AAV can be further subdivided in three diagnostic entities, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) with the presence of ANCA autoantibodies targeting the myeloperoxidase (MPO) or the neutrophil cytoplasmic antigens proteinase 3 (PR3). This evidence concerns the gene PRTN3 and granulomatosis with polyangiitis.