Incidence Probability of Progression to Progressive Fibrosing Interstitial Lung Diseases and Status of Management and Treatments in Patients With Fibrosing Interstitial Lung Diseases Other Than Idiopathic Pulmonary Fibrosis in Japan
Conditions: Lung Diseases, Interstitial
Sponsor: Boehringer Ingelheim

Non-Interventional Study (NIS) Protocol
Document Number: 
NA
BI Study Number: 
1199-0523
BI Investigational
Product(s):
Not applicable
Title:
Incidence probability of progression to progressive fibrosing interstitial 
lung diseases and status of management and treatments in patients 
with fibrosing interstitial lung diseases other than idiopathic pulmonary 
fibrosis in Japan
Brief lay title: 
Study of progression to PF-ILD incidence/management and treatment
Protocol version 
identifier: 
1.4 
Date of last version of 
protocol:
29 Nov. 2023
PASS:
Not applicable
EU PAS register 
number:
Not applicable
Active substance:
Not applicable
Medicinal product:
Not applicable
Product reference:
Not applicable
Procedure number:
Not applicable
Marketing 
authorisation 
holder(s):
Joint PASS:
Not applicable
Research question 
and objectives:
PRIMARY OBJECTIVE 
To investigate the incidence probability of progression to PF-ILDs in 
patients with fibrosing ILD other than IPF in real-world setting in Japan
SECONDARY OBJECTIVE
To investigate the characteristics of procedures for management and 
treatment in patients with fibrosing ILD other than IPF in real-world 
setting in Japan
Country(-ies) of 
study:
Japan
Author: 
Co-Author: 

Marketing 
authorisation 
holder(s): 
In case of PASS, add:
MAH contact person:
Not applicable
In case of PASS, add: 
Not applicable
In case of PASS, add: 
Not applicable
Date: 
29 Nov. 2023
Page 1 of 41
Proprietary confidential information
© 2024 Boehringer Ingelheim Group of companies. All rights reserved.
This document may not - in full or in part - be passed on, reproduced, published or otherwise used 
without prior written permission

1. 
 TABLE OF CONTENTS
1.
TABLE OF CONTENTS...........................................................................................3
2.
LIST OF ABBREVIATIONS......................................................................................4
3.
RESPONSIBLE PARTIES .......................................................................................5
4.
ABSTRACT..............................................................................................................5
5.
AMENDMENTS AND UPDATES.............................................................................8
6.
MILESTONES..........................................................................................................9
7.
RATIONALE AND BACKGROUND .......................................................................10
8.
RESEARCH QUESTION AND OBJECTIVES........................................................11
8.1
PriMary Objective ..............................................................................................11
8.2
Secondary objective ..........................................................................................11
9.
RESEARCH METHODS ........................................................................................11
9.1
STUDY DESIGN................................................................................................11
9.2
SETTING ...........................................................................................................11
9.3
VARIABLES.......................................................................................................13
9.4
DATA SOURCES ..............................................................................................15
9.5
STUDY SIZE......................................................................................................15
9.6
DATA MANAGEMENT ......................................................................................15
9.7
DATA ANALYSIS...............................................................................................15
9.8
QUALITY CONTROL.........................................................................................17
9.9
LIMITATIONS OF THE RESEARCH METHODS ..............................................17
9.10
OTHER ASPECTS ............................................................................................17
10.
PROTECTION OF HUMAN SUBJECTS................................................................17
11.
MANAGEMENT AND REPORTING OF ADVERSE EVENTS/ADVERSE 
REACTIONS..........................................................................................................17
12.
PLANS FOR DISSEMINATING AND COMMUNICATING STUDY RESULTS ......17
13.
REFERENCES ......................................................................................................18
13.1
PUBLISHED REFERENCES.............................................................................18
13.2
UNPUBLISHED REFERENCES........................................................................19
ANNEX 1. LIST OF STAND-ALONE DOCUMENTS......................................................20
ANNEX 2.  INTERSTITIAL LUNG DISEASE PROGRESSION PROXIES.....................21
ANNEX 3. Code for Treatment, Management & clnical examination .............................23
ANNEX 4. Code for Comorbidity and medical history ....................................................24
ANNEX 5.  ALGORITHMS FOR DEFINING uderlying diseases....................................25
ANNEX 6.  Information of the code to define fibrosing ILD and underlying diseases.....27
ANNEX 7. REVIEWERS AND APPROVAL SIGNATURES ...........................................40

2.
LIST OF ABBREVIATIONS
COPD
Chronic Obstructive Pulmonary Disease
CTD-ILD
Connective Tissue Disease-Interstitial Lung Disease
DPC
Diagnosis Procedure Combination
HP
Hypersensitivity Pneumonitis
HRCT/CT
High-Resolution Computed Tomography/Computed Tomography
ILD
Interstitial Lung Disease
IHD
Instant Health Data
IIPs
Idiopathic Interstitial Pneumonias
iNSIP
Idiopathic Nonspecific Interstitial Pneumonia
IPF
Idiopathic Pulmonary Fibrosis
KL-6
Krebs von den Lungen-6
MCTD
Mixed Connective Tissue disease
MDV
Medical Data Vision
PF-ILD
Progressive Fibrosing Interstitial Lung Disease
PM/DM-ILD 
Polymyositis/Dermatomyositis-Interstitial Lung Disease
RA-ILD
Rheumatoid Arthritis-Interstitial Lung Disease
SAS
Statistical Analysis System
SLE-ILD
Systemic Lupus Erythematosus-Interstitial Lung Disease
SP-D
Surfactant Protein D
SSc-ILD
Systemic Sclerosis-Interstitial Lung Disease

