Microscopic polyangiitis (MPA) and myeloperoxidase (MPO)-ANCA positive AAV (MPO-AAV) are predominant in East Asian populations, while granulomatosis with polyangiitis (GPA) and proteinase 3 (PR3)-ANCA positive AAV (PR3-AAV) are common in the populations of European ancestry [2]. Here, PRTN3 is linked to microscopic polyangiitis.