An Open Label, Intra-Subject Dose Escalation Study of CCX140-B in Subjects With Primary Focal Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome
Phase: PHASE2
Conditions: Focal Segmental Glomerulosclerosis
Sponsor: Amgen

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 1 of 109 
PROTOCOL 
 
TITLE PAGE 
Study Title: An Open Label, Intra-Subject Dose Escalation Study of 
CCX140-B in Subjects with Primary Focal Segmental 
Glomerulosclerosis (FSGS) and Nephrotic Syndrome 
Protocol Number: CL012_140 
Investigational Product: CCX140-B, a selective antagonist of human C-C chemokine 
receptor 2 (CCR2)  
Indication: Primary Focal Segmental Glomerulosclerosis (FSGS) 
Sponsor: ChemoCentryx, Inc. 
Development Phase: 2 
IND number 134007 
EUDRACT number 2017-003022-32 
Sponsor's Responsible 
Medical Officer: ChemoCentryx, Inc. 
Mountain View, California 94043, USA 
Sponsor Signatory: 
Vice President, Translational Research and Clinical Development 
ChemoCentryx, Inc. 
Mountain View, California 94043, USA 
Approval Date: 15 December 2017 
17 April 2018    Amendment 1.0 
 
Confidential 
The information contained herein is the property of the Sponsor and may not be reproduced, 
published or disclosed to others without written authorization of the Sponsor. 
This study will be conducted according to the principles of Good Clinical Practice as described 
in International Conference on Harmonization guidelines, including the archiving of essential 
documents. 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 2 of 109 
INVESTIGATOR SIGNATORY PAGE 
Protocol Number: CL012_140 
Protocol Title: An Open-Label, Intra-Subject Dose Escalation Study of CCX140-B in Subjects 
with Primary Focal Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome 
I agree: 
• to assume responsibility for the proper conduct of the study at this site. 
• to conduct the study in compliance with this protocol, any future amendments, and with any 
other study conduct procedures provided by ChemoCentryx, Inc. 
• not to implement any deviations from or changes to the protocol without agreement from the 
sponsor and prior review and written approval from the Institutional Review Board 
(IRB)/Ethics Committee (EC), except where necessary to eliminate an immediate hazard to 
the subjects, or for administrative aspects of the study (where permitted by all applicable 
regulatory requirements). 
• that I am thoroughly familiar with the appropriate use of the investigational drug(s), as 
described in this protocol, and any other information provided by the sponsor including, but 
not limited to the following: the current version of the Investigator’s Brochure prepared by 
ChemoCentryx, Inc. and approved product label, if applicable. 
• that I am aware of and will comply with current ICH/FDA good clinical practices guidelines 
(GCP) and all regulatory requirements. 
• to ensure that all persons assisting me with the study are adequately informed about the 
investigational drug(s) and their study-related duties and function as described in the 
protocol. 
 
__________________________________      
 
___________________ 
Principal Investigator  
 
 
 
 
 
 
 
Date 
 
__________________________________ 
Printed Name 
Address*_____________________________________________________________ 
____________________________________________________________________ 
Phone Number* ___________________________________________ 
* If the address or phone number needs to be changed during the course of the study, this will be 
done by the Investigator, with written notification to the Sponsor, and will not require (a) 
protocol amendment(s). 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 3 of 109 
SPONSOR CONTACT INFORMATION 
Protocol Number: CL012_140 
Protocol Title: An Open-Label, Intra-Subject Dose Escalation Study of CCX140-B in Subjects 
with Primary Focal Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome 
 
Medical Officer 
ChemoCentryx, Inc. 
Mountain View, 
California 94043, USA 
Office telephone number:
Fax number:
Email: 
Clinical Operations 
Manager 
ChemoCentryx, Inc. 
Mountain View, California 94043, USA 
Office telephone number:
Fax number: 
Email: 
 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 5 of 109 
PROTOCOL AMENDMENT 1.0: SUMMARY OF CHANGES 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
Synopsis (Efficacy 
Objectives and 
Endpoints) 
The Primary Efficacy 
Objective is to evaluate 
the effect of CCX140-B 
on proteinuria in 
subjects with primary 
FSGS with nephrotic 
syndrome, assessed as 
achievement of partial 
or complete remission 
of urine protein to 
creatinine ratio (UPCR) 
through Study Week 
12, where partial and 
complete remission are 
defined as follows: 
Partial Remission 
(includes all of the 
following): 
- reduction from 
baseline by ≥50 
percent in UPCR 
-reduction in 
UPCR to a level 
that is < 3.5 g/g 
-serum albumin 
within normal 
range 
-subject may not be a 
treatment failure (e.g. 
has not received rescue 
therapy, has not 
required 
discontinuation of study 
drug) 
 
Complete 
Remission 
(includes all of the 
following): 
-reduction in 
UPCR to < 0.3 g/g 
-serum albumin 
The Primary Efficacy Objective 
is to evaluate the effect of 
CCX140-B on proteinuria in 
subjects with primary FSGS 
with nephrotic syndrome, 
assessed as a median reduction 
from baseline of urine protein to 
creatinine ratio (UPCR) of at 
least 20%, i.e. ≥ 20%, by week 
12. 
Secondary efficacy endpoints of 
this study will be assessed 
through Study Week 12 and 
through End of Treatment and 
include: 
Achievement of partial 
or complete remission 
of urine protein to 
creatinine ratio 
(UPCR):  
Partial Remission (includes all 
of the following) 
• 
reduction from baseline by 
≥50 percent in UPCR 
• 
reduction in UPCR to a 
level that is < 3.5 g/g 
• 
subject may not be a 
treatment failure  
• 
Complete Remission 
(includes all of the 
following) 
• 
reduction in UPCR to < 0.3 
g/g 
• 
serum albumin within 
normal range 
• 
For patients with abnormal 
serum creatinine levels at 
baseline, return to normal 
levels for that age group 
• 
For patients with normal 
Moved partial and 
complete remission from 
primary to secondary 
objective and evaluate 
median reduction of 
UPCR as primary 
efficacy. This was based 
on discussions with lead 
investigators of the study 
and is aligned with 
previously observed 
treatment effects with 
CCX140-B in subjects 
with diabetic 
nephropathy. 
Redefined partial and 
complete remission based 
on expert input from 
investigators. 
 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 6 of 109 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
within normal 
range 
-subject may not be a 
treatment failure (e.g. 
has not received rescue 
therapy and has not 
required 
discontinuation of study 
drug) 
serum creatinine levels at 
baseline, final value within 
20% of baseline levels 
• 
subject may not be a 
treatment failure (e.g. has 
not received rescue therapy 
and has not required 
discontinuation of study 
drug) 
Synopsis: Primary 
Efficacy Objectives  
eGFR, calculated by the 
CKD-EPI equation, 
based on cystatin C 
eGFR, calculated by the CKD-
EPI Cystatin C equation, CKD-
EPI Creatinine equation, CKD-
EPI Creatinine-Cystatin C 
equation and MDRD Creatinine 
equation. 
To allow calculating 
eGFR by several 
equations including 
creatinine instead of 
solely Cystatin C, based 
on FDA recommendation 
in study CL011_140 
Synopsis: Dose and 
dose adjustments 
… 10 mg twice daily, 
then to 15 mg twice 
daily while monitoring 
efficacy … 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
,,, 10 mg twice daily on day 15, 
then to 15 mg twice daily on day 
43 while monitoring efficacy … 
 
If planned dose escalation to 10 
mg (on day 15) or 15 mg twice 
daily (on day 43) was previously 
halted the dose may be escalated 
further if subjects are not 
considered treatment failures and 
who otherwise meet criteria for 
continuation. In such case the 
subject will return approximately 
2 weeks later for blood draws to 
assess PK (AUC0-6). Subjects 
will return 2 weeks after the PK 
assessment to evaluate the safety 
and need for further dose 
adjustment. 
 
Subjects who completed 12 
weeks of treatment and who are 
not considered treatment failures 
and who otherwise meet criteria 
for continuation may be 
extended for up to an additional 
40 weeks to assess degree and 
duration of response. During the 
extension period the dose may 
be adjusted if indicated based on 
Clarification of procedure 
 
 
 
 
 
 
 
 
 
 
 
The full list of conditions 
for subjects being allowed 
to enter the extended 
treatment period was 
inadvertently omitted and 
is now provided. 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 7 of 109 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
 
 
 
 
In subjects who have 
achieved at least a 
Partial Response by 
Week 12 (Study Day 
85), treatment may be 
extended for up to an 
additional 40 weeks to 
assess degree of 
response and duration 
of response. During the 
extension period dose 
may be adjusted if 
indicated based on 
assessment of safety, 
PK and efficacy. 
assessment of safety, PK and 
efficacy. (see Table 1 and 
Figure 1 for guidelines). 
 
Starting with the extended 
treatment period (Day 85 and 
beyond) if the dose is adjusted 
upward or downward for any 
reason at Day 85 or during the 
extended treatment period the 
subject will return approximately 
2 weeks later for blood draws to 
assess PK (AUC 0-6)  
Figure 1 
Blank 
Added Figure 1 
Added Figure 1 to 
accompany Table 1 for 
further clarification  
Table 1: Dose 
Modification Schedule 
and Rules Through 
Study Day 85 
Day 43: At Day 43 
based on results of the 
Day 29 PK, the dose 
may be adjusted to the 
next higher planned 
dose level (15 mg twice 
daily for subjects 
receiving 10 mg twice 
daily; 10 mg twice 
daily for subjects 
receiving 5 mg twice 
daily) if all of the 
following are true: 
Subject is not a 
Treatment Failure, and 
• Subject has not 
achieved and 
maintained at least a 
Partial Remission, and 
• Projected AUC0-12 on 
the next higher planned 
dose level does not 
Day 43: At Day 43 based on 
results of the Day 29 PK, the 
dose may be adjusted to the next 
higher planned dose level (15 
mg twice daily for subjects 
receiving 10 mg twice daily; 10 
mg twice daily for subjects 
receiving 5 mg once daily) if all 
of the following are true: 
• Subject is not a Treatment 
Failure, and 
• Meets all criteria for 
continuation: 
- Subject has not achieved and 
maintained at least a Partial 
Remission, and 
- Projected AUC0-12 on the next 
higher planned dose level does 
not exceed 240 µg•h/mL and 
- The next higher dose does not 
Clarifications and 
standardization of all 
titration requirements on 
day 43 or beyond. 
 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 8 of 109 
Section(s) 
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exceed 240 µg•h/mL 
• The next higher dose 
does not exceed the 
Maximum Tolerated 
Dose (MTD) 
 
Day 71: Based on 
results of Day 57 PK, 
dose will be adjusted to 
the next higher planned 
dose level if all of the 
following are true: 
The next higher dose 
does not exceed 15 mg 
twice daily or the MTD, 
whichever is lower 
• Subject has not 
previously been treated 
at the next higher dose 
and required downward 
dose adjustment 
• Subject is not a 
Treatment Failure, and 
• Subject has not 
achieved and 
maintained at least a 
Partial Remission, and 
• Projected AUC0-12  on 
the next higher planned 
dose level does not 
exceed 240 µg•h/mL 
exceed the Maximum Tolerated 
Dose (MTD) 
 
 
Day 71: Based on results of Day 
57 PK, subjects can be 
considered for dose escalation if 
all of the following are true:  
• Subject is not already treated 
with 15 mg twice daily and 
• 
Subject did not require 
previous downward dose 
adjustment and  
 
• Subject is not a Treatment 
Failure, and• Meets all criteria 
for continuation: 
- Subject has not achieved and 
maintained at least a Partial 
Remission, and 
- Projected AUC0-12  on the next 
higher planned dose level does 
not exceed 240 µg•h/mL and 
- The next higher dose does not 
exceed the Maximum Tolerated 
Dose (MTD) 
Table 1: Dose 
Modification Schedule 
and Rules Through 
Study Day 85 
Day 85 and beyond: If 
dose is adjusted upward 
or downward for any 
reason at Day 85 or 
during the extended 
treatment period the 
subject will return 
approximately 2 weeks 
later for blood draws to 
assess PK (AUC0-6) and 
will return 2 weeks 
after assessment of PK 
to assess safety and 
Day 85 and beyond:  
If dose is adjusted upward or 
downward for any reason at Day 
85 or during the extended 
treatment period the subject will 
return approximately 2 weeks 
later for blood draws to assess 
PK (AUC0-6). Subjects will 
return 2 weeks after the PK 
assessment to evaluate the safety 
and need for further dose 
Administrative change  

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 9 of 109 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
need for further dose 
adjustment. 
adjustment. 
Synopsis: Extended 
Treatment Period 
Subjects who are not 
Treatment Failures as 
of Week 12 may 
continue extended 
treatment under any of 
the following 
conditions 
• Have achieved and 
have sustained Partial 
or Complete Remission 
by Week 12 
• Have achieved a 
significant response of 
at least 30% decline in 
proteinuria by Week 12 
and, in the opinion of 
the Investigator, remain 
candidates for 
investigational 
treatment 
• No longer have 
nephrotic syndrome, 
independent of the % 
reduction in proteinuria 
and, in the opinion of 
the Investigator, remain 
candidates for 
investigational 
treatment 
Subjects are eligible for the 
extended treatment period under 
the following conditions: 
1. Not considered treatment 
failures as of Week 12  
2. And, in the opinion of the 
Investigator, remain candidates 
for investigational treatment 
3. And any of the conditions 
below apply: 
• Partial or Complete Remission 
by Week 12 
• Response with ≥20% reduction 
in UPCR by Week 12  
• No longer have nephrotic 
syndrome 
Clarifications & 
Adjustment from at least 
30% to ≥20% reduction in 
UPCR. This is in 
alignment with lead 
investigators and 
previously observed 
treatment effects in 
patients with diabetic 
nephropathy. 
Synopsis: Extended 
Treatment Period 
Subjects who are 
receiving a dose that is 
less than 15 mg twice 
daily at Week 12 can be 
considered for further 
dose escalation if all of 
the following are true: 
• Subject has not 
previously required 
dose reduction due to 
toxicity or adverse 
event 
• Subject has not 
achieved complete 
Subjects can be considered for 
dose escalation if all of the 
following are true:  
• Subject is not already treated 
with 15 mg twice daily and 
Subject did not require previous 
downward dose adjustment and 
• Subject is not a Treatment 
Failure, and 
• Meets all criteria for 
continuation: 
- Subject has not achieved and 
Clarifications and 
standardization of all 
titration requirements in 
extended treatment period 
with the initial treatment 
period.  

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 10 of 109 
Section(s) 
Original Text 
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Rationale for Change 
remission 
• The next dose has not 
been determined to be 
above the maximally 
tolerated dose 
• Based on PK analysis 
the exposure (AUC0-12) 
at the next dose is not 
projected to exceed 240 
µg•h/mL. 
• Subject is not a 
Treatment Failure 
maintained at least a Partial 
Remission, and 
- Projected AUC0-12  on the next 
higher planned dose level does 
not exceed 240 µg•h/mL and 
- The next higher dose does not 
exceed the Maximum Tolerated 
Dose (MTD) 
Synopsis: Dose 
Modification Rules 
Dose Modification 
Rules for individual 
subjects 
Discontinue treatment 
with CCX140-B when a 
subject experiences any 
of the following events 
confirmed by repeat 
measurement after 2 
weeks: 
50% increase from 
baseline in serum 
creatinine value, 
confirmed by repeat 
measurement after 2 
weeks 
If baseline UPCR < 6.0 
g/g and increase of  
>3.0 g/g; if UPCR ≥6.0 
g/g an increase of 50% 
 
Dose Modification Rules for 
individual subjects 
Discontinue treatment with 
CCX140-B when a subject 
experiences any of the following 
events confirmed by repeat 
measurement after 2 weeks: 
• 
Progression of renal disease, 
defined as eGFR that is both 
below 60 ml/min/1.73 m2 
and is confirmed to 
represent at least 30% 
decline in eGFR from 
baseline  
• 
Requirement for rescue with 
glucocorticoids, other new 
immunomodulatory or 
immunosuppressive therapy, 
plasmapheresis or dialysis 
• 
Pre-specified adverse event 
or laboratory abnormality 
that, per Protocol, requires 
permanent discontinuation 
of study CCX140-B 
Any other treatment-related 
adverse event, laboratory 
evidence of toxicity or 
intolerance that in the judgment 
of the investigator, warrants 
permanent discontinuation of 
Added per 
recommendation from 
FDA 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 11 of 109 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
CCX140-B 
Consider discontinuation of 
treatment when a subject 
experiences any of the following 
events: 
An increase of serum ALT to 
>3xULN should be followed by 
repeat testing within 48 to 72 
hours of all four of the usual 
serum measures (ALT, AST, 
ALP, and TBL) to confirm the 
abnormalities and to determine if 
they are increasing or 
decreasing. 
Discontinuation of treatment 
should be considered if: 
• ALT or AST >8xULN 
• ALT or AST >5xULN for 
more than 2 weeks 
• ALT or AST >3xULN and 
(TBL >2xULN or INR >1.5) 
• ALT or AST >3xULN with the 
appearance of fatigue, nausea, 
vomiting, right upper quadrant 
pain or tenderness, fever, rash, 
and/or eosinophilia (>5%) 
Synopsis (Inclusion and 
Exclusion criteria)  
See body text of the 
protocol 
See body text of the protocol 
 
Synopsis (Efficacy 
Assessment) and Time 
& Events Table  
• Urine 
protein:creatinine ratio 
(UPCR) and 
urine:albumin ratio 
(UACR), assessed in a 
sample drawn from a 24 
hour collection is 
required at baseline and 
Study Week 12 (see 
schedule). At other time 
points defined in the 
Time and Events table, 
24 hour collection is 
preferred. However, at 
these visits, a first 
morning void sample 
• Urine protein:creatinine ratio 
(UPCR) and urine:albumin ratio 
(UACR), assessed in a sample 
drawn from a 24 hour collection 
is required at baseline and Study 
Week 12 (see schedule). At 
other time points defined in the 
Time and Events table, 24 hour 
collection is preferred. However, 
at these visits, a midstream first 
morning void sample may be 
used for assessments if a 24hr 
urine sample is not available  
Emphasize importance of 
24h urine collection. 
Allow for collection of 
midstream urine during 
visit on days when 24h 
urine is not mandatory 
and therefore not 
available. 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 12 of 109 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
may be used for 
assessments. 
Synopsis (Efficacy 
Assessment)  
eGFR calculated from 
serum creatinine 
usingby the chronic 
kidney disease-
epidemiology 
collaboration (CKD-
EPI equation based on 
cystatin C; Levey et al, 
2009) 
Change from baseline in eGFR 
calculated by the CKD-EPI 
Cystatin C equation, CKD-EPI 
Creatinine equation, CKD-EPI 
Creatinine-Cystatin C equation 
and MDRD Creatinine equation 
To allow calculating 
eGFR by several 
equations including 
creatinine instead of 
Cystatin C only, based on 
FDA recommendation in 
study CL011_140 
Time and Events Table: 
Dose 
(Days1-14) 5 mg bid 
 
(Days 15 -29) 10 mg 
bid 
(Days1-14) 5 mg bid  
 
(Days 15 -43) 10 mg bid 
Although PK blood draws 
will occur on Day 29 the 
dose modification is not 
scheduled to occur until 
Day 43. This was an error 
in the Time and Events 
Table 
Time and Events Table: 
ACTG-BPNST 
blank 
Added to study days 1- 52 
ACTG-BPNST evaluation 
was inadvertently omitted 
in Section 6 and the Time 
and Events table 
Time and Events Table: 
Footnote 1 
Organ transplantation 
including renal 
transplantation should 
be captured in medical 
history. 
Removed 
Since organ transplants 
are exclusionary, subjects 
that have had transplants 
will not enroll. Therefore, 
this footnote is not 
applicable. 
Section 2.1 Primary 
Efficacy Objective 
The Primary Efficacy 
Objective is to evaluate 
the effect of CCX140-B 
on proteinuria in 
subjects with primary 
FSGS with nephrotic 
syndrome, assessed as 
achievement of partial 
or complete remission 
of urine protein to 
creatinine ratio (UPCR) 
through Study Week 
12, where partial and 
complete remission are 
defined as follows: 
Partial Remission 
(includes all of the 
following) 
The Primary Efficacy Objective 
is to evaluate the effect of 
CCX140-B on proteinuria in 
subjects with primary FSGS 
with nephrotic syndrome, 
assessed as a median reduction 
from baseline of urine protein to 
creatinine ratio (UPCR) of at 
least 20%, i.e. ≥ 20%, by week 
12. 
Move partial and 
complete remission from 
primary to secondary 
objective and evaluate 
median reduction of 
UPCR as primary 
efficacy. This change is 
based on discussions with 
lead investigators of the 
study and is aligned with 
previously observed 
treatment effects with 
CCX140-B in subjects 
with diabetic 
nephropathy. 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 13 of 109 
Section(s) 
Original Text 
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Rationale for Change 
Reduction from 
baseline by ≥50 percent 
in UPCR 
Reduction in UPCR to 
a level that is <3.5 g/g 
Serum albumin within 
normal range 
Subject may not be a 
treatment failure (e.g. 
has not received rescue 
therapy, has not 
required 
discontinuation of study 
drug) 
Complete Remission 
(includes all of the 
following) 
Reduction in UPCR to 
<0.3 g/g 
Serum albumin within 
normal range 
Subject may not be a 
treatment failure (e.g. 
has not received rescue 
therapy and has not 
required 
discontinuation of study 
drug) 
Section 2.2 Secondary 
Efficacy Objective 
None 
The secondary efficacy 
objectives of this study will be 
assessed at Study Week 12 and 
through End of Treatment and 
include: 
1. Achievement of partial or 
complete remission of urine 
protein to creatinine ratio 
(UPCR) through Study Week 12, 
where partial and complete 
remission are defined as follows: 
Partial Remission (includes all 
of the following) 
• reduction from baseline by ≥50 
Move partial and 
complete remission from 
primary to secondary 
efficacy objective and 
evaluate median reduction 
of UPCR as primary 
efficacy instead. This 
change is based on 
discussions with lead 
investigators of the study 
and is aligned with 
previously observed 
treatment effects with 
CCX140-B in subjects 
with diabetic 
nephropathy. 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 14 of 109 
Section(s) 
Original Text 
Revised Text 
Rationale for Change 
percent in UPCR 
• reduction in UPCR to a level 
that is < 3.5 g/g 
• subject may not be a treatment 
failure (e.g. has not received 
rescue therapy, has not required 
discontinuation of study drug) 
Complete Remission (includes 
all of the following) 
• reduction in UPCR to < 0.3 g/g 
• serum albumin within normal 
range 
• For patients with abnormal 
serum creatinine levels at 
baseline, return to normal levels 
for that age group 
• For patients with normal serum 
creatinine levels at baseline, 
final value within 20% of 
baseline levels  
• subject may not be a treatment 
failure (e.g. has not received 
rescue therapy and has not 
required discontinuation of study 
drug) 
 
Redefined partial and 
complete remission based 
on expert input from 
investigators  
Section 2.2: Secondary 
Efficacy Objective 
eGFR, calculated by the 
CKD EPI equation, 
based on cystatin C 
 
eGFR, calculated by the CKD-
EPI Cystatin C equation, CKD-
EPI Creatinine equation, CKD-
EPI Creatinine-Cystatin C 
equation and MDRD Creatinine 
equation. 
To allow calculating 
eGFR by several 
equations including 
creatinine instead of 
solely Cystatin C, based 
on FDA recommendation 
in study CL011_140 
Section 2.3 Safety 
Objectives 
4. AIDS Clinical Trials 
Group Brief Peripheral 
Neuropathy Screen 
(ACTG BPNS) 
4. AIDS Clinical Trials Group 
Brief Peripheral Neuropathy 
Screening Tool (ACTG 
BPNST): To assess potential 
signs and symptoms of 
peripheral neuropathy the ACTG 
BPNST will be completed by 
study personnel or the study 
investigator as specified in the 
Time and Events table. 
The acronym ACTG-
BPNS was changed to 
ACTG-BPNST as this 
was a typographical error. 
Further specification was 
added that the study 
personnel or study 
investigator will complete 
the ACTG-BPNST. 

CCX140-B 
 
Protocol CL012_140 
Amendment 1.0 (17 April 2018) 
ChemoCentryx, Inc. 
CONFIDENTIAL 
Page 15 of 109 
Section(s) 
Original Text 
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Rationale for Change 
Section 3.1: Initial 
Treatment Phase 
During the initial 12 
weeks of treatment, 
dose will be escalated 
from 5 mg twice daily 
to 10 mg twice daily on 
Study Day 15, then to 
15 mg twice daily on 
Study Day 43, unless 
dose escalation is 
stopped or reversed due 
to any of the following: 
1. Achievement of 
Partial Response 
2. Exposure that 
exceeds, or is projected 
to exceed, the maximal 
allowable exposure 
defined for the study 
3. Safety 
During the initial 12 weeks of 
treatment, dose will be escalated 
from 5 mg twice daily to 10 mg 
twice daily on Study Day 15, 
then to 15 mg twice daily on 
Study Day 43, if subjects are not 
considered treatment failures and 
who otherwise meet criteria for 
continuation (Table 1, Figure 1). 
Clarifications and 
standardization of all 
titration requirements. 
Section 3.1: Initial 
Treatment Phase 
After 12 weeks, dose 
may be escalated 
further if escalation was 
previously halted prior 
to 15 mg twice daily 
solely due to 
achievement of Partial 
Response, and if 
Complete Response has 
not been achieved. 
If planned dose escalation to 10 
mg (on day 15) or 15 mg twice 
daily (on day 43) was previously 
halted the dose may be escalated 
further if subjects are not 
considered treatment failures and 
who otherwise meet criteria for 
continuation (Table 1, Figure 1). 
In such case the subject will 
return approximately 2 weeks 
later for blood draws to assess 
PK (AUC0-6). Subjects will 
return 2 weeks after the PK 
assessment to evaluate the safety 
and need for further dose 
adjustment. 
Language was missing in 
this section although 
synopsis allowed for later 
dose escalation.  
Previous text referring to 
the period (“after 12 
weeks…”) applies to the 
extended treatment phase 
and was therefore deleted 
from Section 3.1.1  
Section 3.1: Table 2: 
Dose Modification 
Schedule and Rules 
Through Study Day 85 
Table 2 was deleted as 
duplication from Table 
1. 
Reference is made to table 1 in 
various sections of the main 
body of the protocol. 
Delete Duplication 
Section 3.1.1: Day 85 
and beyond 
Blank 
If dose is adjusted upward or 
downward for any reason at Day 
85 or during the extended 
treatment period the subject will 
return approximately 2 weeks 
later for blood draws to assess 
PK (AUC0-6). Subjects will 
return 2 weeks after the PK 
Section 3.1.2 was added 
to make synopsis 
consistent with main body 
text of protocol.  

