Granulomatosis With Polyangiitis (Wegener). Description of 15 Cases

Abstract

BackgroundWegener's granulomatosis (WG) is a necrotizing vasculitis of unknown etiology that involves small and medium caliber vessels. It is associated with anti-neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas. ObjectivesTo detail the features of 15 patients with WG diagnosed in a university referral center. Patients and methodsRetrospective study: between 1984 and 2009, 15 patients with WG were diagnosed in our center. Epidemiological, clinical, laboratory tests as well as pathologic studies and treatment were retrospectively analyzed. Biopsy diagnosis of WG was considered as an inclusion criterion. ResultsFifteen patients were diagnosed: 12 men and 3 women. Mean age at diagnosis: 52.2 years (14–78). Twelve patients had a history of smoking. A biopsy was diagnostic in all patients. ANCA were positive in 11 cases, 6 had a cytoplasmic c-ANCA pattern. All patients had pulmonary involvement and seven (40%) had renal involvement. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 5 patients died. ConclusionThe clinical features of this series do not differ from those described by other authors. However, a history of smoking is more common than expected. Frequently used drugs were glucocorticoids and cyclophosphamide (oral and pulse therapy). The course was usually unfavorable, with outbreaks or complications due to immunosuppression, except for those with limited forms. Immunosuppressive therapy should be maintained indefinitely in most cases.