Renal improvement and remission in a patient with refractory ANCA-associated vasculitis treated with avacopan

Abstract Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is associated with end-organ damage resulting in significant morbidity and mortality. Most recently, avacopan, an orally administered selective antagonist of the C5a receptor, was approved by the US Food and Drug Administration as an adjunctive treatment of adult patients with severe, active ANCA-associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis) in combination with standard therapy including glucocorticoids. This case study describes a 58-year-old Asian female with severe ANCA-associated vasculitis and acute renal failure who responded to adjunctive therapy with avacopan despite being refractory to rituximab and glucocorticoid therapy.