Study Of Clinical Features And Survival Time Of Patients In Wegener's Granulomatosis

Objective WG is an antineutrophil cytoplasmic ANCA-associated small-vessel vasculitis of unknown etiology. Although combining corticosteroids and cyclophosphamide has greatly improved the prognosis of WG, mortality remains high with 5-year survival rates. To investigate the clinical features and survival time of Wegener's granalomatosis.Methods 133 WG patients, diagnosed between 1990 to 2009 in Pubmed, were retrospectively studied and their clinical features and survival curve were analyzed. For every patient, the following variables were recorded at diagnosis:age, sex, organs involvement. In addition, all of the laboratory parameters were assessed.Results The median follow-up time was 22 months (1-192 months). The most frequent clinical manifestation of this group was lung involvement (111/133, 83.5%), followed by ENT involvement(104/133,78.2%), kidney involvement (80/133,60.2%), non-specific symptoms (99/133,60.2%).5 patients were MPO-ANCA and p-ANCA positive. Compared with the survival group, age at diagnosed(P=0,019), ascent of CRE(P=0.002), digestion involvement (P=0.048) was higher than those in the group of death. Those features were possible predictors of poor prognosis. In the group of more than 55 years versus the group of less than 55 years,5-year survival rates were 83.5% vs 61.9% (P=0.002). Ascent of CRE (Hazard ratio 3.714, P=0.020) and Gastrointestinal involvement (Hazard ratio 3.498, P=0.019) were the independent factor of survival time. Conclusion This study showed that Wegener's granulomatosis was a multi-system involvement disease. Few patients were MPO-ANCA and p-ANCA positive. Age more than 55, ascent of CRE, Gastrointestinal involvement might be possible predictors of poor prognosis, more active therapy needed to be employed in this kind of patients.