Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare and life-threatening autoimmune disease consisting of three primary types: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The mainstay of treatment is immunosuppression, which has significantly decreased both upfront and long-term mortality [1]. However, most AAV patients require extended duration of maintenance immunosuppression due to a high risk of relapses that can lead to significant irreversible organ damage [2] and other severe complications, including premature death [3]. Despite treatment, approximately 20% of AAV patients will develop end-stage kidney disease (ESKD) requiring chronic dialysis or transplantation [4].

Several studies have suggested that patients with AAV and ESKD have a lower relapse rate compared to other patients with AAV [5], [6], [7], [8], [9]. Furthermore, infections, cardiovascular disease, and malignancies are a major cause of morbidity and mortality in patients with AAV, as well as in patients with ESKD [10], [11], [12]. Due to the perceived lower relapse rate and higher risk of infections or malignancies with immunosuppression, it is recommended to avoid maintenance therapies in AAV patients with ESKD [13], [14], [15]. However, withholding maintenance therapy may increase disease-related complications in AAV patients with extra-renal manifestations. Therefore, the optimal treatment approach for AAV patients with ESKD remains unclear. This systematic review and meta-analysis was undertaken to gather the existing evidence for outcomes in AAV patients after development of ESKD on renal replacement therapy (RRT) to provide guidance for managing these patients and to guide future research opportunities.