Name of company:
Boehringer Ingelheim Co., Ltd.
Name 
of 
finished 
medicinal 
product: Not applicable
Name of active ingredient:
Protocol date:
Study
number:
Version/Revision:
Version/Revision date:
DD Month YYYY
1.4
29 Nov. 2023
Title of study:
Incidence probability of progression to progressive fibrosing interstitial lung 
diseases and status of management and treatments in patients with 
fibrosing interstitial lung diseases other than idiopathic pulmonary fibrosis 
in Japan
Rationale 
and 
background:
While idiopathic pulmonary fibrosis (IPF) is the best-known progressive 
fibrosing interstitial lung disease (PF-ILD), there is a group of patients with 
different underlying clinical interstitial lung disease (ILD) diagnoses other 
than IPF who develop a progressive fibrosing phenotype during the course 
of their disease.
There is minimal literature available to estimate the proportion of patients 
who develop PF-ILDs in patients with fibrosing ILD other than IPF and, the 
current management and treatment for these diseases are not well-known 
in Japan. 
These information are keys to gaining a better understanding of the size of 
this population with high unmet need and for physicians, patients and other 
stakeholders to optimize allocation of resources, diagnostic assessment 
and management of this patient population.
Research 
question 
and 
objectives:
PRIMARY OBJECTIVE 
To investigate the incidence probability of progression to PF-ILDs in 
patients with fibrosing ILD other than IPF in real-world setting in Japan
SECONDARY OBJECTIVE
To investigate the characteristics of procedures for management and 
treatment in patients with fibrosing ILD other than IPF in real-world setting 
in Japan
Study design:
This is a retrospective cohort study using the database from Medical Data 
Vision (MDV).
Data from 01-Jan-2012 to 28-May-2020 will be used. It will be considered 
that each patient is observable from his/her first encounter to his/her last 
encounter in the database. The patients with fibrosing ILD will be identified 
in the period from 01-Jan-2013 to 6 months before 28-May-2020. Each 
patient who meets all the inclusion criteria and none of the exclusion criteria 
will be followed up from the day after the index date until the end of study 
period (i.e., 28-May-2020), the last encounter in the MDV database, in-
hospital death, whichever occurs first.

Population:
Patients who meet all of the inclusion criteria and do not meet any of the 
exclusion criteria will be included in the study population.
Inclusion Criteria:
1) Patients diagnosed with at least two fibrosing ILD codes on different 
dates in the patient identification period from 01-Jan-2013 to 6
months before 28-May-2020  
2) Patients aged 18 years and older on the index date  
3) Patients for whom data for the 12 months prior to the index date can 
be extracted as baseline data
Exclusion Criteria:
1)
Patients grouped into the underlying disease of IPF
2)
Patients who have met PF-ILD progression criteria during the 
baseline period 
Note: only diagnosis records without ‘suspect flag’ are to be considered for 
evaluating diagnosis related in/excl criteria”
Variables:
The following information will be extracted from MDV database in this 
study; sex, age, comorbidity and medical history of interest, Underlying 
disease 
(pre-specified 
ILD), 
clinical 
examination, 
treatment
and
management of interest
Data sources:
The MDV database consists of anonymized administrative claims and 
Diagnosis Procedure Combination (DPC) data for inpatient and outpatient 
settings from acute hospitals (mainly considered as advanced treatment 
hospitals). The database includes >35 million patients from >400 hospitals. 
Patients in Japan have free access to health care facilities and data from 
the other hospitals not included in the database are unavailable.
Study size:
The planned sample size is not based on the formal statistical hypothesis 
testing and statistical precision. Because the MDV database consists of 
>35 million patients, it is expected that the sufficient number of non-IPF 
fibrosing ILD patients will be included in the study.
Data analysis:
Primary outcome 
Incidence probability of progression to PF-ILDs at 6, 12, 18, and 24 
months after the index date.
Cumulative incidence probability will be estimated by using Kaplan-Meier 
method. 
Secondary outcomes
Management and treatment during follow-up period
Frequency of patients with management and treatment of interest during
follow-up period will be analyzed descriptively. 
Milestones:
-
Final protocol: 28 Mar 2023
- 
IRB approval: Prior to study start
- 
Start of data analysis: After the IRB approval
- 
Final results: Q2 2023 
- 
Final study report:  Q3 2023 
- 
Publications: Q3, 2023

5.
AMENDMENTS AND UPDATES
Number
Date
Section 
of 
study 
protocol
Amendment 
or 
update
Reason
1 
28 March 2023
9.2.2.1 
Exclusion 
Criteria
2)Patients 
who 
have met PF-ILD 
progression 
criteria during the 
baseline 
period 
and 3) delete
To further clarify 
and 
simplify 
the 
exclusion criteria 
2 
21 Sep. 2023
ANNEX 
2 
and 3
Add 
Generic 
names 
and 
correct 
ATC 
codes
To correct the drug 
codes
3 
21 Sep. 2023
ANNEX 
2 
and 3
*transdermal 
patch, sublingual 
agent, 
Transmucosal 
agent, 
suppository 
are 
excluded
To 
reflect 
the
updated
definition 
of opioid use
4 
10 Nov. 2023
ANNEX 
2 
and 3
K514-4 
K514-6 
To correct codes for 
lung transplant
5 
29 Nov. 2023
ANNEX 
2 
and 3
N02A0
To correct the drug 
code for opioid

