Rapidly progressive glomerulonephritis
A nephrology emergency. Same-day ANCA and anti-GBM, early steroids, and induction with rituximab or cyclophosphamide to the KDIGO 2024 ANCA guideline.
Tier 1Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- RPGN is a clinical syndrome: loss of at least half the GFR over days to weeks, with haematuria, red cell casts and proteinuria. The biopsy usually shows crescents.
- It is a nephrology emergency. Untreated, most patients reach kidney failure within weeks to months. Delay is the main reason for poor outcomes.
- Send ANCA and anti-GBM the same day and call nephrology. Start IV methylprednisolone without waiting for the biopsy when the picture and serology fit.
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Sources
- KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis. Kidney International 2024;105(3S):S71-S116Kidney Disease Improving Global Outcomes, 2024 (amended: Practice Points 9.3.1.9 and 9.3.3.1 and Figures 6 to 8 and 13 revised)
- KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases, chapter 10 (anti-GBM disease). Kidney International 2021;100(4S):S1-S276Kidney Disease Improving Global Outcomes, 2021
- KDIGO 2025 Clinical Practice Guideline for the Management of IgA Nephropathy and IgA Vasculitis. Kidney International 2025;108(4S):S1-S71Kidney Disease Improving Global Outcomes, 2025
- Bose B, Carter SA, Abdy ME et al. CARI Guidelines commentary on the KDIGO clinical practice guideline for the management of glomerular diseases. Nephrology 2025;30(9):e70119AU/NZCARI Guidelines (Nephrology), 2025
- Avacopan (Tavneos) PBS listing for severe active GPA and MPA, 1 October 2024AU/NZPharmaceutical Benefits Scheme, 2024
- Walsh M et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis (PEXIVAS). N Engl J Med 2020;382:622-31New England Journal of Medicine, 2020
- Jayne DRW et al. Avacopan for the treatment of ANCA-associated vasculitis (ADVOCATE). N Engl J Med 2021;384:599-609. Retracted 29 June 2026 (retraction notice doi:10.1056/NEJMe2608684)New England Journal of Medicine, 2021; retracted 2026
- 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