Background: Since 1951, the year when the Churg-Strauss syndrome was reported, several isolated cases have been reported in the literature but works containing a high number of cases are exceptional. The features of the syndrome as well as diagnostic criteria have not been contrasted in large series. We therefore tried to analyze these parameters in 16 patients diagnosed of Churg-Strauss syndrome at our hospital.
Methods: A retrospective analysis was made of 16 patients diagnosed of Churg-Strauss syndrome at Hospital Universitario La Paz from 1978 at 1998. Data regarding personal antecedents, clinical manifestations, analytical parameters, histology and clinical course were collected. Also, the main diagnostic 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 patients had antecedents of bronchial asthma. Among clinical manifestations, 60% had fever and 50% a general syndrome. The main involved organs by decreasing frequency were lung (100%), nervous system (63%), and heart (44%). The mean eosinophilic count was 7,640/mm3. The histological diagnosis was obtained in 14 out of the 16 patients (88%). The most efficient biopsies corresponded to muscle and nerve. After therapy, no relapse was noted of vasculitis although bronchial asthma persisted in most patients (12). Twelve cases (75%) fulfilled the Lanham diagnostic criteria.
Conclusions: Churg-Strauss syndrome affects mainly women with severe eosinophilia. Electromyogram and muscle and peripheral nerve biopsy are useful tools for diagnosis. The Lanham criteria lead to the diagnosis in most patients.