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assessment to evaluate the safety 
and need for further dose 
adjustment. 
Section 3.1.2: Extended 
Treatment Period 
Subjects who are not 
Treatment Failures as 
of Week 12 may 
continue extended 
treatment under any of 
the following 
conditions: 
• Have achieved and 
have sustained Partial 
or Complete Remission 
by Week 12 
• Have achieved a 
significant response of 
at least 30% decline in 
proteinuria by Week 12 
and, in the opinion of 
the Investigator, remain 
candidates for 
investigational 
treatment 
• No longer have 
nephrotic syndrome, 
independent of the % 
reduction in proteinuria 
and, in the opinion of 
the Investigator, remain 
candidates for 
investigational 
treatment 
Subjects are eligible for the 
extended treatment period under 
the following conditions: 
1) Not considered treatment 
failures as of Week 12  
2) And, in the opinion of the 
Investigator, remain candidates 
for investigational treatment 
3) And any of the conditions 
below apply: 
• Partial or Complete Remission 
by Week 12 
• Response with ≥20% reduction 
in UPCR by Week 12  
• No longer have nephrotic 
syndrome 
Clarifications & 
Adjustment from at least 
30% to ≥20% reduction in 
UPCR. This is in 
alignment with lead 
investigators and 
previously observed 
treatment effects in 
patients with diabetic 
nephropathy. 
Section 3.1.2: Extended 
Treatment Period 
Subjects who are 
receiving a dose that is 
less than 15 mg twice 
daily at Week 12 (Day 
85) can be considered 
for further dose 
escalation if all of the 
following are true: 
• Subject has not 
previously required 
dose reduction due to 
toxicity or adverse 
event 
• Subject has not 
Subjects can be considered for 
dose escalation if all of the 
following are true:  
• Subject is not already treated 
with 15 mg twice daily and 
• Subject did not require 
previous downward dose 
adjustment  
• Subject is not a Treatment 
Failure, and 
• Meets all criteria for 
Clarifications and 
standardization of all 
titration requirements in 
extended treatment period 
with the initial treatment 
period. 

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achieved complete 
remission 
• The next dose has not 
been determined to be 
above the maximally 
tolerated dose 
• Based on PK analysis 
the exposure (AUC0-12) 
at the next dose is not 
projected to exceed 240 
µg•h/mL. 
• Subject is not a 
Treatment Failure 
continuation: 
- Subject has not achieved and 
maintained at least a Partial 
Remission, and 
- Projected AUC0-12  on the next 
higher planned dose level does 
not exceed 240 µg•h/mL and 
- The next higher dose does not 
exceed the Maximum Tolerated 
Dose (MTD) 
Section 3.2: Dose 
Modification Based on 
PK Exposure 
Blank 
If planned dose escalation to 10 
mg (on day 15) or 15 mg twice 
daily (on day 43) was previously 
halted the dose may be escalated 
further if subjects are not 
considered treatment failures and 
who otherwise meet criteria for 
continuation (Table 1, Figure 1). 
In such case the subject will 
return approximately 2 weeks 
later for blood draws to assess 
PK (AUC0-6). Subjects will 
return 2 weeks after the PK 
assessment to evaluate the safety 
and need for further dose 
adjustment. 
Language was missing in 
this section although 
synopsis allowed for later 
dose escalation 
Synopsis and Section 
4.2: Inclusion criteria 
2. Renal biopsy 
findings consistent with 
diagnosis of primary 
focal segmental 
glomerulosclerosis 
(FSGS), including all 
histologic subtypes; 
subjects with genetic 
risk factors with 
presentations that are 
otherwise consistent 
with primary FSGS 
may be enrolled 
2. Renal biopsy findings 
consistent with diagnosis of 
focal segmental 
glomerulosclerosis (FSGS), and 
consistent with primary FSGS 
based on presentation of 
histopathology, medical history, 
and clinical course; subjects with 
genetic risk factors with 
presentations that are otherwise 
consistent with primary FSGS 
may also be enrolled 
Clarification of primary 
FSGS 
Synopsis and Section 
4.2: Inclusion criteria 
2. Renal biopsy 
findings consistent with 
diagnosis of primary 
focal segmental 
glomerulosclerosis 
2. Renal biopsy findings 
consistent with diagnosis of 
focal segmental 
glomerulosclerosis (FSGS), and 
consistent with primary FSGS 
based on presentation of 
Clarification of primary 
FSGS 

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(FSGS), including all 
histologic subtypes; 
subjects with genetic 
risk factors with 
presentations that are 
otherwise consistent 
with primary FSGS 
may be enrolled 
histopathology, medical history, 
and clinical course; subjects with 
genetic risk factors with 
presentations that are otherwise 
consistent with primary FSGS 
may also be enrolled 
Synopsis and Section 
4.2: Inclusion criteria 
4. Estimated glomerular 
filtration rate (eGFR, 
calculated using the 
CKD-EPI creatinine 
equation) >30 
mL/min/1.73m2 
4. Estimated glomerular 
filtration rate (eGFR) >30 
mL/min/1.73m2, as calculated 
using the Chronic Kidney 
Disease Epidemiology 
Collaboration (CKD_EPI) 
equation (using creatinine or 
cystatin C)  
Specify equation source 
for the calculation of 
eGFR 
Synopsis & Section 4.3: 
Exclusion Criteria #4 
Blank 
4. Histological FSGS subtype of 
collapsing variant 
Added exclusion per 
discussion with lead 
investigator as these 
patients will not respond 
to any medical therapy. 
Synopsis & Section 4.3: 
Exclusion Criteria #5 
and #6 related to 
rituximab and other 
anti-CD20 monoclonal 
antibodies 
4. Use of rituximab 
within 20 weeks prior 
to screening with 
recovery of CD20+ B 
cell population; if 
rituximab was used 
between 20 and 52 
weeks prior to 
screening, recovery of 
CD20 positive B cell 
population must be 
confirmed; where 
recovery is defined as 
within normal limits or 
within 70% of pre-
treatment baseline, 
whichever is least 
5. Subjects who initiated, 
discontinued or changed dose of 
rituximab or other anti-CD20 
monoclonal antibodies within 16 
weeks (4 months) prior to 
screening are excluded. Patients 
who initiated treatment with 
rituximab or other anti-CD20 
monoclonal antibodies >16 
weeks (4 months) prior to 
screening are permitted if 
deemed safe by the investigator 
and only if they are intended to 
remain on continued, unchanged 
therapy at a dosing interval that 
has been documented to achieve 
continuous B cell depletion for 
the given patient. UPCR 
assessments up to 1 year prior to 
screening (if available) that were 
performed in these patients as 
part of the clinical routine should 
be documented in the medical 
history. 
6. Subjects who discontinued 
Rituximab or other anti-CD20 
monoclonal antibodies >16 
Exclusion #4 became #5 
and addition of #6 due to 
additional exclusion 
criteria.  
Specify exclusion/use 
Rituximab and other anti-
CD20 monoclonal 
antibodies.  
Added exclusion per 
discussion with lead 
investigator as these 
patients will not respond 
to any medical therapy. 
Per discussion with lead 
investigators a subgroup 
treated chronically with 
anti-CD20 monoclonal 
antibodies should be 
included in the study 
target population as these 
patients have a high 
unmet medical need 
despite antibody 
treatment. 

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weeks (4 months) prior to 
screening without confirmed 
recovery of CD20+ B cell 
population to within normal 
range are excluded.  Patients 
who discontinued rituximab or 
other anti-CD20 monoclonal 
antibodies >16 weeks (4 months) 
prior to screening with 
confirmed recovery of CD20+ B 
cell population to within normal 
range are permitted in the study. 
UPCR and other urine protein 
assessments up to 1 year prior to 
screening (if available) that were 
performed in these patients as 
part of the clinical routine should 
be recorded in the medical 
history. 
Synopsis & Section 4.3: 
Exclusion Criteria - #7 
related to calcineurin 
inhibitors, or other 
immunotherapy 
5. Increase in dose or 
introduction of new 
calcineurin inhibitors, 
or other 
immunotherapy, within 
12 weeks prior to 
screening; dose 
reductions to maintain 
calcineurin inhibitors at 
optimal safe levels are 
permitted 
7. Subjects who initiated or 
increased the dose of calcineurin 
inhibitors, or other 
immunotherapy, within 12 
weeks prior to screening are 
excluded; dose reductions to 
maintain calcineurin inhibitors at 
optimal safe levels are permitted. 
Patients who initiated a 
treatment with calcineurin 
inhibitors, or other 
immunotherapy >12 weeks are 
permitted. UPCR and other urine 
protein assessments up to 1 year 
prior to screening (if available) 
that were performed in these 
patients as part of the clinical 
routine should be recorded in the 
medical history. 
Further clarification on 
intended exclusion/use of 
calcineurin inhibitors, or 
other immunotherapy.  
Synopsis & Section 4.3: 
Exclusion Criteria -  #8 
related to 
Glucocorticoids 
6. Glucocorticoids at 
dose greater than 10 
mg/day prednisone 
equivalent within 4 
weeks prior to 
screening 
8. Subjects taking 
glucocorticoids at dose greater 
than 10 mg/day prednisone 
equivalent within 4 weeks prior 
to screening are excluded. 
Clarification 
Synopsis & Section 4.3: 
Exclusion Criteria -  
#16  
14. Evidence of hepatic 
disease; AST, ALT, 
alkaline phosphatase, or 
bilirubin > 2 x the 
upper limit of normal 
(unless due to Gilbert’s 
16. Evidence of hepatic disease; 
AST, ALT, alkaline phosphatase 
>2x ULN, or total bilirubin > 2x 
ULN or INR > 1.5 x ULN at 
baseline with the exception that 
isolated INR elevation in the 
Change based on FDA 
recommendation to 
include INR. 

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disease) at baseline 
(baseline = within 24 
hours prior to dosing) 
absence of other significant liver 
enzyme abnormalities is 
explained by anticoagulant 
therapy, (e.g. warfarin). 
Synopsis & Section 4.3: 
Exclusion Criteria and 
Section 7.5.1: Dose 
Modification / Stopping 
Rules 
QTc 
QTcF 
Changed all references of 
QTc to QTcF to clarify 
that the Fridericia 
equation will be used to 
calculate the QTc interval. 
Synopsis & Section 4.3: 
Exclusion Criteria 
Evidence of hepatic 
disease; AST, ALT, 
alkaline phosphatase 
>2x ULN, or total 
bilirubin > 2x ULN or 
INR > 1.5 x ULN at 
baseline with the 
exception that isolated 
INR elevation in the 
absence of other 
significant liver enzyme 
abnormalities is 
explained by 
anticoagulant therapy, 
(e.g. warfarin).  
Evidence of hepatic disease; 
AST, ALT, alkaline phosphatase 
>2x ULN, or total bilirubin > 2x 
ULN or INR > 1.5 x ULN at 
screening with the exception that 
isolated INR elevation in the 
absence of other significant liver 
enzyme abnormalities is 
explained by anticoagulant 
therapy, (e.g. warfarin). 
Day 1 blood draws will be 
evaluated by a central 
laboratory and therefore 
won’t be available in time 
for Day 1 dosing. 
Therefore, the screening 
assessments should be 
used. 
Synopsis & Section 4.3: 
Exclusion Criteria -  
#20 and #27 
23. History of alcohol 
or illicit drug abuse or 
of lithium, 
pamidronate, interferon 
or prescribed NSAIDS. 
Recreational use of 
cannabis is not 
excluded where legal. 
20. History of alcohol or illicit 
drug abuse. Recreational use of 
cannabis is not excluded where 
legalized. 
27. Subjects taking lithium, 
pamidromate or interferon; 
subjects taking non-steroidal 
anti-inflammatory agents 
(NSAIDS) chronically 
(intermittent, i.e. occasional 
NSAIDS for pain or fever is 
discouraged, but is not 
excluded). 
Clarify drug exclusion 
and medication exclusion 
with two separate 
exclusion criteria 
Synopsis & Section 4.3: 
Exclusion Criteria -  
#21 
None 
21. History of gastrointestinal 
conditions that may interfere 
with study medication 
compliance, e.g., severe 
gastroparesis, with regurgitation 
of food or oral medication 
Added to make consistent 
with other CCXI 
protocols 
Synopsis & Section 4.3: 
Exclusion Criteria -  
#26 
22. Subject is taking 
strong CYP3A4 
inducers (e.g., 
phenytoin, rifampicin, 
26. Subjects taking strong 
CYP3A4 inducers (e.g., 
phenytoin, rifampicin, 
carbamazepine, St. John’s Wort) 
Strong CYP3A4 
inhibitors are additionally 
excluded as concomitant 
medication due to 

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carbamazepine, St. 
John’s Wort) 
or strong CYP3A4 inhibitors 
(e.g., boceprevir, clarithromycin, 
conivaptan, grapefruit juice, 
indinavir, itraconazole, 
ketoconazole, 
lopinavir/ritonavir, mibefradil, 
nefazodone, nelfinavir, 
posaconazole, ritonavir, 
saquinavir, telaprevir, 
telithromycin, and voriconazole) 
within two weeks prior to 
screening. 
CYP3A4 metabolism of 
CCX_140-B.  
Section. 5.2: Rationale 
for Dose Range 
Pre-existing language in 
the synopsis only. Was 
inadvertently not 
included in section 5.2.   
In all subjects, treatment at the 
selected dose will be continued 
through 12 weeks or until 
declaration of Treatment Failure, 
where Treatment Failure is 
defined as any of the following: 
1) Progression of renal disease, 
defined as eGFR that is both 
below 60 ml/min/1.73 m2 and is 
confirmed to represent at least a 
30% decline in eGFR from 
baseline; 
2) Requirement for rescue with 
glucocorticoids, other new 
immunomodulatory or 
immunosuppressive therapy, 
plasmapheresis, or dialysis, per 
the judgment of the Investigator; 
3) Pre-specified adverse event or 
laboratory abnormality that, per 
Protocol, requires permanent 
discontinuation of study 
CCX140-B 
4) Any other treatment-related 
adverse event, laboratory 
evidence of toxicity, or 
intolerance that, in the judgment 
of the Investigator, warrants 
permanent discontinuation of 
CCX140-B. 
Pre-existing language 
from the synopsis was 
inadvertently omitted in 
main body of the 
protocol. 
Section 6: Study 
procedures and Time 
and Events table 
Blank 
The following item was added to 
sections 6.1 through 6.17 (day 1 
through week 52): 
AIDS Clinical Trials Group 
ACTG-BPNST evaluation 
was inadvertently omitted 
in Section 6 and the Time 
and Events table  

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Brief Peripheral Neuropathy 
Screening Tool (ACTG 
BPNST). 
Synopsis and Section 
6.1: Screening 
Assessments (Day -28 
to Day 0) (and Time 
and Events Table where 
applicable) 
10. Concomitant 
medications including 
medications taken for 
up to 12 weeks prior to 
screening Day 1 should 
be captured on the 
eCRF. Rituximab 
within 52 weeks of 
screening must be 
captured on the eCRF 
(note that there must be 
documented evidence 
of CD20+ B cells when 
subject has history of 
use or Rituximab) 
10. Concomitant medications 
including medications taken for 
up to 12 weeks prior to 
screening Day 1 should be 
captured on the eCRF.  
Prior treatment with rituximab or 
other anti-CD20 monoclonal 
antibodies and corresponding 
levels of CD20+ B cells need to 
be recorded up to 1 year prior to 
screening Day 1. Eligible 
patients who have entered the 
study on a continuous cycle 
therapy of rituximab or other 
anti-CD20 monoclonal 
antibodies need to remain on an 
unchanged therapy throughout 
the study at a dosing interval that 
has been documented to achieve 
continuous B cell depletion for 
the given patient unless 
prohibited due to safety 
considerations. 
Record UPCR and urine protein 
assessments up to 1 year prior to 
screening (if available) in the 
medical history if patients were 
treated with Rituximab, other 
CD20+ monoclonal antibodies 
or calcineurin inhibitors, or other 
immunotherapy. 
To record CD20+ 
monoclonal antibodies 
levels or urine protein 
levels in the medical 
history in conjunction 
with prior medications 
Section 6.2: Study Day 
1 and Section 6.8: Study 
Week 12 
Blank 
Please be reminded that a 24 hr 
urine collection at baseline 
(study day 1) and week 12 are 
mandatory 
Highlighting the 
importance of 24 hr. urine 
collection for study days 1 
and week 12 as the 
defined efficacy endpoints 
Section 6.5 and Section 
6.6 and Time and 
Events table 
Day 43:  
no PK collection 
Day  57: 
Pharmacokinetics (Cmin) 
– sample is to be 
collected at time 0 (just 
prior to taking the first 
Day 43:  
4. Pharmacokinetics (Cmin) – 
sample is to be collected at time 
0 (just prior to taking the next) 
 
Day 57:  
 

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dose of CCX140-B).  
However, if there was a 
dose increase in the 
prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 
6 hours post dose) is to 
be collected instead. 
The time 0 sample is to 
be collected just prior to 
administration of the 
first dose of CCX140-
B. Subjects must fast 
prior to the time 0, 0.5 
and 1 hour PK and can 
consume food 
immediately after the 1 
hour PK blood draw. 
8. Pharmacokinetics (time 0, 0.5, 
1, 2, 3, 4 & 6 hours post dose), 
the time 0 sample is to be 
collected just prior to 
administration of the next dose 
of CCX140-B. Subjects must 
fast prior to the time 0, 0.5 and 1 
hour PK and can consume food 
immediately after the 1 hour PK 
blood draw. 
Section 7.3.1: Adverse 
Event Severity 
Reporting 
The severity of each 
adverse event will be 
determined by the 
investigator using 
CTCAE v4.0. If and 
event is not defined in 
the CTCAE criteria, use 
the following scale: 
The severity of each adverse 
event will be determined by the 
investigator using the following 
scale: 
Removed references to 
the CTCAE per feedback 
from the FDA 
Section 7.3.5: 
Pregnancies 
Any pregnancies that 
occur in female subjects 
or partners of male 
study subjects must be 
reported to the Safety 
team within 24 hours of 
awareness as indicated 
in Section 7.3.3. All 
pregnancies must be 
followed up until 
conclusion and the 
outcome of the 
pregnancy reported 
within 24 hours of 
awareness to the Safety 
team as indicated in 
Section 7.3.3 
Any pregnancies that occur in 
female subjects or partners of 
male study subjects must be 
reported to 
 Clinical 
Safety within 2 hours of 
awareness to the contacts details 
outlined in Section 7.3.3. An 
exposure in Utero form will then 
be forwarded for completion as 
soon as possible. All pregnancies 
must be followed up until 
conclusion and the outcome of 
the pregnancy reported within 24 
hours of awareness to 
 
Clinical Safety. Should the
outcome of the pregnancy meet 
criteria for an SAE, this should 
be reported as indicated in 
Section 7.3.3 
Updated guidelines for 
reporting of pregnancies 
and special situation 
reporting 
Section 7.3.6: Special 
Situation Reporting 
Blank 
Special situation reports include 
reports of overdose, misuse and 
abuse of the IMP: 
• Overdose: refers to the 
administration of a quantity of a 
Updated guidelines for 
reporting of pregnancies 
and special situation 
reporting 

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medicinal product given per 
administration or cumulatively 
(accidentally or intentionally), 
which is above the maximum 
recommended dose according to 
the protocol. Clinical Judgment 
should always be applied. In 
cases of a discrepancy in the 
drug accountability, overdose 
will be established only when it 
is clear that the subject has taken 
excess dose(s) or the investigator 
has reason to suspect that the 
subject has taken additional dose 
(s). 
• Misuse: refers to situations 
where the medicinal product is 
intentionally and inappropriately 
used not in a way that is not in 
accordance with the protocol 
instructions or local prescribing 
information and may be 
accompanied by harmful 
physical or psychological 
effects. 
• Abuse: is defined as persistent 
or sporadic, intentional 
excessive use of a medicinal 
product, which is accompanied 
by harmful physical and/or 
psychological effects. 
• Medication Error: Medication 
error is any unintentional error in 
the prescribing, dispensing or 
administration of a medicinal 
product by a healthcare 
professional, patient or 
consumer, respectively. The 
administration or consumption 
of the unassigned treatment and 
administration of an expired 
product are always reportable as 
medication errors, cases of 
subjects missing doses of 
investigational product are not 
considered reportable as 
medication error  
• All special situation reports 
must be reported on the special 
situations report form and 

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forwarded to 
 within 24 hour
All adverse events (AEs) 
associated with these special 
situation reports should be 
reported as AEs or SAEs at the 
same time using the AE eCRF 
and/or the SAE report form. 
Details of the symptoms and 
signs, clinical management and 
outcome will be reported, when 
available. 
Section 7.3.7 Serious 
Adverse Event 
Reporting 
Contact details are as 
follows: 
– 
New Zealand: 
Telephone: +
Facsimile: 
e-mai
 
 SAE hotline – 
Europe: 
Te ph ne: 
Fax: 
 
e-mail
Telephone: 
 
 or 
 
 
Fac m e: 
e-mail: 
Updated contact
information
for SAE repor n

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Section 7.5 Dose 
Modification Rules  
Table 2 Dose 
Modifications for 
Individual Subjects  
(was table 3 before) 
Assessment 
ALT, AST  
 
Event 
ALT or AST ≥ 3x ULN  
 
Timing  
Halt treatment then 
alert Sponsor (who will 
alert the DMc) and 
reassess after 1 week 
Response 
If ALT or AST and 
Total bilirubin 
normalize discuss next 
steps for resumption of 
treatment with Medical 
Monitor 
Assessment 
ALT, AST  
 
Event 
ALT or AST ≥ 8x ULN  
 
Timing  
Confirmed within 72 hours  
(ALT, ASP, ALT and Total 
Bilirubin should all be re-tested) 
 
Response 
Consider 
Discontinuation 
of 
Treatment 
Added and modified per 
FDA recommendation  
Section 7.5 Dose 
Modification Rules  
Table 2 Dose 
Modifications for 
Individual Subjects  
(was table 3 before) 
New Information 
Assessment 
ALT, AST  
Event 
ALT or AST ≥ 5x ULN  
Timing  
Confirmed within 2 weeks  
(ALT, ASP, ALT and Total 
Bilirubin should all be re-tested) 
Response 
Consider Discontinuation of 
Treatment 
Added per 
recommendation from the 
FDA 
Section 7.5 Dose 
Modification Rules  
Table 2 Dose 
Modifications for 
New Information 
Assessment 
ALT, AST , Total Bilirubin and 
INR 
Added per 
recommendation from the 
FDA 

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Individual Subjects  
(was table 3 before) 
Event 
ALT or AST ≥ 3x ULN  and 
Total Bilirubin > 2 x ULN or 
INR > 1.5 
Timing  
Confirmed within 72 hours 
(ALT, ASP, ALT and Total 
Bilirubin and INR should all be 
re-tested) 
Response 
Consider Discontinuation of 
Treatment 
Section 7.5 Dose 
Modification Rules  
Table 2 Dose 
Modifications for 
Individual Subjects  
(was table 3 before) 
New Information 
Assessment 
ALT, AST  
Event 
ALT or AST ≥ 3x ULN with the 
appearance of fatigue, nausea, 
vomiting, right upper quadrant 
pain or tenderness, fever, rash 
and / or eosinophilia (>5%) 
Timing  
Confirmed within 72 hours 
(ALT, ASP, ALT and Total 
Bilirubin should all be re-tested) 
Response 
Consider Discontinuation of 
Treatment 
Added per 
recommendation from the 
FDA 
Section 7.5.1 Dose 
modification / Stopping 
Rules for overall studies 
A Safety Review 
Committee (SRC) 
comprising the Study 
Director, Medical 
Monitor, at least one 
external expert 
nephrologist, and Study 
Statistician will review 
all safety events … 
A Safety Review Committee 
(SRC) comprising the Study 
Director, 
Medical 
Monitor, 
Clinical Pharmacologist, at least 
one external expert nephrologist, 
and 
Study 
Statistician 
will 
review all safety events … 
Addition of Clinical 
Pharmacologist to SRC to 
advise on 
pharmacokinetic findings 
and projections  
Section 7.7 
Concomitant 
Blank 
In addition, subjects taking 
strong CYP3A4 inhibitors (e.g 
Exclusion of CYP3A4 
inhibitors due to 

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Revised Text 
Rationale for Change 
Medications and 
Restrictions (and Time 
and Events table where 
applicable) 
 
 
 
 
 
 
 
 
 
 
 
Also, rituximab should 
be recorded up to 52 
weeks prior to 
screening Day 1 with 
evidence for recovery 
of CD20+ B cell 
population. 
boceprevir, clarithromycin, 
conivaptan, grapefruit juice, 
indinavir, itraconazole, 
ketoconazole, 
lopinavir/ritonavir, 
mibefradil,nefazodone, 
nelfinavir, posaconazole, 
ritonavir, saquinavir, telaprevir, 
telithromycin, and voriconazole) 
will be excluded as these drugs 
may substantially increase the 
CCX140-B plasma 
 
Prior treatment with rituximab or 
other anti-CD20 monoclonal 
antibodies and corresponding 
levels of CD20+ B cells need to 
be recorded up to 1 year prior to 
screening Day 1. Eligible 
patients who have entered the 
study on a continuous cycle 
therapy of rituximab or other 
anti-CD20 monoclonal 
antibodies need to remain on an 
unchanged therapy throughout 
the study at a dosing interval that 
has been documented to achieve 
continuous B cell depletion for 
the given patient unless 
prohibited due to safety 
considerations. Their levels of 
CD20+ B cells that are routinely 
evaluated per standard of care 
with anti-CD20 monoclonal 
antibody therapy need to be 
recorded throughout the study. 
Record UPCR and urine protein 
assessments up to 1 year prior to 
screening (if available) in the 
medical history if patients were 
treated with Rituximab, other 
CD20+ monoclonal antibodies 
or calcineurin inhibitors, or other 
immunotherapy. 
metabolism of CCX140-B 
 
 
 
 
 
Additional instructions 
needed because not all 
patients treated with 
rituximab or other anti-
CD20 monoclonal 
antibodies are excluded 
per revised exclusion 
criteria 
Additional instructions for 
the collection of levels of 
CD20+ B cells, UPCR 
and urine protein 
assessments due to 
revised exclusion criteria. 
Section 7.7 
Concomitant 
Medications and 
Restrictions (and Time 
and Events table where 
Blank 
Rescue Therapy:  
If the investigator decides that a 
subject needs to receive rescue 
therapy the subject will be 
Added per feedback from 
the FDA 

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Section(s) 
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Revised Text 
Rationale for Change 
applicable) 
considered a treatment failure. 
Rescue therapy is considered 
any new therapy or required 
dose increase of an existing 
concomitant therapy (other than 
allowed per section 7.7. 
Concomitant Medications and 
Restrictions) that is required for 
the safety of the patient and to 
treat the underlying disease of 
FSGS. 
This would include but is not 
limited to the initiation of high 
dose glucocorticoid therapy, new 
immunosuppressive agents such 
as calcineurin inhibitors, 
cyclophosphamide, MMF, 
monoclonal antibodies, or other 
major treatments (e.g. 
plasmapheresis, dialysis). 
Section 8.2.1: Primary 
Endpoint Analysis 
The primary efficacy 
endpoint is the 
proportion of subjects 
with partial or complete 
remission at any time 
during the treatment 
period.  
Partial Remission is 
defined as … 
Figure 1…. The 
number and percent of 
subjects with partial 
and complete response 
as of week 12 will be 
summarized including 
2-sided 90% and 95% 
confidence intervals 
(CIs). Statistical 
inference will be made 
based off of decision 
rules of the Fleming 
two-stage design. 
The Primary Efficacy endpoint 
is to evaluate the effect of 
CCX140-B on proteinuria in 
subjects with primary FSGS 
with nephrotic syndrome, 
assessed as a median reduction 
from baseline of urine protein to 
creatinine ratio (UPCR) of at 
least 20%, i.e. ≥ 20%, by week 
12. 
The assumed treatment effect for 
this study is based on efficacy 
findings with 5 and 10 mg 
CCX140 once daily in study 
CL005_140 in patients with 
diabetic nephropathy and 
proteinuria. In a pre-specified 
subset analysis, the greatest 
percentage improvement of 
UACR at approximately 12 
weeks from baseline was 
observed in subjects who 
presented with the highest 
baseline UACR (801-3000 
mg/g) and eGFR of at least 60 
mL/min/1.73m2. This subgroup 
receiving 5 (N=10) or 10 mg 
(N=13) of CCX140 once daily 
showed a median reduction in 
UACR of approximately 20%-
30% receiving CCX140 while 
Change primary endpoint 
“partial and complete 
remission” to secondary 
endpoint and evaluate 
“median reduction of 
UPCR” as primary 
efficacy instead. Existing 
text is modified or deleted 
accordingly. 
This change is based on 
discussions with lead 
study investigators and is 
based on previously 
observed treatment effects 
of CCX140-B for the 
reduction of proteinuria in 
subjects with diabetic 
nephropathy. 

