Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of systemic vasculitis characterized by inflammation of small vessels and in most of the cases presence of autoantibodies against neutrophil cytoplasmic antigens (proteinase 3 PR3-ANCA and myeloperoxidase MPO-ANCA). The gene discussed is PRTN3; the disease is necrotizing vasculitis.