Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels associated with ANCA specific for myeloperoxidase (MPO) or proteinase 3 (PR3). Here, PRTN3 is linked to anti-neutrophil cytoplasmic antibody-associated vasculitis.