6.
MILESTONES
Milestone
Planned Date
IRB/IEC approval
Prior to study start
Start of Data Analysis
After the IRB approval
Interim Report
NA
Final report of study results:
Q3 2023

7.
RATIONALE AND BACKGROUND
The term interstitial lung disease (ILD) encompasses a large group of over 200 
pulmonary disorders. While idiopathic pulmonary fibrosis (IPF) is the best-known 
progressive fibrosing ILD (PF-ILD), there is a group of patients with different underlying 
clinical ILD diagnoses other than IPF who develop a progressive fibrosing phenotype during 
the course of their disease (e.g., idiopathic interstitial pneumonias [IIPs] such as  idiopathic 
nonspecific interstitial pneumonia [iNSIP] and unclassifiable IIP; hypersensitivity 
pneumonitis [HP]; autoimmune interstitial lung disease [connective tissue disease-ILDs
(CTD-ILD) such as rheumatoid arthritis-ILD (RA-ILD), systemic sclerosis-ILD (SSc-ILD) and 
anti-synthetase syndrome]; Sarcoidosis; Pneumoconiosis/exposure related ILD) [P17-
10582, P18-04729, P19-01738, R19-0854]. These patients demonstrate a number of 
similarities to patients with IPF, with their disease being defined by the presence of 
progressive pulmonary fibrosis, worsening respiratory symptoms, declining lung function 
despite immunomodulatory therapies and, ultimately, early mortality.
Although there had been no indicated therapies for patients with chronic fibrosing 
ILDs with a progressive phenotype (other than IPF), Nintedanib has just been approved for 
SSc-ILD in the end of Dec. 2019 based on the results of the SENSCIS trial (NCT02597933) 
[P19-04387] and for PF-ILDs in the end of May 2020 in Japan based on the results of the 
INBUILD trial (NCT02999178) [P19-08802, P20-02333]. 
There is minimal literature available to estimate the proportion of patients who 
develop PF-ILDs in patients with fibrosing ILD other than IPF in Japan. In addition, the 
current management and treatment for these diseases are not well-known. These 
information are keys to gaining a better understanding of the size of this population with high 
unmet need and for physicians, patients and other stakeholders to optimize allocation of 
resources, diagnostic assessment and management of this patient population.
Therefore, this study will use the database from Medical Data Vision (MDV) to 
investigate the incidence probability of progression to PF-ILDs according to each co-existing 
underlying diseases and the characteristics of procedures for management and treatment 
in patients with fibrosing ILD other than IPF. 

9.2.2
Study population
9.2.2.1
Eligibility criteria
Patients who meet all of the inclusion criteria and do not meet any of the exclusion criteria 
will be included in the study population.
Inclusion Criteria:
1) Patients diagnosed with at least two fibrosing ILD codes*1 on different dates in the 
patient identification period from 01-Jan-2013 to 6 months before 28-May-2020
2) Patients aged 18 years and older on the index date  
3) Patients for whom data for the 12 months prior to the index date can be extracted as 
baseline data
*1
International Statistical Classification of Diseases and Related Health Problems 
(ICD-10) code or disease code: J84.1, J84.9, 8844510, 8848382, 8844341,
8848278, 8836513, 8848267, 8848302, 8839362, 8848283, 8837013, 8834158,
8848245, 8840951, 8847737  
Exclusion Criteria:
1) Patients grouped into the underlying disease of IPF*1  
2) Patients who have met PF-ILD progression criteria during the baseline period  
*1
Please refer to the ANNEX 5 for algorithm to define underlying diseases
Note: only diagnosis records without ‘suspect flag’ are to be considered for evaluating 
diagnosis related in/excl criteria”

9.4
DATA SOURCES
The MDV database consists of anonymized administrative claims and DPC data for inpatient 
and outpatient settings from acute hospitals (mainly considered as advanced treatment 
hospitals). At present, the database includes >35 million patients from >400 hospitals. 
Patients in Japan have free access to health care facilities and data from the other hospitals 
not included in the database are unavailable.
9.5
STUDY SIZE
The planned sample size is not based on the formal statistical hypothesis testing and 
statistical precision. Because the MDV database consists of >35 million patients, it is 
expected that the sufficient number of non-IPF fibrosing ILD patients will be included in the 
study. 
9.6
DATA MANAGEMENT
The anonymised data received from hospitals has been checked by data managers in MDV 
to update the database. The database Boehringer Ingelheim (BI) received from MDV will be 
used.
The analyses will be performed using Instant Health Data (IHD) platform, SAS statistical 
software (version 9.3 or higher) and/or R.
9.7
DATA ANALYSIS
9.7.1
Common handling for statistical analysis
Unless otherwise specified, descriptive analysis is planned. 
The summary statistics for continuous variables are N, mean, standard deviation, minimum, 
25th percentile (Q1), median, 75th percentile (Q3), and maximum.
Tabulations of frequencies for categorical variables will include all possible categories and 
will display the number of observations in a category as well as percentage (%) relative to 
the population analysed. The precision for percentage will be one decimal point.
9.7.2
Statistical analysis  
9.7.2.1
Patient background characteristics and treatment
y Patient demographics (sex, age at the index date)
y Comorbidity and medical history during 12 months prior to the index date (included)
y Clinical examination, management, and treatment during 6 months prior to the index 
date (included)
y Duration of follow-up period, which is defined as “end of follow-up period” – “start of 
follow-up period” + 1 day
y Underlying diseases 
These will also be analysed in patients with progression to PF-ILD and those without 
progression to PF-ILD respectively. 
9.7.2.2
Primary outcome
- 
Incidence probability of progression to PF-ILDs at 6, 12, 18, and 24 months after the 
index date

