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EGPA — SCE Rheumatology MCQ

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ModerateVasculitisEGPASCE Rheumatology

A 46-year-old woman with adult-onset asthma and chronic rhinosinusitis develops a painful right foot drop and numbness in the left hand. Nerve-conduction studies demonstrate an asymmetric axonal neuropathy affecting separate peripheral nerves. Examination shows palpable purpura and expiratory wheeze. Her blood eosinophil count is 2.8 × 10^9/L and an antigen-specific assay detects MPO-ANCA. What is the most likely diagnosis?

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Correct answer: D — Eosinophilic granulomatosis with polyangiitis

The diagnosis is eosinophilic granulomatosis with polyangiitis (EGPA). Adult-onset asthma and chronic rhinosinusitis establish the characteristic airways phenotype, while marked eosinophilia, mononeuritis multiplex and palpable purpura indicate eosinophilic systemic small-vessel vasculitis. MPO-ANCA is present in only a minority of EGPA cases but is associated particularly with neuropathic, cutaneous and renal vasculitic manifestations. Microscopic polyangiitis may be MPO-ANCA positive but does not typically cause this marked eosinophilic asthma phenotype. Granulomatosis with polyangiitis is more often associated with PR3-ANCA and destructive granulomatous ENT or pulmonary disease. Hypereosinophilic syndrome can cause eosinophilic organ injury, but the purpura, mononeuritis multiplex and MPO-ANCA strongly favour EGPA. Sarcoidosis does not explain the eosinophilia and vasculitic neuropathy.

Reference: Emmi G, et al. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis, 2023. https://pubmed.ncbi.nlm.nih.gov/35106968/