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Microscopic Polyangiitis — RACP Adult Medicine MCQ

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ModerateRheumatologyMicroscopic PolyangiitisRACP Adult Medicine

A 65-year-old woman presents with rapidly progressive glomerulonephritis and pulmonary haemorrhage. She has no upper airway symptoms. p-ANCA (anti-MPO) is positive. Kidney biopsy shows pauci-immune necrotising crescentic glomerulonephritis. What is the most likely diagnosis?

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Correct answer: A — Microscopic polyangiitis

Pulmonary-renal syndrome with p-ANCA/anti-MPO and pauci-immune crescentic GN without upper airway granulomatous disease is MPA. Unlike GPA, MPA lacks the granulomatous upper airway involvement. Treatment is the same as for GPA: rituximab or cyclophosphamide plus glucocorticoids for induction, followed by rituximab or azathioprine for maintenance.

Reference: EULAR – 2024 – AAV Guidelines; KDIGO 2024