Evolution of Therapy for ANCA-Associated Vasculitis with Kidney Involvement.

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) belongs to a group of small-vessel systemic vasculitides characterized by granulomatous and neutrophilic inflammation of various tissues. Patients often have circulating autoantibodies targeting neutrophilic antigens. Although AAV was once associated with severe end-organ damage and extremely high mortality rates, the use of glucocorticoids and cyclophosphamide led to a paradigm change in its treatment. Over the last 20 years, significant progress in understanding the immunopathogenesis of AVV has enabled development of targeted immunotherapies, providing a much better prognosis for patients. This review describes the evolution of treatment of AAV, particularly for patients with kidney involvement.