Anti-neutrophil cytoplasmic antibodies (ANCA) are autoantibodies, the major ANCA antigen targets in inflammatory vasculitides is the proteinase-3, which are cytoplasmic granular with accentuation between nuclear lobes (cANCA), wherever in IBD the myeloperoxidase are the target, which are fine homogenous, diffuse rim-like staining of perinuclear cytoplasm (pANCA) [4–6].The atypical pattern of ANCA is described as fine rime-like staining with intranuclear foci or broad nonhomogenous rim-like staining of the nuclear periphery as a mixture of c-ANCA and p-ANCA [7, 8]. The gene discussed is PRTN3; the disease is inflammatory bowel disease.