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GPA — RACP Adult Medicine MCQ

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ModerateRheumatologyGPARACP Adult Medicine

A 55-year-old man presents with haemoptysis, rapidly progressive glomerulonephritis (creatinine rising from 100 to 400 μmol/L over 2 weeks), and constitutional symptoms. Urinalysis shows dysmorphic RBCs and RBC casts. c-ANCA/anti-PR3 is strongly positive. CT chest shows pulmonary haemorrhage. Renal biopsy shows pauci-immune crescentic glomerulonephritis. What is the most likely diagnosis?

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Correct answer: C — Granulomatosis with polyangiitis (GPA)

Pulmonary-renal syndrome (haemoptysis + RPGN), c-ANCA/anti-PR3 positivity, and pauci-immune crescentic GN is granulomatosis with polyangiitis (GPA, formerly Wegener). GPA classically involves upper airways (sinusitis, saddle-nose deformity), lungs (haemorrhage, nodules, cavities), and kidneys. Treatment: induction with rituximab or cyclophosphamide + prednisolone (RAVE trial: rituximab non-inferior to cyclophosphamide). Plasma exchange for severe renal disease (PEXIVAS trial: no mortality benefit but may benefit severe AKI).

Reference: ACR/EULAR – 2022 – AAV; RAVE Trial; eTG Rheumatology 2024