Figure 2.

Histopathological and immunohistochemistry of pancreatic tissues from CALCB mutations (scale bar: 50 μm). (a) Typical features of pancreatic inflammation (fibrosis) and autoimmune disease (inflammatory infiltrates and vasculitis which depicted by bracket) (HE × 100). (b) Perineural inflammation: nerve fibers surrounded by an attenuated inflammatory infiltrate, concentric circle permutation (HE × 40) and nerve fiber crosses section (inset) (HE × 200). (c) Abundant IgG-positive plasma cells infiltration. (d) IgG4-positive lymphoplasmacytic cells. Enlarge as depicted by arrowheads and inset (× 200). (e) Electron microscopic examination indicates that dense lamina propria fibrosis and plasma cells and lymphocyte cells infiltration in the patient with the homozygous S30P CALCB mutation (× 3 800). (f) Collagen fiber diffuses hyperplasia, lymphocyte infiltration, and venous sinus occlusion (× 3 800). Immunohistochemical staining showed: CD3-, CD20-, CD138-, and CD68-positive infiltrating cells, which surrounded pancreatic nerve fibers. (g) CD3-positive T-lymphocytes are present within the lesion. (h) CD20 immunostaining demonstrates multiple B-cell aggregates. (i) CD138-positive are present around nerve fibers demonstrates that the infiltrating cells contain a large number of plasma cells. (j) CD68-positive is present macrophage. Polyclonality is established by the presence of both kappa (k) and lamda (l) light chain staining. βCGRP immunohistochemical analysis in pancreas from AIP: (m and n) βCGRP fails to stain nerve fibers from CALCB mutual pancreatic tissues, (m and n) βCGRP strong express in the inflammatory cells around the nerve fibers and (o) vasculature, (p) Immunofluorescence microscopy showed IgG-surrounded perivascular, resulting in obliterative vasculitis