Analysis of factors predictive of survival based on 49 patients with systemic Wegener's granulomatosis and prospective follow-up

Objectives. This prospective study attempted to determine factors predictive of survival in systemic Wegener's granulomatosis (WG) based on 49 patients. Patients and methods. All patients had previously untreated systemic WG. Treatment was with oral or pulse cyclophosphamide plus corticosteroids. Univariate and multivariate analyses of survival were performed using 13 parameters evaluated at diagnosis. Results. The mortality rate was 37% during a mean follow-up period of 1.9 yr. Among the 13 parameters evaluated, univariate analysis selected the following factors as predictors of a poor outcome: serum creatinine greater than or equal to 18.1 mg/dl, age greater than or equal to 57 yr, and erythrocyte sedimentation rate (ESR) greater than or equal to 90 mm 1st h. The absence of ear, nose and throat (ENT) involvement also tended to predict a greater risk of mortality. Glomerulonephritis, when present and regardless of creatininaemia and pulmonary involvement had no significant effect. Multivariate analysis retained serum creatinine greater than or equal to, 18.1 mg/dl and age greater than or equal to 57 yr as significant predictors of pool prognosis. Conclusions. Our results suggest that impaired renal function and older age are independent factors predicting poor outcome in WG. ESR proved to be a good marker of disease severity. Conversely, univariate analysis indicated that patients with ENT involvement tended to have a better outcome, suggesting a more benign evolution of granulomatous disease compared with more aggressive vasculitis.