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Section(s) 
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Revised Text 
Rationale for Change 
patients receiving placebo 
(N=12) showed an increase of 
approximately 12%. 
Based on these data, it is 
assumed that the median 
reduction from baseline UPCR is 
at least 20% by week 12 in the 
planned study CL012_140.  If 
the final observed data are 
consistent with this assumption, 
i.e. the previous positive study 
results are replicated in the 
FSGS population, the study will 
be considered positive in 
assessing the efficacy of 
CCX140. 
Otherwise, if the observed data 
are consistent with the median 
reduction of below 20%, the 
study would not be considered 
positive. 
Section 8.2.2: 
Secondary Endpoint 
Secondary endpoints 
include: 
• Change from  baseline 
of UPCR in subjects 
treated with CCX140-B 
over time … 
 
 
 
 
 
 
 
 
 
 
Secondary endpoints include: 
• Partial Remission (includes all 
of the following)  
- reduction from baseline by ≥50 
percent in UPCR  
- reduction in UPCR to a level 
that is < 3.5 g/g at week 12 
- subject may not be a treatment 
failure (e.g. has not received 
rescue therapy, has not required 
discontinuation of study drug) 
• Complete Remission (includes 
all of the following) at week 12 
- reduction in UPCR to < 0.3 g/g 
- serum albumin within normal 
range 
- for patients with abnormal 
serum creatinine levels at 
baseline, return to normal levels 
for that age group 
The definition of previous 
primary endpoint “partial 
and complete remission” 
was moved to secondary 
endpoint section because 
the primary endpoint was 
changed as described 
above. 
 
 
 
 
 
 
 
 
 
 

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Section(s) 
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Revised Text 
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The proportion with 
partial remission 
defined as reduction 
from baseline by ≥50 
percent in UPCR, 
normalization of serum 
albumin and subject 
may not be a treatment 
failure (e.g. has not 
received rescue therapy, 
has not required 
discontinuation of study 
drug) 
• The proportion with 
complete remission at 
any time during the 
treatment period, 
defined as achievement 
of UPCR ≤ 0.3 g/g and 
normal eGFR or no 
progression of renal 
failure 
- for patients with normal serum 
creatinine levels at baseline, 
final value within 20% of 
baseline levels 
- subject may not be a treatment 
failure (e.g. has not received 
rescue therapy and has not 
required discontinuation of study 
drug) 
• Change from baseline of 
UPCR in subjects treated with 
CCX140-B over time … 
• The proportion of patients with 
partial remission at any time 
during the treatment period 
• The proportion of patients with 
complete remission at any time 
during the treatment period 
 
 
 
 
 
 
 
 
Deleted inconsistencies 
within definitions of 
partial and complete 
remission 
 
Section 8.2.2: 
Secondary Endpoint 
Analysis 
Change from baseline 
in renal function, based 
on eGFR (calculated 
with the CKD EPI 
equation using the 
CKD-EPI Cystatin C 
equation, CKD-EPI 
Creatinine equation, 
CKD-EPI Creatinine-
Cystatin C equation and 
MDRD Creatinine 
equation over time 
Change from baseline in renal 
function, based on eGFR 
(calculated using the CKD-EPI 
Cystatin C equation 
(Dharnidharka VR, et al, 2002), 
the CKD-EPI Creatinine 
equation (Schwartz GJ, et al. 
2016, Selistre L et al 2016), 
CKD-EPI Creatinine-Cystatin C 
equation (Stevens et al., 2008) 
and MDRD Creatinine equation 
(Levey et al. 1999, Levey et al. 
2006) over time 
Added references for each 
of the 4 eGFR equations 

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Section(s) 
Original Text 
Revised Text 
Rationale for Change 
Section 8.4: Sample 
Size Assumptions 
Six (6) male or female 
adult subjects with 
biopsy-proven primary 
FSGS and nephrotic 
syndrome will be 
enrolled into the first 
stage of this study. 
Analysis of data in this 
study will follow the 
Fleming’s two-stage 
design. The null 
hypothesis that the true 
response (partial or 
complete remission) 
rate is 0.25 will be 
tested against a one-
sided alternative 
assuming a true 
response rate of 0.65. In 
the first stage, 6 
subjects will be treated. 
After all of the initial 6 
subject have completed 
their 12-week of 
treatment the study will 
be paused and assessed 
safety and efficacy. If 
the overall study results 
are deemed safe and the 
subjects’ responses to 
treatment are 
considered clinically 
meaningful seven 
additional subjects will 
be treated for a total of 
13 subjects. The null 
hypothesis will be 
rejected if 6 or more 
responses are observed 
in 13 subjects. This 
design yields a one-
sided type I error rate of 
0.05 and 80% power. 
Six (6) male or female adult 
subjects with biopsy-proven 
primary FSGS and nephrotic 
syndrome will be enrolled into 
the first stage of this study, 
which is followed by another 
seven subjects after the data 
review of the initial 6 patients. 
The relatively small sample size 
is selected based on the 
exploratory safety and efficacy 
nature of the study. After all of 
the initial 6 subject have 
completed their 12-week of 
treatment the study will be 
paused and assessed safety and 
efficacy. If the overall study 
results are deemed safe and the 
subjects’ responses to treatment 
are considered clinically 
meaningful seven additional 
subjects will be treated for a 
total of 13 subjects.  
This section was updated 
to reflect changes in the 
primary and secondary 
endpoints and to allow for 
the exploration of safety 
and efficacy in the study 
more appropriately. 
Section 10.8: Case 
Report Form 
Completion 
It is the policy of the 
Sponsor that study data 
must be verifiable to the 
source data, which 
necessitates access to 
all original recordings, 
laboratory reports, and 
subjects’ records. 
It is the policy of the Sponsor 
that study data must be verifiable 
to the source data, which 
necessitates access to all relevant 
source documents, laboratory 
reports, and subjects’ records. 
Although original 
documentation is the 
primary source, certified 
copies from an EMR 
captured in each subject’s 
printed chart can be 
utilized for SDV. 

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Section(s) 
Original Text 
Revised Text 
Rationale for Change 
References 
Blank 
Added reference #s  
13 (Dharnidharka VR, et al. 
(2002) 
22 Levey AS, et al. (1999) 
23 Levey AS, et al. (2006) 
26 Schwartz GJ, et al. (2009) 
27 Selistre L, et al. (2016) 
Added references for the 
4 different eGFR 
calculations utilized for 
this study 
Appendix A 
Statement of 
Obligations of Sponsor, 
Monitor and Clinical 
Investigator, 
Removed 
The Statement of 
Obligations is redundant 
with language on  FDA 
Form 1572 and is 
therefore not required as 
an appendix in the 
protocol 

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TABLE OF CONTENTS 
INVESTIGATOR SIGNATORY PAGE .........................................................................................2 
SPONSOR CONTACT INFORMATION .......................................................................................3 
SPONSOR SIGNATURE FOR APPROVAL .................................................................................4 
PROTOCOL AMENDMENT 1.0: SUMMARY OF CHANGES ...................................................5 
I. STUDY SYNOPSIS ...................................................................................................................38 
II. TIME AND EVENTS TABLE .................................................................................................53 
III. LIST OF ABBREVIATIONS AND ACRONYMS ................................................................56 
1. 
INTRODUCTION ......................................................................................................58 
1.1. 
Background Information .............................................................................................58 
1.2. 
Potential Role of CCR2 in Pathogenesis of FSGS .....................................................58 
1.3. 
CCR2 Inhibitor CCX140-B: Non-Clinical Safety ......................................................60 
1.4. 
CCR2 Inhibitor CCX140-B: Clinical Experience ......................................................61 
1.5. 
Rationale for Study in FSGS: Unmet Medical Need ..................................................62 
1.6. 
Risk-Benefit Assessment ............................................................................................62 
2. 
OBJECTIVES .............................................................................................................63 
2.1. 
Primary Efficacy Objective ........................................................................................63 
2.2. 
Secondary Efficacy Objectives ...................................................................................63 
2.3. 
Safety Objectives ........................................................................................................64 
2.4. 
Exploratory Objectives ...............................................................................................64 
3. 
STUDY DESIGN AND DOSING REGIMEN ..........................................................65 
3.1. 
Initial Treatment Phase ...............................................................................................65 
3.1.1. 
Day 85 and Beyond ....................................................................................................66 
3.1.2. 
Extended Treatment Period ........................................................................................66 
3.1.3. 
Follow-Up ...................................................................................................................67 
3.2. 
Dose Modification Based on PK Exposure ................................................................67 
3.3. 
Dose Modifications During the Extended Treatment Period .....................................68 
4. 
STUDY POPULATION .............................................................................................68 
4.1. 
Size of Population .......................................................................................................68 
4.2. 
Inclusion Criteria ........................................................................................................69 
4.3. 
Exclusion Criteria .......................................................................................................69 
5. 
STUDY TREATMENTS............................................................................................72 

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5.1. 
Product Characterizations ...........................................................................................72 
5.2. 
Rationale for Dose Range ...........................................................................................72 
5.2.1. 
Safety ..........................................................................................................................72 
5.2.2. 
Efficacy .......................................................................................................................74 
5.3. 
Drug Supply ................................................................................................................74 
5.3.1. 
Packaging and Labeling ..............................................................................................74 
5.3.2. 
Storage ........................................................................................................................74 
5.3.3. 
Blinding ......................................................................................................................74 
6. 
STUDY PROCEDURES ............................................................................................75 
6.1. 
Screening Assessments (Day-28 to Day 0) ................................................................75 
6.2. 
Study Day 1 (After Confirmation of Eligibility) ........................................................75 
6.3. 
Study Day 15 (Safety, Efficacy and PK) ....................................................................76 
6.4. 
Study Day 29 (Safety, Efficacy and PK) ....................................................................77 
6.5. 
Study Day 43- evaluate results from Day 29 (modify dose per dose 
modification guidelines based on PK results) ............................................................77 
6.6. 
Study Day 57( +/- 3 Days)-Safety, Efficacy, PK........................................................78 
6.7. 
Study Day 71( +/- 3 Days)-Safety, Efficacy, PK........................................................78 
6.8. 
Study Week 12 or Day 85 (+/- 3 days) (Safety & Efficacy)- See Section 6.19 
for PK elimination visit guidelines .............................................................................79 
6.9. 
Week 16 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........80 
6.10. 
Week 20 (+/- 1 week) .................................................................................................80 
6.11. 
Week 24 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........81 
6.12. 
Week 28 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........82 
6.13. 
Week 32 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........82 
6.14. 
Week 36 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........83 
6.15. 
Week 40 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........84 
6.16. 
Week 44 (+/-1 week) (See Section 6.19 for PK elimination visit guidelines) ...........84 
6.17. 
Week 48 (+/- 1 week) (See Section 6.19 for PK elimination visit guidelines) ..........85 
6.18. 
Week 52 or Termination Visit (See Section 6.19 for PK elimination visit 
guidelines) ...................................................................................................................86 
6.19. 
Post Treatment Elimination Blood Draws ..................................................................86 
6.20. 
Follow-Up Visits - Four Weeks After Final Dose of Study Medication For 
Any Reason .................................................................................................................86 
7. 
STUDY ASSESSMENTS ..........................................................................................87 

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7.1. 
Physical Examinations, Vital Signs, and ECGs..........................................................87 
7.2. 
Clinical Safety Laboratory Assessments ....................................................................87 
7.3. 
Adverse Events ...........................................................................................................88 
7.3.1. 
Adverse Event Severity Assessment ..........................................................................89 
7.3.2. 
Causality Assessment .................................................................................................89 
7.3.3. 
Serious Adverse Events ..............................................................................................89 
7.3.4. 
SARs and SUSARs .....................................................................................................90 
7.3.5. 
Pregnancies .................................................................................................................90 
7.3.6. 
Special Situation Reporting ........................................................................................90 
7.3.7. 
Serious Adverse Event Reporting ...............................................................................91 
7.4. 
Study Completion and Withdrawal ............................................................................92 
7.5. 
Dose Modification Rules ............................................................................................92 
7.5.1. 
Dose Modification / Stopping Rules for the Overall Study ........................................94 
7.5.2. 
Drug Accountability ...................................................................................................94 
7.6. 
Treatment Compliance ................................................................................................94 
7.7. 
Concomitant Medications and Restrictions ................................................................95 
7.7.1. 
Rescue Therapy ..........................................................................................................95 
8. 
STATISTICAL METHODS .......................................................................................96 
8.1. 
Demographics and Baseline Characteristics ...............................................................96 
8.2. 
Efficacy Analysis ........................................................................................................96 
8.2.1. 
Primary Endpoint Analysis .........................................................................................96 
8.2.2. 
Secondary Endpoint Analysis .....................................................................................97 
8.3. 
Safety Analysis ...........................................................................................................98 
8.4. 
Sample Size Assumptions...........................................................................................99 
8.5. 
Subject Populations ....................................................................................................99 
8.5.1. 
Modified Intent-to-Treat Population ...........................................................................99 
8.5.2. 
Per Protocol Population ............................................................................................100 
8.5.3. 
Safety Population ......................................................................................................100 
8.6. 
Demographics and Baseline Characteristics .............................................................100 
8.7. 
Subject Disposition ...................................................................................................100 
8.8. 
Treatment Compliance ..............................................................................................100 
8.9. 
Protocol Deviations ..................................................................................................100 
8.10. 
Prior and Concomitant Medications .........................................................................101 

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8.11. 
Pharmacokinetic and Pharmacodynamic marker analysis ........................................101 
9. 
STUDY COMPLETION AND TERMINATION ....................................................102 
9.1. 
Study Completion .....................................................................................................102 
9.2. 
Study Termination ....................................................................................................102 
10. 
REGULATORY AND ADMINISTRATIVE REQUIREMENTS ..........................102 
10.1. 
Investigator Responsibilities .....................................................................................102 
10.2. 
Institutional Review Board or Ethics Committee .....................................................102 
10.3. 
Informed Consent .....................................................................................................103 
10.4. 
Protocol Modifications .............................................................................................103 
10.5. 
Regulatory Documentation .......................................................................................103 
10.6. 
Subject Identification Register .................................................................................104 
10.7. 
Record Retention ......................................................................................................104 
10.8. 
Case Report Form Completion .................................................................................104 
10.9. 
Monitoring ................................................................................................................104 
10.10. 
On-site Visits and Audits ..........................................................................................105 
10.11. 
Use of Information and Publication ..........................................................................105 
11. 
REFERENCES .........................................................................................................106 
APPENDIX A. Subject Instructions ............................................................................................109 
Subject Instructions for Collection of 24 Hour Urine Samples ...................................................109 
Subject Instructions for Dosing of Study Medication on Visit Days...........................................109 
 
LIST OF TABLES 
Table 1: 
Dose Modification Schedule and Rules Through Study Day 85 ................................42 
Table 2: 
Dose Modification Rules for Individual Subjects ......................................................92 
 
LIST OF FIGURES 
Figure 1: 
Study Schematic .........................................................................................................52 
 

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I. STUDY SYNOPSIS 
Name of Sponsor 
ChemoCentryx, Inc. 
Name of Active 
Ingredient 
CCX140-B 
Study number  
CL012_140 
Title 
An Open-Label, Intra-Subject Dose Escalation Study of CCX140-B in Subjects with Primary 
Focal Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome 
Investigators 
TBD 
Study centers 
Up to 10 sites planned in North America and Europe  
Primary Study Period 
• Participation time for each subject: 
o Up to 4 weeks screening 
o Up to 12 weeks initial treatment 
o 4 weeks follow-up after last dose administered 
Extended Study Period 
• Subjects who meet pre-defined response criteria at 12 weeks may 
enter an extended treatment period for up to an additional 40 weeks 
Phase of 
development 
Phase 2 
Efficacy Objectives and Endpoints 
The Primary Efficacy Objective is to evaluate the effect of CCX140-B on proteinuria in 
subjects with primary FSGS with nephrotic syndrome, assessed as a median reduction from 
baseline of urine protein to creatinine ratio (UPCR) of at least 20%, i.e. ≥ 20%, by week 12. 
Secondary efficacy endpoints of this study will be assessed through Study Week 12 and through 
End of Treatment and include: 
• Achievement of partial or complete remission of urine protein to creatinine ratio (UPCR): 
Partial Remission (includes all of the following) 
• reduction from baseline by ≥50 percent in UPCR 
• reduction in UPCR to a level that is < 3.5 g/g 
• subject may not be a treatment failure 
Complete Remission (includes all of the following) 

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• reduction in UPCR to < 0.3 g/g 
• Serum albumin within normal range 
• For patients with abnormal serum creatinine levels at baseline, return to normal levels for 
that age group 
• For patients with normal serum creatinine levels at baseline, final value within 20% of 
baseline levels 
• subject may not be a treatment failure 
• Change from baseline in UPCR over time 
• Time to and proportion with achievement of partial remission during the treatment period 
• Time to and proportion with achievement of complete remission during the treatment period 
• Time to rescue therapy, based on Investigator or physician initiation of glucocorticoids or 
new immunosuppressive agents or new major treatment modalities (e.g. plasmapheresis, 
dialysis) 
• Changes over time in other laboratory and other parameters related to renal function, 
including: 
− Serum albumin 
− Creatinine 
− Cystatin C 
− eGFR, calculated by the CKD-EPI Cystatin C equation, CKD-EPI Creatinine equation, 
CKD-EPI Creatinine-Cystatin C equation and MDRD Creatinine equation. 
− urine albumin to creatinine ratio (UACR) 
− total 24 hour urine protein excretion 
Quality of Life endpoints include: 
• Change in factors associated with quality of life from baseline over time as assessed using 
the SF-36 v2 
• Change in factors associated with quality of life from baseline over time as assessed using 
the EQ-5D-5L 
Safety will be evaluated via the following endpoints: 
• Adverse events 
• Vital signs 
• Electrocardiograms 
• AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
• Changes in laboratory parameters including: 

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− hematology, including complete blood count, reticulocyte count, smear evaluation 
− serum haptoglobin, lactate dehydrogenase 
− serum chemistry, expanded to include serum transaminases, CPK 
− urinalysis 
− coagulation factors 
Pharmacokinetics will be evaluated based on: 
Exposure (AUC, Cmax, Cmin) 
 
Exploratory objectives include: 
• Assessment of biomarkers associated with disease activity over time, including blood lipid 
profile, urinary MCP-1, and potentially other biomarkers of renal inflammation, fibrosis and 
injury; 
• Assessment of biomarkers associated with risk of severity of disease, which may include 
allelic variations associated with the pathogenesis and/or prognosis of FSGS such as 
NPHS1, NPHS2, WT-1, LAMB2, CD2AP, TRPC6, ACTN4, INF2, APOL1. 
• Assessment of blood and/or urinary biomarkers associated with CCR2 biology, which may 
include peripheral blood leukocyte subsets and selected cytokines and chemokines 
• Assessment of mechanisms by which nephrotic syndrome may potentially impact 
pharmacokinetics of CCX140, including assessment of CCX140 in urine 
• Assessment of edema as measured by physical examination, leg circumference and body 
weight 
• Assessment of concentration of CCX140-B in urine 
 
Background 
Focal segmental glomerulosclerosis (FSGS) may present with nephrotic syndrome, with marked 
proteinuria, reduced plasma albumin, edema, hyperlipidemia and a high risk for progression to 
end stage renal disease. Current therapeutic recommendations include supportive measures and 
consideration of a trial of high dose glucocorticoids. Glucocorticoid treatment for durations of 4 
to 24 months has been reported to achieve partial remission in 28% to 74% of patients, but is 
associated with significant morbidity, incomplete response in most patients, and a high risk of 
recurrence following withdrawal (Korbet et al., 2012; Banfi et al., 1991; Cattran and Rao, 1998; 
Rydel et al., 1995; Gipson et al., 2016). 
CCR2 has emerged as a potential target for treatment of FSGS based on (a) expression of CCR2 
in diseased kidney, (b) association between disease activity and elevated urinary levels of 
macrophage chemo-attractant protein-1 (MCP-1, ligand of CCR2), and (c) efficacy of CCR2 
blockade in animal models with features of FSGS. 
CCX140-B is an orally administered, selective antagonist of CCR2, which has exhibited 
efficacy in murine models of renal injury. In patients with diabetic nephropathy, administration 

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of CCX140-B at 5 mg daily or 10 mg daily for up to 52 weeks was associated with a favorable 
safety profile and a significant reduction from baseline in proteinuria (Study CL005_140; de 
Zeeuw, et al., 2015). 
Based on the preclinical rationale, favorable safety profile, and signals of efficacy in diabetic 
subjects with marked proteinuria, ChemoCentryx plans to assess CCX140-B in FSGS. 
Methodology 
This is an open label clinical trial to test the efficacy, safety, pharmacokinetics and tolerability 
of ascending doses of CCX140-B in subjects with primary FSGS and nephrotic syndrome. 
Throughout the treatment period, any changes in background medications should be limited to 
settings in which the Investigator deems it to be necessary for the safety of the subject. 
Eligibility requirements are detailed in the inclusion and exclusion criteria. 
The screening period will be up to 28 days. 
Dose and Dose Adjustments 
Eligible subjects will start treatment with CCX140-B at 5 mg twice daily. CCX140-B will be 
taken orally without food, at least one hour before a meal. The dose may be increased in a 
stepwise manner to 10 mg twice daily on day 15, then to 15 mg twice daily on day 43 while 
monitoring efficacy, PK and safety. Dose escalations will occur in accordance with the rules 
and schedule detailed in Table 1, Figure 1 and Section 7.5.1. Dose may be adjusted downward 
in the event of intolerance, laboratory evidence of toxicity, or a measured or projected steady 
state exposure that exceeds the pre-specified maximum safe exposure based on the No 
Observed Adverse Event Level (NOAEL) established in the non-clinical toxicology program. 
In all subjects, treatment at the selected dose will be continued through 12 weeks or until 
declaration of Treatment Failure, where Treatment Failure is defined as any of the following: 
1) Progression of renal disease, defined as eGFR that is both below 60 ml/min/1.73 m2 and is 
confirmed to represent at least a 30% decline in eGFR from baseline; 
2) Requirement for rescue with glucocorticoids, other new immunomodulatory or 
immunosuppressive therapy, plasmapheresis, or dialysis, per the judgment of the 
Investigator; 
3) Pre-specified adverse event or laboratory abnormality that, per Protocol, requires permanent 
discontinuation of study CCX140-B 
4) Any other treatment-related adverse event, laboratory evidence of toxicity, or intolerance 
that, in the judgment of the Investigator, warrants permanent discontinuation of CCX140-B. 
 
If planned dose escalation to 10 mg (on day 15) or 15 mg twice daily (on day 43) was 
previously halted the dose may be escalated further if subjects are not considered treatment 
failures and who otherwise meet criteria for continuation. In such case the subject will return 
approximately 2 weeks later for blood draws to assess PK (AUC0-6). Subjects will return 2 
weeks after the PK assessment to evaluate the safety and need for further dose adjustment. 
Subjects who completed 12 weeks of treatment are not considered treatment failures and who 
otherwise meet criteria for continuation may be extended for up to an additional 40 weeks to 
assess degree and duration of response. During the extension period the dose may be adjusted if 
indicated based on assessment of safety, PK and efficacy. (see Table 1 and Figure 1 for 

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guidelines). 
Starting with the extended treatment period (Day 85 and beyond) if the dose is adjusted upward 
or downward for any reason at Day 85 or during the extended treatment period the subject will 
return approximately 2 weeks later for blood draws to assess PK (AUC 0-6)  
Table 1: 
Dose Modification Schedule and Rules Through Study Day 85 
Study Day 
Dose and Requirements for Dose Modification 
1 
Initiate dosing at 5 mg twice daily 
15 
At Day 15, dose will be increased to 10 mg twice daily if all of the 
following are true: 
• Subject has not achieved and maintained at least Partial Remission 
and 
• Subject is not a Treatment Failure 
29 
At Day 29, PK samples will be collected to measure AUC0-6 and enable 
projection of AUC0-12 at both the current dose level and the next higher 
planned dose level AUC0-12 
43 
At Day 43 based on results of the Day 29 PK, the dose may be adjusted 
to the next higher planned dose level (15 mg twice daily for subjects 
receiving 10 mg twice daily; 10 mg twice daily for subjects receiving 
5 mg twice daily) if all of the following are true: 
• Subject is not a Treatment Failure, and 
• Meets all criteria for continuation: 
o Subject has not achieved and maintained at least a Partial 
Remission, and 
o Projected AUC0-12 on the next higher planned dose level does 
not exceed 240 µg•h/mL and 
o The next higher dose does not exceed the Maximum 
Tolerated Dose (MTD) 
NOTE: If the projected AUC0-12 on the current dose exceeds 
240 µg•h/mL, dose will be de-escalated to the next lower dose level 
57 
At Day 57, PK samples will be collected to measure AUC0-6 and enable 
projection of AUC0-12 
Day 71 
Based on results of Day 57 PK, subjects can be considered for dose 
escalation if all of the following are true: 
• Subject is not already treated with 15 mg twice daily and  
• Subject did not require previous downward dose adjustment and 
• Subject is not a Treatment Failure, and 
• Meets all criteria for continuation: 
o Subject has not achieved and maintained at least a Partial 

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Remission, and 
o Projected AUC0-12 on the next higher planned dose level does 
not exceed 240 µg•h/mL and 
o The next higher dose does not exceed the Maximum 
Tolerated Dose (MTD) 
NOTE: If  the projected AUC0-12 on the current dose exceeds 240 
µg•h/mL, dose will be de-escalated to the next lower dose level 
Day 85 and 
beyond 
If dose is adjusted upward or downward for any reason at Day 85 or 
during the extended treatment period the subject will return 
approximately 2 weeks later for blood draws to assess PK (AUC0-6). 
Subjects will return 2 weeks after the PK assessment to evaluate the 
safety and need for further dose adjustment. 
Blood will be drawn to evaluate trough exposure in accordance with the Time and Events 
Schedule. In addition, blood will be drawn to assess exposure (AUC0-12), extrapolated from 
measured AUC0-6), at the following times to guide dosing decisions: 
• Study Day 1 prior to first dose of CCX140, and at intervals through 6 hours following the 
initial dose 
• On study Day 29, two weeks following initiation of 10 mg twice daily, to assess exposure at 
steady state, and to confirm that exposure is within the safety margins based on non-clinical 
NOAEL 
• If dose was adjusted on Day 43, blood will be collected on study Day 57 to assess AUC0-6 
• Additional assessments of exposure may be scheduled if indicated. Indications may include: 
o Subjects receiving a total dose greater than 10 mg daily who have experienced at 
least 50% reduction in proteinuria since the previous PK assessment, to assess 
impact of change in proteinuria on exposure and to ensure exposure is within safety 
margins 
o Subjects who experienced a potentially dose-limiting safety event 
o Subjects who required a dose reduction for higher than planned exposure; PK will be 
assessed approximately 2 weeks after reduction 
o Subjects for whom dose is adjusted for any other reason; PK will be assessed 
approximately 2 weeks after reduction 
If measured or projected steady state PK exposure (AUC0-12) exceeds 240 µg•h/mL, which is 
approximately half of the rat NOAEL AUC0-24, the dose will be reduced to the dose projected to 
provide exposure within the target range. 
 
