Mapping a path forward: Addressing disease burden, pathways and solutions in ANCA-associated vasculitis

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a frequently relapsing systemic autoimmune disorder characterized by inflammation and destruction of small- to medium-sized blood vessels resulting in potentially life-threatening organ damage. Of the three AAV subtypes, granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are the most common. The aims of treatment are to rapidly control active disease with induction therapy (typically rituximab [the new standard-of-care] or cyclophosphamide alongside glucocorticoids [GC] and avacopan), followed by less aggressive maintenance strategies to reduce the risk of relapse. International and national guidelines for the treatment of GPA/MPA are generally aligned, with all guidelines highlighting a need to reduce treatment-related adverse events through rapid GC tapering and the use of GC-sparing avacopan treatment. Guidelines will continue to evolve as ongoing studies provide new insights into alternative (GC-sparing) treatment options and optimal RTX-based treatment regimens.