Management and Outcomes of ANCA-Associated Vasculitis at a Tertiary Healthcare Facility

Abstract

Purpose of the Study. Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a rare multisystem autoimmune condition with an incidence of 0.4–24 per 1 million person- years. The severity of renal involvement predicts prognosis. Limited data are available on its management and outcomes; thus, we aim to assess this at our tertiary-care renal facility. Study Design. Retrospective data were collected using our national renal electronic database (eMED) to identify AAV patients over a period of 6 years. Outcomes included progression to end-stage renal disease (ESRD) or death, chronic kidney disease (CKD), and preservation of renal function. Results. Thirty-six patients were included in the final study. Cyclophosphamide was used in 24 patients (66.7%) and, comparatively, rituximab in 7 patients (19.4%) for induction. Seven patients (19.4%) had a documented relapse, and six patients (85.7%) had rituximab as induction therapy for relapse. The majority of patients were on azathioprine (61.1%, 57.1% relapse population) as maintenance therapy. 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. Conclusions. While cyclophosphamide remains the choice of induction immunosuppression therapy, we favour rituximab as an induction agent in the relapse of AAV. Despite aggressive immunosuppression therapy, the incidence of ESRD and death remains high in these patients.

The Efficacy and Safety of Rituximab in ANCA-Associated Vasculitis: A Systematic Review

“…In a prospective study on the RAVE trial’s patients, using RTX showed promising results with a safe and more effective outcome in relapsed patients who were treated with either RTX or CYC [ 40 ]. In another comparative study of RTX and CYC on 16 GPA and 20 MPA patients, RTX was favorable for the induction of remission in the refractory AAV patients [ 96 ]. RTX is considered a promising treatment for elderly patients due to the higher risk of treatment-related side effects in AAV patients under CYC or GC treatment [ 83 ].…”

“…CR was achieved in 93% of the RTX group and 70% in the conventional group; however, no significant difference was observed between these two groups in 6-month and 12-month renal recovery. Another study on 36 AAV patients with renal involvement showed that RTX is a favorable induction agent in relapsed AAV patients, and 41.7% and 28.6% of AAV patients under CYC and RTX faced ESRD or death, respectively [ 96 ]. Moura et al [ 98 ] investigated the efficacy of RTX and CYC in 251 patients with severe disease and showed that no significant difference was observed between RTX and CYC in the induction of remission.…”

Anti-neutrophil cytoplasmic antibody associated vasculitis following rituximab: Outcomes of 50 patients in a tertiary single centre

“…Their incidence increases with age, reaching a high level in the 60-70-year-old age group. 6,7 Immunosuppressive drugs are used to induce remission and for long-term maintenance therapy. Cyclophosphamide or rituximab with glucocorticoids is recommended as induction therapy for severe life-threatening AAV.…”