Extended Treatment Period 
• Subjects are eligible for the extended treatment period under the following conditions: 
1. Not considered treatment failures as of Week 12 and 
2. In the opinion of the Investigator, remain candidates for investigational treatment 
and 
3. Any of the conditions below apply: 

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• Partial or Complete Remission by Week 12 
• Response with ≥20% reduction in UPCR by Week 12 
• No longer have nephrotic syndrome 
These subjects will return to clinic for safety, PK and efficacy measurements every 4-8 weeks. 
Subjects taking CCX140-B at 15 mg twice daily will be maintained at that dose unless de-
escalation is required based on safety signal or steady state PK exposure (AUC0-12) exceeds 
240 µg•h/mL. 
 
Subjects can be considered for dose escalation if all of the following are true: 
• Subject is not already treated with 15 mg twice daily and 
• Subject did not require previous downward dose adjustment and 
• Subject is not a Treatment Failure, and 
• Meets all criteria for continuation: 
o Subject has not achieved and maintained at least a Partial Remission, and 
o Projected AUC0-12  on the next higher planned dose level does not exceed 240 
µg•h/mL and 
o The next higher dose does not exceed the Maximum Tolerated Dose (MTD) 
Subjects who undergo dose escalation will return to clinic approximately 2 weeks after 
initiation of the increase for assessment of PK. 
Dose modification rules that applied during the initial dose escalation period also apply to dose 
escalation after 12 weeks. 
Follow-Up 
Following completion of treatment or early termination of treatment all subjects should be 
encouraged to return for the termination visit, and 7 and 14 days after their last dose for blood 
draws to assess elimination pharmacokinetics, and 28 days after their last dose for follow-up 
and End of Study visit assessments. The subject’s condition will be evaluated by the 
Investigator at the end of study drug administration and again during the post 4 week follow-up 
visit. Appropriate standard of care medical treatment should be provided to all subjects as 
needed. 
Subjects will be discharged from the study when all the visit procedures have been completed. 
Rationale for Dose Range 
Safety: 
A dose of 15 mg twice daily in humans is projected to produce a steady state AUC0-12 of 
72 μg•hr/mL, based on modeling from Phase 1 PK data. The safety margin associated with this 
exposure is approximately 3-fold below the NOAEL exposure in rats, and 5-8-fold below the 
NOAEL exposure established in dogs. 
 
Efficacy: 
This study is designed to test CCX140-B at doses ranging from 5 mg twice daily through 15 mg 
twice daily, a dose projected to provide continuous high level receptor engagement and 

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antagonism. 
The targeted dose was projected from observations in a murine model with features of FSGS, 
the adriamycin-induced nephropathy mouse model (Lee, et al., 2011). CCX140-B does not bind 
murine CCR2 and human CCR2 with the same potency, therefore the model was established in 
a CCR2 knockout and human CCR2 knock-in (hCCR2-KI) mouse. Mice were treated with 10, 
30, and 90 mg/kg of CCX140-B once daily for 9 days. The 24 hour post last dose blood 
samples from 90 mg/kg dosing resulted in close to 100% receptor coverage. The mean 
CCX140-B concentration at trough was 3.3 µg/mL (6.6 µM). 
In the absence of proteinuria, the human trough concentration with 15 mg twice daily dosing is 
projected to be ~5.5 µg/mL, which is higher than the 3.3 µg/mL required for 100% CCR2 
blockade in the above described hCCR2 mouse model. However, the projected exposures and 
safety margins were established for humans without proteinuria. Because CCX140-B is highly 
protein bound, subjects with nephrotic-level proteinuria may have a lower exposure at a given 
dose due to potential drug loss with protein in urine or impact of disease on metabolism. A post-
hoc analysis of PK in diabetic nephropathy subjects of trial CL005_140 who entered in study 
with albuminuria of 2 – 3 grams indicated that exposure was approximately 30% below the 
mean for the treatment group as a whole (~0.8 µg/mL versus ~1.2 µg/mL). 
Therefore 15 mg twice daily was selected as the upper limit for dose ranging for subjects with 
nephrotic level proteinuria. 
Dose Modification Rules 
Dose Modification Rules for individual subjects 
Discontinue treatment with CCX140-B when a subject experiences any of the following events 
confirmed by repeat measurement after 2 weeks: 
• Progression of renal disease, defined as eGFR that is both below 60 ml/min/1.73 m2 and is 
confirmed to represent at least 30% decline in eGFR from baseline 
• Requirement for rescue with glucocorticoids, other new immunomodulatory or 
immunosuppressive therapy, plasmapheresis or dialysis 
• Pre-specified adverse event or laboratory abnormality that, per Protocol, requires permanent 
discontinuation of study CCX140-B 
• Any other treatment-related adverse event, laboratory evidence of toxicity or intolerance 
that in the judgment of the investigator, warrants permanent discontinuation of CCX140-B 
Halt treatment then alert sponsor and reassess after 1 week when there is a Clinical diagnosis of 
hemolysis in the judgment of the investigator, based on elevation of LDH, elevation of 
reticulocyte count, elevation of bilirubin and or other laboratory indications. 
 
Consider discontinuation of treatment when a subject experiences any of the following events: 
An increase of serum ALT to >3xULN should be followed by repeat testing within 48 to 72 
hours of all four of the usual serum measures (ALT, AST, ALP, and TBL) to confirm the 
abnormalities and to determine if they are increasing or decreasing. 

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Discontinuation of treatment should be considered if: 
• 
ALT or AST >8xULN 
• 
ALT or AST >5xULN for more than 2 weeks 
• 
ALT or AST >3xULN and (TBL >2xULN or INR >1.5) 
• 
ALT or AST >3xULN with the appearance of fatigue, nausea, vomiting, right upper 
quadrant pain or tenderness, fever, rash, and/or eosinophilia (>5%) 
 
Halt treatment then alert sponsor and reassess after 2 weeks whenever a subject has either 
• Hemoglobin reduced from baseline by ≥ 2 g/L and deemed clinically significant by the PI 
• An increase in reticulocyte count by ≥ 2% (absolute) from baseline that is not otherwise 
explained 
• ≥ moderate symptoms limiting instrumental activities of daily living or asymptomatic 
(based on diagnostic evaluation only) and deemed clinically significant by the investigator 
• Any adverse events assessed as moderate, related to study drug, and clinically significant 
 
Dose Modification / Stopping Rules for the overall study 
A dose will be considered as potentially exceeding the maximum tolerated dose should any of 
the following events occur: 
• 2 or more subjects with confirmed QTcF prolongation ≥ 450 msec and an increase from 
baseline of ≥ 30 msec, based on triplicate 12-lead ECGs that is not otherwise explained 
• 2 or more subjects with clinically significant hemolytic anemia, defined as hemoglobin that 
has declined from baseline by at least 2 grams/deciliter and is less than or equal to 8 g/dL 
and is assessed by the investigator and is not otherwise explained 
• 2 or more subjects with bilirubin greater than 3 times upper limit of normal, and not 
ascribable to Gilbert’s disease 
• 2 or more subjects with new motor neuropathy or sensory neuropathy that limits 
instrumental activities of daily living and is assessed by the Investigator as clinically 
significant 
• 2 or more subjects who require dose reduction or discontinuation due to adverse events 
assessed as possibly related to Study Drug, where the adverse events are medically similar 
events in the same System Organ Class based on MedDRA criteria 
If a dose is assessed to be above the maximum tolerated dose, dosing will be reduced to the next 
lower dose. 
A Safety Review Committee (SRC) comprising the Sponsor Study Director, Medical Monitor, 
at least one external expert nephrologist, and the Study Statistician will review all safety events 
described in the dose modification rules and all serious adverse events as they occur. The SRC 
will periodically review all adverse events and all laboratory abnormalities. The SRC may 
declare a dose to be above the maximally tolerated dose based either on the defined rules or on 
other considerations. 

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Number of Subjects 
Six (6) male or female adult subjects with biopsy-proven primary FSGS and nephrotic 
syndrome will be enrolled in the first stage of this study. An additional 7 subjects may be 
enrolled in the second stage of this study based on observations in the first 6 subjects, as 
described in the sample size section of this protocol. 
Main Criteria for Inclusion 
1. Male or female subjects aged 18 years and older 
2. Renal biopsy findings consistent with diagnosis of primary focal segmental 
glomerulosclerosis (FSGS) and consistent with primary FSGS based on presentation of 
histopathology, medical history and clinical course; subjects with genetic risk factors with 
presentations that are otherwise consistent with primary FSGS may also be enrolled. 
3. Urinary total protein:creatinine ratio (UPCR) ≥3.5 g protein/g creatinine at screening, based 
on sample drawn from a 24-hour collection 
4. Hypoalbuminemia of less than 3.5 g/dL 
5. Estimated glomerular filtration rate (eGFR) >30 mL/min/1.73m2, as calculated using the 
Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation (using creatinine 
or cystatin C) 
6. If used, RAAS inhibitor dose, including doses of angiotensin converting enzyme inhibitor 
and/or angiotensin II receptor antagonist, must have been started at least 4 weeks prior to 
Study Day 1, and projected to remain stable throughout the course of the study unless 
adjustment is required for management of hypertension 
7. Female subjects of childbearing potential may participate if adequate contraception is used 
during, and for at least 5 half-lives after last dose of study drug. Male subjects with partners 
of childbearing potential may participate in the study if they had a vasectomy at least 6 
months prior to randomization or if adequate contraception is used during, and for at least 
one month after the last dose of study drug. Adequate contraception is defined as resulting 
in a failure rate of less than 1% per year (combined estrogen and progestogen [oral, 
intravaginal, or transdermal], or progestogen-only hormonal contraception (oral, injectable, 
or implantable), intra-uterine device, intra-uterine hormone releasing system, bilateral tubal 
occlusion, vasectomized partner, or sexual abstinence). In addition, a barrier method (i.e. 
cervical cap, diaphragm or condom) must be used during intercourse between a male subject 
and a female of child-bearing potential. 
8. Willing and able to give written Informed Consent and to comply with the requirements of 
the study protocol. 
9. Judged to be otherwise fit for the study by the Investigator, based on medical history, 
physical examination, and clinical laboratory assessments. Subjects with clinical laboratory 
values that are outside of normal limits (other than those specified in the Exclusion Criteria) 
and/or with other abnormal clinical findings that are judged by the Investigator not to be of 
clinical significance, may be entered into the study. 
Main Criteria for Exclusion 
1. Pregnant or nursing 
2. History of organ transplantation, including renal transplantation 
3. Currently on an organ transplant waiting list or there is a reasonable possibility of getting an 

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organ transplant within 6 months of screening. 
4.  Histological FSGS subtype of collapsing variant 
5. Subjects who initiated, discontinued or changed dose of rituximab or other anti-CD20 
monoclonal antibodies within 16 weeks (4 months) prior to screening are excluded. Subjects 
who initiated treatment with rituximab or other anti-CD20 monoclonal antibodies >16 
weeks (4 months) prior to screening are permitted if deemed safe by the investigator and 
only if they are intended to remain on continued, unchanged therapy at a dosing interval that 
has been documented to achieve continuous B cell depletion for the given patient. UPCR 
and other urine protein assessments up to 1 year prior to screening (if available) that were 
performed in these patients as part of the clinical routine should be recorded in the medical 
history. 
6. Subjects who discontinued Rituximab or other anti-CD20 monoclonal antibodies >16 weeks 
(4 months) prior to screening without confirmed recovery of CD20+ B cell population to 
within normal range are excluded. Subjects who discontinued rituximab or other anti-CD20 
monoclonal antibodies >16 weeks (4 months) prior to screening with confirmed recovery of 
CD20+ B cell population to within normal range are permitted in the study. UPCR and 
other urine protein assessments up to 1 year prior to screening (if available) that were 
performed in these patients as part of the clinical routine should be recorded in the medical 
history. 
7. Subjects who initiated or increased the dose of calcineurin inhibitors, or other 
immunotherapy, within 12 weeks prior to screening are excluded; dose reductions to 
maintain calcineurin inhibitors at optimal safe levels are permitted. Subjects who initiated a 
treatment with calcineurin inhibitors, or other immunotherapy >12 weeks are permitted. 
UPCR and other urine protein assessments up to 1 year prior to screening (if available) that 
were performed in these patients as part of the clinical routine should be recorded in the 
medical history. 
8. Subjects taking glucocorticoids at dose greater than 10 mg/day prednisone equivalent within 
4 weeks prior to screening are excluded. 
9. Plasmapheresis within 12 weeks prior to screening 
10. Body Mass Index (BMI) ≥40 
11. Participated in any clinical study of an investigational product within 12 weeks prior to 
screening or within 5 half-lives after taking the last dose 
12. Currently on dialysis or likely to require dialysis during the study. 
13. History or presence of any form of cancer within the 5 years prior to screening, with the 
exception of excised basal cell or squamous cell carcinoma of the skin, or carcinoma in situ 
such as cervical or breast carcinoma in situ that has been excised or resected completely and 
is without evidence of local recurrence or metastasis 
14. Positive HBV, HCV, or HIV viral screening test. Subjects who have received highly 
effective therapy for HCV demonstrated to have negative viral titers for at least 6 months 
following discontinuation of treatment, will be considered to have a negative HCV 
screening test weeks prior to screening 
15. Evidence of tuberculosis based on interferon γ release assay (IGRA), tuberculin purified 
protein derivative (PPD) skin test, or chest radiography done during screening or within 6 
weeks prior to screening 
16. Evidence of hepatic disease; AST, ALT, alkaline phosphatase >2x ULN, or total bilirubin > 
2x ULN or INR > 1.5 x ULN at screening with the exception that isolated INR elevation in 

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the absence of other significant liver enzyme abnormalities is explained by anticoagulant 
therapy, (e.g. warfarin). 
17. Clinically significant peripheral neuropathy 
18. Hematologic abnormalities as follows: Hb <8 g/dL, platelets <50,000, ANC <1000 
cells/μL) at baseline 
19. Abnormality of ECG at screening, assessed by the Investigator as clinically significant (e.g. 
QTcF greater than 450 msec). 
20. History of alcohol or illicit drug abuse. Recreational use of cannabis is not excluded where 
legalized. 
21. History of gastrointestinal conditions that may interfere with study medication compliance, 
e.g., severe gastroparesis, with regurgitation of food or oral medication 
22. Known hypersensitivity to CCX140-B or inactive ingredients of the CCX140-B tablets 
(including microcrystalline cellulose, starch, crospovidone, magnesium stearate, or silicon 
dioxide) 
23. Renal disease associated with disorders other than FSGS (e.g. lupus nephritis, C3 
glomerulonephropathy) that is active, or has significant risk of progressing to end stage 
renal disease during the 12-week initial treatment phase of the study 
24. History or presence of systemic disorder other than FSGS that requires, or is expected to 
require, systemic glucocorticoids or immune modulators; topical or inhaled corticosteroids 
and immune modulators are not excluded 
25. History or presence of any medical condition or disease which, in the opinion of the 
Investigator, may place the subject at unacceptable risk for study participation 
26. Subjects taking strong CYP3A4 inducers (e.g., phenytoin, rifampicin, carbamazepine, St. 
John’s Wort) or strong CYP3A4 inhibitors (e.g., boceprevir, clarithromycin, conivaptan, 
grapefruit juice, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, 
nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, and 
voriconazole) within two weeks prior to screening. 
27.  Subjects taking lithium, pamidronate or interferon; subjects taking non-steroidal anti-
inflammatory agents (NSAIDS) chronically (intermittent, i.e. occasional NSAIDS for pain 
or fever is discouraged, but is not excluded). 
Study Medication 
CCX140-B will be administered as 5 mg CCX140-B tablets 
Duration of Treatment and Observation 
Subjects will be screened within a period not to exceed 28 days prior to Day 1. 
The initial treatment period, including dose escalation, will be up to 12 weeks. Subjects who are 
not Treatment Failures and who otherwise meet criteria for continuation may continue treatment 
for up to an additional 40 weeks to assess achievement of remission and duration of response. 
All subjects will be followed for 4 weeks (28 days) after the last CCX140-B dose administered. 
To the extent possible, any adverse events that are deemed study drug-related and are ongoing 
at discharge will be followed up to resolution or until a determination is made that the 
unresolved event is stable. The subject’s condition will be evaluated by the Investigator at the 
end of the clinical trial and appropriate standard of care medical treatment will be provided to 
all subjects as needed. 

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Safety Assessments 
Safety assessments include adverse events, physical examination abnormalities , vital signs, 
ECGs, clinical laboratory tests (including blood chemistry, hematology, and urinalysis), and the 
AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST), a 
validated assessment tool for early detection of peripheral neuropathy. 
Efficacy Assessments 
Primary and Secondary Efficacy assessments include: 
• Urine protein:creatinine ratio (UPCR) and urine:albumin ratio (UACR), assessed in a 
sample drawn from a 24 hour collection is required at baseline and Study Week 12 (see 
schedule). At other time points defined in the Time and Events table, 24 hour collection is 
preferred. However, at these visits, a midstream void sample may be used for assessments if 
a 24hr urine sample is not available. 
• 24 hour urine protein excretion to be collected at baseline (pre-dose on Day 1), Day 15, Day 
29, Day 57 and Day 85 (12 weeks) (same as previous) 
• Change from baseline in eGFR calculated by the CKD-EPI Cystatin C equation, CKD-EPI 
Creatinine equation, CKD-EPI Creatinine-Cystatin C equation and MDRD Creatinine 
equation 
• Serum albumin level 
• Health-related quality of life based on the SF-36 v2 and EQ-5D-5L 
Exploratory Assessments 
MCP-1 excreted in urine and biomarker analysis including genetic mutations associated with 
the pathogenesis and/or prognosis of FSGS, biomarkers associated with CCR2 biology and 
biomarkers of renal inflammation, fibrosis and injury and leg circumference measurements. 
Pharmacokinetic Assessments and Analysis 
Plasma samples will be collected according to the Time and Events Table to determine the PK 
profile of CCX140. Individual plasma concentrations of CCX140 will be listed, plotted, and 
summarized descriptively and graphically. PK parameters will be calculated based on plasma 
CCX140 concentrations at the time of sample collection in relation to time of administration of 
the most recent dose of study medication. The following parameters will be determined, where 
possible: 
Concentrations of CCX140 (neutral form of CCX140-B which is a sodium salt) will be 
determined in plasma according to the Time and Events Table. The following parameters will 
be determined, where possible: 
Cmax 
Maximum plasma concentration 
Tmax 
Time of maximum plasma concentration 
AUC0-6 Area under the plasma concentration-time curve from Time 0 to Hour 6 for all 
subjects, after the first dose of 5 mg QD, two weeks after 10 mg BID, and four 
weeks after 15 mg BID 

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AUC0-12 Area under the plasma concentration-time curve from Time 0 to Hour 12, two 
weeks after 10 mg BID and four weeks after 15 mg BID, will be extrapolated for 
all subjects. 
Cmin 
Trough level plasma concentrations at visits when AUC0-6 is not collected 
Plasma/serum and urinary PD markers will be summarized and may be analyzed using methods 
analogous to the efficacy parameters. 
The relationships among PK parameters, UPCR and renal function based on eGFR will be 
evaluated. The data may also be used to evaluate the PK/PD relationship of CCX140-B 
treatment. To this end, the change and/or percent change from baseline in UPCR, eGFR, and 
other biomarkers may be used as PD markers. 
If there are sufficient data, population PK analysis will be performed. 
Pharmacodynamic Markers and Analysis 
• Blood will be collected according to Time and Event Table for leukocyte absolute counts 
and percentage as well as lymphocyte subsets. Gene mutations associated with prevalence 
and prognosis of FSGS may be evaluated as well. 
• Plasma/serum samples will be collected according to the Time and Events Table for 
pharmacodynamic marker measurements, which may include, for example, inflammatory 
cytokine and chemokine levels 
• Urine samples will be collected according to the Time and Events Table for biomarker 
analysis including, for example, markers for renal inflammation, fibrosis and injury. 
The change and/or percent change from baseline in UPCR, eGFR, and other biomarkers may be 
used as PD markers. 
Statistical Methods 
Details of the statistical analysis will be provided in a separate statistical analysis plan (SAP), 
which will be written, finalized, and approved prior to database lock and will be included in the 
Clinical Study Report (CSR) for this protocol. The SAP will supersede the statistical analysis 
methods described in this clinical protocol. Any deviation from the protocol will be documented 
and described in the final report. If changes to principal features stated in the protocol are 
required, these will be documented in a protocol amendment. The final SAP will take into 
account any amendment to the protocol. Data analysis and writing of a CSR for all study data 
will be performed by the designated CRO in accordance with its SOPs. Analysis of PK and PD 
data, and writing of PK and PD reports will be performed by designated PK and PD teams in 
accordance with their standard operating procedures. 
All continuous study assessments will be summarized by visit (as applicable) and change from 
baseline (baseline is typically defined as pre-dose evaluation on day 1 unless otherwise 
specified) to each post-baseline visit using descriptive statistics (n, mean, median, standard 
deviation, minimum, and maximum). All categorical study assessments will be summarized by 
visit (as applicable) using frequency counts and percentages. 

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Figure 1: 
Study Schematic 
 

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II. TIME AND EVENTS TABLE 
Visit  
D(ays) +/-3 days 
&  
W(eeks) +/- 1 
week 
Scr 
D
1 
D 
15 
D 
29 
D 
43 
D 
57 
D 
71 
D 
85 
or 
w
k 
12 
Wk 
16 
Wk
20 
Wk 
24 
Wk 
28 
Wk 
32 
Wk 
36 
Wk 
40 
Wk 
44 
Wk 
48 
Wk 52 
or Early 
Term 
Visit (for 
any 
reason) 
7 and 
14 
days 
post 
final 
dose  
Follow 
up Visit 
(4 wks 
post final 
dose) 
Dose 
 
(Days1-14) 
 5 mg bid 
(Days 15 -43)   
10 mg bid 
Starting on D43, dose 
modifications based 
on PK and safety 
results in accordance 
with Table 1. Dose 
will not exceed 15 mg 
bid 
 
During the extended treatment period, further dose modifications may be 
made based on efficacy, PK and safety results, in accordance with  
Table 1. Dose will not exceed 15 mg bid2 
Single 
blood 
draw to 
assess 
elimina
tion 
 
Informed consent 
X 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Demog, Med Hx1 
X 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Screening for TB, 
HIV,HBV, HCV  
X 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Renal biopsy to 
confirm FSGS if 
not done prior to 
entry 
X 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Enroll Eligible 
Subject 
 
X 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
PE & Vital signs,  
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
Neuropathy 
Screening Tool 
(ACTG BPNST) 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
Assessment of 
body fluid via 
measurement of leg 
circumference & 
bio-impedance 
X 
X 
 
 
 
X 
 
X 
 
 
 
X 
 
 
X 
 
 
X 
 
X 

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Visit  
D(ays) +/-3 days 
&  
W(eeks) +/- 1 
week 
Scr 
D
1 
D 
15 
D 
29 
D 
43 
D 
57 
D 
71 
D 
85 
or 
w
k 
12 
Wk 
16 
Wk
20 
Wk 
24 
Wk 
28 
Wk 
32 
Wk 
36 
Wk 
40 
Wk 
44 
Wk 
48 
Wk 52 
or Early 
Term 
Visit (for 
any 
reason) 
7 and 
14 
days 
post 
final 
dose  
Follow 
up Visit 
(4 wks 
post final 
dose) 
Triplicate 12-lead 
ECGs (triplicates, 
performed within a 
5 minute interval). 
All ECGs must be 
performed prior to 
any IV access or 
blood draws. 
X3 
X 
 
X 
 
X 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
PG test  
X 
X 
 
X 
 
X 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
Hematology, serum 
chemistry, lipids, 
X 
X 
X 
X 
 
X 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
Urinalysis 
X 
X
X 
X 
 
X 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
24-hour urine for 
UPCR, UACR & 
total protein4 
 
X 
X 
X 
 
X 
 
X 
 
 
 
X 
 
 
X 
 
 
X 
 
 
Midstream spot 
urine void for 
UPCR during site 
visit 
X 
X 
X 
X 
 
X 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
SF-36 v2 & EQ-
5D-5L 
 
X 
 
 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
 
Dispense CCX140-B  
 
→  
→ 
 
→  
→ → 
→ 
→ 
→ 
→ 
→ 
→ 
→ 
→ 
 
 
 
CCX140-B 
accountability 
 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
 
Whole blood for 
DNA 
 
X 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
PD 
plasma/serum/urine 
sample collection5 
 
X 
X 
X 
 
X 
 
X 
 
X 
 
X 
 
 
X 
 
 
X 
 
X 
Lymphocyte 
Subset6 
 
X 
X 
X 
 
X 
 
X 
 
X 
 
X 
 
 
X 
 
 
X 
 
X 

CCX140-B 
 
 
 
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Page 55 of 109 
Visit  
D(ays) +/-3 days 
&  
W(eeks) +/- 1 
week 
Scr 
D
1 
D 
15 
D 
29 
D 
43 
D 
57 
D 
71 
D 
85 
or 
w
k 
12 
Wk 
16 
Wk
20 
Wk 
24 
Wk 
28 
Wk 
32 
Wk 
36 
Wk 
40 
Wk 
44 
Wk 
48 
Wk 52 
or Early 
Term 
Visit (for 
any 
reason) 
7 and 
14 
days 
post 
final 
dose  
Follow 
up Visit 
(4 wks 
post final 
dose) 
PK AUC plasma 
sample 7 
 
X 
 
X 
 
X 
Pharmacokinetics (Cmin) – sample is to be collected. However, if there was a dose 
increase in the prior 2 weeks, PK AUC sample is to be collected instead. 
 
