Evolution of Therapy for ANCA-Associated Vasculitis with Kidney Involvement

Abstract

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.

Antineutrophil cytoplasmic antibody-associated vasculitis

“…Both generalized and severe descriptions include the involvement of at least one vital organ with dysfunction or failure. Hence, nephrologists manage generalized, severe, and refractory AAV cases [8,9].…”

“…The pivotal role of CYC in remission induction was established in a series of National Institutes of Health trials highlighting its efficacy but also cumulative toxicities [8,14]. The CYCLOPS trial compared i.v.…”

“…route of CYC treatment, and shorter duration of induction. Maintenance therapy is used to reduce the risk of relapses [8,42].…”