Clinical Trials

Inclsuion Criteria: 

• • • • •  
• A diagnosis of GPA or MPA according to the Chapel-Hill Consensus Conference definitions (Jennette et al, 2013) and ACR/EULAR 2022 criteria (Robson et al, 2022; Suppiah et al, 2022)
  • Evidence of active disease based on the BVAS v3 with a score of at least 1 and life- or organ-threatening GPA/MPA, OR risk of progression to life- or organ-threatening GPA/MPA at the time of commencing avacopan.
  • Examples of risk of progression to life- or organ-threatening GPA/MPA include but are not limited to:
    • • Severe sinonasal disease with risk of chondritis progressing to saddle nose deformity, bony damage from sinusitis, or nasal septal perforation.
      • Any evidence of current or prior renal involvement by GPA/MPA (e.g., a patient presenting with a sinonasal flare and lung nodules who also previously had renal involvement but no evidence of renal involvement at this time).
      • Subglottic stenosis at risk of progression to airway obstruction based on the degree of narrowing or with limitation of physical activity.
      • Scleritis at risk for vision loss based on the severity of the inflammatory disease.
      • Severe skin disease with deep ulceration or coalescing purpura limiting physical activity.
      • • •  
      • Prior severe disease with organ- or life-threatening manifestation such that insufficient treatment could lead to recurrence of severe disease.
• Receiving standard therapy for the treatment of GPA or MPA
• Initiating avacopan for clinical purposes within +/- 14 days of consent. Those who do not initiate avacopan within 14 days of consent, can re-screen for participation within 14 days of avacopan initiation.
• With an intended course of avacopan use of at least 6 months
• At least 18 years of age at the time of consent
• Willing and able to give written Informed Consent and to comply with the requirements of the study protocol
• Judged by the investigator to be otherwise fit for the study, based on medical history, physical examination, and clinical laboratory assessment.
• 1. 1.  

Exclusion Criteria:

• • A diagnosis of EGPA
  • Patients with alveolar hemorrhage requiring invasive pulmonary ventilation anticipated to last beyond screening or patients requiring avacopan to be administered by methods other than oral capsule form.
  • Known pregnancy or breast-feeding
  • Interstitial lung disease as the primary manifestation of GPA/MPA
• Any other known autoimmune disease (excluding Hashimoto’s thyroiditis)
  • Malignancy receiving treatment (excluding 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)
• Untreated hepatitis C virus infection
• Active or latent hepatitis B virus infection
• Human immunodeficiency virus infection
• Active or latent tuberculosis infection
• Liver function test abnormalities (AST, ALT, alkaline phosphatase, or bilirubin > 3 times the upper limit of normal before start of avacopan)
• History or presence of any medical condition or disease which, in the opinion of the Investigator, may place the patient at unacceptable risk for study participation
• Currently involved in an interventional study and/or concurrently using experimental agents/medications
• Has previously received avacopan for treatment of GPA/MPA (including relapse) for at least 30 days.
• Prior discontinuation of avacopan because of an adverse event or lack of response.
• History of cocaine/levamisole use

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

Eligibility last updated 1/17/2025. Questions regarding updates should be directed to the study team contact.