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GPA — RACP Adult Medicine MCQ

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ModerateRheumatologyGPARACP Adult Medicine

A 60-year-old man presents with recurrent epistaxis, chronic sinusitis with nasal crusting, cough with haemoptysis, and rapidly progressive renal failure. Urinalysis shows RBC casts. CT sinuses shows mucosal thickening and bony erosion. c-ANCA (anti-PR3) is positive. Kidney biopsy shows pauci-immune crescentic glomerulonephritis. What is the most likely diagnosis?

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Correct answer: D — Granulomatosis with polyangiitis

The triad of upper airway disease (sinusitis, nasal crusting, epistaxis), lower airway involvement (haemoptysis), and pauci-immune crescentic GN with positive c-ANCA/anti-PR3 is diagnostic of GPA (formerly Wegener granulomatosis). Induction therapy is rituximab or cyclophosphamide plus glucocorticoids. Plasma exchange is considered for severe renal disease.

Reference: EULAR – 2024 – AAV Guidelines; ACR/VF 2021 AAV