A 34-year-old man with adult-onset asthma and recurrent nasal polyposis presents with palpable purpura on the legs, asymmetrical sensorimotor neuropathy, and weight loss. Total leukocyte count is 16,000/mm3 with 45 percent eosinophils. Chest CT shows fleeting migratory infiltrates. ANCA is positive against myeloperoxidase. What is the most likely diagnosis?
- A Granulomatosis with polyangiitis
- B Eosinophilic granulomatosis with polyangiitis ✓
- C Hypereosinophilic syndrome
- D Allergic bronchopulmonary aspergillosis
Explanation
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) progresses through three phases: asthma and allergic rhinitis, then eosinophilic organ infiltration, then systemic vasculitis with neuropathy, purpura, and cardiac involvement. Marked peripheral eosinophilia and migratory infiltrates during the vasculitic phase are classic, and roughly half of patients are MPO-ANCA positive. Granulomatosis with polyangiitis features PR3-ANCA, cavitating lung lesions, and destructive sinonasal disease rather than asthma and hypereosinophilia. Hypereosinophilic syndrome lacks vasculitic features such as neuropathy and purpura, and ABPA is confined to airways disease.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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