On Oct 8, 2021, the US Food and Drug Administration issued its first new drug approval for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in a decade. The first-in-class approval allows the orally administered complement 5a receptor inhibitor avacopan to be used as an adjunct to standard therapy for the two main forms of ANCA-associated vasculitis (microscopic polyangiitis and granulomatosis with polyangiitis), making avacopan the first drug to be approved with ANCA-associated vasculitis as the primary indication.