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ANCA-associated vasculitis (GPA, MPA, EGPA) — the autoimmune disease that inflames small blood vessels including the kidneys. Rapidly progressive glomerulonephritis is the signature kidney emergency.
Evidence reviewed & updated: 2026-08 — reflects the latest published trials and guidelines.
ANCA-associated vasculitis is an autoimmune disease where antibodies (ANCA) attack small blood vessels — most dangerously in the kidneys and lungs. The kidney form (pauci-immune crescentic glomerulonephritis) can destroy function within weeks, making it a true emergency: early diagnosis and induction therapy save both life and kidney.
ANCA vasculitis is a small-vessel vasculitis driven by antibodies against neutrophil enzymes (PR3-ANCA in GPA, MPO-ANCA in MPA). The immune attack inflames vessel walls — in the kidney, this produces crescentic glomerulonephritis that can halve kidney function in weeks.
It classically affects middle-aged to older adults but can strike at any age; GPA adds the classic triad of airway (sinusitis, nodules, saddle-nose), lung, and kidney involvement.
The kidney signs: microscopic blood in urine (often with red cell casts), proteinuria (usually modest, unlike nephrotic syndrome), and a rapidly rising creatinine. 'Rapidly progressive glomerulonephritis' — eGFR falling over weeks — is the signature emergency.
Because the window to save kidneys is weeks, ANY combination of new kidney failure + blood in urine + constitutional symptoms (fever, weight loss, joint pain) or lung symptoms should trigger an urgent ANCA workup.
Induction (first 3-6 months): high-dose steroids plus rituximab (now first-line in most) or cyclophosphamide — the RAVE trial showed rituximab equals cyclophosphamide for remission with fewer side effects. Plasma exchange is added in severe disease (dialysis-dependent or pulmonary hemorrhage).
Maintenance (2+ years): rituximab or azathioprine with low-dose steroids, plus trimethoprim-sulfamethoxazole when appropriate (PJP prophylaxis and reduced GPA airway relapses). Relapse is common (~50% in 5 years) — ANCA titers, urine, and eGFR are tracked for life.
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