Cumulative incidence probability of progression to PF-ILD at 6, 12, 18 and 24 months after 
the index date will be estimated by using Kaplan-Meier method. Greenwood’s variance 
estimate will be used for calculating 95% confidence interval. Kaplan-Meier curve will also 
be displayed.
Of the patients with progression to PF-ILD, frequency of patients meeting each criteria of 
progression to PF-ILD will be analysed descriptively. 
9.7.2.3
Secondary outcomes
- 
Management and treatment during follow-up period
Frequency of patients with management and treatment during follow-up period will be 
analyzed.
9.7.3
Subgroup analysis
The three subgroup analyses by underlying diseases defined by disease codes on the 
index date and 12-months lookback period as described in Annex 5 will be performed for 
patient background characteristics and treatment, primary outcome, and secondary 
outcomes.
<Subgroup Patterns>
Underlying Autoimmune Disease (Subgroup1): Autoimmune ILD, Non-autoimmune ILD, 
Multiple, Unknown 
ILD Category (Subgroup 2): Hypersensitive pneumonitis, Exposure-related ILD, iNSIP, 
Unclassifiable IIP, Sarcoidosis, RA-ILD, SSc-ILD, MCTD-ILD, Other autoimmune ILD, 
Other ILD, Multiple, Unknown
Pre-Specified ILD Clinical Diagnosis (Subgroup 3): Hypersensitive pneumonitis, Exposure-
related ILD, iNSIP, Unclassifiable IIP, Sarcoidosis, RA-ILD, SSc-ILD, MCTD-ILD, SLE-ILD, 
PM/DM-ILD, Sjogren ILD, Other autoimmune ILD, Other ILD, Multiple, Unknown
9.7.4
Sensitivity analysis
1) The end of data extraction period will be changed from 28-May-2020 to the latest data 
cut off. OFEV was approved for PF-ILD on 29-May-2020, and the characteristics of 
claims data before and after the above approval for new indications might not be 
different. To increase the sample size, all the analyses will be repeated with this data 
extraction period.
2) The index date will be changed from the date of the second code of fibrosing ILD to the 
date of the first code of fibrosing ILD because there might be patients who are asked to 
visit hospitals annually after the first diagnosis. All the analyses will be repeated with 
this index date. 
In addition, all the analyses will be repeated with both data extraction period and index 
date changed.
After exploring the proxy patterns among patients meeting the criteria of progression to 
PF-ILD as described in Section 9.7.2.2, a sensitivity analysis with modified progression 
criteria might be performed.  

9.8
QUALITY CONTROL
The analysis program and the data codes used in the analysis will be checked by someone 
different from the creator.
9.9
LIMITATIONS OF THE RESEARCH METHODS
The MDV database does not have pulmonary function test results and there have been no 
evidence on the validated algorithm to define fibrosing ILD patients other than IPF and 
progression to PF-ILD as events by using claims data. Therefore, the estimated incidence
in this study might be biased and the sensitivity analysis by using different algorithm is 
planned to assess the robustness of the results.
Due to the characteristics of health care system in Japan as described in Section 9.4, data 
generated in the clinics/hospitals other than hospitals included in the MDV database are not 
captured and the incidence probability and frequency might be underestimated.  
9.10
OTHER ASPECTS
9.10.1
Data quality assurance
A quality assurance audit/inspection of this study may be conducted by the sponsor or 
sponsor’s designees. The quality assurance auditor will have access to all study-related files.
9.10.2
Study records
Not applicable. (This study will not use Case Report Forms.)
9.10.3
Completion of study
Not applicable.
9.10.4
Protocol deviations
Deviations from the research protocol that may occur in this study include deviations from 
the procedures for handling databases and methods of analysis and reporting. Any 
deviations should be reported to the principal investigator by the co-investigator or contract 
research organization.
9.10.5
Compensation available to the patient in the event of study related injury
Not applicable.
10.
PROTECTION OF HUMAN SUBJECTS
The MDV data used in this study is existing information and does not involve the acquisition 
of new information. In addition, it is anonymized processed information and cannot identify 
individuals. Therefore, the protection of subjects does not apply to this study.
11.
MANAGEMENT 
AND 
REPORTING 
OF 
ADVERSE 
EVENTS/ADVERSE REACTIONS
Not applicable based on secondary use of data without any potential that any employee of 
BI or agent working on behalf of BI will access individually identifiable patient data.
12.
PLANS FOR DISSEMINATING AND COMMUNICATING STUDY 
RESULTS

The results of this research will be presented at conferences and published in peer-reviewed 
journals.
13. 
REFERENCES
13.1
PUBLISHED REFERENCES
P17-10582  Flaherty KR, Brown KK, Wells AU, et al Design of the PF-ILD trial: a double-
blind, randomised, placebo-controlled phase III trial of nintedanib in patients 
with progressive fibrosing interstitial lung disease BMJ Open Respiratory 
Research 2017;4:e000212. doi: 10.1136/bmjresp-2017-000212
 
