0-10 
0-10 
X 
Wrist Extensors 
0-10 
0-10 
X 
Quadriceps femoris 
0-10 
0-10 
X 
Ankle dorsiflexors 
0-10 
0-10 
X 
 
 
 
 
Supine 
 
 
 
Neck Flexors 
X 
X 
0-10 
 
 
 
 
Sidelying 
 
 
 
Gluteus medius 
0-10 
0-10 
X 
 
 
 
 
Prone 
 
 
 
Gluteus maximus 
0-10 
0-10 
X 
 
 
MMT-8 score (0 – 150) 
0-70 
0-70 
0-10 
 
Muscle groups should be performed in the order listed in table. Muscle groups are tested 
bilaterally, except neck flexors (axial); Total MMT-8 score: 0 – 150 potential range. 
 
 
 
 
 
IMACS Form 04: Manual Muscle Testing Scoring Sheet 
Modified for MMT-8 evaluation, ie, removal of muscle groups associated with MMT26 
(score range 0 – 260) 

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Health Assessment Questionnaire (HAQ) 
Please check the response which best describes your usual abilities 
OVER THE PAST WEEK: 
 
Without ANY 
difficulty 
With SOME 
difficulty 
With MUCH 
difficulty 
UNABLE to 
do 
DRESSING & 
GROOMING 
Are you able to: 
 
 
 
 
Dress yourself, including 
tying shoelaces, and 
doing buttons? 
 
 
 
 
Shampoo your hair? 
 
 
 
 
ARISING 
Are you able to: 
 
 
 
 
Stand up from a straight 
chair? 
 
 
 
 
Get in and out of bed? 
 
 
 
 
EATING 
Are you able to: 
 
 
 
 
Cut your meat? 
 
 
 
 
Lift a full cup or glass to 
your mouth? 
 
 
 
 
Open a milk carton? 
 
 
 
 
WALKING 
Are you able to: 
 
 
 
 
Walk outdoors on flat 
ground 
 
 
 
 
Climb up five steps? 
 
 
 
 
Please check any AIDS OR DEVICES that you usually use for any if these activities: 
      Cane 
     Devices used for dressing (button hook, zipper pull, shoe horn, etc.) 
      Walker 
      Special or built up utensils 
      Crutches 
      Special or built up chair 
      Wheelchair 
      Other (specify:  
) 

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Please check any categories for which you usually need HELP FROM ANOTHER PERSON: 
      Dressing and Grooming 
      Eating 
      Arising 
      Walking 
Please check the response which best describes your usual abilities 
OVER THE PAST WEEK: 
 
Without ANY 
difficulty 
With SOME 
difficulty 
With MUCH 
difficulty 
UNABLE to 
do 
HYGIENE 
Are you able to: 
 
 
 
 
Wash and dry your body? 
 
 
 
 
Take a tub bath 
 
 
 
 
Get on and off the toilet 
 
 
 
 
REACH 
Are you able to: 
 
 
 
 
Reach and get down a 
5-pound object (such as a 
bag of sugar) from just 
above your head? 
 
 
 
 
Bend down to pick up 
clothing from floor? 
 
 
 
 
GRIP 
Are you able to: 
 
 
 
 
Open car doors? 
 
 
 
 
Open jars which have 
been previously opened? 
 
 
 
 
Turn faucets on and off? 
 
 
 
 
ACTIVITIES 
Are you able to: 
 
 
 
 
Run errands and shop? 
 
 
 
 
Get in and out of a car? 
 
 
 
 
Do chores such as 
vacuuming or yardwork? 
 
