FRI0293 FACTORS ASSOCIATED WITH FIRST-YEAR AND OVERALL MORTALITY IN ANCA ASSOCIATED VASCULITIS; PATIENTS WITH RENAL LIMITED VASCULITIS MAKES NO BETTER THAN MICROSCOPIC POLYANGIITIS

Abstract

Background Overall mortality in ANCA-associated vasculitis (AAV) over the last two decades has been reported to be decreasing with the use of immunosuppressive therapies. However, mortality rates remain high and most of the deaths occur in the first year after diagnosis despite treatment. Objectives In this study, we aimed to determine the prevalence of mortality in our AAV patients and to investigate the factors that may be associated with first-year and overall mortality. Methods Patients followed up in one center with the diagnosis of AAV were included in the study. Diagnostic subgroups were; granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal-limited vasculitis (RLV). The clinical and demographic characteristics of the patients were collected retrospectively. Factors predictive of mortality were evaluated by Kaplan-Meier method and the Cox proportional hazard model. Results In total 104 (45 [43%] female and mean age 54.9 ± 15.4 years) AAV patients (51 GPA; 25 MPA; 27 RLV and 1 EGPA) were included in the analysis. ANCA positivity was detected in 81 (77.9%) patients with IIF and/or ELISA. ESRD had developed in 31 (32.3%) of AAV patients and in 37.8% of patients with renal involvement. Mortality rate was 14.4% in the first year after diagnosis and 27.9% during a median follow-up period of 1289 (5-5804) days. The age at diagnosis was the only significant predictor of first-year mortality (p=0.006). Univariate analysis revealed that overall mortality was associated with AAV subgroups (p = 0.035), renal (p = 0.046) (figure) and, ENT (ear-throat) involvement (p = 0.025), ESRD (p = 0.025) and hemodialysis at the time of diagnosis (p = 0.040). However Cox regression analysis showed age at diagnosis as the only significant predictor also of the overall mortality (P=0,001). Conclusion Our findings suggest that there could be some survival differences between AAV subgroups and patients with or without renal and ENT involvement. However, the age at diagnosis seems to be the only significant predictor of first-year and overall mortality in our AAV patients. *AAV: ANCA-Associated Vasculitis; GPA: Granulomatosis with PolyAngiitis; MPA: Microscopic PolyAngiitis; RLV: Renal Limited Vasculitis Disclosure of Interests Onay Gercik: None declared, Zeki Soypacaci: None declared, Fulya Cakalagaoglu: None declared, Gokhan Kabadayi: None declared, Idil Kurut: None declared, Dilek Solmaz: None declared, Servet Akar Grant/research support from: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Speakers bureau: Pfizer