A Study of CCX140-B in Subjects With Primary FSGS and Nephrotic Syndrome

An Open Label, Intra-Subject Dose Escalation Study of CCX140 B in Subjects with Primary Focal
Segmental Glomerulosclerosis (FSGS) and Nephrotic Syndrome. The aim of this study is to
explore the effect of CCX140-B, a selective antagonist of C-C chemokine receptor type 2, on
proteinuria in subjects with FSGS.

Inclusion Criteria:

1. Male or female subjects aged 18 years and older

2. Primary FSGS based on renal biopsy findings consistent with FSGS and based on
presentation of histopathology, medical history and clinical course OR subjects with
genetic risk factors with presentations that are otherwise consistent with primary
FSGS

3. Urinary total protein:creatinine ratio (UPCR) ≥ 3.5 g protein/g creatinine at
screening

Exclusion Criteria:

1. Pregnant or nursing

2. History of organ transplantation, including renal transplantation

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 anti-CD20 monoclonal
antibodies within 16 weeks (4 months) prior to screening are excluded. Subjects who
initiated treatment with anti-CD20 monoclonal antibodies >16 weeks (4 months) prior to
screening are permitted if deemed safe by the investigator and only if they intend to
remain on continued, unchanged therapy at a dosing interval that has been documented
to achieve continuous B cell depletion for the given patient.

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. Body Mass Index (BMI) ≥ 40