 
 
PK Cmin sample  
 
 
X 
 
X 
 
 
 
 
PK concentration 
sample 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
X 
X 
 
Concomitant 
medications8 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
X 
X 
X 
 
X 
Adverse event 
assessment 
 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
X 
 
X 
X 
X 
X 
 
X 
1. Record UPCR and urine protein assessments up to 1 year prior to screening (if available) in the medical history if patients were treated with Rituximab, other CD20+ 
monoclonal antibodies or calcineurin inhibitors, or other immunotherapy. Plasmapheresis within 12 weeks of screening should be captured in medical history.  
2. The decision to modify the dose (i.e.: either to maintain, reduce or increase) will be made 2 weeks after each subject escalates to the 15 mg bid dose. This time point could 
vary for each subject.  
3. Only the Screening 12-lead ECG can be single. The remainder should be triplicate 12 lead ECGs  
4. 24 hr UPCR is be required for each dose escalation, and to confirm response at current dose. Total volume should be recorded and a 2 ml aliquot samples should be 
collected for PK assessment. Sites need to contact the patient prior to the visit day as a reminder to start the 24 hr urine collection. In the rare event that a patient does not 
bring in a 24-hour sample, a 24 hour sample needs to be started on the visit day and returned to the site upon completed collection. Urine protein:creatinine ratio (UPCR) and 
urine:albumin ratio (UACR), assessed in a sample drawn from a 24 hour collection is required at baseline and Study Week 12 (see schedule). At other time points defined in 
the Time and Events table, 24 hour collection is preferred. However, at these visits, a midstream void sample may be used for assessments if a 24hr urine sample is not 
available. 
5. PD samples must be collected before administration of the morning dose. Include urine samples for MCP-1 and MCP-1/creatinine ratio 
6. Whole blood for analysis of absolute count and percentages of T cells, B cells and Natural Killer Cells. 
7. PK samples are to be collected from time 0 through 6 hours (0, 0.5, 1, 2, 3, 4 & 6) on study day 1 and on the relevant study day for each dose escalation (time 0 occurs 
immediately before the 1st dose and subsequent samples are collected at 0.5, 1, 2, 3, 4 & 6 hours after dosing and at least 2 weeks after the subject has completed dose 
escalation/adjustment and is receiving a continuous stable dose. Subjects can eat 1 hour after the time 0 blood draws have been completed (immediately after the 1 hour blood 
draw). 
8. Medications taken up to 12 weeks prior to screening should be captured as concomitant medications. Prior treatment with rituximab or other anti-CD20 monoclonal 
antibodies and corresponding levels of CD20+ B cells need to be recorded up to 1 year prior to screening. Eligible patients who have entered the study on a continuous cycle 
therapy of rituximab or other anti-CD20 monoclonal antibodies need to remain on an unchanged therapy at a dosing interval that has been documented to achieve continuous 
B cell depletion for the given patient unless prohibited due to safety considerations. Their levels of CD20+ B cells that are routinely evaluated per standard of care with anti-
CD20 monoclonal antibody therapy need to be recorded throughout the study. 
 

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III. LIST OF ABBREVIATIONS AND ACRONYMS 
ACTG-BPNST 
AIDS Clinical Trials Group – Brief Peripheral Neuropathy Screening 
Tool 
ADLs 
Activities of Daily Living 
ADR 
Adriamycin 
ARB 
angiotensin receptor II blocker 
AUC 
area under the curve 
AUC0-6 
area under the curve, from time of dosing to 6 hours post-dose 
BID 
two doses per day; also represented as b.i.d. 
BMI 
Body mass index 
CCL2 
CC Chemokine Ligand 2 (also known as MCP-1), main chemokine for 
CCR2 
CCR2 
CC Chemokine Receptor 2 
CCX140-B 
sodium salt of CCX140 
CKD-EPI 
chronic kidney disease epidemiology collaboration 
Cmin 
Minimum (minimal) plasma concentration 
Cmax 
maximum (maximal) plasma concentration 
CR 
Complete remission 
CTCAE 
Common Terminology (or Toxicity) Criteria for Adverse Events 
CYP 
cytochrome P450 
DN 
Diabetic nephropathy 
ECG 
Electrocardiogram 
eGFR 
Estimated glomerular filtration rate 
EQ-5D-5L 
EuroQo1-5 Dimensions-5 Levels 
ESRD 
End Stage Renal Disease 
FDA 
Food and Drug Administration 
FSGS 
Focal Segmental Glomerulosclerosis 
GI 
Gastrointestinal 
GLP 
Good Laboratory Practices 
GMP 
Good Manufacturing Practices 
hERG 
potassium channel encoded by the human ether-à-gogo related gene 
HIV 
Human immunodeficiency virus 
ICH 
International Conference on Harmonization 
IgG 
Immunoglobulin G 
IgM 
Immunoglobulin M 

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Amendment 1.0 (17 April 2018) 
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IGRA 
Interferon γ Release Assay 
IMP 
Investigational medicinal product 
IND 
Investigational New Drug Application 
INR 
International Normalized Ratio 
KDIGO 
Kidney Disease Improving Global Outcomes 
LLN 
Lower Limit of Normal 
LOCF 
Last observation carried forward 
MCP-1 
monocyte chemoattractant protein 1, also known as CCL2 
mITT 
Modified Intent to treat 
MMRM 
Mixed-effects model for repeated measures 
NOAEL 
no-observed-adverse-effect level 
NOEL 
no-observed-effect level 
PK 
pharmacokinetic(s) 
PO 
oral (per os); also represented as “p.o.” 
PPD 
Protein purified derivative 
PR 
Partial remission 
PT 
Preferred Term 
PT 
Prothrombin time 
PTT 
Partial thromboplastin time 
QD 
one dose per day; also represented as “q.d.” 
RAAS 
Renin-angiotensin-aldosterone system 
RBC 
red blood cell(s) 
SAE 
Serious adverse event 
SF-36 
Short form (36) Health Survey 
SOC 
System Organ Class 
t1/2 
half-life 
T2DM 
type 2 diabetes mellitus 
TEAE 
Treatment emergent adverse event 
Tmax 
time of maximal concentration 
UACR 
urinary albumin:creatinine ratio 
UAER 
urinary albumin excretion rate 
ULN 
Upper Limit of Normal 
UPCR 
Urinary protein:creatinine ratio 
μM 
Micromolar 
 

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1. 
INTRODUCTION 
1.1. 
Background Information 
Focal segmental glomerulosclerosis (FSGS) describes a group of disorders that present clinically 
with proteinuria and progressive renal insufficiency and share a common histologic lesion 
characterized by scarring that appears on light microscopy to occur in a subset of glomeruli and 
to affect only part of the involved glomeruli. 
Primary FSGS comprises a subset that is currently defined by ruling out other forms of FSGS, 
where the latter include genetic, adaptive, viral, and medication-associated or toxin-associated 
disease. Clinically, primary FSGS commonly presents with nephrotic-range proteinuria, reduced 
plasma albumin levels, and hyperlipidemia. Electron microscopy often reveals generalized 
effacement of podocytes, despite the focal and segmental appearance of lesions with light 
microscopy. Current therapeutic recommendations include a trial of high dose glucocorticoids; 
treatment for durations of 4 to 24 months has been reported to achieve complete or partial 
remission in 28% to 74% of patients (Korbet et al., 2012; Banfi et al., 1991; Cattran and Rao, 
1998; Rydel et al., 1995; Gipson et al., 2016). 
Limitations of current therapy include inadequate control for a significant proportion of subjects, 
high risk of relapse, and toxicity associated with treatment; the latter includes metabolic, 
cardiovascular, neuropsychiatric and bone toxicity with glucocorticoids, nephrotoxicity with 
calcineurin inhibitors and increased risk of infection and/or neoplasm associated with immune 
suppression. There is a need for therapeutic approaches that induce a more rapid response in a 
greater proportion of subjects with less toxicity, and for well-tolerated agents that can be used 
chronically to reduce the risk of relapse. 
CCR2 has emerged as a potential target for treatment of FSGS based on expression of CCR2 in 
diseased kidney, association between disease activity and efficacy of CCR2 blockade in animal 
models with features of FSGS. 
CCX140-B is an orally administered, selective antagonist of CCR2, which has exhibited efficacy 
in murine models of renal injury as assessed by reduction in proteinuria and improvement in 
renal histology. In patients with diabetic nephropathy, administration of CCX140-B at 5 mg 
daily or 10 mg daily for up to 52 weeks was associated with and favorable safety profiled and 
significant reduction from baseline in proteinuria (Study CL005_140; de Zeeuw, et al., 2015). 
Based on the preclinical rationale, favorable safety profile, and signals of efficacy in diabetic 
subjects with marked proteinuria, ChemoCentryx plans to assess CCX140-B in subjects with 
FSGS. The present study will evaluate the effect of ascending doses of CCX140-B in subjects 
with nephrotic syndrome who are assessed to be sufficiently stable to delay initiation of high 
dose glucocorticoids and other immunosuppressant therapy This population has a high unmet 
medical need for new therapeutic options. 
1.2. 
Potential Role of CCR2 in Pathogenesis of FSGS 
A potential role for CCR2 in the pathogenesis of FSGS is supported by clinical and 
histopathologic observations in subjects with renal disease, and findings with in vitro studies and 
in vivo mouse models of FSGS. 

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CCR2 is a major driver of monocyte migration and activation, and has been shown to mediate 
renal interstitial inflammation and tubular atrophy in chronic renal diseases by recruiting 
monocytes to the renal interstitium (Yadav et al; 2010). Increased urinary levels of MCP-1, the 
ligand for CCR2, is associated with increased interstitial macrophage infiltration in subjects with 
chronic kidney disease (Eardley et al, 2006) and with increased degree of proteinuria in children 
with FSGS (Wasilewska, et al, 2011). There is a well-characterized DNA polymorphism (MCP1 
2518 A/G) that has been associated with elevated MCP-1 levels and greater risk for development 
of renal failure in FSGS, as well as in IgA nephropathy, diabetic nephropathy and lupus nephritis 
(Besbas et al, 2015). 
In vitro experiments support a role for the CCR2/MCP-1 pathway in the purported disease 
acceleration role ascribed to proteinuria. Specifically, it is reported that tubular epithelial cells 
release MCP-1 (CCL2) when exposed to serum proteins on the apical side (Burton et al, 1999). 
In vivo, targeting the MCP-1/CCR2 system in preclinical models of CKD demonstrates benefit. 
MCP-1 deficient mice are resistant to the development of albuminuria following diabetes 
induction by streptozotocin and this protective phenotype is marked by a lack of monocyte 
recruitment into the glomeruli (Chow et al., 2006). MCP-1 deficiency also prevents diabetic 
db/db mice from developing albuminuria (Chow et al., 2007). Pharmacological treatment with a 
CCR2 antagonist prevents the onset of albuminuria in db/db mice and this benefit correlates with 
a lack of monocyte recruitment to the glomeruli and amelioration of histological damage (Kang 
et al., 2010). ChemoCentryx conducted an extensive series of preclinical efficacy studies with 
CCX140-B and surrogate CCR2 antagonists. Efficacy was observed in mouse models of 
diabetic, non-diabetic nephropathy and in models with features of FSGS induced by partial 
nephrectomy or by exposure to Adriamycin (ADR). Rapid and significant reduction in 
proteinuria as assessed by measurements of urinary albumin excretion rate (UAER) and urinary 
albumin:creatinine ratio (UACR) was observed in all animal models, the reduction in proteinuria 
are sustained throughout the duration of the studies. Histological parameters were also improved 
by treatment with CCR2 inhibitors and in combination with RAAS blockade. These histological 
parameters include reduced glomerular hypertrophy, mesangial expansion, glomerular sclerosis, 
and increased tubular and glomerular integrity. In addition, histopathology reveals preservation 
of glomerular architecture and restoration of normal tubular appearance with CCR2 blockade in 
the 5/6 nephrectomy mouse model. The presence of intra-glomerular CCR2 positive cells has 
been demonstrated in the animal models (PC0684_140). Moreover, renal protective effects are 
associated with CCR2 inhibition in the FSGS models. Notably, the number of podocytes per 
glomerulus is significantly higher with CCR2 inhibitor therapy than in vehicle-treated matched 
controls (See Investigator’s Brochure). 
Ex vivo, evidence for a role for CCR2 in FSGS in humans comes from unpublished studies 
conducted by ChemoCentryx. Expression of CCR2 protein was assessed using 
immunohistochemistry methods in renal biopsies from normal kidney and from subjects with 
primary FSGS. Both showed expression of CCR2 in cells implicated in pathogenesis, including 
parietal epithelial cells, tubular epithelial cells and infiltrating macrophages. 
Difference in expression between healthy and diseased kidney was observed in the tubular 
epithelium, consistent with prior data showing that proteinuria induces CCL2 (MCP-1, the ligand 
for CCR2) expression in tubules (Burton et al; 1999). 

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Finally, support derives from the demonstration that CCX140-B was associated with clinically 
meaningful reduction in proteinuria in a Phase 2 study in subjects with diabetic nephropathy (de 
Zeeuw, et al., 2015). The pathogenic mechanisms that contribute to proteinuria and progressive 
renal dysfunction in diabetic nephropathy are shared, at least in part, with mechanisms in FSGS 
(Jefferson et al., 2008). Specifically, a decrease in podocyte number has been shown in subjects 
with both type I and type II diabetes mellitus, reduced podocyte number may precede the onset 
of clinically detectable albuminuria and/or proteinuria by several years, foot process effacement 
is observed, and experimental and clinical studies have shown a direct correlation between the 
magnitude of proteinuria and the extent of effacement and decrease in podocyte number. Further, 
as in FSGS, increased glomerular proteinuria leads to enhanced tubular cell uptake of protein, 
leading to complement activation, tubulointerstitial inflammation and release of fibrogenic 
growth factors and inflammatory cytokines. 
These observations support the rationale that selective CCR2 inhibition with CCX140-B may 
provide therapeutic benefit in subjects with FSGS. 
1.3. 
CCR2 Inhibitor CCX140-B: Non-Clinical Safety 
The nonclinical safety of CCX140-B was studied in vitro and in vivo in mice, rats, dogs and 
Cynomolgus monkeys. 
In repeat dose toxicity studies (up to 26 weeks in rats and 39 weeks in dogs) two adverse 
nonclinical findings of concern were revealed: 
• Anemia (red blood cell [RBC] parameter findings, with reticulocytosis) was observed across 
all species studied; rapid recovery of the RBC findings and reticulocytosis was observed 
following discontinuation of treatment. 
• Peripheral axonopathy was observed at high exposures only in dogs; histological findings 
showed a likely cessation of active damage with decreased immune cell infiltrates following 
discontinuation of treatment; however, recovery was incomplete during the observation 
period. 
The NOAEL in the 26-week rat studies of 15 mg/kg/day (based on reversible anemia) was 
associated with systemic AUC0-24 total plasma exposures to CCX140 of 464.6 and 
493.7 µg•hr/mL, for males and females, respectively, and the Cmax concentrations was 32 µg/mL. 
The NOAEL in the dog studies (based on neuropathy) is higher than the NOAEL established in 
rat. Thus, for dose escalation in human subjects, the more sensitive rat-based NOAEL will be 
used: total plasma AUC0-24 exposures of 465 µg•hr/mL, with total Cmax exposures of 32 µg/mL. 
These exposures are approximately 3- to 4-fold higher than the predicted human exposures with 
a 15 mg twice daily (b.i.d.), the highest dose of CCX140-B to be tested in the current study. 
Anemia findings are likely translatable to humans, but are verifiable, reversible, and there was an 
acceptable safety margin across all species (see below). To ensure early detection of anemia in 
the Phase 2 clinical trial, blood samples will be drawn at baseline, at 6 hours following 
administration of the first dose, at Study Days 8, 15 and 29, then at every 4 week intervals 
throughout the treatment and follow up period for assessment of complete blood count, 
reticulocyte count and bilirubin. 

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Neuronal findings were observed only in dogs, and may not be translatable to humans. 
Specifically, in the 39-week dog study, the highest tested dose of CCX140-B was associated with 
slight to mild neuronal cell changes on histology, with no clinically-observable changes. Off-
target binding assays, in vivo immune mechanism assessments, and immunohistochemistry 
evaluations did not reveal a mechanism, but may rule out direct CCR2 receptor inhibition. In in 
vitro assessments, there were no metabolites that were unique to dogs, but there was a higher 
percentage of free CCX140 in plasma in dogs which was unique among studied species, 
including humans. Thus a threshold effect of free drug concentration cannot be ruled out. 
Subjects in the Phase 2 study will be monitored for adverse events related to the nervous system, 
and will be evaluated for early symptoms using a questionnaire validated for early detection of 
peripheral neuropathy in studies of patients with HIV infection. 
1.4. 
CCR2 Inhibitor CCX140-B: Clinical Experience 
CCX140-B has been assessed in 4 Phase 1 clinical trials in healthy volunteers, one Phase 2 trial 
in subjects with diabetes, and two Phase 2 trials in subjects with diabetic nephropathy. A tabular 
summary of clinical studies conducted with CCX140-B and submitted to IND 114469 are 
provided in the Investigator’s Brochure. 
Efficacy findings in study CL005_140 in patients with diabetic nephropathy and proteinuria 
provide support for exploring CCX140-B in FSGS. In that trial, 192 subjects with diabetic 
nephropathy received uninterrupted dosing for up to 52 weeks with placebo, 5 mg daily of 
CCX140-B or 10 mg daily of CCX140-B. Compared to placebo, CCX140-B was associated with 
greater reduction in albuminuria. Specifically, changes from baseline in the ratio of urinary 
albumin to creatinine (UACR) averaged -2% in the placebo group (95% CI -11% to +9%), -18% 
in the CCX140-B 5 mg group (95% CI -26% to -8%), and -11% in the CCX140-B 10 mg group 
(95% CI -20% to -1%). The decrease in UACR was observed at the earliest measured time point 
(Day 15) and reached a maximum decrease by the Day 85 visit (de Zeeuw et al., 2015). Of note, 
subjects entering the study were stratified based on the level of proteinuria at baseline. In a pre-
specified subset analysis, the greatest percentage improvement in UACR from baseline was 
observed in subjects who presented with the highest baseline UACR (801-3000 mg/g) and eGFR 
of at least 60 mL/min/1.73m2. In this subset mean changes in UACR from baseline were +45 % 
in the placebo group (95% CI -6% to +121%, n=8), -23% in the CCX140-B 5 mg group (95% CI 
-55% to +32%, n=5; p = 0.03 vs control), and -39% in the CCX140-B 10 mg group (95% CI -
61% to -7%; n=8; p = 0.004 vs control). 
The pathogenic mechanisms that contribute to proteinuria and progressive renal dysfunction in 
diabetic nephropathy are shared, at least in part, with mechanisms in FSGS (Jefferson et al., 
2008). Specifically, a decrease in podocyte number has been shown in subjects with both type I 
and type II diabetes mellitus, reduced podocyte number may precede the onset of clinically 
detectable albuminuria and/or proteinuria by several years, foot process effacement is observed, 
and experimental and clinical studies have shown a direct correlation between the magnitude of 
proteinuria and the extent of effacement and decrease in podocyte number. Further, as in FSGS, 
increased glomerular proteinuria leads to enhanced tubular cell uptake of protein, leading to 
complement activation, tubulointerstitial inflammation and release of fibrogenic growth factors 
and inflammatory cytokines. 

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When taken together with results from the pre-clinical studies, and a favorable clinical safety 
profile (see Investigator’s Brochure), these observations further support evaluation of CCX140-B 
in highly proteinuric renal diseases, including FSGS. 
1.5. 
Rationale for Study in FSGS: Unmet Medical Need 
Supportive management for FSGS includes renin-angiotensin aldosterone system inhibitors 
(RAAS) blockers for management of proteinuria and hypertension when indicated, and statins 
for management of dyslipidemia. For patients presenting with primary FSGS and proteinuria, 
glucocorticoids at doses equivalent to 0.3 to 1.5 mg/kg/d prednisone for durations of 4 to 24 
months have been reported to achieve complete or partial remission in 28% to 74% of patients 
(Korbet et al., 1994; Banfi et al., 1991; Cattran and Rao, 1998; Rydel et al., 1995; Gipson et al., 
2016; Cattran et al., 2008). 
Limitations of current treatment include inadequate response in a significant proportion of 
patients, frequent recurrence of disease is responders, and progression of renal insufficiency in 
most patients over time. The most informative published data on rate of progression derives from 
the Toronto Glomerulonephritis Registry (Cattran et al, 2008). Among 370 patients with FSGS, 
eGFR declined by 5 to 18 g/ml/1.75m2/year in the subset with nephrotic level proteinuria. 
Among patients with high levels of proteinuria at the time of presentation, 50% progressed to 
ESRD within 3 to 8 years (D’Agati, 2011). In the US, the estimated incidence of newly-
recognized FSGS, and the reported incidence end stage renal disease (ESRD) due to FSGS, are 
both approximately 7/million/year (Sim et al., 2016). Thus current therapies may be delaying but 
not preventing, renal failure (Troyanov et al., 2005). 
Additional limitations of current therapy include significant morbidity and premature mortality 
ascribable to the neurologic, metabolic and bone toxicity associated with high dose 
glucocorticoids, renal toxicity associated with calcineurin inhibitors, and to immunosuppression. 
There is a need for therapeutic approaches that induce and maintain response in a greater 
proportion of subjects with less toxicity. 
1.6. 
Risk-Benefit Assessment 
For subjects with primary FSGS who do not achieve sustained response with 
immunosuppression, as assessed by reduction in proteinuria there are no effective therapies 
available. Histologic recurrence in renal transplants is very high, with high levels of proteinuria 
portending a poor renal prognosis (Besbas et al, 2015). For subjects who do achieve response, 
the increased risk of infection associated with corticosteroids is a significant concern, as are the 
metabolic and neurologic toxicities associated with corticosteroids; these toxicities become 
increasingly concerning for subjects who require continuous treatment to maintain control 
(corticosteroid/ immunosuppressant dependent) or who experience frequent relapses and require 
recurrent treatment. 
To date, CCX140-B has been well-tolerated in the 7 completed clinical studies with administered 
doses ranging from 0.05 mg to 15 mg QD for 12 days and 10 mg QD for 52 weeks. 
The rationale for exploring this agent in FSGS is strong. Blockade of CCR2 in murine models of 
FSGS is associated with reduced proteinuria, improved renal function, and more normal 
histology. In subjects with diabetic nephropathy, treatment with CCX140-B was associated with 

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rapid and sustained reduction in proteinuria. If similar findings are observed in clinical trials, 
CCX140-B may comprise a novel podocyte protective agent to reduce morbidity associated with 
nephrotic syndrome and sustain renal function, with a favorable safety profile compared to 
current best standards of care. 
2. 
OBJECTIVES 
2.1. 
Primary Efficacy Objective 
The Primary Efficacy Objective is to evaluate the effect of CCX140-B on proteinuria in subjects 
with primary FSGS with nephrotic syndrome, assessed as a median reduction from baseline of 
urine protein to creatinine ratio (UPCR) of at least 20%, i.e. ≥ 20%, by week 12. 
2.2. 
Secondary Efficacy Objectives 
The secondary efficacy objectives of this study will be assessed at Study Week 12 and through 
End of Treatment and include: 
1. Achievement of partial or complete remission of urine protein to creatinine ratio (UPCR) 
through Study Week 12 and through the end of treatment, where partial and complete 
remission are defined as follows: 
Partial Remission (includes all of the following) 
• reduction from baseline by ≥50 percent in UPCR 
• reduction in UPCR to a level that is < 3.5 g/g 
• subject may not be a treatment failure  
Complete Remission (includes all of the following) 
• reduction in UPCR to < 0.3 g/g 
• serum albumin within normal range 
• For patients with abnormal serum creatinine levels at baseline, return to normal 
levels 
• For patients with normal serum creatinine levels at baseline, final value within 
20% of baseline levels 
• subject may not be a treatment failure 
2. Assessment of change from baseline in UPCR over time 
3. Assessment of time to, and proportion of subjects with achievement of partial remission 
4. Assessment of time to, and proportion of subjects with achievement of complete 
remission  
5. Assessment of time to rescue therapy, based on Investigator or physician initiation of 
glucocorticoids or new immunosuppressive agents or new major treatment modalities 
(e.g. plasmapheresis, dialysis) 

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6. Changes over time in other laboratory parameters related to renal function, including: 
• Serum albumin 
• Creatinine 
• Cystatin C 
• eGFR, calculated by the CKD-EPI Cystatin C equation, CKD-EPI Creatinine 
equation, CKD-EPI Creatinine-Cystatin C equation and MDRD Creatinine equation. 
• urine albumin to creatinine ratio (UACR) 
• total 24 hour urine protein excretion 
7. Quality of Life endpoints include: 
• Change in factors associated with quality of life from baseline over time as assessed 
using the SF-36 v2 
• Change in factors associated with quality of life from baseline over time as assessed 
using the EQ-5D-5L 
2.3. 
Safety Objectives 
The safety objectives of CCX140-B will be assessed via the following endpoints: 
1. Adverse events 
2. Vital signs 
3. Electrocardiograms 
4. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST): 
To assess potential signs and symptoms of peripheral neuropathy the ACTG BPNST will be 
completed by study personnel or the study investigator as specified in the Time and Events 
table. 
5. Changes in laboratory parameters including: 
• Hematology including complete blood count, reticulocyte count, smear evaluation 
• Serum haptoglobin, lactate dehydrogenase 
• Serum chemistry including serum transaminases and CPK 
• Urinalysis 
• Coagulation factors (Prothrombin Time [PT], Partial Thromboplastin Time [PTT], 
International Normalization Ratio [INR]) 
2.4. 
Exploratory Objectives 
Whole blood, plasma, serum and urine will be collected at baseline and at intervals over the 
course of the treatment period for exploratory analyses. Parameters will include: 
1. Assessment of biomarkers associated with disease activity over time, including blood lipid 
profile, MCP-1 excreted in urine, and potentially other biomarkers of renal inflammation, 
fibrosis and injury 
2. Assessment of biomarkers associated with risk of severity of disease, which may include 
allelic variations associated with the pathogenesis and/or prognosis of FSGS such as NPHS1, 
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3. Assessment of blood and/or urinary biomarkers associated with CCR2 biology, which may 
include peripheral blood leukocyte subsets and selected cytokines and chemokines 
4. Assessment of mechanisms by which nephrotic syndrome may potentially impact 
pharmacokinetics of CCX140, including assessment of CCX140 in urine. 
5. Assessment of edema as measured by physical examination, leg circumference and body 
weight 
6. Assessment of concentration of CCX140-B in urine 
3. 
STUDY DESIGN AND DOSING REGIMEN 
3.1. 
Initial Treatment Phase 
This is an open label clinical trial to test the efficacy, safety, pharmacokinetics and tolerability of 
ascending doses of CCX140-B in subjects with primary FSGS and nephrotic syndrome. 
The screening period will be up to 28 days. Subjects will visit the study center during Screening 
and on Day 1 (baseline), weekly throughout Dose Escalation, then every 2 weeks through Study 
Week 12. 
Eligible subjects will start treatment with CCX140-B at 5 mg twice daily. CCX140-B will be 
taken orally without food, at least one hour before meal. The dose will be increased in a stepwise 
manner to 15 mg twice daily while monitoring safety, in accordance with the rules (Table 1 and 
Figure 1) and schedule detailed in the Time and Events Table. Treatment at the selected dose 
will be continued up to Week 12 or until declaration of Treatment Failure. 
This study incorporates intra-subject dose escalation and dose modification. Four factors guide 
dose modification including: 
1. Time 
2. Response 
3. Exposure 
4. Safety 
During the initial 12 weeks of treatment, dose will be escalated from 5 mg twice daily to 10 mg 
twice daily on Study Day 15, then to 15 mg twice daily on Study Day 43, if subjects are not 
considered treatment failures and who otherwise meet criteria for continuation (Table 1, 
Figure 1). 
If planned dose escalation to 10 mg (on day 15) or 15 mg twice daily (on day 43) was previously 
halted the dose may be escalated further if subjects are not considered treatment failures and who 
otherwise meet criteria for continuation (Table 1, Figure 1). In such case the subject will return 
approximately 2 weeks later for blood draws to assess PK (AUC0-6). Subjects will return 2 weeks 
after the PK assessment to evaluate the safety and need for further dose adjustment. 
At any time during the study dose may be de-escalated for safety, or if projected exposure 
exceeds the maximum allowable exposure defined for the study. Because exposure may be 
impacted by changes in proteinuria, the protocol requires assessment of exposure if proteinuria 
declines significantly. 

