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

Abstract

Objective. To determine predictors of survival of patients with systemic Wegener's granulomatosis (WG).Methods. 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. Results. 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 mole/liter (P < 0.001); age >52 years (P < 0.002); absence of ear, nose, and throat (ENT) involvement (P < 0.001); and hemoglobin <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). Conclusion. 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.

Long‐Term Outcomes Among Participants in the WEGENT Trial of Remission‐Maintenance Therapy for Granulomatosis With Polyangiitis (Wegener's) or Microscopic Polyangiitis

“…According to the findings of our multivariate analysis, advancing age was the sole factor significantly associated with death, as has been identified in previous studies of patients with GPA (2,3,31). The only other study that identified prognostic baseline factors for survival in a cohort of patients with GPA receiving the same care regimen found age, kidney involvement, and lung involvement to be associated with poorer survival (31).…”

Hypertrophic pachymeningitis is a characteristic manifestation of granulomatosis with polyangiitis: A retrospective study of anti‐neutrophil cytoplasmic antibody‐associated vasculitis

“…3 Regarding the pathological description of GPA, the predominance of granulomatosis is obvious in the respiratory tract lesions, while small-vessel vasculitis principally concerns the pathogenesis of renal involvement. 8,25,26 Considering these indications, existence of granulomatous lesions may be required for the formation of HP in AAV. In fact, all patients with HP in the present study significantly indicated paranasal sinus involvement since the early phases of the illness, and a pulmonary nodular lesion was detected in one patient without paranasal sinus involvement.…”

Entwicklung von Morbidität und Mortalität bei ANCA-assoziierten Vaskulitiden

“…Unter Therapie mit CYC ist die Infektionsrate höher als unter mittelpotenten Immunsuppressiva, insbesondere bei eingeschränkter Nierenfunktion [2]. Obwohl Infektionen unverändert ein zentrales Problem bei der Behandlung der AAV darstellen, sind auf diesem Gebiet große Fortschritte zu verzeichnen.…”