An interdisciplinary approach to the care of patients with Wegener's granulomatosis - Long-term outcome in 155 patients

Objective. To examine the outcome in 155 consecutive patients with Wegener' s granulomatosis (WG) followed up for a median of 7 years. Methods. Treatment was adapted to the activity and extent of disease, with regular evaluation by an interdisciplinary team accompanied by group educat ion about vasculitis, Results. The estimated median survival time was 21.7 years (95% confidence interval [95% CI] 15.60-27.86). Twenty-two patients died; 19 deaths were at tributable to WG and/or its treatment. Significant predictors of survival a t diagnosis were age >50 years (hazard ratio [HR] 5.45, 95% CI 1.97-15.02), kidney involvement with impaired renal function (HR 5.42, 95% CI 1.76-16.6 8), and lung involvement (HR 3.75, 95% CI 1.26-11.16), At some stage, 142 p atients received prednisone and cyclophosphamide (CYC), usually as daily CY C plus mesna as uroprotection, 50 patients received trimethoprim/sulfametho xazole, and 45 received methotrexate, Complete remission was achieved in 83 of the 155 patients. One or more relapses occurred in 99 patients after ei ther complete or partial remission. CYC-induced cystitis and myelodysplasti c syndrome occurred in 17 and 11 patients, respectively. A cumulative dose of 100 gm or more of CYC resulted in a 2-fold greater risk of CYC-related m orbidity than with lower CYC doses. Serious infections occurred in 41 patie nts. Conclusion. An interdisciplinary approach to the care of 155 WG patients re sulted in a median survival of >21 years. Kidney or lung involvement at dia gnosis was predictive of a >3-fold higher mortality, Although CYC remains e ssential in the treatment of WG, it was administered as briefly as possible and under close surveillance to avoid permanent CYC-related morbidity, whi ch can lead to serious therapeutic problems in chronic relapsing WG.