#3468 Relapse of ANCA-associated vasculitis in patients undergoing renal replacement therapy: a rare but severe complication. Case Series

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Abstract Background and Aims Renal involvement is one of the most severe and common manifestations in patients with ANCA-associated vasculitis (AAV), progressing to end-stage kidney disease (ESKD, stage G5T) in 20–25% of cases. Due to its recurrent nature, immunosuppressive treatment must be maintained for at least two years, which can increase morbidity and mortality due to its potential adverse effects. Although the risk of relapse after initiating renal replacement therapy (RRT) is lower, relapses can present with extrarenal manifestations, leading to potentially severe complications. Method Here, we describe our experience with four patients who developed an AAV relapse after initiating RRT between 2020 and 2024. Results The results are summarized in Table 1. All patients had MPO-ANCA positivity. Two patients were undergoing hemodialysis, one was on peritoneal dialysis, and one experienced relapse within the first few weeks post-kidney transplantation. Of the four patients, only one was receiving maintenance therapy after initiating RRT. The time on RRT at the moment of relapse varied widely; however, all patients had elevated ANCA titers at the time of relapse. In three cases, alveolar hemorrhage was the predominant clinical manifestation. All cases were treated with plasma exchange, corticosteroids, and rituximab ± cyclophosphamide, with favorable clinical outcomes. Conclusion Our experience highlights that, although uncommon, AAV relapses can occur in patients undergoing RRT and may be potentially severe. The management of this condition poses the challenge of balancing the risks and benefits of long-term immunosuppression. Although there is no established consensus on the follow-up of these patients, maintaining regular laboratory monitoring and a high clinical suspicion for warning symptoms is crucial to enable early and effective treatment of relapse.

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