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). The gene discussed is PRTN3; the disease is anti-neutrophil cytoplasmic antibody-associated vasculitis.