A Phase 2 Trial of the Safety and Efficacy of Bardoxolone Methyl in Patients With Rare Chronic Kidney Diseases - PHOENIX

Inclusion Criteria:

- Male and female patients 18 ≤ age ≤ 65 upon study consent;

- Screening eGFR (average of Screen A and Screen B eGFR values) ≥ 30 and ≤ 90
mL/min/1.73 m2. The two eGFR values collected at Screen A and Screen B visits used to
determine eligibility must have a percent difference ≤ 25%;

- Albumin to creatinine ratio (ACR) ≤ 2500 mg/g at Screen B visit;

- If receiving an angiotensin-converting enzyme (ACE) inhibitor and/or an angiotensin II
receptor blocker (ARB), patients should be prescribed the maximally tolerated labeled
daily dose (MTLDD) for at least 6 weeks prior to the Screen A visit;

- For patients enrolling in T1D Cohort: Diagnosis of type 1 diabetes confirmed by
fasting C-peptide level. Diagnosis must have been made ≤ 35 years of age; and
prescribed stable dose of insulin to maintain adequate glucose control for at least 6
months prior to the Screen A visit;

- For patients enrolling in IgAN Cohort: Biopsy-confirmed IgA nephropathy;

- For patients enrolling in FSGS Cohort: Biopsy-confirmed FSGS that is not due to known
secondary causes including morbid obesity, decreased renal mass, viral infections,
drug-induced nephrotoxicity, or prior history of vasculitis;

- For patients enrolling in ADPKD Cohort: Genetic confirmation of PKD1 mutation;

- Adequate bone marrow reserve and organ function at the Screen A visit as follows:
Hematologic: Absolute neutrophil count > 1.5 x 109/L, platelets > 100 x 109/L,
hemoglobin (Hgb) ≥ 9 g/dL; and Hepatic: Total bilirubin (TBL) ≤ 1.5 times the upper
limit of normal (ULN), alanine aminotransferase (ALT) and aspartate aminotransferase
(AST) ≤ 1.5 times ULN.

Exclusion Criteria:

- Kidney or any other solid organ transplant recipient or a planned transplant during
the study;

- B-type natriuretic peptide (BNP) level > 200 pg/mL at Screen A visit;

- Acute dialysis or acute kidney injury within 12 weeks prior to Screen A visit or
during Screening;

- Serum albumin < 3 g/dL at Screen A visit;

- Systemic immunosuppression for more than 2 weeks, cumulatively, within the 12 weeks
prior to randomization or anticipated need for immunosuppression during the study;

- For patients enrolling in IgAN Cohort: Systemic manifestations of Henoch-Schonlein
purpura within 1 year prior to Screen A visit; or have used belimumab, eculizumab, or
rituximab within 6 months prior to Screen A visit;

- For patients enrolling in ADPKD Cohort: Receiving tolvaptan;

- Cerebrovascular event (stroke, transient ischemic attack) or aneurysm within 6 months
prior to Screen A visit or during Screening;

- History of clinically significant left-sided heart disease and/or clinically
significant cardiac disease;

- Uncontrolled systemic hypertension;

- Systolic BP < 90 mm Hg at Screen A visit after a period of rest;

- History of malignancy within 2 years prior to Screen A visit, with the exception of
localized skin or cervical carcinomas;

- Uncontrolled diabetes (HbA1c > 10.0%) at Screen A visit;

- Untreated or uncontrolled active bacterial, fungal, or viral infection;

- Participation in other interventional clinical studies within 30 days prior to Day 1;

- Unwilling to practice acceptable methods of birth control (both males who have
partners of child-bearing potential and females of childbearing potential) during
Screening, while taking study drug, and for at least 30 days after the last dose of
study drug is ingested;

- Women who are pregnant or breastfeeding.