Churg-Strauss syndrome. Our two-decade experience

Background. Since 1951, the year when the Churg-Strauss syndrome was report ed, several isolated cases have been reported in the literature but works c ontaining a high number of cases are exceptional. The features of the syndr ome as well as diagnostic criteria have not been contrasted in large series . We therefore tried to analyze these parameters in 16 patients diagnosed o f Churg-Strauss syndrome at our hospital. Methods. A retrospective analysis was made of 16 patients diagnosed of Chur g-Strauss syndrome at Hospital Universitario La Pat from 1978 at 1998. Data regarding personal antecedents, clinical manifestations, analytical parame ters, histology and clinical course were collected. Also, the main diagnost ic criteria reported in the literature were applied to each case. Results. Of the 16 cases, thirteen (81%) were women, six were diagnosed at an age between 21 and 40 years; other six cases from 51 to 70 years. All pa tients had antecedents of bronchial asthma. Among clinical manifestations, 60% had fever and 50% a general syndrome. The main involved organs by decre asing frequency were lung (100%), nervous system (63%), and heart (44%). Th e mean eosinophilic count was 7,640/mm(3). The histological diagnosis was o btained in 14 out of the 16 patients (88%). The most efficient biopsies cor responded to muscle and nerve. After therapy, no relapse was noted of vascu litis although bronchial asthma persisted in most patients (12). Twelve cas es (75%) fulfilled the Lanham diagnostic criteria. Conclusions. Churg-Strauss syndrome affects mainly women with severe eosino philia. Electromyogram and muscle and peripheral nerve biopsy are useful to ols for diagnosis. The Lanham criteria lead to the diagnosis in most patien ts.