P18-04729  Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal Victor J. What’s in a 
name? That which we call IPF, by any other name would act the same. 
European Respiratory Journal. 2018;51(5). Prevalence and characteristics of 
progressive fibrosing interstitial lung disease in a prospective registry
R11-4318   Guidelines for Good Pharmacoepidemiology Practices (GPP) (revision 2: April 
2007). http://www.pharmacoepi.org/resources/guidelines_08027.cfm (access 
date: 
13 
September 
2011) 
; 
Bethesda: 
International 
Society 
for 
Pharmacoepidemiology (ISPE) (2007)
R15-4859 
 Guidance for industry and FDA staff: best practices for conducting and 
reporting pharmacoepidemiologic safety studies using electronic healthcare 
data 
(May 
2013, 
drug 
safety). 
http://www.fda.gov/downloads/drugs/guidancecomplianceregulatoryinformati
on/guida nces/ucm243537.pdf (access date: 28 August 2015) ; U.S. 
Department of Health and Human Services, Food and Drug Administration, 
Center for Drug Evaluation and Research (CDER), Center for Biologics 
Evaluation and Research (CBER) (2013)
R15-4870]   European Medicines Agency (EMA). The ENCePP code of conduct for 
scientific 
independence 
and 
transparency 
in 
the 
conduct 
of 
pharmacoepidemiological and pharmacovigilance studies (London, 21 
November 
2011, 
EMA/929209/2011). 
http://www.encepp.eu/code_of_conduct/documents/CodeofConduct_Rev2.pd
f (access date: 28 August 2015) ; London: European Medicines Agency (EMA), 
European 
Network 
of 
Centres 
for 
Pharmacoepidemiology 
and 
Pharmacovigilance (ENCePP) (2011)
P19-01738
Cottin V, Wollin L, Fischer A, Quaresma M, Stowasser S, Harari S. Fibrosing 
interstitial lung diseases: knowns and unknowns. Eur Resp Rev 
2019;28:180100.
R19-0854
Kolb M, Vasakova M. The natural history of progressive fibrosing interstitial 
lung diseases. Respir Res (Lond) 2019;20(1):57
P19-08802
Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al, 
INBUILD Trial Investigators. Nintedanib in progressive fibrosing interstitial lung 
diseases. New England Journal of Medicine, published on September 29, 
2019, doi: 10.1056/NEJMoa1908681; 2019. p. 1718-1727.
P20-02333
Wells AU, Flaherty KR, Brown KK, Inoue Y, Devaraj A, Richeldi L, et al, 
INBUILD Trial Investigators. Nintedanib in patients with progressive fibrosing 
interstitial lung diseases - subgroup analyses by interstitial lung disease 
diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, 
parallel-group trial. Lancet Respiratory Medicine, Published Online March 5, 
2020, doi: 10.1016/S2213-2600(20)30036-9; 2020. p. 453-460.

13.2
UNPUBLISHED REFERENCES
None.

ANNEX 1. LIST OF STAND-ALONE DOCUMENTS
Number
Document 
Reference Number
Date
Title
1
Annex 1
20 February 2023
List of stand-alone documents
2 
Annex 2
20 February 2023
INTERSTITIAL LUNG DISEASE 
PROGRESSION PROXIES
3
Annex 3
20 February 2023
CODE FOR TREATMENT, 
MANAGEMENT & CLNICAL 
EXAMINATION
4 
Annex 4
20 February 2023
CODE FOR COMORBIDITY 
AND MEDICAL HISTORY
5 
Annex 5
20 February 2023
ALGORITHMS FOR DEFINING 
UDERLYING DISEASES
6 
Annex 6 
20 February 2023
INFORMATION OF THE CODE 
TO DEFINE FIBROSING ILD 
AND UNDERLYING DISEASES
7 
Annex 7 
20 February 2023
REVIEWERS AND APPROVAL 
SIGNATURES

N02A0 [Excluding 
methadone, 
buprenorphine,  
naloxonecombinations 
(Suboxone)] * 
*transdermal patch, 
sublingual agent, 
Transmucosal agent, 
suppository are excluded
lung transplant during follow-up period
K514-4 
K514-6 
QHZFODLPIRULPPXQRVXSSUHVVLYHdrugs during follow-up
period with at least two out of the three test codes** within 3 
months before new claims for immunosuppressive drugs
Rituximab
Tacrolimus 
Mycophenolate
Cyclosporine
Cyclophosphamide
Azathioprine
Tocilizumab
*: Not received in the 12 months prior to the index date
**: ձ Pulmonary function tests, ղ HRCT/CT, ճ KL-6 or SP-D 
Rituximab/ L01G1
Tacrolimus/ L04X0
Mycophenolate/ L04X0
Cyclosporine or 
(Ciclosporin, Cyclosporin) /
L04X0
Cyclophosphamide/ L01A0
Azathioprine/ L04X0
Tocilizumab/ M01C0 &
L04C0
(Generic names/ MphMRA 
ATC codes which each 
drug is under)
QHZFODLPIRURUDOFRUWLFRVWHURLGZLWKDprednisone-equivalent 
dose of >20 mg during follow-up period and with at least two out of 
the three test codes* within 3 months before new claims for oral 
corticosteroid
*: Not received in the 12 months prior to the index date
**: ձ Pulmonary function tests, ղ HRCT/CT, ճ KL-6 or SP-D
H02A2 excluding Florinef
FODLPIRU1LQWHGDQLE* during follow-up period
*: Patients meeting this criterion will be counted only if patients with 
fibrosing ILDs meet this criterion on or after 29-May-2020, because 
nintedanib was approved for PF-ILD on 29-May-2020. Even if
patients with fibrosing ILDs meet this criterion on or before 28-May-
2020, they will not be counted.
Nintedanib/ L01H9 &
R07D0
(Generic name/ MphMRA 
ATC codes which 
nintedanib is under)

