A Case of Churg-Strauss Syndrome Revealed by Eosinophilic Gastroenteritis

Abstract

Churg-Strauss syndrome (CSS) is a rare and usually underrecognized entity that often manifests late in the process of the disease. The syndrome is a small vessel-necrotizing vasculitis characterized by asthma, extravascular necrotizing granulomas, hypereosinophilia, and lung infiltrates [1]. According to the classification of CSS by the American College of Rheumatology, the presence of four or more of the criteria required to establish a diagnosis of CSS yield a sensitivity of 99.7% for CSS [2, 3] (Table 1). The annual incidence of the disease ranges between 0.5 and 6.8 per million inhabitants. The average age of the patient at diagnosis is 50 years; there is no sex predominance, and the annual incidence among asthma patients ranges from 0 to 97 per million [1]. The pathogenesis and etiology of CSS remain unclear, but the disease is most likely autoimmune, at least for those 30–40% anti-neutrophil cytoplasmic antibodies (ANCA)positive patients [4].