Detailed analysis of the ANCAs found in the CIMDL and GPA patients showed that none of the eight perinuclear ANCAs in CIMDL patients reacted with MPO, while four reacted with PR3, three with human neutrophil elastase (HNE), and two had double positivity to PR3 and HNE. Here, PRTN3 is linked to granulomatosis with polyangiitis.