ANNEX 5.  ALGORITHMS FOR DEFINING UDERLYING DISEASES
The following three subgroups will be defined to assess the distribution of underlying diseases and 
to perform subgroup analysis by underlying diseases. 
Subgroup1: Autoimmune ILD, Non-autoimmune ILD, Multiple, Unknown, IPF#
Subgroup2: Hypersensitive pneumonitis, Exposure-related ILD, iNSIP, Unclassifiable IIP, 
Sarcoidosis, RA-ILD, SSc-ILD, MCTD-ILD, Other autoimmune ILD, Other ILD, Multiple, 
Unknown, IPF#
Subgroup3: Hypersensitive pneumonitis, Exposure-related ILD, iNSIP, Unclassifiable IIP, 
Sarcoidosis, RA-ILD, SSc-ILD, MCTD-ILD, SLE-ILD, PM/DM-ILD, Sjogren ILD, Other 
autoimmune ILD, Other ILD, Multiple, Unknown, IPF#
#Note: Patients grouped into IPF are excluded from study population according to the exclusion 
criteria.
The patients will be grouped according to the following three steps. 
Step 1: Patients will be grouped based on the fibrosing-ILD code on the index date 
If two or more different (1) disease codes specified in this step 1 exist on index date 
, then subgroup1=subgorup2=subgrou3=Multiple 
else if disease code on index date=8844510, then Subrgoup1=Autoimmune ILD, subgroup2=SSc-
ILD, subgroup3=SSc-ILD
else if disease code on index date=8847737, then subgroup1=Autoimmune ILD, subgroup2=RA-
ILD, subgorup3=RA-ILD
else if disease code on index date=8848382, then subrgoup1=Autoimmune ILD, subgroup2=Other 
autoimmune ILD, subgroup3=Other autoimmune ILD 
else if disease code on index date=in(8848278, 8836513, 8844341), then subgroup1=Autoimmune 
ILD, subgroup2=Other autoimmune ILD. Subgroup3=SLE-ILD
else if disease code on index date=in(8848267, 8848302, 8839362, 8848283, 8837013), then 
subgroup1=Autoimmune ILD, subgroup2=Other autoimmune ILD, subgroup3=PM/DM ILD 
else if disease code on index date=8834158, then subgroup1=Autoimmune ILD, subgroup2=Other 
autoimmune ILD, subgroup3=Sjogren ILD 
(1)Note: The disease codes in the same conditional statement are NOT considered different. For 
example, disease codes of 8848278 and 8836513 are in the same conditional statement and these are 
not considered different disease codes. On the other hand, disease codes of 8848278 and 8848267 
are in the different conditional statement and these are considered different disease codes.
Step 2: Patients not grouped in the step 1 will be grouped based on the earliest underlying disease 
code in the lookback period and fibrosing-ILD code on the index date 
else if two or more different (2) earliest underlying disease codes specified in this step 2 exist AND 
disease code on index date=in(J84.1, J84.9), then subgroup1=subgorup2=subgrou3=Multiple 
else if earliest underlying disease code=in(D86.0, D86.1, D86.3, D86.8, D86.9) AND disease code 
on index date=in(J84.1, J84.9), then subgroup1=Non-autoimmune ILD, subgroup2=Sarcoidosis, 
subgroup3=Sarcoidosis 
else if earliest underlying disease code=in(J67.0, J67.1, J67.2, J67.3, J67.4, J67.5, J67.6, J67.7, 
J67.8, J67.9) AND disease code on index date=in(J84.1, J84.9), then subgroup1=Non-autoimmune 
ILD, subgroup2=Hypersensitive pneumonitis, subgroup3=Hypersensitive pneumonitis 
else if earliest underlying disease code=J84.0 AND disease code on index date=in(J84.1, J84.9), 
then subgroup1=Non-autoimmune ILD, subgroup2=Other ILD, subgroup3=Other ILD 
else if earliest underlying disease code=in(J60, J61, J62.8, J63.0, J63.1, J63.2, J63.3, J63.4, J63.5, 
J64, J65, J66.0, J66.1, J68.0, J68.4, J70.1, J70.3, J70.4, J70.8) AND disease code on index 
date=in(J84.1, J84.9), then subgroup1=Non-autoimmune ILD, subgroup2=Exposure-related ILD, 
subgroup3=Exposure-related ILD