 
 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 170 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
Please check any AIDS or DEVICES that you usually use for any activities: 
      Raised toilet seat 
      Bathtub bar 
      Bathtub seat 
      Long-handled appliances for reach 
      Jar opener (for jars previously opened) 
      Long-handled appliances in bathroom 
 
      Other (specify: 
 
) 
Please check any categories for which you usually need HELP FROM ANOTHER PERSON: 
      Hygiene 
      Gripping and opening things 
      Reach 
      Errands and chores 
We are also interested in learning whether or not you are affected by pain because of your illness. 
How much pain have you had because of your illness IN THE PAST WEEK: 
PLACE A VERTICAL (    |    ) MARK ON THE LINE TO INDICATE THE SEVERITY 
OF PAIN 
 
NO PAIN 
SEVERE PAIN 
0 
100 
 
 
 
IMACS FORM 5a: Health Assessment Questionnaire (HAQ) 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 171 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 
Serum Levels of Muscle Enzymes 
 
Blood Laboratories 
Result 
 
Normal Range 
Creatine kinase (IU/L) 
 
 
 
Aldolase (IU/L) 
 
 
 
SGOT* (IU/L) 
 
 
 
SGPT* (IU/L) 
 
 
 
LDH (IU/L) 
 
 
 
Creatinine* (mg/dL) 
 
 
 
 
 
 
 
 
 
* Creatinine will not be used for TIS calculation, per Aggarwal 2017, p8. 
In addition, the central laboratory will use ALT (SGPT) and AST (SGOT) acronyms for these 
tests in this study. ALT = alanine aminotransferase; AST = aspartate aminotransferase; 
LDH = lactate dehydrogenase. 
IMACS FORM 06: SERUM LEVELS OF MUSCLE ENZYMES 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 172 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 
Cutaneous Dermatomyositis Disease Area and Severity 
Index (CDASI) ver02 
Select the score in each anatomical location that describes the most severely affected 
dermatomyositis-associated skin lesion 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 173 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 5-D Pruritus Scale 
 
Source: Elman et al, 2010. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 174 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 Timed Up and Go 
General 
• 
The patient should sit on a standard armchair, placing his/her back against the chair and resting 
his/her arms chair’s arms. Any assistive device used for walking should be nearby. 
• 
Regular footwear and customary walking aids should be used. 
• 
The patient should walk to a line that is 3 meters (9.8 feet) away, turn around at the line, walk 
back to the chair, and sit down. 
• 
The test ends when the patient’s buttocks touch the seat. 
• 
Patients should be instructed to use a comfortable and safe walking speed. A stopwatch should 
be used to time the test (in seconds). 
Set-up 
• 
Measure and mark a 3 meter (9.8 feet) walkway. 
• 
Place a standard height chair (seat height 46 cm, arm height 67 cm) at the beginning of the 
walkway. 
Patient Instructions  
• 
Instruct the patient to sit on the chair and place his/her back against the chair and rest his/her 
arms chair’s arms. 
• 
The upper extremities should not be on the assistive device (if used for walking), but it should be 
nearby. 
• 
Demonstrate the test to the patient. 
• 
When the patient is ready, say “Go”. 
• 
The stopwatch should start when you say go, and should be stopped with the patient’s buttocks 
touch the seat. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 175 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
Timed Up and Go Testing Form 
Name:___________________________________________________________  
Assistive Device and/or Bracing Used:__________________________________  
Date:_______________ TUG Time:___________  
Date:_______________ TUG Time:___________  
Date:_______________ TUG Time:___________  
Date:_______________ TUG Time:___________  
Date:_______________ TUG Time:___________  
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Source: Podsiadlo and Richardson, 1991. 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 176 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 EuroQol 5-Dimension Questionnaire (EQ-5D-5L) 
Under Each heading, please tick ONE box that best describes your health TODAY. 
 
Mobility 
 
I have no problems in walking about 
  
I have slight problems in walking about 
  
I have moderate problems in walking about 
  
I have severe problems in walking about 
  
I am unable to walk about 
  
Self-Care 
I have no problems washing or dressing myself 
  
I have slight problems washing or dressing myself  
  
I have moderate problems washing or dressing myself 
  
I have severe problems washing or dressing myself 
  
I am unable to wash or dress myself 
  
Usual Activities (eg, work, study, housework, family or leisure activities) 
I have no problems doing my usual activities 
  
I have slight problems doing my usual activities 
  
I have moderate problems doing my usual activities 
  
I have severe problems doing my usual activities 
  
I am unable to do my usual activities 
  
Pain/Discomfort 
I have no pain or discomfort 
  
I have slight pain or discomfort 
  
I have moderate pain or discomfort 
  
I have severe pain or discomfort 
  
I have extreme pain or discomfort 
  

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 177 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
Anxiety/Depression 
I am not anxious or depressed 
  
