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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Definition: a multisystem disease characterized by necrotizing granulomatous vasculitis with eosinophilia, which most commonly involves the lungs and the skin but can also affect the renal, cardiovascular, gastrointestinal, central, and peripheral nervous systems.

Etiology: unknown

Clinical features
• Severe allergic asthma attacks (chief complaint)
• Allergic rhinitis/sinusitis
• Skin nodules, palpable purpura
• Pericarditis
• Gastrointestinal involvement: bleeding, ischemia, perforation
• Pauci-immune glomerulonephritis
• CNS: impaired mental status, mononeuritis multiplex (loss of motor and sensory function)

Diagnostics
• Peripheral blood eosinophilia
• ↑ IgE level
• Circulating MPO-ANCA/pANCA (∼ 40% of cases)
• Biopsy (confirmatory test): tissue eosinophilia and necrotizing granuloma

Treatment: immunosuppression with glucocorticoids; possible in combination with cyclophosphamide

Churg-Strauss syndrome = pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules
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