Typically, proteinase 3 (pr3)-ANCA and cytoplasmic (c-ANCA) patterns are found in GPA, whereas myeloperoxidase (MPO)-ANCA and perinuclear (p-ANCA) patterns dominate in MPA and about 40% of EGPA cases [4,5]. Here, PRTN3 is linked to granulomatosis with polyangiitis.