Small vessel vasculitides, such as Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA), are strongly associated with antineutrophil cytoplasmic antibodies (ANCA), which are either directed to myeloperoxidase (MPO) or proteinase 3 (PR3) [1-3]. This evidence concerns the gene PRTN3 and granulomatosis with polyangiitis.