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

Abstract

Background. The aim of this study was to evaluate the clinical course of patients with Wegener’s granulomatosis and renal involvement, with special reference to relapse rate, renal and patient survival and morbidity Introduction from serious infections. Methods. A retrospective analysis was carried out of Wegener’s granulomatosis is a necrotizing granulomat108 patients presenting with Wegener’s granulomatosis ous small-vessel vasculitis that affects mainly the upper and active renal disease in eight hospitals in Norway and lower respiratory tract and the kidneys. Although between 1988 and 1998. Multivariate analysis was used the prognosis has improved dramatically over the last to investigate whether selected variables predicted decades, the disease still carries a high mortality and relapse, renal and patient survival and serious morbidity rate. In the 1950s, the median patient surinfections. vival was 5 months, and 1 year mortality was 80% [1]. Results. Median follow-up was 41.5 months. Twenty- The introduction of aggressive treatment with high two patients (20.4%) were admitted with a need for doses of glucocorticosteroids combined with cyclophodialysis. Complete remission was obtained in 81.5% sphamide has improved the prospects for these after a median of 4 months, and 54.7% relapsed after patients. Fauci and co-workers induced remission in a median of 22.5 months. Two- and five-year renal 93% out of 85 patients with Wegener’s granulomatosis, survival was 86 and 75%, respectively, and 22.8% and 88% were still alive after a mean follow-up of 51 developed end-stage renal disease ( ESRD). Two- and months [2]. In the classical study by Hoffman and five-year patient survival was 88 and 74%, respectively, co-workers, 80% of the 158 patients were alive after 8 and the cumulative mortality was 3.8 times higher than years [3]. expected. The relative risk of relapse increased with The epidemiology of Wegener’s granulomatosis is the use of intravenous pulse cyclophosphamide com- largely unknown. The incidence is low, diagnostic pared with daily oral cyclophosphamide. Initial renal criteria vary and studies often come from tertiary function predicted renal survival, and low serum albu- referral hospitals introducing the possibility of selection min and high age at treatment start increased the bias. In the 1970s, the reported annual incidence was mortality risk. Thirty one per cent of the patients were between 0.4 and 4 per million [4,5]. The estimates have hospitalized for serious infections during follow-up. increased over the last decade, which may be attributed Old age increased the risk of having an infection. to an increased awareness of the disease among cliniConclusions. The current treatment of Wegener’s gran- cians, but also to the introduction of assays for antiulomatosis does not prevent relapse, development of neutrophil cytoplasmic autoantibodies (ANCA) in ESRD and serious treatment-induced infections in a 1985 [6 ]. Thus, a British study from 1995 reported an considerable fraction of the patients. Alternative strat- annual incidence of 8.5 per million [7]. egies for the management of this disease will be an The proportion of patients with renal involvement important objective for further studies. at disease presentation has varied between studies from <20% to 80%, but invariably increases to 80–94% during follow-up [3,8]. Renal involvement in Wegener’s granulomatosis heralds a more severe out