Note: Disease codes of 8840951 and 8848245 are added to the coitional statement.   
else if earliest underlying disease code=in(M05.0, M05.1, M05.2, M05.3, M05.8, M05.9, M06.0, 
M06.2, M06.3, M06.4, M06.8, M06.9) AND disease code on index date=in(J84.1, J84.9, 8840951, 
8848245), then subgroup1=Autoimmune ILD, subgroup2=RA-ILD, subgroup3=RA-ILD
else if earliest underlying disease code=in(M32.1, M32.9) AND disease code on index 
date=in(J84.1, J84.9, 8840951, 8848245), then subgroup1=Autoimmune ILD, subgroup2=Other 
autoimmune ILD, subgroup3=SLE-ILD
else if earliest underlying disease code=in(M33.0, M33.1, M33.2, M33.9) AND disease code on 
index date=in(J84.1, J84.9, 8840951, 8848245), then subgroup1=Autoimmune ILD,
subgroup2=Other autoimmune ILD, subgroup3= PM/DM ILD 
else if earliest underlying disease code=in(M34.0, M34.1, M34.8, M34.9) AND disease code on 
index date=in(J84.1, J84.9, 8840951, 8848245), then subgroup1=Autoimmune ILD,
subgroup2=SSc-ILD, subgroup3=SSc-ILD
else if earliest underlying disease code=M35.0 AND disease code on index date=in(J84.1, J84.9, 
8840951, 8848245), then subgroup1=Autoimmune ILD, subgroup2=Other autoimmune ILD, 
subgroup3=Sjogren ILD
else if earliest underlying disease code=7109008 AND disease code on index date=in(J84.1, J84.9, 
8840951, 8848245), then subgroup1=Autoimmune ILD, subgroup2=MCTD-ILD,
subgroup3=MCTD-ILD
else if earliest underlying disease code=in(8841317, 8850417, 8848245, M35.2, M35.3, M35.4, 
M35.5, M35.6, M35.7, M35.8, M35.9, M30.0, M30.1, M30.2, M30.3, M30.8, M31.0, M31.1, 
M31.2, M31.3, M31.4, M31.6, M31.7, M31.8, M31.9) AND disease code on index date=in(J84.1, 
J84.9, 8840951, 8848245), then subgroup1= Autoimmune ILD, subgroup2=Other autoimmune 
ILD, subgroup3=Other autoimmune ILD 
(2)Note: The earliest underlying disease codes in the same conditional statement are NOT 
considered different. For example, earliest underlying disease codes of D86.0 and D86.1 are in the 
same conditional statement and these are not considered different earliest underlying disease codes. 
On the other hand, earliest underlying disease codes of D86.0 and J67.0 are in the different 
conditional statement and these are considered different earliest underlying disease codes. 
Step 3: Patients not grouped in the step 1 and 2 will be grouped based on the fibrosing-ILD code on 
the index date 
else if disease code on index date=in(5163005, 5150011, 8849927) AND no other fibrosing-ILD 
code on the index date, then subgroup1=subgroup2=subgroup3=IPF. These patients will be
excluded from study population. 
else if two or more different (3) disease codes including disease code of IPF specified in this step 3 
exist on index date, then subgroup1=subgorup2=subgrou3=Multiple 
else if disease code on index date=in(8850547, 8845727), then subgroup1=Non-autoimmune ILD, 
subgroup2=iNSIP, subgroup3=iNSIP 
else if disease code on index date=in(5168008, 5168009, 5150001, 8845731, 8845640ˈJ84.9), 
then subgroup1=Non-autoimmune ILD, subgroup2=Unclassifiable IIP, subgroup3=Unclassifiable 
IIP
else if disease code on index date=in(1363003, 8845663, 8845714, 8845719, 5168010), then 
subgroup1=Non-autoimmune ILD, subgroup2=Other ILD, subgroup3=Other ILD 
else if disease code on index date=in(8840951, 8848245), then subgroup1=Autoimmune ILD, 
subgroup2=Other autoimmune ILD, subgroup3=Other autoimmune ILD 
else subgroup1=subgroup2=subgroup3=Unknown 
(3)Note: The disease codes in the same conditional statement are NOT considered different. For 
example, disease codes of 8850547 and 88457271 are in the same conditional statement and these 
are not considered different disease codes. On the other hand, disease codes of 8850547 and 
5168008 are in the different conditional statement and these are considered different disease codes.
END

nonspecific interstitial pneumonia
J841
8845727
Non-autoimmune
iNSIP
iNSIP
acute interstitial pneumonia
J84.1
1363003
Non-autoimmune
Other IIPs
Other IIPs
respiratory bronchiolitis-associated interstitial lung disease
J841
8845663
Non-autoimmune
Other IIPs
Other IIPs
cryptogenic organizing pneumonia
J841
8845714
Non-autoimmune
Other IIPs
Other IIPs
desquamative interstitial pneumonia
J841
8845719
Non-autoimmune
Other IIPs
Other IIPs
lymphocytic interstitial pneumonia
J841
5168010
Non-autoimmune
Other IIPs
Other IIPs
- 
- 
- 
Non-autoimmune
Unclassifiabl
e 
Unclassifiable
interstitial pneumonia
J849
4860015
Non-autoimmune
Unclassifiabl
e 
Unclassifiable
interstitial lung disease
J849
5168003
Non-autoimmune
Unclassifiabl
e 
Unclassifiable
respiratory disorder in dermatosclerosis
 M348
8844510
SSc-ILD
SSc-ILD
respiratory disorder complicated by granulomatosis with 
polyangiitis
 M313
8848382
other CTD-
ILD
other CTD-ILD
lupus pneumonia
 M321
8844341
SLE-ILD
SLE-ILD

