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Granulomatosis with polyangiitis — ESENeph MCQ

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HardGlomerulonephritisGranulomatosis with polyangiitisESENeph

A 44-year-old man develops rapidly progressive renal failure after 2 weeks of sinusitis and otitis media. Creatinine is 298 micromol/L and urine microscopy shows red cell casts. ANCA is positive with proteinase-3 specificity; anti-GBM antibody is negative. Renal biopsy shows necrotising crescentic glomerulonephritis with little immune deposition. What is the most appropriate management?

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Correct answer: D — Glucocorticoid plus rituximab or cyclophosphamide induction

PR3-ANCA disease with pauci-immune necrotising crescents requires remission induction with glucocorticoids plus rituximab or cyclophosphamide. Antibiotics alone would miss a rapidly progressive immune-mediated glomerulonephritis. Plasma exchange is no longer routine for every ANCA renal presentation and is considered selectively, such as severe pulmonary haemorrhage. The pearl is that ENT disease plus pauci-immune crescentic GN strongly suggests granulomatosis with polyangiitis.

Reference: KDIGO 2024 ANCA Vasculitis Guideline