Clinical Trials

Inclusion Criteria  

Each patient must meet the following criteria to be enrolled in this study.

1. Subjects aged 18 or greater.
2. Fulfillment of the definitions of the Second Chapel Hill Consensus Conference for ANCA-associated vasculitis (either granulomatosis with polyangiitis (GPA) or microscopic polyangiitis MPA).
1. Positivity for ANCA, directed against PR3 or MPA.
2. Diffuse alveolar hemorrhage.
3. Respiratory failure requiring mechanical ventilation.
4. Severe newly diagnosed disease or severe relapsing disease.  Severe relapsing disease is defined as at least one major BVAS/WG item or a score ≥ 3 and the investigator deems standard treatment for severe disease is necessary.
5. Minimum BVAS/WG of 3.
6. Requirement of standard-of-care therapy for active severe ANCA-associated vasculitis (GPA or MPA).

Exclusion Criteria

Patients who present any of the following criteria will be excluded from the study.

1. Diagnosis with eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) as defined by the Chapel Hill Consensus Conference.
2. Any of the co-morbidities:

• Allergies: a history of severe allergic reactions to avacopan.
• Liver disease: acute or chronic liver disease that is deemed sufficiently severe to impair their ability to participate in the trial.
• Renal disease: a history of documented antiglomerular basement membrane disease (anti-GBM disease).
• Other uncontrolled diseases, including any uncontrolled psychiatric disorders, drug and alcohol abuse, that could interfere with participation in the trial according to the protocol.

3. Aspartate aminotransferase or alanine aminotransferase or amylase > 2.5 times the upper limit of normal, unless attributed to vasculitis.

Note: Other protocol defined Inclusion/Exclusion Criteria may apply.

Eligibility last updated 9/3/2024. Questions regarding updates should be directed to the study team contact.