Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of necrotizing vasculitides predominantly affecting small- to medium-sized blood vessels, in the presence of proteinase-3 (PR3) and myeloperoxidase (MPO) ANCAs (1). Here, PRTN3 is linked to anti-neutrophil cytoplasmic antibody-associated vasculitis.