#5679 PATIENT OUTCOME IN A SWEDISH SINGLE-CENTRE COHORT OF ANCA-ASSOCIATED VASCULITIS WITH RENAL INVOLVEMENT

Abstract

Abstract Background and Aims End-stage kidney disease and premature death are common in patients with antineutrophil cytoplasmic antibody (ANCA) -associated vasculitis (AAV) with renal involvement. Poor patient outcome is attributed to multiple factors comprising a delay in diagnosis and treatment, inadequate efficacy of treatments, and complications due to toxic effects of given immunosuppressive therapy. The aim of the present study was to evaluate clinical and prognostic features of a Swedish cohort of patients with renal AAV and to investigate the pattern of immunosuppressive treatment and predictors of renal and patient survival. Method Ninety-one patients diagnosed with AAV with renal involvement between 01 March 2002 and 30 October 2018 in a single-centre were included in the present retrospective study. Results Among these ninety-one patients, 52 (57%) were diagnosed with PR3-ANCA, and 39 (43%) with MPO-ANCA. Patients with PR3-ANCA and MPO-ANCA were received comparable induction therapy. However, the frequency of PLEX was significantly higher in patients with PR3-ANCA versus MPO-ANCA (44% vs. 21% respectively; P = 0.025). Overall renal survival at 1- and 5-year was 91% and 69% respectively with no significant differences between patients with PR3-ANCA and MPO-ANCA. Overall, 1-year and 5-year patient survival was 92% and 77% respectively. The overall survival time mean was11.5 years and 95% confidence interval (CI) of 9.9 to 13.0 years. Mean survival time in patients with PR3-ANCA was 10.8; 95% CI 8.8 to 12.7 years versus 11.7; 95% CI 9.6 to 13.8 years in those with PR3-ANCA. Cox regression analysis showed that advancing age significantly predicted higher mortality risk, whereas MPO-ANCA subtype significantly predicted lower mortality risk (Figure 1). Infection (25%), malignancy (22%), and cardiovascular events (16%) were the major causes of death in the present cohort. In a subgroup of thirty-two patients with eGFR <15 ml/min per 1.73 m2 or undergoing dialysis at time of diagnosis. Twenty-three patients (72%) were treated with PLEX, whereas 9 patients (28%) did not receive PLEX as a part of remission induction therapy. Treatment with PLEX had no effects on overall 1-year patient and renal survival Conclusion In this cohort of patients with AAV with renal involvement the overall 1-year renal and patient survival were high. Advancing age significantly predicted higher mortality risk, while MPO-ANCA subtype significantly predicted lower mortality risk. Treatment with PLEX had no effects on 1-year patient and renal survival in a subgroup of patients with severe renal involvement with AAV.