Challenges and advances in the management of antineutrophil cytoplasmic antibody vasculitis in 2025.

Purpose of review: Antineutrophil cytoplasmic antibody (ANCA) vasculitides are complex, immune mediated conditions of significant morbidity and mortality. This review highlights the evolving therapeutic landscape and emerging sub-phenotypes of the disease group to support development of more personalised interventions for people living with ANCA vasculitides.

Recent findings: The advances in management include steroid-sparing strategies, with avacopan offering complement-based neutrophil inhibition. Rituximab has become central in induction and maintenance therapy, reducing cyclophosphamide use and replacing azathioprine. Cyclophosphamide is still used but often as an additional induction agent in the combination with rituximab to speed up disease control and to reduce glucocorticoid burden in severe kidney disease. Evidence gaps remain, particularly regarding long-term safety of newer agents, the optimal duration of maintenance therapy, and the benefits of plasma exchange in select populations. Recognition of distinct disease trajectories such as slowly sclerosing kidney disease in opposition to rapidly progressive glomerulonephritis highlight the need for more stratified treatment.

Summary: Early disease detection, shared decision-making and personalised care are keys to improving outcomes in ANCA vasculitis. Future directions are risk stratifications that incorporate biomarker and novel diagnostic techniques to guide treatment intensity. Identification of key service components associated with improved outcomes such as multidisciplinary care is essential to support equitable translation of medical advances into clinical practice, ensuring both efficacy and safety in this high-risk population.