ANCA vasculitis: what is in the pipeline

Purpose of review – Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is now recognized as a complex immune-mediated inflammatory disorder rather than a purely pauci-immune small-vessel vasculitis. Advances in understanding ANCA specificity, complement activation, neutrophil extracellular trap formation, genetic susceptibility, and environmental triggers have reshaped concepts of disease classification, prognosis, and therapy. A focused review of these developments is timely because they are directly informing current treatment strategies, disease stratification, and future drug development. Recent findings – Treatment has shifted from conventional cyclophosphamide and glucocorticoids toward targeted, steroid-sparing approaches. Rituximab is now central to remission induction and maintenance, while avacopan in GPA (granulomatosis with polyangiitis) and MPA (microscopic polyangiitis) and interleukin (IL)-5 blockade in EGPA (eosinophilic granulomatosis with polyangiitis) further reduce glucocorticoid exposure and toxicity. Emerging therapies include next-generation anti-CD20 monoclonal antibodies such as obinutuzumab, CAR-T cell strategies, and other precision immunologic approaches targeting B-cell pathways, including BAFF/APRIL blockade, tyrosine kinase inhibitors (TKIs) and complement-directed therapies designed to more selectively interrupt pathogenic inflammation. Summary – Improved biologic insight is enabling biomarker-driven classification and mechanism-based therapy selection. These advances may reduce treatment-related morbidity, improve durability of remission, and support a more personalized approach to clinical care and translational research in AAV.