Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a group of autoimmune disorders, including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA), characterized by necrotizing small-vessel vasculitis with serum autoantibodies mainly against proteinase 3 (PR3) or myeloperoxidase (MPO). Here, PRTN3 is linked to granulomatosis with polyangiitis.