ANCA-associated vasculitis
GPA, MPA and EGPA. Telling them apart, PR3 and MPO by immunoassay, the biopsy, rituximab or cyclophosphamide with reduced-dose steroids, the avacopan withdrawal, rituximab maintenance, anti-IL-5 drugs in EGPA (MANDARA), and the 2025 BSR update.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Necrotising vasculitis of small vessels with few immune deposits (pauci-immune). Three diseases: GPA, MPA and EGPA.
- Peak age 60 to 75. Fever, weight loss and arthralgia for weeks, then organ damage.
- Send PR3 and MPO by immunoassay, a urinalysis, and imaging of the chest. Tissue confirms.
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Sources
- Therapeutic Guidelines: Rheumatology (systemic vasculitis)AU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- KDIGO 2024 Clinical Practice Guideline for the Management of ANCA-Associated Vasculitis. Kidney Int 2024;105(3S):S71-S116 (with 2024 and 2025 corrigenda)Kidney Disease Improving Global Outcomes, 2024
- Bose B et al. CARI Guidelines commentary on the KDIGO clinical practice guideline for the management of glomerular diseases. Nephrology 2025;30(9):e70119 (commentary on KDIGO 2021, not the 2024 AAV update)AU/NZCARI Guidelines (Nephrology), 2025
- Avacopan (Tavneos) PBS listing for severe active GPA and MPA, 1 October 2024AU/NZPharmaceutical Benefits Scheme, 2024
- Jayne DRW et al. Avacopan for the treatment of ANCA-associated vasculitis (ADVOCATE). N Engl J Med 2021;384:599-609. Retracted 29 June 2026New England Journal of Medicine, 2021 (retracted 2026)
- Tavneos (avacopan) and Health Canada's review of data integrity concernsHealth Canada, 2026
- Hellmich B et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis 2024;83:30-47EULAR, 2022 update
- Biddle K et al. The 2025 British Society for Rheumatology management recommendations for ANCA-associated vasculitis. Rheumatology (Oxford) 2025;64:4470-94British Society for Rheumatology, 2025
- Chung SA et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of ANCA-associated vasculitis. Arthritis Rheumatol 2021;73:1366-83American College of Rheumatology, 2021
- Robson JC et al. 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis 2022;81:315-20ACR and EULAR, 2022
- Wechsler ME et al. Benralizumab versus mepolizumab for eosinophilic granulomatosis with polyangiitis (MANDARA). N Engl J Med 2024;390:911-21New England Journal of Medicine, 2024
- Wechsler ME et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis (MIRRA). N Engl J Med 2017;376:1921-32New England Journal of Medicine, 2017
- Withdrawal of TAVNEOS (avacopan)AU/NZTherapeutic Goods Administration, 16 October 2026
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Personal emails, About story and Australian-style copy