Churg-Strauss Syndrome: The Clinical Features And Long-term Follow-up Of 15 Patients

Background Churg-Strauss syndrome (CSS) is a rare necrotizing multi-system vasculitis which affects the small- to medium-sized blood vessels and is characterized by asthma, eosinophilia and extravascular eosinophilic granulomas. Asthma is a cardinal feature of CSS and usually precedes overt vasculitis by an average of 3-8 years. Asthma is not clinically controlled, regardless of the prescription of appropriate doses of inhaled corticosteroids and long-acting bronchodilators, which is often mistaken in diagnosis as refractory asthma.Objective To describe the clinical features, treatment outcome, and long-term follow-up of CSS from Peking Union Medical College Hospital.Methods and Results We studied 15 patients diagnosed with CSS at Peking Union Medical College Hospital Between 2001 and 2007. The diagnosis of CSS is based on the classification criteria of the American Collage of Rheumatology. All patients had asthma. As in other case series, the lung, peripheral nervous system, and skin were the most commonly involved organs. During the active stage of the disease, most of the patients exhibited peripheral blood eosinophilia and an elevated serum total IgE level. Two patients were treated with pulses of methylprednisolone followed by tapering and cyclophosphamide, eight patients were treated with corticosteroids and cyclophosphamide, and the others were treated with corticosteroids alone. All patients gained remission when their first discharges. The outcomes after long-term follow-up were generally good. Two patients were withdrew. Seven patients sustained remission. Five patients suffered relapses during the follow-up period.Conclusions CSS was highly variable in its presentation and course. The manifestations may range from mild symptoms to life-threatening conditions. Corticosteroids and cyclophosphamide were effective remedies. The outcome after long-term follow-up was good in general.