Wegener's granulomatosis: clinical course in 108 patients with renal involvement

Background. The aim of this study was to evaluate the clinical course of pa tients with Wegener's granulomatosis and renal involvement, with special re ference to relapse rate, renal and patient survival and morbidity from seri ous infections. Methods. A retrospective analysis was carried out of 108 patients presentin g with Wegener's granulomatosis and active renal disease in eight hospitals in Norway between 1988 and 1998. Multivariate analysis was used to investi gate whether selected variables predicted relapse, renal and patient surviv al and serious infections. Results. Median follow-up was 41.5 months. Twenty-two patients (20.4%) were admitted with a need for dialysis. Complete remission was obtained in 81.5 % after a median of 4 months, and 54.7% relapsed after a median of 22.5 mon ths. Two- and five-year renal survival was 86 and 75%, respectively, and 22 .8% developed end-stage renal disease (ESRD). Two- and five-year patient su rvival was 88 and 74%, respectively, and the cumulative mortality was 3.8 t imes higher than expected. The relative risk of relapse increased with the use of intravenous pulse cyclophosphamide compared with daily oral cyclopho sphamide. Initial renal function predicted renal survival, and low serum al bumin and high age at treatment start increased the mortality risk. Thirty one per cent of the patients were hospitalized for serious infections durin g follow-up. Old age increased the risk of having an infection. Conclusions. The current treatment of Wegener's granulomatosis does not pre vent relapse, development of ESRD and serious treatment-induced infections in a considerable fraction of the patients. Alternative strategies for the management of this disease will be an important objective for further studi es.