Based on the presence of specific ANCAs and clinical symptoms, AAV can be subdivided into microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA), which is predominantly associated with the presence of PR3-ANCAs (1). This evidence concerns the gene PRTN3 and granulomatosis with polyangiitis.