Predicting mortality in systemic Wegener's granulomatosis: a survival analysis based on 93 patients

To determine predictors of survival of patients with systemic Wegener's granulomatosis (WG).

We retrospectively studied 93 patients (median age 52 years, male/female ratio 1.7) with systemic WG. All subjects received cytotoxic drugs. Survival was evaluated as a function of the main clinical and laboratory parameters and 2 disease activity scores assessed at diagnosis. Statistical analyses used the multivariate Cox proportional hazards regression model.

The mean followup was 4.5 years; 25 (27%) patients died. According to univariate analysis, a pejorative prognostic value was attributed to serum creatinine >160 micromole/liter (P < 0.001); age >52 years (P < 0.002); absence of ear, nose, and throat (ENT) involvement (P < 0.001); and hemoglobin < or =11.8 gm/dl (P = 0.02). Multivariate analysis retained age >52 years (hazard ratio [HR] = 3.40, P = 0.04) as an independent predictor of poor outcome, whereas the presence of ENT involvement was associated with a longer survival (HR = 0.31, P = 0.02).

Our results suggest that an older age and the absence of ENT involvement at diagnosis independently predict an increased risk of mortality for WG patients. These findings could indicate that the prognosis of WG might be governed by the balance between the granulomatosis as opposed to the vasculitic disease process.