A 65-year-old woman with known GPA (granulomatosis with polyangiitis) develops severe alveolar hemorrhage with diffuse pulmonary infiltrates and haemoptysis, along with creatinine rising to 3.8 mg/dL and ANCA (PR3) strongly positive. Which treatment is currently preferred as adjunct to standard induction (rituximab or cyclophosphamide + steroids) for life-threatening disease?
- A Intravenous immunoglobulin (IVIG)
- B Plasma exchange (PLEX)
- C Avacopan (C5a receptor inhibitor) ✓
- D Azathioprine pulse therapy
Correct answer: C. Avacopan (C5a receptor inhibitor)
Explanation
Avacopan, a selective C5a receptor blocker, received FDA approval in 2021 as an adjunct to rituximab or cyclophosphamide for induction of remission in severe ANCA-associated vasculitis (ADVOCATE trial). It allows significant glucocorticoid reduction while achieving non-inferior and sustained remission rates. Plasma exchange was evaluated in the PEXIVAS trial (2020), which demonstrated no benefit on renal survival or death when added to standard induction in ANCA vasculitis, largely ending its routine use. IVIG and azathioprine are not used for acute induction.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.