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Blood will be drawn to evaluate trough exposure in accordance with the Time and Events 
Schedule. In addition, as detailed in Table 1, blood will be drawn to assess exposure (AUC0-12, 
extrapolated from measured AUC0-6), at the following times to guide dosing decisions: 
• Study Day 1 prior to first dose of CCX140, and at intervals through 6 hours following the 
initial dose 
• On study Day 29, two weeks following initiation of 10 mg twice daily, to assess exposure at 
steady state, and to confirm that exposure is within the safety margins based on non-clinical 
NOAEL 
• If dose was adjusted on Day 43, blood will be collected on study Day 57 to assess exposure 
• Additional assessments of exposure may be scheduled if indicated. Indications may include: 
o Subjects receiving a total dose greater than 10 mg daily who have experienced at least 
50% reduction in proteinuria since the previous PK assessment, to assess impact of 
change in proteinuria on exposure and to ensure exposure is within safety margins 
o Subjects who experienced a potentially dose-limiting safety event 
o Subjects who required a dose reduction for higher than planned exposure; PK will be 
assessed approximately 2 weeks after reduction 
o Subjects for whom dose is adjusted for any other reason; PK will be assessed 
approximately 2 weeks after reduction 
If measured or projected steady state PK exposure (AUC0-12) exceeds 240 µg•h/mL, which is 
approximately half of the rat NOAEL AUC0-24, the dose will be reduced to the dose projected to 
provide exposure within the target range. 
3.1.1. 
Day 85 and Beyond 
If dose is adjusted upward or downward for any reason at Day 85 or during the extended 
treatment period the subject will return approximately 2 weeks later for blood draws to assess PK 
(AUC0-6). Subjects will return 2 weeks after the PK assessment to evaluate the safety and need 
for further dose adjustment. 
3.1.2. 
Extended Treatment Period 
Subjects are eligible for the extended treatment period under the following conditions: 
1) Not considered treatment failures as of Week 12 and 
2) In the opinion of the Investigator, remain candidates for investigational treatment and 
3) Any of the conditions below apply: 
• Partial or Complete Remission by Week 12 
• Response with ≥20% reduction in UPCR by Week 12 
• No longer have nephrotic syndrome 
These subjects will return to the clinic for safety, PK and efficacy measurements every 4 weeks. 
Subjects taking CCX140-B at 15 mg twice daily will be maintained at that dose unless de-
escalation is required based on safety signal or steady state PK exposure (AUC0-12) that exceeds 
240 µg•h/mL. 

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Subjects can be considered for dose escalation if all of the following are true: 
• Subject is not already treated with 15 mg twice daily and  
• Subject did not require previous downward dose adjustment and 
• Subject is not a Treatment Failure, and 
• Meets all criteria for continuation: 
o Subject has not achieved and maintained at least a Partial Remission, and 
o Projected AUC0-12  on the next higher planned dose level does not exceed 240 
µg•h/mL and 
o The next higher dose does not exceed the Maximum Tolerated Dose (MTD) 
Subjects who undergo dose escalation will return to clinic approximately 2 weeks after initiation 
of the increase for assessment of PK. 
Dose modification rules that applied during the initial dose escalation period also apply to dose 
escalation after 12 weeks. 
3.1.3. 
Follow-Up 
Following completion of treatment, or early termination of treatment, all subjects should be 
urged to return for the termination visit and again 7 and 14 days after their last dose for blood 
draws to assess elimination pharmacokinetics, and 28 days after their last dose for follow-up and 
End of Study visit assessments. Appropriate standard of care medical treatment should be 
provided to all subjects as needed. 
After Study Day 43, dose escalation can occur by not more than one step monthly. Within 2 
weeks following each dose escalation, PK must be assessed to ensure that the projected AUC0-12 
does not exceed 240 µg•h/mL. Unscheduled visits for assessment of PK may be required. 
Stopping rules and dose reduction rules that applied during the 12 week initial dose escalation 
period also apply during dose escalation after 12 weeks (See Table 2). 
Throughout the treatment period, any changes in background medications should be limited to 
settings in which the Investigator deems it to be necessary for the safety of the subject. 
Subjects will be discharged from the study when all the visit procedures have been completed. 
3.2. 
Dose Modification Based on PK Exposure 
Blood will be drawn to evaluate trough exposure in accordance with the Time and Events 
Schedule. In addition, blood will be drawn to assess exposure (AUC0-12, extrapolated from 
measured AUC0-6) at the following times to guide dosing decisions: 
• Study Day 1 prior to first dose of CCX140-B, and at intervals through 6 hours following the 
initial dose 
• Two weeks (Day 29) following initiation of 10 mg twice daily, to assess exposure at steady 
state, and to confirm that exposure is within the safety margins based on non-clinical 
NOAEL 
• Approximately 2 weeks following initiation of 15 mg twice daily (Day 57, if dose was 
increased at Day 43), to assess exposure at steady state, and to confirm that exposure is 

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within the safety margins (projected AUC0-12 less than or equal to 240 µg•h/mL) based on 
non-clinical NOAEL 
• At Study Day 85 PK will be assessed to guide dosing beyond Week 12. 
• Additional assessments of exposure may be scheduled if indicated. Indications may include: 
o Subjects receiving a total dose greater than 10 mg daily who have experienced at least 
50% reduction in proteinuria since the previous PK assessment, to assess impact of 
change in proteinuria on exposure and to ensure exposure is within safety margins 
o Subjects who experienced a potentially dose-limiting safety event 
o Subjects who required a dose reduction for higher than planned exposure; PK will be 
assessed approximately 2 weeks after reduction 
o Subjects for whom dose is adjusted for any other reason; PK will be assessed 
approximately 2 weeks after reduction 
If planned dose escalation to 10 mg (on day 15) or 15 mg twice daily (on day 43) was previously 
halted the dose may be escalated further if subjects are not considered treatment failures and 
otherwise meet criteria for continuation (Table 1, Figure 1). In such cases the subject will return 
approximately 2 weeks later for blood draws to assess PK (AUC0-6). Subjects will return 2 weeks 
after the PK assessment to evaluate the safety and need for further dose adjustment. 
3.3. 
Dose Modifications During the Extended Treatment Period 
If measured or projected exposure steady state PK exposure (AUC0-12) exceeds 240 µg•h/mL, 
which is approximately half of the rat NOAEL AUC0-24, the dose will be reduced to the dose 
projected to provide exposure within the target range. 
For subjects who escalate dose to 10 mg twice daily on Study Day 15, PK samples from the dose 
two weeks after 10 mg BID dosing (planned for Day 29) will be analyzed expeditiously and the 
PK data will be used for projecting the steady state PK exposure. If the projected steady state PK 
exposure (AUC0-12) is higher than 240 µg•h/mL, which is half of the rat NOAEL steady state 
AUC0-24, the dose will be reduced to 5 mg BID for the remainder of the study (Table 2). 
If the projected steady state PK exposure (AUC0-12) is lower than 240 µg•h/mL, the steady PK 
exposure at 15 mg BID will be projected from the 10 mg BID Day 43 PK data. If the projected 
PK exposure (AUC0-12) at 15 BID is lower than 240 µg•h/mL, the dose will be escalated to 15 
BID. If the projected PK exposure (AUC0-12) is higher than 240 µg•h/mL, the dose will not 
escalate to 15 mg BID but will stay at 10 mg BID for the remainder of the study. 
4. 
STUDY POPULATION 
4.1. 
Size of Population 
Approximately 13 male or female adult subjects with biopsy-proven primary FSGS and 
nephrotic syndrome will be enrolled in the study. In the first stage 6 subjects will be treated. 
After all of the initial 6 subjects have completed their 12-weeks of treatment the study will be 
paused and assessed. If the overall study results are deemed safe and the subjects’ responses to 

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treatment are considered clinically meaningful, seven additional subjects will be treated for a 
total of 13 subjects. 
4.2. 
Inclusion Criteria 
1. Male or female subjects aged 18 years and older 
2. Renal biopsy findings consistent with diagnosis of focal segmental glomerulosclerosis 
(FSGS), and consistent with primary FSGS based on presentation of histopathology, 
medical history, and clinical course; subjects with genetic risk factors with presentations 
that are otherwise consistent with primary FSGS may also be enrolled. 
3. Urinary total protein:creatinine ratio (UPCR) ≥ 3.5 g protein/g creatinine at screening, 
based on sample drawn from a 24-hour collection 
4. Hypoalbuminemia of less than 3.5 g/dL 
5. Estimated glomerular filtration rate (eGFR) >30 mL/min/1.73m2, as calculated using the 
Chronic Kidney Disease Epidemiology Collaboration (CKD_EPI) equation (using 
creatinine or cystatin C) 
6. If used, RAAS inhibitor dose, including doses of angiotensin converting enzyme inhibitor 
and/or angiotensin II receptor antagonist must have been started at least 4 weeks prior to 
Study Day 1, and projected to remain stable throughout the course of the study unless 
adjustment is required for management of hypertension. 
7. Female subjects of childbearing potential may participate if adequate contraception is 
used during, and for at least 5 half- lives after last dose of study drug. Male subjects with 
partners of childbearing potential may participate in the study if they had a vasectomy at 
least 6 months prior to randomization or if adequate contraception is used during, and for 
at least one month after the last dose of study drug. Adequate contraception is defined as 
resulting in a failure rate of less than 1% per year (combined estrogen and progestogen 
[oral, intravaginal, or transdermal], or progestogen-only hormonal contraception (oral, 
injectable, or implantable), intra-uterine device, intra-uterine hormone releasing system, 
bilateral tubal occlusion, vasectomized partner, or sexual abstinence). In addition, a 
barrier method (i.e. cervical cap, diaphragm or condom) must be used during intercourse 
between a male subject and a female of child-bearing potential. 
8. Willing and able to give written Informed Consent and to comply with the requirements 
of the study protocol 
9. Judged to be otherwise fit for the study by the Investigator, based on medical history, 
physical examination, and clinical laboratory assessments. Subjects with clinical 
laboratory values that are outside of normal limits (other than those specified in the 
Exclusion Criteria) and/or with other abnormal clinical findings that are judged by the 
Investigator not to be of clinical significance, may be entered into the study. 
4.3. 
Exclusion Criteria 
1. Pregnant or nursing 
2. History of organ transplantation, including renal transplantation 

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3. Currently on an organ transplant waiting list or there’s a reasonable possibility of getting 
an organ transplant within 6 months of screening 
4. Histological FSGS subtype of collapsing variant 
5. Subjects who initiated, discontinued or changed dose of rituximab or other anti-CD20 
monoclonal antibodies within 16 weeks (4 months) prior to screening are excluded. 
Subjects who initiated treatment with rituximab or other anti-CD20 monoclonal 
antibodies >16 weeks (4 months) prior to screening are permitted if deemed safe by the 
investigator and only if they are intended to remain on continued, unchanged therapy at a 
dosing interval that has been documented to achieve continuous B cell depletion for the 
given patient. UPCR and other urine protein assessments up to 1 year prior to screening 
(if available) that were performed in these patients as part of the clinical routine should 
be recorded in the medical history. 
6. Subjects who discontinued Rituximab or other anti-CD20 monoclonal antibodies >16 
weeks (4 months) prior to screening without confirmed recovery of CD20+ B cell 
population to within normal range are excluded. Subjects who discontinued rituximab or 
other anti-CD20 monoclonal antibodies >16 weeks (4 months) prior to screening with 
confirmed recovery of CD20+ B cell population to within normal range are permitted in 
the study. UPCR and other urine protein assessments up to 1 year prior to screening (if 
available) that were performed in these patients as part of the clinical routine should be 
recorded in the medical history. 
7. Subjects who initiated or increased the dose of calcineurin inhibitors, or other 
immunotherapy, within 12 weeks prior to screening are excluded; dose reductions to 
maintain calcineurin inhibitors at optimal safe levels are permitted. Subjects who initiated 
a treatment with calcineurin inhibitors, or other immunotherapy >12 weeks are permitted. 
UPCR and other urine protein assessments up to 1 year prior to screening (if available) 
that were performed in these patients as part of the clinical routine should be recorded in 
the medical history. 
8. Subjects taking glucocorticoids at dose greater than 10 mg/day prednisone equivalent 
within 4 weeks prior to screening are excluded. 
9. Plasmapheresis within 12 weeks prior to screening 
10. Body Mass Index (BMI) ≥ 40 
11. Participated in any clinical study of an investigational product within 12 weeks prior to 
screening or within 5 half-lives after taking the last dose 
12. Currently on dialysis or likely to require dialysis during the study 
13. History or presence of any form of cancer within the 5 years prior to screening, with the 
exception of excised basal cell or squamous cell carcinoma of the skin, or carcinoma in 
situ such as cervical or breast carcinoma in situ that has been excised or resected 
completely and is without evidence of local recurrence or metastasis 
14. Positive HBV, HCV, or HIV viral screening test. Subjects who have received highly 
effective therapy for HCV demonstrated to have negative viral titers for at least 6 months 

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following discontinuation of treatment, will be considered to have a negative HCV 
screening test weeks prior to screening 
15. Evidence of tuberculosis based on interferon γ release assay (IGRA), tuberculin purified 
protein derivative (PPD) skin test, or chest radiography done during screening or within 6 
weeks prior to screening 
16. Evidence of hepatic disease; AST, ALT, alkaline phosphatase >2x ULN, or total bilirubin 
> 2x ULN or INR > 1.5 x ULN at screening with the exception that isolated INR 
elevation in the absence of other significant liver enzyme abnormalities is explained by 
anticoagulant therapy, (e.g. warfarin). 
17. Clinically significant peripheral neuropathy 
18. Hematologic abnormalities as follows: Hb <8 g/dL, platelets <50,000, ANC <1000 
cells/μL) at baseline 
19. Abnormality of ECG at screening, assessed by the Investigator as clinically significant 
(e.g. QTcF greater than 450 msec). 
20. History of alcohol or illicit drug abuse. Recreational use of cannabis is not excluded 
where legalized. 
21. History of gastrointestinal conditions that may interfere with study medication 
compliance, e.g., severe gastroparesis, with regurgitation of food or oral medication 
22. Known hypersensitivity to CCX140-B or inactive ingredients of the CCX140-B tablets 
(including microcrystalline cellulose, starch, crospovidone, magnesium stearate, or 
silicon dioxide) 
23. Renal disease associated with disorders other than FSGS (e.g. lupus nephritis, C3 
glomerulonephropathy) that is active, or has significant risk of progressing to end state 
renal disease during the 12-week initial treatment phase of the study 
24. History or presence of systemic disorder other than FSGS that requires, or is expected to 
require, systemic corticosteroids or immune modulators; topical or inhaled corticosteroids 
and immune modulators are not excluded 
25. History or presence of any medical condition or disease which, in the opinion of the 
Investigator, may place the subject at unacceptable risk for study participation 
26. Subjects taking strong CYP3A4 inducers (e.g., phenytoin, rifampicin, carbamazepine, St. 
John’s Wort) or strong CYP3A4 inhibitors (e.g., boceprevir, clarithromycin, conivaptan, 
grapefruit juice, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, 
nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, and 
voriconazole) within two weeks prior to screening. 
27. Subjects taking lithium, pamidronate or interferon; subjects taking non-steroidal anti-
inflammatory agents (NSAIDS) chronically (intermittent, i.e. occasional NSAIDS for 
pain or fever is discouraged, but is not excluded). 

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5. 
STUDY TREATMENTS 
5.1. 
Product Characterizations 
The investigational medicinal products (IMP) are oral film coated tablets containing 5 mg of 
CCX140-B. The tablets are manufactured under current good manufacturing practice. The 
clinical trial material is packaged in high density polyethylene (HDPE) bottles containing 30 
tablets. 
5.2. 
Rationale for Dose Range 
5.2.1. 
Safety 
The three active treatment doses proposed for the study are 5 mg administered twice daily, 10 
mg administered twice daily and 15 mg administered twice daily. 
Five (5) mg twice daily is selected as the lowest dose for exploration of dose response. The 
projected exposure at this dose is well below the NOAEL established in non-clinical toxicology 
studies. Specifically, using the rat as the more sensitive species based on exposure, the NOAEL 
in the 26-week rat study was 15 mg/kg/day. The NOAEL in the 13-week and 26-week rat studies 
were associated with systemic exposures to CCX140 which were about 18-20 times higher than 
the projected human exposure associated with a daily 5 mg dose, and about 8-10 fold a 10 mg 
BID dose. 
Ten (10) mg twice daily is selected as an intermediate dose for exploration of dose response and 
to guard against losing the efficacious dose if 15 mg twice daily is associated with unexpected or 
intolerable toxicity. Using the rat as the more sensitive species based on exposure, the NOAEL 
in the 26-week rat study was 15 mg/kg/day. The NOAEL in the 13-week and 26-week rat studies 
were associated with systemic exposures to CCX140 which were about 9-10 times higher than 
the human exposure associated with a daily 10 mg dose, and about 4-5 fold a 10 mg BID dose. 
Fifteen (15) mg twice daily is selected as the highest dose for this protocol, which is projected to 
provide exposure at both peak and trough that is within the safety margins established in the 
toxicology program, and that is projected to maintain a high level of target engagement, as 
summarized below and detailed in the Investigator’s Brochure. The projected clinical exposure 
with 15 mg twice daily dosing is 3-fold below the NOAEL exposure established in the most 
sensitive species tested in the nonclinical toxicity studies (rat 26-week, PO, QD) The safety 
margin associated with this exposure is approximately 3-fold below the NOAEL established in 
rat, and 5-8-fold below the exposure established in dog. 
The projected exposures and safety margins were established for humans without proteinuria. 
Because CCX140-B is highly protein bound, nephrotic subjects may have a lower exposure at a 
given dose due to potential drug loss with protein in urine. 
In humans, CCX140-B has been administered and was well tolerated at 15 mg administered once 
daily for up to 10 days in healthy volunteers, and at 10 mg administered once daily for up to 52 
weeks in subjects with Diabetic Nephropathy (DN). Administration of 15 mg twice daily in 
humans without proteinuria is projected to achieve a peak concentration of ~6.5 µg/mL (~13 
µM) and a trough concentration of ~5.5 µg/mL (~11µM) at steady state. Total exposure with 15 
mg twice daily is projected to be ~2-fold higher than that achieved with 15 mg daily 

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administration in healthy volunteers and 3.07-fold higher than that observed with 10 mg daily 
exposure in subjects with DN. Trough exposure with 15 mg twice daily is projected to be 2.4-
fold higher than trough exposure achieved in DN. 
The projected clinical exposure with 15 mg BID dosing is 3-fold below the NOAEL exposure 
established in the most sensitive species tested in the nonclinical toxicity studies (rat 26-week, 
PO, QD), as detailed in the Investigator’s Brochure. 
Rationale for selection of 15 mg twice daily is to ensure adequate exposure margin to address: 
1. Loss of albumin-bound drug through proteinuria; a post-hoc analysis PK in subjects 
of trial #CL005_140 who entered in study with albuminuria >1 gram indicated 
exposure was approximately 30% below the mean for the treatment group as a whole 
2. Increased binding competition from endogenous ligand; MCP-1 is reported to be 
increased in serum and urine in subjects with FSGS 
Rationale for administration of the highest dose as a divided dose (15 mg twice daily), as 
opposed to a single dose (30 mg once daily) include: 
1. Ease of administration (3 tablets twice daily vs 6 tablets once daily) 
2. 15 mg administered as a single daily dose has previously been tested and tolerated in 
healthy volunteers, while 30 mg has not been previously dosed 
3. 15 mg twice daily will result in a lower Cmax than 30 mg once daily, mitigating the 
potential for Cmax-driven safety concerns 
To ensure early detection of dose-related safety signals, the proposed protocol will incorporate: 
1. Frequent clinical visits and safety laboratory assessments, including application of a 
standard health assessment and a questionnaire to detect symptoms of neuropathy at 
regular intervals as detailed in the Time and Events Table. 
2. Safety monitoring by a Safety Review Committee throughout the study. 
In all subjects, treatment at the selected dose will be continued through 12 weeks or until 
declaration of Treatment Failure, where Treatment Failure is defined as any of the following: 
1. Progression of renal disease, defined as eGFR that is both below 60 ml/min/1.73 m2 
and is confirmed to represent at least a 30% decline in eGFR from baseline; 
2. Requirement for rescue with glucocorticoids, other new immunomodulatory or 
immunosuppressive therapy, plasmapheresis, or dialysis, per the judgment of the 
Investigator; 
3. Pre-specified adverse event or laboratory abnormality that, per Protocol, requires 
permanent discontinuation of study CCX140-B 
4. Any other treatment-related adverse event, laboratory evidence of toxicity, or 
intolerance that, in the judgment of the Investigator, warrants permanent 
discontinuation of CCX140-B. 

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5.2.2. 
Efficacy 
This study is designed to test CCX140-B at doses ranging from 5 mg twice daily, through 15 mg 
bid, a dose projected to provide continuous high level receptor engagement and antagonism. 
The targeted dose was derived from observations in a murine model with features of FSGS, the 
Adriamycin-induced nephropathy mouse model (Lee, et al., 2011). CCX140 does not bind 
murine CCR2 and human CCR2 with the same potency. Therefore the model was established in 
a CCR2 knockout and human CCR2 knock-in (hCCR2-KI) mouse. Mice were treated with 10, 
30, and 90 mg/kg of CCX140 once daily for 9 days. The 24 hour blood samples from 90 mg/kg 
dosing resulted in close to 100% receptor coverage. The mean CCX140 concentration at trough 
was 3.3 µg/mL. 
In the absence of proteinuria, the human trough concentration with 15 mg twice daily dosing is 
projected to be ~5.5 µg/mL, which is higher than the 3.3 µg/mL required for 100% CCR2 
blockade in the above described hCCR2 mouse model. However, the projected exposures and 
safety margins were established for humans without proteinuria. Because CCX140-B is highly 
protein bound, subject with nephrotic-level proteinuria may have a lower exposure at a given 
dose due to potential drug loss with protein in urine or impact of disease on metabolism. A post-
hoc analysis of PK in diabetic nephropathy subjects of trial CL005_140 who entered in study 
with albuminuria of 2 – 3 grams indicated that exposure was approximately 30% below the mean 
for the treatment group as a whole (~0.8 µg/mL versus ~1.2 µg/mL). 
Therefore 15 mg twice daily was selected as the upper limit for dose ranging for subjects without 
nephrotic level proteinuria. 
5.3. 
Drug Supply 
5.3.1. 
Packaging and Labeling 
The investigational medicinal products (IMP) are oral film coated tablets containing 5 mg of 
CCX140-B. The tablets are manufactured under current good manufacturing practice. The 
clinical trial material is packaged in high density polyethylene (HDPE) bottles containing 30 
tablets. 
5.3.2. 
Storage 
CCX140-B tablets will be stored according to label instructions. Access should be restricted to 
pharmacy staff or to the designated responsible member of the Investigator’s staff, and to the 
study monitor. The Investigator agrees that neither s/he nor any of the study staff will supply 
study medication to any persons other than those enrolled in the study. 
5.3.3. 
Blinding 
Since this study is open-label blinding guidelines are not applicable. 

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6. 
STUDY PROCEDURES 
6.1. 
Screening Assessments (Day-28 to Day 0) 
Prior to any study related assessments review the Informed Consent and have the subject 
acknowledge their understanding, then provide a copy to the subject 
1. Demographics, Medical History (verify plasmapheresis status within 12 weeks of screening) 
2. Screening for Tuberculosis, HIV, HBC & HCV if status is unknown 
3. Full Physical Examination and Vital Signs including height, weight, heart rate, respiratory 
rate and body temperature 
4. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
5. Leg circumference and bioimpedance to assess edema 
6. Renal biopsy for FSGS confirmation if status is unknown 
7. Hematology, Serum Chemistry, Urinalysis 
8. Pregnancy Test for women of childbearing potential 
9. 12-lead ECG (no triplicate ECG is necessary for screening) 
10. Concomitant medications including medications taken for up to 12 weeks prior to screening 
Day 1 should be captured on the eCRF. Prior treatment with rituximab or other anti-CD20 
monoclonal antibodies and corresponding levels of CD20+ B cells need to be recorded up to 
1 year prior to screening Day 1. Eligible patients who have entered the study on a continuous 
cycle therapy of rituximab or other anti-CD20 monoclonal antibodies need to remain on an 
unchanged therapy throughout the study at a dosing interval that has been documented to 
achieve continuous B cell depletion for the given patient unless prohibited due to safety 
considerations. Their levels of CD20+ B cells that are routinely evaluated per standard of 
care with anti-CD20 monoclonal antibody therapy need to be recorded throughout the study. 
Record UPCR and urine protein assessments up to 1 year prior to screening (if available) in 
the medical history if patients were treated with Rituximab, other CD20+ monoclonal 
antibodies, calcineurin inhibitors, or other immunotherapy 
6.2. 
Study Day 1 (After Confirmation of Eligibility) 
All evaluations on day 1 (unless otherwise specified in the Day 1 section) will occur PRIOR 
to dosing to serve as baseline evaluations. 
1. Enroll study subject and obtain enrollment ID code 
2. Full Physical Examination and Vital Signs including height, weight, heart rate, respiratory 
rate and body temperature 
3. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection 
4. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 

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5. Leg circumference and bioimpedance to assess edema 
6. Triplicate 12-Lead ECG 
7. Pregnancy Test for women of childbearing potential 
8. Hematology, Serum Chemistry, Urinalysis 
9. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection. 
Please be reminded that a 24 hr. urine collection at baseline (Study day 1) and at week 12 
are mandatory. 
10. SF-36 v2 and EQ-5D-5L 
11. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
12. Pharmacokinetics (time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose), the time 0 sample is to be 
collected just prior to administration of the first dose of CCX140-B. Subjects must fast prior 
to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
13. PD (plasma, serum and urine and whole blood for the lymphocyte subset and DNA) 
14. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
15. Adverse Events- record all adverse events starting with first dose of study medication and 
serious adverse events starting after administration of Informed Consent. 
6.3. 
Study Day 15 (Safety, Efficacy and PK) 
1. Symptom directed abbreviated physical examination and vital signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST). 
3. Leg circumference and bioimpedance to assess edema 
4. Hematology, Serum Chemistry, Urinalysis 
5. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection 
6. Review diary and used study medication with each subject. Confirm dosing compliance. 
7. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of 10 mg CCX140-B) 
8. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 

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9. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
10. Adverse Events- record all adverse events starting with first dose of study medication. 
6.4. 
Study Day 29 (Safety, Efficacy and PK) 
1. Symptom directed abbreviated physical examination and vital signs including weight, heart 
rate, respiratory rate and body temperature.  
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 
6. Hematology, Serum Chemistry, Urinalysis 
7. Review diary and used study medication with each subject. Confirm dosing compliance. 
8. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
Discuss new potential dose when relevant. 
9. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24 hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24 hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection 
10. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 
13. Pharmacokinetics (time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose), the time 0 sample is to be 
collected just prior to administration of the first dose of CCX140-B. Subjects must fast prior 
to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
6.5. 
Study Day 43- evaluate results from Day 29 (modify dose per dose 
modification guidelines based on PK results) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. AIDS Clinical Trials Group Brief Peripheral 
Neuropathy Screening Tool (ACTG BPNST) 
2. First morning void for UPCR measurement 
3. Review diary and used study medication with each subject. Confirm dosing compliance. 
4. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the next) 

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5. Dispense diaries and Directions for Use along with CCX140-B bottles. Discuss new potential 
dose when relevant. 
6. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
7. Adverse Events- record all adverse events. 
6.6. 
Study Day 57( +/- 3 Days)-Safety, Efficacy, PK 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 
6. Review diary and used study medication with each subject. Confirm dosing compliance. 
7. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
Discuss new potential dose when relevant. 
8. Hematology, Serum Chemistry, Urinalysis 
9. Pharmacokinetics (time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose), the time 0 sample is to be 
collected just prior to administration of the next dose of CCX140-B. Subjects must fast prior 
to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
10. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 
11. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24 hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24 hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection 
12. SF-36 V2 and EQ-5D-5L 
13. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
14. Adverse Events- record all adverse events. 
6.7. 
Study Day 71( +/- 3 Days)-Safety, Efficacy, PK 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature.  
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Review diary and used study medication with each subject. Confirm dosing compliance. 

