Points to consider in the management of ANCA-associated vasculitis

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by inflammation and necrosis of small to medium blood vessels that frequently present with organ- or life-threatening manifestations. Although significant therapeutic advances have improved outcomes, many areas in the management of AAV remain controversial or insufficiently defined. Key areas of debate include the choice of induction therapy—particularly the potential benefits of combining rituximab and cyclophosphamide—and the role of adjunctive therapies such as avacopan and plasma exchange. Additional challenges involve determining the optimal duration and dosing of maintenance therapy, strategies for relapse prediction and prevention, appropriate glucocorticoid tapering, the use of fixed versus biomarker-guided maintenance regimens, and the potential for withholding maintenance therapy in select patient populations. Despite multiple high-quality randomized controlled trials and international guidelines, clinical practice remains heterogeneous. The emergence of novel therapies is promising in helping to address these gaps and may provide effective treatment options for patients with resistant or refractory disease. This review summarizes current controversies and challenges in the treatment of AAV and provides a practical, evidence-based framework to support clinical decision-making.