Targeted Therapy for ANCA Associated Vasculitis: Where Have We Been?

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a complex group of autoimmune diseases characterized by systemic small-vessel inflammation. Although glucocorticoids combined with conventional immunosuppressants can induce remission, their significant toxicity and high relapse rates remain major clinical challenges. With the deepening understanding of the pathogenesis of AAV, particularly the growing insights into neutrophils, B cells, T cells, the complement system, and key cytokine pathways, the application of targeted biologics has emerged as a new paradigm in treatment. This review systematically elucidates the progress in targeted therapies based on different immunopathological pathways. However, biologics still face challenges, including high costs, individual variability in efficacy, infection risks, and a lack of predictive biomarkers. Future treatment strategies must evolve toward personalized precision medicine, aiming to achieve long-term glucocorticoid-free remission for a higher proportion of patients through the exploration of combination therapies, the discovery of novel biomarkers, and the optimization of treatment selection.