The prognosis for patients with Wegener's granulomatosis has improved considerably over the last decades. The aim of the study was to assess the early death risk based on the prediction model in a population-based cohort of 60 patients with Wegener's granulomatosis. Clinical analysis has been conducted using the disease extent index (DEI) and Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) questionnaires for disease activity. Logistic regression analysis and a Wilcoxon test were included into the statistics. Survival time and death risk were assessed using the Kaplan-Meier estimator and the Cox proportional hazard model. An Receiver Operating Characteristic curve or ROC curve was employed to estimate the value of logistic regression. The early death risk was 16 times higher (P<0.02) in the dialyzed patients and 15 times higher (P<0.05) in the patients with cough as compared with the patients without those predicaments. Predictors of early death are: disease duration, hemoglobin concentration, necessity of dialysis and occurrence of cough. Simultaneous renal and respiratory tract involvement is associated with the highest early death risk.