I am slightly anxious or depressed 
  
I am moderately anxious or depressed 
  
I am severely anxious or depressed 
  
I am extremely anxious or depressed 
  
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 178 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 
 
 
© 2009 EuroQol Research Foundation. EQ-5D™ is a trade mark of the EuroQol Research Foundation. 
UK (English) v1.1 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 179 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 Work Productivity and Activity Impairment Questionnaire: 
General Health v2.0 (WPAI-GH) 
 

CSL Behring 
Study Number: 
IgPro20_3007 
 
Study Product: 
IgPro20  
 
Protocol version: Amendment 4 
 
Page 180 of 186 
Date: 20 October 2022 
Confidential 
CS-SOP-04-T02 Version 2.0 
 
 
Effective Date: Week commencing 25-June-2018 
 
 
 
 
 
 
 
 
 
 
 
 
 
Source: Reilly et al, 1993. 

C S L Be hri n g  
St u d y N u m ber:  I g Pr o 2 0 _ 3 0 0 7 
 
St u d y Pr o d uct:  I g Pr o 2 0  
 
Pr ot oc ol versi o n: A me n d me nt  4 
 
Pa ge 1 8 1  of 1 8 6  
Date: 2 0 Oct o ber 2 0 2 2  
C o nfi de ntial  
C S -S O P -0 4 -T 0 2 Versi o n 2. 0  
 
 
Effecti ve Date: Wee k c o m me nci n g 2 5-J u ne-2 0 1 8  
 
 A b bre vi ate d Tre at me nt S atisf acti o n Q uesti o n n aire f or 
Me dic ati o n ( T S Q M -9)  
 
I nstr ucti o ns: Ple ase t a ke s o me ti me t o t hi n k a b o ut y o ur le vel of s atisf acti o n or diss atisf acti o n 
wit h t he me dic ati o n y o u are t a ki n g i n t his cli nic al st u d y. We are i ntereste d i n y o ur e v al u ati o n of 
t he effecti ve ness a n d c o n ve nie nce of t he me dic ati o n o ver t he l ast t w o t o t hree wee ks, or si nce 
y o u l ast use d it. F or e ac h q uesti o n, ple ase pl ace a si n gle c hec k m ar k ne xt t o t he res p o nse t h at 
m ost cl osel y c orres p o n ds t o y o ur o w n e x perie nces.  
1. H o w s atisfi e d or diss atisfi e d ar e y o u wit h t h e a bilit y of t h e m e dic ati o n t o pr e v e nt or tr e at 
y o ur c o n diti o n?  
 
E xtre mel y Dissatisfie d  
1 
 
Ver y Dissatisfie d  
2 
 
Dissatisfie d  
3 
 
S o me w hat Satisfie d  
4 
 
Satisfie d  
5 
 
Ver y Satisfie d  
6 
 
E xtre mel y Satisfie d  
7 
 
2. H o w s atisfi e d or diss atisfi e d ar e y o u wit h t h e w a y t h e m e dic ati o n r eli e v es y o ur s y m pt o ms?  
 
E xtre mel y  Dissatisfie d  
1 
 
Ver y Dissatisfie d  
2 
 
Dissatisfie d  
3 
 
S o me w hat Satisfie d  
4 
 
Satisfie d  
5 
 
Ver y Satisfie d  
6 
 
E xtre mel y Satisfie d  
7 
 
3 . H o w s atisfi e d or diss atisfi e d ar e y o u wit h t h e a m o u nt of ti m e it t a k es t h e m e dic ati o n t o st art 
w or ki n g?  
 
