As such, the successful phase III study and the recent FDA-approval of the C5aR1 inhibitor avacopan in ANCA-associated vasculitis (96) and a promising phase II study with the LTB4/C5a inhibitor nomacopan in BP (unpublished), that led to the initiation of a phase III trial, may pave the way for effective complement-related therapies for this disease. This evidence concerns the gene C5AR1 and anti-neutrophil cytoplasmic antibody-associated vasculitis.