OBJECTIVE To determine if elderly patients with Wegener's granulomatosis (WG) exhibit distinctive clinical features or outcomes compared with patients whose conditions were diagnosed at younger ages. METHODS Retrospective cohort study. METHODS University medical center. METHODS Thirty-three patients with WG diagnosed when 60 years old or older and 34 patients with WG diagnosed at age younger than 60 years, identified by record review of all WG patients seen over an 11-year period. RESULTS The prevalence of specific clinical features, progression to end-stage renal disease, mortality rate, and infectious and noninfectious complications of therapy were examined. The prevalence of upper respiratory tract involvement (rhinitis, sinusitis, otitis, epistaxis) and hemoptysis were significantly less common as initial manifestations in the elderly patients, although pulmonary infiltrates were seen more commonly during the course of their disease. Renal insufficiency was more common at the time of diagnosis in the elderly patients (64% vs 35%; p < 0.05). Most notably, CNS involvement was 4.5-fold more common in elderly patients (27% vs 6%; p = 0.02). The overall incidence of infectious and noninfectious complications of therapy was similar between the groups, although the mortality rate was markedly higher in the elderly patients (54% vs 19%; p < 0.01). Almost all deaths were due to overwhelming infection. CONCLUSIONS Elderly patients with WG present with distinctive clinical features, particularly a relatively low incidence of upper respiratory tract complaints and a high incidence of CNS involvement. The mortality risk from infectious complications of WG is substantially higher in elderly patients, although this cannot be attributed directly to adverse affects of therapy.