Antineutrophil cytoplasmic autoantibodies binding to cellular targets, including proteinase 3, activates neutrophils and monocytes; antineutrophil cytoplasmic autoantibodies-activated neutrophils release neutrophil extracellular traps with neutrophil serine proteases, and neutrophil serine proteases are used by myeloid effector cells to induce necrotizing vasculitis (68, 69). Here, PRTN3 is linked to necrotizing vasculitis.