Presentation of 50 cases of Wegener's granulomatosis from São Paulo, Brazil

The purpose of this study is to analyze 50 cases of Wegener's granulomatosis admitted at Hospital das Clínicas (São Paulo, Brazil) from 1985 to 2000, regarding the clinical presentation, sites of accomitment, diagnostic procedures, therapeutics and prognosis of this systemic vasculitis that involves preferentially the upper airways, lungs and kidneys.

The initial clinical presentation was characterized by upper airways involvement in 42/50, pulmonary 39/50, kidney 31/50, ocular manifestations 23/50, osteoarticular in 21/50, cutaneous 21/50 and nervous system involvement in 15/50. The mean age of the patients was 39 ± 15 years, and 26 were female. The diagnosis was confirmed in biopsies or necropsies in 44/50 patients. C-ANCA was positive in 26/40 and P-ANCA in 7/40 patients. The initial pulmonary manifestations were characterized by nodules with or without cavitation in 27/50, masses with or without cavitation in 13/50, and alveolar hemorrhage in 9/50. The treatment was prednisone (1 mg/kg) and cyclophosphamide (2-3 mg/kg). The severe cases also received 3 days methylprednisolone 1 g/day. From the 50 patients attended 11 died, 10 lost follow up, and 29 are in remission. The pneumologist should be aware of the pulmonary and systemic presentation of WG in order to diagnose and treat these patients properly.