E xtre mel y Dissatisfie d  
1 
 
Ver y Dissatisfie d  
2 
 
Dissatisfie d  
3 
 
S o me w hat Satisfie d  
4 
 
Satisfie d  
5 
 
Ver y Satisfie d  
6 
 
E xtre mel y Satisfie d  
7 
 

C S L Be hri n g  
St u d y N u m ber:  I g Pr o 2 0 _ 3 0 0 7 
 
St u d y Pr o d uct:  I g Pr o 2 0  
 
Pr ot oc ol versi o n: A me n d me nt  4 
 
Pa ge 1 8 2  of 1 8 6  
Date: 2 0 Oct o ber 2 0 2 2  
C o nfi de ntial  
C S -S O P -0 4 -T 0 2 Versi o n 2. 0  
 
 
Effecti ve Date: Wee k c o m me nci n g 2 5-J u ne-2 0 1 8  
 
4. H o w e as y or diffic ult is it t o us e t h e m e dic ati o n i n its c urr e nt f or m?  
 
E xtre mel y Diffic ult  
1 
 
Ver y Diffic ult  
2 
 
Diffic ult  
3 
  
S o me w hat Eas y  
4 
 
Eas y  
5 
  
Ver y Eas y  
6 
  
E xtre mel y Eas y  
7 
 
5. H o w e as y or diffic ult is it t o pl a n w h e n y o u will us e  t h e m e dic ati o n e ac h ti m e? 
 
E xtre mel y Diffic ult  
1 
 
Ver y Diffic ult  
2 
 
Diffic ult  
3 
  
S o me w hat Eas y  
4 
 
Eas y  
5 
  
Ver y Eas y  
6 
  
E xtre mel y Eas y  
7 
 
6. H o w c o n v e ni e nt or i nc o n v e ni e nt is it t o t a k e t h e m e dic ati o n as i nstr uct e d?  
 
E xtre mel y I nc o n ve nie nt 
1 
  
Ver y I nc o n ve nie nt  
2 
  
I nc o n ve nie nt  
3 
  
S o me w hat C o n ve nie nt  
4 
  
C o n ve nie nt  
5 
  
Ver y C o n ve nie nt  
6 
 
E xtre mel y C o n ve nie nt  
7 
 
7. O v er all, h o w c o nfi d e nt ar e y o u t h at t a ki n g t his m e dic ati o n is a g o o d t hi n g f or y o u?  
 
N ot at All C o nfi de nt  
1 
 
A Little C o nfi de nt  
2 
  
S o me w hat C o nfi de nt 
3 
  
Ver y C o nfi de nt  
4 
  
E xtre mel y C o nfi de nt  
5 
 
8. H o w c ert ai n ar e y o u t h at t h e g o o d t hi n gs a b o ut y o ur m e dic ati o n o ut w ei g h t h e b a d t hi n gs?  
 
N ot at All Certai n  
1 
 
A Little Certai n  
2 
 
S o me w hat Certai n  
3 
  
Ver y Certai n  
4 
 
E xtre mel y Certai n  
5 
 

C S L Be hri n g  
St u d y N u m ber:  I g Pr o 2 0 _ 3 0 0 7 
 
St u d y Pr o d uct:  I g Pr o 2 0  
 
Pr ot oc ol versi o n: A me n d me nt  4 
 
Pa ge 1 8 3  of 1 8 6  
Date: 2 0 Oct o ber 2 0 2 2  
C o nfi de ntial  
C S -S O P -0 4 -T 0 2 Versi o n 2. 0  
 
 
Effecti ve Date: Wee k c o m me nci n g 2 5-J u ne-2 0 1 8  
 
9. T a ki n g all t hi n gs i nt o acc o u nt, h o w s atisfi e d or diss atisfi e d ar e y o u wit h t his m e dic ati o n?  
 
E xtre mel y Dissatisfie d  
1 
 
Ver y Dissatisfie d  
2 
 
Dissatisfie d  
3 
 
S o me w hat Satisfie d  
4 
 
Satisfi e d  
5 
 
Ver y Satisfie d  
6 
 
E xtre mel y Satisfie d  
7 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
S o urce: B har mal  et al, 2 0 0 9. 
 
Q ui ntiles ©  2 0 0 7 

Version: 2.0
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Print Date: 31-Oct-2022 04:57:37 GMT-04:00
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IgPro20_3007 - Protocol Amendment - 4 - 20Oct2022
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Approved-
28-Oct-2022 06:56:57
Approved-
28-Oct-2022 07:09:28
Approved-
28-Oct-2022 07:20:07
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28-Oct-2022 07:53:30
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