Avacostar - (PASS)

The Avacostar PASS is a non-interventional, multi-national, prospective cohort study that will collect data from 2 cohorts of patients: those treated with avacopan for active severe AAV, and a second cohort treated with a cyclophosphamide or rituximab-based induction regimen without avacopan for active severe AAV. The overall study duration is anticipated to be up to 7 years, including a recruitment period of approximately 3 years.

Inclusion Criteria: * Diagnosis of AAV (MPA or GPA), as determined by the Investigator according to their usual practice. * Active, severe AAV at the time of commencing avacopan or non-avacopan SoC induction therapy, in the opinion of the Investigator. * Age ≥18 years of either sex. * Has provided written informed consent. * Has commenced within the previous 6 months, or is planned to commence avacopan, cyclophosphamide or rituximab for the treatment of severe, active AAV outside of an interventional clinical study. Exclusion Criteria: * Concurrent participation in an interventional study, unless prospectively discussed and agreed with the Medical Monitor.