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4. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B).  However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw.  
5. First morning void for UPCR measurement 
6. SF-36 V2 and EQ-5D-5L 
7. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
8. Adverse Events- record all adverse events. 
6.8. 
Study Week 12 or Day 85 (+/- 3 days) (Safety & Efficacy)- See 
Section 6.19 for PK elimination visit guidelines 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature.  
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 
6. Hematology, Serum Chemistry, Urinalysis 
7. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24 hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24 hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection. 
Please be reminded that a 24 hr. urine collection at baseline (study day 1) at week 12 are 
mandatory. 
8. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
9. SF-36 V2 and EQ-5D-5L 
10. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 
11. Review diary and used study medication with each subject. Confirm dosing compliance. 
12. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 

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13. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
14. Adverse Events- record all adverse events. 
6.9. 
Week 16 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 
6. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B).  However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
7. First morning void for UPCR measurement 
8. SF-36 V2 and EQ-5D-5L 
9. Review diary and used study medication with each subject. Confirm dosing compliance. 
10. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 
6.10. 
Week 20 (+/- 1 week) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 
6. First morning void for UPCR measurement 
7. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B).  However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 

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be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
8. SF-36 V2 and EQ-5D-5L 
9. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 
10. Review diary and used study medication with each subject. Confirm dosing compliance. 
11. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
12. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
13. Adverse Events- record all adverse events. 
6.11. 
Week 24 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 
6. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B).  However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
7. First morning void for UPCR measurement 
8. SF-36 V2 and EQ-5D-5L 
9. Review diary and used study medication with each subject. Confirm dosing compliance. 
10. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 

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6.12. 
Week 28 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 
6. Hematology, Serum Chemistry, Urinalysis 
7. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr. urine collection 
8. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
9. SF-36 V2 and EQ-5D-5L 
10. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 
11. Review diary and used study medication with each subject. Confirm dosing compliance. 
12. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
13. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
14. Adverse Events- record all adverse events. 
6.13. 
Week 32 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 

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6. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
7. First morning void for UPCR measurement 
8. SF-36 V2 and EQ-5D-5L 
9. Review diary and used study medication with each subject. Confirm dosing compliance. 
10. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 
6.14. 
Week 36 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 
6. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw.  
7. First morning void for UPCR measurement 
8. SF-36 V2 and EQ-5D-5L 
9. Review diary and used study medication with each subject. Confirm dosing compliance. 
10. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 

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6.15. 
Week 40 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 
6. Hematology, Serum Chemistry, Urinalysis 
7. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24 hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24 hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr urine collection. 
8. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
9. SF-36 V2 and EQ-5D-5L 
10. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0. 
11. Review diary and used study medication with each subject. Confirm dosing compliance. 
12. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
13. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
14. Adverse Events- record all adverse events. 
6.16. 
Week 44 (+/-1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 

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6. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
7. First morning void for UPCR measurement 
8. SF-36 V2 and EQ-5D-5L 
9. Review diary and used study medication with each subject. Confirm dosing compliance. 
10. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 
6.17. 
Week 48 (+/- 1 week) (See Section 6.19 for PK elimination visit 
guidelines) 
1. Symptom directed abbreviated physical examination and Vital Signs including weight, heart 
rate, respiratory rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Triplicate 12-Lead ECG 
4. Pregnancy Test for women of childbearing potential 
5. Hematology, Serum Chemistry, Urinalysis 
6. Pharmacokinetics (Cmin) – sample is to be collected at time 0 (just prior to taking the first 
dose of CCX140-B). However, if there was a dose increase in the prior 2 weeks, PK AUC 
(time 0, 0.5, 1, 2, 3, 4 & 6 hours post dose) is to be collected instead. The time 0 sample is to 
be collected just prior to administration of the first dose of CCX140-B. Subjects must fast 
prior to the time 0, 0.5 and 1 hour PK and can consume food immediately after the 1 hour PK 
blood draw. 
7. First morning void for UPCR measurement 
8. SF-36 V2 and EQ-5D-5L 
9. Review diary and used study medication with each subject. Confirm dosing compliance. 
10. Dispense diaries and Directions for Use along with a 30 day supply of CCX140-B bottles. 
11. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
12. Adverse Events- record all adverse events. 

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6.18. 
Week 52 or Termination Visit (See Section 6.19 for PK elimination 
visit guidelines) 
1. Full Physical Examination and Vital Signs including height, weight, heart rate, respiratory 
rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 
6. Hematology, Serum Chemistry, Urinalysis. 
7. 24 hour urine collection for UPCR, UACR and total protein. Subject starts 24hr. collection 
after the first void in the morning of the day prior to the visit day and should bring the 
24 hr collection (including first morning void on the visit day) to the visit. Sites need to 
contact the subject prior to the visit day as a reminder to start the 24 hr urine collection 
8. SF-36 V2 and EQ-5D-5L 
9. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0 
10. PK concentration sample 
11. Review diary and used study medication with each subject. Confirm dosing compliance. 
12. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
13. Adverse Events- record all adverse events that are ongoing at the time of this visit and 
determine if the subject needs to be further evaluated 
6.19. 
Post Treatment Elimination Blood Draws 
At the termination visit and again 7 and 14 days following the subject’s final dose of study 
medication record the date/time of last dose and collect a blood sample for drug elimination 
assessment. 
6.20. 
Follow-Up Visits - Four Weeks After Final Dose of Study Medication 
For Any Reason 
1. Full Physical Examination and Vital Signs including height, weight, heart rate, respiratory 
rate and body temperature. 
2. AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
3. Leg circumference and bioimpedance to assess edema 
4. Triplicate 12-Lead ECG 
5. Pregnancy Test for women of childbearing potential 

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6. PD (plasma, serum and urine and whole blood for the lymphocyte subset). Samples are to be 
collected at time 0 
7. Hematology, Serum Chemistry, Urinalysis 
8. First morning void for UPCR measurement 
9. Concomitant medications- verify and document any new medications that subject has started 
since the previous visit. 
10. Adverse Events- record all adverse events that are ongoing at the follow up visit and 
determine if the subject needs to be further evaluated. 
7. 
STUDY ASSESSMENTS 
7.1. 
Physical Examinations, Vital Signs, and ECGs 
A complete physical examination (including evaluation of general appearance/mental status, 
HEENT [head, eyes, ears, nose, throat], and the following body systems: dermatologic, 
cardiovascular, respiratory, gastrointestinal, musculoskeletal and neurologic) for safety, 
assessments will be performed at visits indicated in the Time and Events Table. 
Body weight will be measured as part of the physical examinations. Height needs to be recorded 
at screening only. BMI will be calculated from the body weight and height measurements. 
Vital signs and body weight will be measured during screening and on each scheduled study day 
as indicated in the Time and Events Table. Blood pressure, pulse rate, and body temperature will 
be measured. All vital signs assessments will be performed after the subject has rested for at least 
three minutes, while subject is seated. Body weight should be measured without shoes and the 
subject clothed in under garments or hospital gown only using a calibrated scale. 
Leg circumference will be measured during screening, Day 1 and each scheduled visit as 
indicated in the Time and Events Table. A supplied measuring tape will be used to measure the 
leg circumference in each leg at the point of widest circumference. The measurement should be 
made with the subject standing with weight distributed equally on each foot. 
The AIDS Clinical Trials Group Brief Peripheral Neuropathy Screening Tool (ACTG BPNST) 
will be used for the evaluation of peripheral neuropathy. 
Bioimpedance will be measured during screening, Day 1 and each scheduled visit as indicated in 
the Time and Events Table. 
A baseline 12-lead ECG, after resting for at least 3 minutes, will be performed at screening and 
assessed for any clinically significant abnormalities. When required (Time and Events Table), 
triplicate ECGs should be performed with a 1 minute interval between tracings. All abnormalities 
will be recorded in the EDC system. Assuming the ECG abnormality does not preclude study 
entry, an ECG may be repeated as clinically indicated. 
7.2. 
Clinical Safety Laboratory Assessments 
The following tests will be performed at the visits identified in the Time and Events Table. 

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Hematology: hemoglobin, hematocrit, RBC count, WBC count with differential, platelet count, 
mean cell hemoglobin, mean cell hemoglobin concentration, mean corpuscular volume, 
haptoglobin and schistocytes. 
Serum Chemistry: liver panel (total and direct bilirubin, lactate dehydrogenase [LDH], AST, 
ALT), renal panel (BUN, creatinine), creatine phosphokinase (CPK), albumin, sodium, 
potassium, magnesium, bicarbonate, chloride, calcium, inorganic phosphorus, glucose, total 
protein, alkaline phosphatase, coagulation panel (PT, PTT, INR), lipid panel (HDL, non-HDL 
LDL, Triglycerides, Total Cholesterol), uric acid, serum amylase, serum lipase, LDH and 
cystatin C. 
Urinalysis: At the central laboratory, nitrite, blood, and protein, will be tested. If positive, 
microscopy will be performed. 
Virology (measured only at screening and may be measured at the local laboratory): hepatitis B 
surface antigen, hepatitis C antibodies, HIV 1 and 2 antibodies; virology tests done within 6 
weeks prior to screening are acceptable for eligibility assessment. 
TB screen: Only one of the following is needed: interferon γ release assay (IGRA), tuberculin 
purified protein derivative (PPD) skin test, or chest radiograms (X rays or CT scan); chest 
radiography done within 6 weeks prior to screening is allowed for eligibility assessment. Chest 
radiography at subsequent visits will only be performed if deemed clinically necessary by the 
Investigator to assess safety. 
Pharmacodynamics: urinary MCP-1, and potentially other biomarkers of renal inflammation, 
fibrosis and injury. Assessment of blood and/or urinary biomarkers associated with CCR2. 
biology, which may include peripheral blood leukocyte subsets and selected cytokines and 
chemokines. 
Genetic Markers: Assessment of biomarkers associated with risk of severity of disease, which 
may include allelic variations associated with the pathogenesis and/or prognosis of FSGS such as 
NPHS1, NPHS2, WT-1, LAMB2, CD2AP, TRPC6, ACTN4, INF2, APOL1. 
7.3. 
Adverse Events 
An adverse event (AE) is defined as any untoward medical occurrence associated with the use of 
a drug in humans, whether or not considered drug related. An adverse event could therefore be 
any unfavorable and/or unintended sign (including abnormal laboratory findings), symptom, or 
disease temporally associated with the use of the drug, whether or not considered related to the 
drug. This definition includes inter-current illnesses or injuries, and exacerbation of pre-existing 
conditions. 
An unexpected adverse event is an adverse event that is not identified in nature, severity, or 
frequency in the current Investigator’s Brochure, or that is of greater severity than expected 
based on the information in the Reference Safety Information listing within the Investigator’s 
Brochure. 
All adverse events occurring in subjects who have been treated with CCX140-B will be recorded 
in the EDC system and will be reported in accordance with regulatory requirements. Adverse 
events reported prior to commencement of administration of study medication will be considered 

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pre-treatment events. If subject stops taking CCX140-B, adverse event reporting should be 
continued as long as study is active. 
All adverse events will be monitored until resolution or, if the adverse event is determined to be 
chronic, until a cause is identified. If an adverse event remains unresolved at the conclusion of 
the study, a clinical assessment will be made by the Investigator and the Sponsor’s Medical 
Monitor to determine whether continued follow-up of the adverse event is warranted. 
7.3.1. 
Adverse Event Severity Assessment 
The severity of each adverse event will be determined by the investigator using the following 
scale: 
• Mild (Grade 1):  no limitation of usual activities 
• Moderate (Grade 2):  some limitation of usual activities 
• Severe (Grade 3):  inability to carry out usual activities 
• Life-threatening (Grade 4):  an immediate risk of death 
• Death (Grade 5) 
7.3.2. 
Causality Assessment 
The relationship of CCX140-B to an adverse event will be determined by the Investigator and 
Sponsor based on the following definitions: 
• Probably Not Related: the adverse event was more likely explained by causes other than 
study drug CCX140-B. 
• Possibly Related: there is evidence for a reasonable possibility that study drug CCX140-B 
administration caused the adverse event. 
7.3.3. 
Serious Adverse Events 
A serious adverse event (SAE) is defined as any untoward medical occurrence that at any dose: 
• Results in death; 
• Is life-threatening (i.e., the subject was, in the opinion of the Investigator, at immediate risk 
of death from the event as it occurred); 
• Requires or prolongs hospitalization; 
• Results in persistent or significant disability or incapacity; 
• Is a congenital anomaly or birth defect; 
• Is an important and significant medical event that, based on appropriate medical judgment, 
may jeopardize the subject and/or may require medical or surgical intervention to prevent 
one of the other outcomes defining serious. 
Elective surgery already known during screening to occur in the course of the study, and elective 
hospitalizations for convenience of the subject which are clearly unrelated to any medical 
condition, and agreed upon between the Investigator and the subject, will not have to be reported 

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as SAEs. Hospital stays on the evening of Day 1 (or beyond) will also not be considered an SAE, 
unless other SAE criteria are met. 
7.3.4. 
SARs and SUSARs 
A serious adverse reaction (SAR) is defined as an SAE for which there is at least a reasonable 
possibility that the study drug CCX140-B caused the event. 
A suspected unexpected serious adverse reaction (SUSAR) is defined as an SAE for which there 
is at least a reasonable possibility that the study drug CCX140-B caused the event, and the SAE 
is ‘unexpected’, i.e., not described in terms of nature, severity, or frequency in the Reference 
Safety Information within the current Investigator’s Brochure. 
‘Reasonable possibility’ means that there is evidence to suggest a causal relationship between the 
study drug and the adverse event. Within the reporting requirements, the following examples 
illustrate the types of evidence that would suggest a causal relationship: 
• A single occurrence of an event that is uncommon and known to be strongly associated 
with drug exposure (e.g., angioedema, hepatic injury, Stevens-Johnson Syndrome); 
• One or more occurrences of an event that is not commonly associated with drug 
exposure, but is otherwise uncommon in the population exposed to the drug (e.g., tendon 
rupture); 
• An aggregate analysis of specific events observed in a clinical trial (such as known 
consequences of the underlying disease or condition under investigation or other events 
that commonly occur in the study population independent of drug therapy) that indicates 
those events occur more frequently in the drug treatment group than in a concurrent or 
historical control group. 
Events related to the underlying disease, such as relapses or worsening of disease will not be 
considered as SUSARs, unless there is a reasonable possibility that CCX140-B use was 
associated with these events. 
7.3.5. 
Pregnancies 
Any pregnancies that occur in female subjects or partners of male study subjects must be 
reported to 
 Clinical Safety within 24 hours of awareness to the contacts details outlined 
in Section 7.3.3 An exposure in Utero form will then be forwarded for completion as soon as 
possible. All pregnancies must be followed up until conclusion and the outcome of the 
pregnancy reported within 24 hours of awareness to
Clinical Safety. Should the 
outcome of the pregnancy meet criteria for an SAE, th
ould be reported as indicated in 
Section 7.3.3. 
7.3.6. 
Special Situation Reporting 
Special situation reports include reports of overdose, misuse and abuse of the IMP: 
• Overdose: refers to the administration of a quantity of a medicinal product given per 
administration or cumulatively (accidentally or intentionally), which is above the maximum 
recommended dose according to the protocol. Clinical Judgment should always be applied. In 
cases of a discrepancy in the drug accountability, overdose will be established only when it is 

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clear that the subject has taken excess dose(s) or the investigator has reason to suspect that 
the subject has taken additional dose (s). 
• Misuse: refers to situations where the medicinal product is intentionally and inappropriately 
used not in a way that is not in accordance with the protocol instructions or local prescribing 
information and may be accompanied by harmful physical and/or psychological effects. 
• Abuse: is defined as persistent or sporadic, intentional excessive use of a medicinal product, 
which is accompanied by harmful physical or psychological effects. 
• Medication Error: Medication error is any unintentional error in the prescribing, dispensing 
or administration of a medicinal product by a healthcare professional, patient or consumer, 
respectively. The administration or consumption of the unassigned treatment and 
administration of an expired product are always reportable as medication errors, cases of 
subjects missing doses of investigational product are not considered reportable as medication 
error. 
All special situation reports must be reported on the special situations report form and forwarded 
to 
 Clinical Safety within 24 hours. 
All adverse events (AEs) associated with these special situation reports should be reported as 
AEs or SAEs at the same time using the AE eCRF and/or the SAE report form. Details of the 
symptoms and signs, clinical management and outcome will be reported, when available. 
7.3.7. 
Serious Adverse Event Reporting 
Any serious adverse event occurring from screening through the end of the treatment period, 
whether or not considered study related, will be reported immediately (within 24 hours) to the 
Safety team. Reporting is done by completing the SAE form in the EDC system. If it is not 
possible to access the EDC system, the Investigator will send an email to the appropriate regional 
clinical safety mailbox (see information below) or call their regional SAE hotline and fax the 
completed SAE report form within 24 hours of awareness. When access to the EDC system is 
resumed, the SAE information should be entered as soon as possible. Contact details are as 
follows: 
 
Clinical Safety 
Telephone: 
 or 
 or 
Facsimile: 
 or 
or 
e-mail: 
Any medication or other therapeutic measures used to treat the event, in addition to the outcome 
of the adverse event, will be recorded in the EDC system. 
Follow-Up Reports 
The investigator must continue to follow the subject until the SAE has subsided or until the 
condition becomes chronic in nature, stabilizes (in the case of persistent impairment), or the 
subject dies. 

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Within 24 hours of receipt of follow-up information, the investigator must update the SAE form 
electronically in the EDC system for the study and submit any supporting documentation (e.g., 
subject discharge summary or autopsy reports) to 
 Clinical Safety via fax or e-mail. If it 
is not possible to access the EDC system, refer to the procedures outlined above for initial 
reporting of SAEs. 
SAE reporting should continue for 90 days after stopping study drug. The Sponsor should be 
notified if the investigator becomes aware of any SAE that occurs after the end of the AE 
reporting period, if they believe that an event is related to the prior study drug treatment. 
The Sponsor or its representatives will report all SUSARs to national health authorities and 
ethics committees in an expedited manner in accordance with Clinical Trial Directive, Articles 
16 and 17, ICH Guideline E2A and ENTR CT3 on the reporting of all SUSARs. 
7.4. 
Study Completion and Withdrawal 
The Week 12 visit will be the final treatment day and the week 16 visit will be the last Study Day 
for all subjects, except for those participating in the Open-Label Extension (last visit will be 
Week 52). Procedures for this day will be completed per the Time and Events Table. Each 
subject’s condition will be evaluated by the Investigator at the end of the clinical trial and 
appropriate standard of care medical treatment will be provided to all subjects as needed. For 
early withdrawals from the study, the procedures for the Early Termination visit will be 
performed, including blood draws during the termination visit and again 7 & 14 days after the 
final dose, when possible. The clinical trial will be terminated early if there is a safety concern. 
7.5. 
Dose Modification Rules 
Table 2: 
Dose Modification Rules for Individual Subjects 
Assessment 
Event 
Timing 
Response 
Serum Creatinine 
50% increase from 
baseline value 
If confirmed by 
repeat measurement 
after 2 weeks 
If confirmed by repeat 
measurement after 2 weeks 
Urine 
Protein:Creatinine 
ratio (UPCR) 
If baseline UPCR       
< 6.0 g/g an increase 
of > 3.0 g/g; if UPCR 
≥ 6.0 g/g an increase 
of 50%  
If confirmed by 
repeat measurement 
after 2 weeks 
If confirmed by repeat 
measurement after 2 weeks 
ALT, AST  
ALT or AST ≥ 8x 
ULN  
Confirmed within 72 
hours (ALT, ASP, 
ALP and Total 
Bilirubin should all 
be re-tested)  
Consider Discontinuation of 
Treatment 
ALT, AST  
ALT or AST ≥ 5x 
ULN  
Confirmed within 2 
weeks (ALT, ASP, 
ALP and Total  
Bilirubin should all 
be re-tested) 
Consider Discontinuation of 
Treatment 
ALT, AST, Total 
ALT or AST ≥ 3x 
Confirmed within 72 
Consider Discontinuation of 

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Bilirubin and INR 
ULN  and Total 
Bilirubin > 2 ULN or 
INR > 1.5 
hours (ALT, ASP, 
ALP, Total Bilirubin 
and INR should all 
be re-tested) 
Treatment 
ALT, AST  
ALT or AST ≥ 3x 
ULN with the 
appearance of fatigue, 
nausea, vomiting, right 
upper quadrant pain or 
tenderness, fever, rash 
and /or eosinophilia 
(>5%) 
Confirmed within 72 
hours (ALT, ASP, 
ALP and Total 
Bilirubin should all 
be re-tested) 
Consider Discontinuation of 
Treatment 
Hemolysis  
Clinical diagnosis of 
hemolysis in the 
judgment of the 
investigator, based on 
elevation of LDH, 
elevation of 
reticulocyte count, 
elevation of bilirubin 
and or other laboratory 
indications. 
Halt treatment then 
reassess after 1 week 
If normalized, continue to treat at 
reduced dose, defined as the next 
lower dose. Otherwise, contact 
Medical Monitor to discuss next 
steps 
Anemia 
Hemoglobin reduced 
from baseline by ≥ 2 
g/L and deemed 
clinically significant 
by the PI 
Halt treatment then 
reassess after 2 
weeks 
If normal, continue to treat at 
reduced dose, defined as next 
lower dose. Otherwise, contact 
Medical Monitor to discuss next 
steps 
Reticulocyte Count 
Increase in 
reticulocyte count by 
≥2% (absolute) from 
baseline that is not 
otherwise explained 
Halt treatment then 
reassess after 2 
weeks 
If within normal range for the 
hematocrit, continue to treat at 
reduced dose, defined as next 
lower dose. Otherwise, contact 
Medical Monitor to discuss next 
steps 
Peripheral Neuropathy 
≥ moderate symptoms 
limiting  (limiting 
instrumental activities 
of daily living or 
asymptomatic (based 
on diagnostic 
evaluation only) 
and  deemed clinically 
significant by the 
investigator 
Halt treatment then 
reassess after 2 
weeks 
Contact Medical Monitor to 
discuss next steps 
Other Clinically 
Significant Events 
Any adverse events 
assessed as moderate, 
related to study drug, 
and clinically 
significant 
Halt treatment then 
reassess after 2 
weeks 
Contact Medical Monitor to 
discuss next steps 

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7.5.1. 
Dose Modification / Stopping Rules for the Overall Study 
A dose will be considered as potentially exceeding the maximum tolerated dose and 
consideration will be given for stopping the study should any of the following events occur: 
• 2 or more subjects with QTcF prolongation ≥ 450 msec and an increase from baseline of ≥ 30 
msec, based on triplicate 12-lead ECGs that is not otherwise explained 
• 2 or more subjects with clinically significant hemolytic anemia, defined as hemoglobin that 
has declined from baseline by at least 2 grams/deciliter and is less than or equal to 8 g/dL and 
is assessed by the investigator and is not otherwise explained 
• 2 or more subjects with bilirubin greater than 3 times upper limit of normal, and not 
ascribable to Gilbert’s disease 
• 2 or more subjects with new motor neuropathy or sensory neuropathy that limits instrumental 
activities of daily living and is assessed by the Investigator as clinically significant 
• 2 or more subjects who require dose reduction or discontinuation due to adverse events 
assessed as possibly related to Study Drug, where the adverse events are medically similar in 
the same System Organ Class based on MedDRA criteria. 
If a dose is assessed to be above the maximally tolerated dose, dosing and dose escalation will be 
limited to the next lower dose. 
A Safety Review Committee (SRC) comprising the Study Director, Medical Monitor, Clinical 
Pharmacologist, at least one external expert nephrologist, and Study Statistician will review all 
safety events described in the stopping rules and all serious adverse events as they occur. The 
SRC will periodically review all adverse events and all laboratory abnormalities. The SRC may 
declare a dose to be above the maximally tolerated dose based on the defined rules, or based on 
other considerations. 
7.5.2. 
Drug Accountability 
The study pharmacist and Investigator must maintain accurate records of dates and quantities of 
product(s) received, to whom dispensed (subject-by-subject accounting), and accounts of any 
product accidentally or deliberately destroyed. The Investigator must retain all unused and/or 
expired study supplies until the study monitor has confirmed the accountability data. 
7.6. 
Treatment Compliance 
Subjects will be screened within a period not to exceed 28 days prior to Day 1. 
The initial treatment period, including dose escalation, will be up to 12 weeks. Subjects who are 
not Treatment Failures and who otherwise meet criteria for continuation may continue treatment 
for up to an additional 40 weeks to assess achievement of remission and duration of response. 
All subjects should be followed for 4 weeks (28 days) after the last CCX140-B dose 
administered. 
The CCX140-B tablets will be self-administered by participating study subjects. The morning 
dose of study drug on Day 1 will be taken in the presence of study site personnel. Subjects will 
be provided with dosing instructions at the start of the study, and will be encouraged by study 
site personnel to take the study medication according to the instructions for the duration of the 

