A case of Churg-Strauss syndrome affecting lung and neuromuscular system

Abstract

Churg-Strauss syndrome(CSS) is a systemic vascular disorder that has an unknown cause with multiorgan involvement and diverse presentations. The three main histologically distinct phases were necrotizing vasculitis, tissue eosinophilia and extravascular granulomas. A diagnosis of CSS can be made on four or more of the following six criteria: 1) asthma, 2) peripheral eosinophilia >10% on the differential leukocyte count, 3) mononeuropathy (including multiple) or polyneuropathy, 4) paranasal sinus abnormalities, 5) nonfixed pulmonary infiltrates, and 6) biopsy evidence of extravascular eosinophils in the skin, the nerves, or the lungs. CSS has a good prognosis with systemic steroid therapy. The 5 year survival is approximately 70%. We experienced a 66-year-old man who presented with cough, sputum, edema and numbness in both legs. He presented with all of the 6 CSS criteria. A nerve and muscle biopsy confirmed the diagnosis. Here, we report this case with a review of the relevant literatures.