A Case of Churg-Strauss Syndrome / 대한류마티스학회지

ABSTRACT

Churg-Strauss syndrome(CSS) or the syndrome of allergic granulomatosis and angiitis is a kind of rare systemic vasculitis characterized by bronchilal asthma, peripheral eosinophilia, paranasal sinus abnormality, mono or polyneuropathy, pulmonary infiltrates nonfixed and pathological findings such as prominent eosinophilic infiltration, necrotizing angiitis and extravascular granuloma formation. Clinical features of CSS includes asthma, pulmonary infiltrates, cutaneous lesions, neuropathy, cardiac involvement, gastrointeastinal involvement, renal involvement and musculoskeletal involvement. The diagnosis of CSS is made on the basis of both clinical and pathologic features. We experienced a 30-year-old man who presented peripheral eos'lnophilia, asthma of two year's duration, lower extremity weakness and lung infiltrations. His lung and skin biopsy showed eosinophilic infiltrates and eosinophilic vasculitis. He was diagnosed as Churg-Strauss syndrome based on the clinical manifestations and pathologic finding of lung and skin. He was managed with 3 times of plasmapheresis and methylprednisolone 1.0 g/day of 3 days followed by oral prednisolone 50rag/day and cyclophosphamide 2mg/kg. Clinical symptoms, laboratory and X-ray findings of the patient were improved at 7 days after treatment. After discharge, he has been treated with cyclophosphamide and low dose prednisolone. We report a case CSS with review of the literatures.