systemic lupus erythematosus interstitial pneumonia
 M321
8848278
SLE-ILD
SLE-ILD
respiratory disorder in systemic lupus erythematosus
 M321
8836513
SLE-ILD
SLE-ILD
interstitial pneumonia in juvenile dermatomyositis
 M330
8848267
PM/DM-ILD
PM/DM-ILD
interstitial pneumonia in dermatomyositis
 M331
8848302
PM/DM-ILD
PM/DM-ILD
respiratory disorder in dermatomyositis
 M331
8839362
PM/DM-ILD
PM/DM-ILD
interstitial pneumonia in polymyositis
 M332
8848283
PM/DM-ILD
PM/DM-ILD
respiratory disorder in polymyositis
 M332
8837013
PM/DM-ILD
PM/DM-ILD
respiratory disorder in Sjogren's syndrome
 M350
8834158
Sjogren-ILD
Sjogren-ILD
interstitial pneumonia due to collagen disease
 M351
8848245
other CTD-
ILD
other CTD-ILD
rheumatoid lung disease
 M0510
8840951
other CTD-
ILD
rheumatoid arthritis interstitial pneumonia
 M0510
8847737
RA-ILD
RA-ILD
N-Total number of study patients in each ILD underlying autoimmune disease category, IPF – Idiopathic Pulmonary Fibrosis, iNSIP  – Idiopathic Nonspecific Interstitial 
Pneumonia, IIP – Idiopathic Interstitial Pneumonia, SSc-ILD – Systemic Sclerosis- Associated ILD, RA-ILD – Rheumatoid-Associated ILD, SLE-ILD – Systemic Lupus 
Erythematosus ILD, PM/DM ILD – Polymyositis/Dermatomyositis ILD

overlap syndrome
M351
8841317
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
anti-ARS antibody 
syndrome
M351
8850417
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
mixed connective 
tissue disease
M351
7109008
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
MCTD-ILD
MCTD-ILD
interstitial pneumonia 
due to collagen 
disease
M351
8848245
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
Behcet's disease
M352
1361002
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
vulval Behcet's 
disease
M352
1361011
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD

ocular Behcet's 
disease
M352
8845881
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
vasculo-Behcet's 
disease
M352
1361009
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
oral Behcet's disease
M352
1361010
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
neuro-Behcet disease
M352
1361005
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
entero-Behcet 
disease
M352
8842203
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
incomplete Behcet 
disease
M352
8846052
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
polymyalgia 
rheumatica
M353
7250004
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD

diffuse eosinophilic 
fasciitis
M354
8839540
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
eosinophilic fasciitis
M354
7294009
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
acrosclerosis
M355
7108005
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
multifocal 
fibrosclerosis
M355
8836987
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
Weber-Christian 
disease
M356
8830767
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
hyperkinetic 
syndrome
M357
7285007
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD

familial ligament 
relaxation
M357
8831279
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
eosinophilia-myalgia 
syndrome
M358
7101038
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
IgG4-related disease
M359
8848113
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
systemic autoimmune 
disease
M359
8836526
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
collagen disease
M359
7109004
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
anemia associated 
with collagen 
disease
M359
8833428
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
pericarditis of 
collagen disease
M359
4239017
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmune/CTD 
ILD
Other-
autoimmune/CTD 
ILD
polyarteritis nodosa
M300
8833125
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD

eosinophilic 
granulomatosis with 
polyangiitis
M301
8848338
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
juvenile multiple 
arteritis
M302
8835258
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
acute febrile 
mucocutaneous 
lymph node 
syndrome
M303
4461009
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
Kawasaki's disease
M303
4461003
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
ischemic heart 
disease due to 
Kawasaki's disease
M303
8831474
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
Kawasaki disease-
related coronary 
artery aneurysm
M303
4461004
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
incomplete Kawasaki 
disease
M303
8846336
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
polyangiitis overlap 
syndrome
M308
8837016
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD

skin polyarteritis 
nodosa
M308
8846209
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
cutaneous arteritis
M308
8849445
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
Goodpasture's 
syndrome
M310
4462001
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
hypersensitivity 
vasculitis
M310
4462002
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
cutaneous 
leukocytoclastic 
vasculitis
M310
8851019
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
drug-induced 
hypersensitivity 
vasculitis
M310
8844108
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
thrombotic 
thrombocytopenic 
purpura
M311
4466002
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
thrombotic 
microangiopathy
M311
8833136
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
acquired thrombotic 
thrombocytopenic 
purpura
M311
8849965
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
congenital thrombotic 
thrombocytopenic 
purpura
M311
8847881
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
gangrenous rhinitis
M312
4609006
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD

localized 
granulomatosis with 
polyangiitis
M313
8848336
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
generalized 
granulomatosis with 
polyangiitis
M313
8848371
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
granulomatosis with 
polyangiitis
M313
8848381
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
respiratory disorder 
complicated by 
granulomatosis with 
polyangiitis
M313
8848382
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
Takayasu's arteritis
M314
8848380
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
aortitis syndrome
M314
4467003
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
- 
- 
- 
- 
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
giant cell arteritis
M316
4465001
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD

microscopic 
polyangiitis
M317
8842086
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
ANCA-associated 
vasculitis
M318
8845513
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
hypocomplementemic 
urticarial vasculitis
M318
8849413
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
necrotizing angitis
M319
4460015
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
polyangiitis
M319
4460017
J84.1, J84.8, J84.9, 
J99.0, J99.1
Autoimmune
Other-
autoimmue/CTD 
ILD
Other-
autoimmue/CTD 
ILD
N-Total number of study patients in each ILD underlying autoimmune disease category, 
SSc-ILD – Systemic Sclerosis- Associated ILD, RA-ILD – Rheumatoid-Associated ILD, SLE-ILD – Systemic Lupus Erythematosus ILD, PM/DM ILD – 
Polymyositis/Dermatomyositis ILD
CTD-ILD – connective tissue disease- associated ILD, MCTD-ILD – Mixed Connective Tissue Disease-ILD

BOEHRINGER INGELHEIM Group of Companies
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