Abstract

Anti neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare disorder with annual incidence estimated to be around 1.3 per 100,000 population. Renal involvement is one of the main predictors of mortality and morbidity, with approximately 30% of patients with renal involvement progressing to ESRD after 5 years. Renal biopsy can provide a definite diagnosis, and may predict the renal prognosis in AAV. Limited data is available on its management and outcomes, thus we aim to assess this at our tertiary care renal facility. This was a retrospective cohort study. We included patients with a documented diagnosis of ANCA positive vasculitis between 1st of January 2012 to the 31st December 2017, with a follow up period until 31st December 2019. Using a standardized data collection form we recorded the number of patients who had a renal biopsy, the induction and maintenance therapies used, along with relapse induction and maintenance therapies. At the end of the follow-up period outcomes were divided into progression to end-stage renal disease (ESRD), death, established chronic kidney disease (CKD), and preservation of renal function. Data of thirty-six patients were included in the final study. The patient baseline characteristics are listed in Table 1. Thirty-two patients (94.1%) had a documented renal biopsy. The majority of patients (66.7%) had cyclophosphamide for induction, followed by rituximab (19.4%). Seven patients (19.4%) had a documented relapse during the study period, 4 MPO+ and 3 PR3+. Six patients (85.7%) had rituximab as an induction therapy for relapse. The majority of patients were on azathioprine (61.1%) as maintenance therapy after induction. This was similar to the maintenance therapy used after relapse (57.1%). Progression to ESRD occurred in 11 (30.6%), death in 4 (11.1%), established CKD in 15 (41.7%), and preservation of renal function in 6 (16.7%) patients by the end of the follow up period. While cyclophosphamide remains the choice of induction immunosuppresion therapy, we favour rituximab as an induction agent in relapse of AAV. However despite aggressive immunosuppresion therapy the incidence of ESRD and death remains high in these patients. The current management and outcomes are comparable to present international guidelines and cohorts. We look forward to new updates and therapies to improve outcomes in AAV.

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