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EGPA — RACP Adult Medicine MCQ

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HardRheumatologyEGPARACP Adult Medicine

A 45-year-old man with late-onset severe asthma, eosinophilia (6.5 × 10⁹/L), migratory pulmonary infiltrates, and mononeuritis multiplex (foot drop) develops RPGN. p-ANCA/anti-MPO is positive. He has nasal polyps and maxillary sinusitis. IgE is elevated. Renal biopsy shows pauci-immune crescentic GN with eosinophilic infiltration. What is the most likely diagnosis?

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Correct answer: D — Eosinophilic granulomatosis with polyangiitis (EGPA)

Late-onset asthma, marked eosinophilia (>1.5 × 10⁹/L), migratory pulmonary infiltrates, mononeuritis multiplex, p-ANCA positivity (~40-60% of EGPA), nasal polyps, elevated IgE, and eosinophilic tissue infiltration is eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss). EGPA progresses through allergic (asthma), eosinophilic (infiltrates), and vasculitic (neuropathy, GN) phases. Treatment: corticosteroids (often dramatic response); mepolizumab (anti-IL-5 — MIRRA trial) for non-organ-threatening disease; cyclophosphamide for severe organ involvement.

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