Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients

Objectives. To study the clinical spectrum and evolution of Churg-Strauss s yndrome in order to assess the clinicopathological features of the disease, the response to treatment and the long-term outcome. Methods. Thirty-two patients with proven allergic and granulomatous angiiti s (Churg-Strauss syndrome) and followed up at a single institution were eva luated. They were recruited between 1977 and 1999 from internal medicine de partments. Data were obtained retrospectively from medical files in 15 case s and prospectively, using a standardized form, for the remaining patients. Results. All patients had asthma and hypereosinophilia. The lungs, skin and peripheral nervous system were the organs most frequently involved. Antine utrophil cytoplasmic antibodies with antimyeloperoxidase specificity (MPO-A NCA) were detected in 77.8% of tested patients but they were not useful for monitoring disease activity. Extravascular granulomas were rarely seen in tissue biopsies. Forty per cent of the patients were treated with steroids alone. Immunosuppressive agents were added to the treatment when severe neurologic al, cardiac or gastrointestinal involvement was present. The outcome and lo ng-term survival were good. Clinical relapse was rare after the first year of therapy. Dysaesthesiae of the distal limbs, neurophatic pain and cardiac failure were the most frequent sequelae. Conclusions. Churg-Strauss syndrome is a rare disorder characterized by hyp ereosinophilia and systemic vasculitis occurring in patients with asthma an d allergic rhinitis. Vasculitis commonly affects the lungs, skin and periph eral nervous system. Outcome and long-term survival is usually good with st eroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides.