Addressing clinical challenges in ANCA-associated vasculitis with real-world evidence

Abstract Key challenges in the management of ANCA-associated vasculitis (AAV) include the need to achieve more rapid and sustained remission, reduce exposure to glucocorticoids (GC) and reliably monitor and predict treatment response. Clinical trials in patients receiving rituximab or cyclophosphamide for AAV show that the adjunctive use of avacopan (a novel complement 5a receptor 1 [C5aR1] antagonist) for up to 1 year enables sustained AAV remission, considerable reductions in GC exposure, and greater recovery of kidney function, especially in patients with acute kidney injury. Additional real-world evidence suggests avacopan can be used to replace GC in patients with GC toxicity and supports the use of avacopan in AAV patients with rapidly progressing glomerulonephritis, pulmonary hemorrhage and/or refractory AAV. Future studies are needed to investigate the benefits of extending avacopan treatment beyond 1 year and in specific populations.