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study. Subjects will be instructed to bring the assigned bottles of study medication to the site 
staff at each study visit, whether empty or not. The Investigator or designee should review each 
subject’s dosing diary at each relevant visit, reconcile them against the study medication that 
each subject returns, document reasons for any discrepancies and calculate the dosing 
compliance rate. This information will be recorded and entered into the electronic data capture 
(EDC) system. Subjects that do not take at least 85% of the study medication can be 
discontinued from the study for non-compliance. 
7.7. 
Concomitant Medications and Restrictions 
The doses of RAAS inhibitors must be stable prior to Day 1, and remain stable throughout the 
course of the study, unless adjustment is required for the safety of the subject. For eligible 
subjects willing to participate in the Open-Label Extension (after Week 12), the doses of RAAS 
inhibitors and other concomitant drugs may be modified per physician judgment. Subjects who 
require addition of a new immunosuppressive or immunomodulatory agent may continue 
treatment with CCX140-B if they have not met criteria for treatment failure, the case has been 
discussed with the study director and there is agreement to continue treatment. Plasmapheresis is 
not allowed at any point during the course of the study. 
CCX140-B is primarily cleared through CYP3A4 metabolism. Subjects taking strong CYP3A4 
inducers (e.g., phenytoin, rifampicin, carbamazepine, St. John’s Wort) will be excluded from this 
clinical trial since these drugs may substantially reduce the CCX140-B plasma levels. In 
addition, subjects taking strong CYP3A4 inhibitors (e.g., boceprevir, clarithromycin, conivaptan, 
grapefruit juice, indinavir, itraconazole, ketoconazole, lopinavir/ritonavir, mibefradil, 
nefazodone, nelfinavir, posaconazole, ritonavir, saquinavir, telaprevir, telithromycin, and 
voriconazole) will be excluded as these drugs may substantially increase the CCX140-B plasma 
levels. 
All concomitant medications taken during the course and up to 12 weeks prior to screening Day 
1 study should be recorded meticulously on the concomitant medication pages of the CRF. 
Prior treatment with rituximab or other anti-CD20 monoclonal antibodies and corresponding 
levels of CD20+ B cells need to be recorded up to 1 year prior to screening. 
Eligible patients who have entered the study on a continuous cycle therapy of rituximab or other 
anti-CD20 monoclonal antibodies need to remain on an unchanged therapy  throughout the study 
at a dosing interval that has been documented to achieve continuous B cell depletion for the 
given patient unless prohibited due to safety considerations. Their levels of CD20+ B cells that 
are routinely evaluated per standard of care with anti-CD20 monoclonal antibody therapy need to 
be recorded throughout the study. Record UPCR and urine protein assessments up to 1 year prior 
to screening (if available) in the medical history if patients were treated with Rituximab, other 
CD20+ monoclonal antibodies, calcineurin inhibitors or other immunotherapy. 
7.7.1. 
Rescue Therapy 
If the investigator decides that a subject needs to receive rescue therapy the subject will be 
considered a treatment failure. Rescue therapy is considered any new therapy or required dose 
increase of an existing concomitant therapy (other than allowed per Section 7.7. Concomitant 

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Medications and Restrictions) that is required for the safety of the patient and to treat the 
underlying disease of FSGS. 
This would include but is not limited to the initiation of high dose glucocorticoid therapy, new 
immunosuppressive agents such as calcineurin inhibitors, cyclophosphamide, MMF, monoclonal 
antibodies, or other major treatments (e.g. plasmapheresis, dialysis). 
8. 
STATISTICAL METHODS 
Details of the statistical analysis will be provided in a separate statistical analysis plan (SAP), 
which will be written, finalized, and approved prior to database lock and will be included in the 
Clinical Study Report (CSR) for this protocol. The SAP will supersede the statistical analysis 
methods described in this clinical protocol. Any deviation from the protocol will be documented 
and described in the final report. If changes to principal features stated in the protocol are 
required, these will be documented in a protocol amendment. The final SAP will take into 
account any amendment to the protocol. Data analysis and writing of a CSR for all study data 
will be performed by the designated CRO in accordance with its SOPs. Analysis of PK and PD 
data, and writing of PK and PD reports will be performed by designated PK and PD teams in 
accordance with their standard operating procedures. 
All continuous study assessments will be summarized by visit (as applicable) and change from 
baseline to each post-baseline visit using descriptive statistics (n, mean, median, standard 
deviation, minimum, and maximum). All categorical study assessments will be summarized by 
visit (as applicable) using frequency counts and percentages. 
8.1. 
Demographics and Baseline Characteristics 
All subject baseline characteristics and demographic data (age, sex, race, ethnicity, weight, 
height, body mass index, viral test results, serum creatinine, FSGS duration [from time of first 
diagnosis based on renal biopsy]), eGFR, proteinuria (UPCR), physical examination 
abnormalities, medical history, previous (within 6 months of screening) and concomitant 
medications (including other treatments for FSGS) at study entry will be listed by study center 
and subject number, and will also be summarized. 
8.2. 
Efficacy Analysis 
8.2.1. 
Primary Endpoint Analysis 
The Primary Efficacy Objective is to evaluate the effect of CCX140-B on proteinuria in subjects 
with primary FSGS with nephrotic syndrome, assessed as a median reduction from baseline of 
urine protein to creatinine ratio (UPCR) of at least 20%, i.e. ≥ 20%, by week 12. 
The assumed treatment effect for this study is based on efficacy findings with 5 and 10 mg 
CCX140-B once daily in study CL005_140 in patients with diabetic nephropathy and 
proteinuria. In a pre-specified subset analysis, the greatest percentage improvement of UACR at 
approximately 12 weeks from baseline was observed in subjects who presented with the highest 
baseline UACR (801-3000 mg/g) and eGFR of at least 60 mL/min/1.73m2. This subgroup 
receiving 5 (N=10) or 10 mg (N=13) of CCX140-B once daily showed a median reduction in 

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UACR of approximately 20%-30% receiving CCX140-B while patients receiving placebo 
(N=12) showed an increase of approximately 12%. 
Based on these data, it is assumed that the median reduction from baseline UPCR is at least 20% 
by week 12 in the planned study CL012_140. If the final observed data are consistent with this 
assumption, i.e. the previous positive study results are replicated in the FSGS population, the 
study will be considered positive in assessing the efficacy of CCX140-B. 
Otherwise, if the observed data are consistent with the median reduction of below 20%, the study 
would not be considered positive. 
After all of the initial 6 subjects have completed their 12-week of treatment the study will be 
paused and assessed. If the overall study results are deemed safe and the subjects’ responses to 
treatment are considered clinically meaningful seven additional subjects will be treated for a total 
of 13 subjects. 
8.2.2. 
Secondary Endpoint Analysis 
Secondary endpoints include: 
• Achievement of Partial Remission (includes all of the following) 
− reduction from baseline by ≥50 percent in UPCR 
− reduction in UPCR to a level that is < 3.5 g/g at week 12 
− subject may not be a treatment failure  
• Achievement of Complete Remission (includes all of the following) at week 12 
− reduction in UPCR to < 0.3 g/g 
− serum albumin within normal range 
− for patients with abnormal serum creatinine levels at baseline, return to normal levels for 
that age group 
− for patients with normal serum creatinine levels at baseline, final value within 20% of 
baseline levels 
− subject may not be a treatment failure 
• Change from baseline of UPCR in subjects treated with CCX140-B over time 
• The proportion of subjects with partial remission at any time during the treatment period. 
• The proportion of subjects with complete remission at any time during the treatment period. 
• Time to Partial Remission (PR) or Complete Remission (CR) 
• Change from baseline in renal function, based on eGFR (calculated using the CKD-EPI 
Cystatin C equation (Dharnidharka VR, et al, 2002), the CKD-EPI Creatinine equation 
(Schwartz GJ, et al. 2016, Selistre L et al 2016), CKD-EPI Creatinine-Cystatin C equation 
(Stevens et al., 2008) and MDRD Creatinine equation (Levey et al. 1999, Levey et al. 2006) 
over time 
• Change over time in total 24 hour urine protein excretion 

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• Change from baseline in serum albumin over time 
• Change in UACR from baseline over time 
• Time to investigator or physician initiation of rescue therapy including glucocorticoids, new 
immunosuppressive agents or new major treatment modalities (e.g. plasmapheresis, dialysis) 
• Duration between achievement of remission and relapse or treatment failure 
• Changes over time in Health Quality Assessment (SF-36 v2 and EQ-5D-5L) 
Change from baseline will be summarized with descriptive statistics (mean, SD, median and 
range) and 95% confidence interval for the mean. Baseline is defined as the last assessment prior 
to the first dose of the study drug. 
Time to PR or CR and duration between achievement of remission and relapse or treatment 
failure will be summarized with the Kaplan-Meier estimate for the median and quartiles. 
The proportion of subjects who achieved either partial or complete remission and the proportion 
of subjects with treatment failures as of week 12 will be summarized separately similarly to the 
primary analysis. Formal statistical inference will not be made on these separate endpoints. 
Serum albumin, proteinuria, eGFR, serum lipids, edema (body weight, leg circumference and 
bioimpedance), weight and albuminuria will be summarized by visit and for change from 
baseline to each visit using continuous summary statistics and 90% and 95% confidence 
intervals. [Timeframe: Baseline to week12 and Baseline through end of treatment] 
Time to partial or complete remission will be summarized using Kaplan-Meier methods to 
estimate median time to remission as well as the 25th and 75th percentiles. Associated 95% 
confidence intervals, estimated using the method of Brookmeyer and Crowley (1982) will also 
be provided. Duration of remission will be summarized similarly. [Timeframe: Baseline to week 
12 and Baseline through end of treatment] 
Additionally, time to partial or complete remission will be summarized categorically by the first 
visit the subject showed partial or complete remission. [Timeframe: Baseline to week 12 and 
Baseline through end of treatment] 
The Health Quality Assessment (SF-36 V2 and EQ-5D-5L) will be summarized by visit and for 
change from baseline to each visit using continuous summary statistics and 90% and 95% 
confidence intervals for the overall score and for each of the 8 domain scores: Vitality, Physical 
Functioning, Bodily Pain, General Health Perceptions, Physical Role Functioning, Emotional 
Role Functioning, Social Role Functioning, and Mental Health. [Timeframe: Baseline to week 
12 and Baseline through end of treatment] 
8.3. 
Safety Analysis 
Safety endpoints include: 
• Subject incidence of treatment-emergent serious adverse events, adverse events, and dose 
interruptions and / or withdrawals due to adverse events; 
• Change from baseline and shifts from baseline in all safety laboratory parameters; 
• Change from baseline in vital signs or clinically significant ECG changes. 

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All clinical safety and tolerability data will be listed by subject. [Timeframe: Baseline-week 12 
and Day 85 through end of treatment] 
Subject incidence of treatment-emergent adverse events (TEAE) will be tabulated by MedDRA 
System Organ Class (SOC) and preferred term (PT), by relatedness and by maximum severity. 
[Timeframe: Baseline-week 12 and Day 85 through end of treatment] 
Treatment-emergent serious adverse events (TESAE) and TEAE leading to withdrawal will be 
listed. The listing will include MedDRA SOC, PT, and verbatim term, AE start/stop day, 
severity, relationship to study drug, action taken, and outcome. [Timeframe: Baseline-week 12 
and Day 85 through end of treatment] 
Individual vital signs and change from baseline in vital signs will be listed by subject and study 
visit, and summarized by visit. [Timeframe: Baseline-week 12 and Day 85 through end of 
treatment] 
Individual abnormalities recorded on physical examination at baseline, and change from 
baseline, will be listed by subject and study visit, and summarized by visit. [Timeframe: 
Baseline-week 12 and Day 85 through end of treatment] 
Individual ECG findings and change from baseline in ECG findings will be listed by subject and 
study visit, and summarized by visit. [Timeframe: Baseline-week 12 and Day 85 through end of 
treatment] 
Laboratory data (actual values and change from baseline) will be listed by subject and study 
visit. Abnormal laboratory values will be flagged. Shift tables will be generated for shifts in 
laboratory parameters by study visit. [Timeframe: Baseline-week 12 and Day 85 through end of 
treatment] 
8.4. 
Sample Size Assumptions 
Six (6) male or female adult subjects with biopsy-proven primary FSGS and nephrotic syndrome 
will be enrolled into the first stage of this study, which is followed by another seven subjects 
after the data review of the initial 6 patients. The relatively small sample size is selected based on 
the exploratory safety and efficacy nature of the study. After all of the initial 6 subject have 
completed their 12-week of treatment the study will be paused and assessed safety and efficacy. 
If the overall study results are deemed safe and the subjects’ responses to treatment are 
considered clinically meaningful seven additional subjects will be treated for a total of 13 
subjects. 
Further details will be specified in the SAP. 
8.5. 
Subject Populations 
8.5.1. 
Modified Intent-to-Treat Population 
The modified Intent-to-Treat (mITT) Population will include all subjects who have received a 
dose of the study drug, and have at least one post-baseline efficacy assessment. 

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8.5.2. 
Per Protocol Population 
The Per Protocol (PP) population will consist of all mITT subjects who do not have protocol 
deviations that could significantly affect the interpretation of the results for the primary 
endpoints. Subjects’ inclusion/exclusion from the PP population will be determined and 
documented prior to the database lock. Subjects’ inclusion into the PP population will also 
require drug compliance of ≥85%. 
8.5.3. 
Safety Population 
The safety population will include all subjects who have received at least one dose of study drug. 
8.6. 
Demographics and Baseline Characteristics 
All subject baseline characteristics and demographic data (age, sex, race, ethnicity, weight, 
height, body mass index, viral test results, FSGS duration [from time of first diagnosis based on 
renal biopsy]), eGFR, proteinuria (UPCR), physical examination abnormalities, medical history, 
previous (within 6 months of screening) and concomitant medications (including other 
treatments for FSGS) at study entry will be listed by study center and subject number, and will 
also be summarized. 
8.7. 
Subject Disposition 
The number of subjects who were screened, who screen failed (by reason), who completed Week 
12, who completed the 52 week extension study, who withdrew early from the study, along with 
the reasons for withdrawal, will be presented. 
8.8. 
Treatment Compliance 
Exposure will be summarized by dose at each visit as well as total dose and average weekly 
dose. 
CCX140-B plasma concentration measurements over the course of the study may also be used to 
assess subject compliance. Any events of non-compliance to the protocol will be documented in 
the study records. Non-compliant study subjects whose dosing compliance rate is less than 85% 
may be withdrawn from study. 
8.9. 
Protocol Deviations 
Significant protocol deviations (major and critical) will be listed and summarized by category. 
The effect of significant protocol deviations on the safety and efficacy outcomes will be assessed 
by conducting sensitivity analyses excluding subjects and/or study visits with significant 
protocol deviations. 
Examples of major deviations may include but are not limited to: 
• Failure to properly consent each study subject (i.e., using the correct version of the ICF in the 
appropriate language prior to initiation of study procedures, etc.) 
• Enrollment of ineligible subjects 
• Performance of procedures not defined in the approved protocol 

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• Study medication dispensing or dosing errors 
• Failure of a study subject to return unused study medication after completing the trial 
• Failure to report SAE to sponsor and/or regulatory agencies within specified timelines 
• Use of recruitment procedures not approved by the IRB/EC 
• Continuing research activities after IRB approval has expired 
• Enrolling more subjects than the IRB has approved 
The Sponsor will assess any protocol deviation and decide whether any of these should be 
reported to Competent Authorities as a serious breach of GCP and the protocol. 
8.10. 
Prior and Concomitant Medications 
Prior medication is defined as the medication started prior to the first administration of the study 
drug. Concomitant medication is defined as the medication with stop date on or after the first 
administration of the study drug. With the exception of Rituximab, which must be captured up to 
1 year prior to screening, all other concomitant medications taken by study subjects must be 
captured starting 12 weeks prior to screening, throughout their participation in the study and 
through the follow-up visit, which should occur 4 weeks after each subject takes their final dose 
of study medication. 
Subject incidence of the prior and concomitant medications will be tabulated by the WHODD 
Anatomical Therapeutic Chemical Classification and Preferred Term. 
8.11. 
Pharmacokinetic and Pharmacodynamic marker analysis 
Plasma samples will be collected according to the Time and Events Table to determine the PK 
profile of CCX140. Individual plasma concentrations of CCX140 will be listed, plotted, and 
summarized descriptively and graphically. PK parameters will be calculated based on plasma 
CCX140 concentrations at the time of sample collection in relation to time of administration of 
the most recent dose of study medication. The following parameters will be determined, where 
possible: 
Cmax 
Maximum plasma concentration 
Tmax 
Time of maximum plasma concentration  
AUC0-6 Area under the plasma concentration-time curve from Time 0 to Hour 6 for all 
subjects, after the first dose of 5 mg BID, two weeks after 10 mg BID and four 
weeks after 15 mg BID 
AUC0-12 Area under the plasma concentration-time curve from Time 0 to Hour 12, two 
weeks after 10 mg BID and four weeks 15 mg BID, will be extrapolated for all 
subjects. 
Cmin 
Trough level plasma concentrations at visits when AUC0-6 is not collected 
 
If there are sufficient data, population PK analysis will be performed. 

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Plasma/serum and urinary PD markers will be summarized and may be analyzed using methods 
analogous to the efficacy parameters. 
The relationships among PK parameters, UPCR and renal function based on eGFR will be 
evaluated. The data may also be used to evaluate the PK/PD relationship of CCX140-B 
treatment. To this end, the change and/or percent change from baseline in UPCR, eGFR, and 
other biomarkers may be used as PD markers. 
9. 
STUDY COMPLETION AND TERMINATION 
9.1. 
Study Completion 
A subject has completed the study when s/he has completed the study procedures per protocol. 
9.2. 
Study Termination 
The end of study is defined as the last study visit of the last clinical trial subject. All subjects 
who took at least a single dose of CCX140-B should be urged to complete a study termination 
visit and to return 7 and 14 days after their final dose of study medication to have a blood draw 
to measure concentrations of CCX140 for the purpose of assessing its elimination characteristics. 
They should also be encouraged to complete a termination visit within 4 weeks of their final dose 
of study medication, for assessment of ongoing adverse events. 
10. 
REGULATORY AND ADMINISTRATIVE REQUIREMENTS 
10.1. 
Investigator Responsibilities 
Prior to trial initiation, the Investigator will provide the Sponsor with a fully executed and signed 
FDA Form 1572, a Financial Disclosure Form, and a curriculum vitae. Financial Disclosure 
Forms also will be completed for all Sub-Investigators listed on the Form 1572 who will be 
involved directly in the treatment or evaluation of research subjects in this trial. 
The study will be conducted in accordance with the Declaration of Helsinki (amended by the 59th 
World Medical Association General Assembly, October 2008) and Good Clinical Practice (GCP) 
according to International Conference on Harmonisation (ICH) guidelines. Specifically, the 
study is based on adequately performed laboratory and animal experimentation; the study will be 
conducted under a protocol reviewed by a properly constituted IRB/EC; the study will be 
conducted by scientifically and medically qualified persons; the benefits of the study are in 
proportion to the risks; the rights and welfare of the subjects will be respected; and each subject 
or his/her legal guardian will give his/her written Informed Consent before any protocol-specific 
tests or evaluations are performed. 
10.2. 
Institutional Review Board or Ethics Committee 
Prior to initiating the study, the Investigator will obtain written confirmation from the IRB/EC 
that the IRB/EC was properly constituted and met the definition of all United States Code of 
Federal Regulations Title 21, Section 312.3(b) and Part 56, and/or the applicable local, regional 

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or national Regulatory requirements. A copy of the confirmation will be provided to the Sponsor. 
The Principal Investigator will provide the IRB/EC with all appropriate materials, including the 
protocol and Informed Consent documents. The trial will not be initiated until IRB/EC approval 
of the protocol, the Informed Consent document, and all recruiting materials are obtained in 
writing by the Investigator and copies are received by the Sponsor. Appropriate reports on the 
progress of the study will be made to the IRB/EC and the Sponsor by the Principal Investigator 
in accordance with applicable governmental regulations and in agreement with the policy 
established by the Sponsor. 
10.3. 
Informed Consent 
A properly executed, written, and appropriately explained Informed Consent Form, in 
compliance with the Declaration of Helsinki, ICH GCP, and US Code of Federal Regulations for 
Protection of Human Subjects (21 CFR 50.25[a,b], CFR 50.27, and CFR Part 46, Subpart A), 
will be signed by each subject or his/her legal guardian prior to entering the trial. Either the 
Investigator or the Investigator’s designee will obtain the consent of the study subject. The 
subject will be provided as much time as necessary to review the document, to inquire about 
details of the trial, and to decide whether or not to participate in the study. The Informed Consent 
will be signed and dated by the study subject and by the person who conducted the Informed 
Consent discussion. The Investigator will provide a copy of the signed Informed Consent Form 
to each subject and will maintain a copy in the subject’s record file. 
10.4. 
Protocol Modifications 
Only the Sponsor may modify the protocol. The only exception is when the Investigator 
considers that a subject’s safety would be compromised without immediate action. In this 
circumstance, immediate approval of the chairperson of the IRB/EC must be sought, and the 
Investigator should inform the Sponsor’s Medical Monitor and the full IRB/EC within five 
working days after the emergency occurred. All other amendments that have an impact on 
subject risk or the study objectives, and/or that require revision of the Informed Consent Form, 
must receive approval from the IRB/EC prior to their implementation, except when the changes 
involve only logistical or administrative aspects of the trial. The IRB/EC must be notified of 
changes that are made to study contact personnel, but IRB/EC review or approval of these 
changes is not required. If protocol amendments are substantial and are likely to have an impact 
on the safety of the trial subjects or to change the interpretation of the scientific documents in 
support of the conduct of the trial, or if they are otherwise significant, the sponsor shall notify the 
FDA and other competent authorities concerned of the reasons for, and content of, these 
amendments according to the European Directive “Detailed guidance on the request to the 
competent authorities for authorization of a clinical trial on a medical products for human use, 
the notification of substantial amendments and the declaration of the end of trial (CT-1)(2010/C 
82/01)” and other regulatory guidance. 
10.5. 
Regulatory Documentation 
All regulatory documentation including regulatory submissions, 1572 forms, and correspondence 
regarding this study will be kept by the Sponsor. The CRO that will conduct the study on behalf 

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of the Sponsor will maintain all study documentation according to their SOPs. Clinical trial 
related documents will be archived for the longest of: 
1. 10 years according to national Swedish and EU regulations (LVFS 2003:3), or 
2. For 2 years following the date a full marketing application is approved, or 
3. For 2 years after the FDA is notified that the IND is discontinued if there is no marketing 
application. 
10.6. 
Subject Identification Register 
The Investigator agrees to complete a subject identification register, which will be used for the 
purpose of long term follow-up, if needed. This form will be treated as confidential, and will be 
filed by the Investigator in a secure locked place. Otherwise, all reports and communications 
relating to the study will identify participants by initials and/or assigned number only. 
10.7. 
Record Retention 
The Investigator must retain all study records required by the Sponsor and by the applicable 
regulations in a secure and safe facility. The Investigator must consult a Sponsor CRA before 
disposal of any study records, and must notify the Sponsor of any change in the location, 
disposition, or custody of the study files. Clinical trial related documents will be archived for the 
longest of: 
1. 
10 years according to national Swedish and EU regulations (LVFS 2003:3), or 
2. 
For 2 years following the date a full marketing application is approved, or 
3. 
For 2 years after the FDA is notified that the IND is discontinued if there is no marketing 
application. 
10.8. 
Case Report Form Completion 
Electronic Case Report Forms (CRFs) will be generated for each subject. The electronic data 
capture (EDC) system will comply with CFR 21 Part 11. 
It is the policy of the Sponsor that study data must be verifiable to the source data, which 
necessitates access to all relevant source documents, laboratory reports, and subjects’ records. 
The Investigator must therefore agree to allow access to subjects’ records, and source data must 
be made available for all study data. The subjects (or their legal representatives) must also allow 
access to the subjects’ medical records, and they will be informed of this requirement and will 
indicate their agreement when giving Informed Consent. Upon completion of the study, 
electronic copies of the CRFs will be provided to the investigators and should be included as part 
of his/her study files and retained as per FDA or local regulations. 
10.9. 
Monitoring 
At intervals during the study, and after the completion of subject enrollment, the study center 
will be monitored by a CRA for compliance, which will include ensuring that accurate and 
complete data are promptly recorded in EDC, reviewing source documentation and drug 
accountability records and reviewing all essential regulatory documents for accuracy and 

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completeness. The study will be conducted according to the principles of GCP as accepted in the 
United States and according to CPMP/ICH/135/95. 
10.10. 
On-site Visits and Audits 
The Sponsor’s representatives will visit the study center prior to initiation of the study to provide 
on-site training of the center personnel and to provide information regarding the investigational 
agent, protocol requirements, monitoring requirements, requirements for maintenance of 
essential regulatory documentation and reporting of serious adverse events. 
In certain circumstances, a secondary audit may be conducted by members of a Quality 
Assurance group designated by the Sponsor. The Investigator will be informed if this is to occur 
and will be advised as to the nature of the audit. Representatives of the Food and Drug 
Administration (FDA) and/or representatives of other regulatory authorities may also conduct an 
audit of the study. If informed of such an audit, the Investigator should notify the Sponsor 
immediately. 
10.11. 
Use of Information and Publication 
It is understood by the Investigator that the information generated in this study will be used by 
the Sponsor in connection with the development of the product and therefore may be disclosed to 
government agencies in various countries. To allow for the use of information derived from the 
study, it is understood that the Investigator is obliged to provide the Sponsor with complete test 
results, all study data, and access to all study records. 
The Sponsor recognizes the importance of communicating study data and will disclose or publish 
the results in a suitable form regardless of outcome. The Sponsor will post the clinical trial 
information on appropriate registries, e.g., clinicaltrials.gov prior to enrollment of the first 
subjects, and publish the results of this study in scientific journals, at seminars or conferences, 
and/or in other manner(s) it so chooses. Results from this study shall not be made available to 
any third party by the investigating team without the express permission of the Sponsor. 

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11. 
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17. Investigators Brochure, Edition 9.0. 
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33. Rydel J et al. (1995). Focal segmental glomerular sclerosis in adults: presentation, 
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36. Sim, JJ et al. Distribution of Biopsy-Proven Presumed Primary Glomerulonephropathies 
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37. Stevens LA, Coresh J, Schmid CH, Feldman HI, Froissart M, Kusek J, Rossert J, Van 
Lente F, Bruce RD, 3rd, Zhang YL, Greene T, Levey AS (2008) Estimating GFR using 
serum cystatin C alone and in combination with serum creatinine: a pooled analysis of 
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APPENDIX A. Subject Instructions 
Subject Instructions for Collection of 24 Hour Urine Samples 
Subjects will be asked to fast (not eat anything) for at least 10 hours before the study visit. 
Subjects will collect the urine sample at home according to the instructions they were given. On 
some days they will be asked to collect their urine for a full day, starting with the second urine 
on the day before their visit and including the first urine on the day of their visit. The study staff 
will provide containers for collecting these urine samples. 
 
Subject Instructions for Dosing of Study Medication on Visit Days 
On the day of each study visit, the subject should not take their morning study medication until 
they get to the clinic. They will then take it in the presence of the study staff. On the days when 
they do not have a study visit, they will take their first dose of study medication in the morning at 
approximately the same time each day. Their second dose should be taken approximately 12 
hours after the first dose.