Histological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with...

Autoimmune pancreatitis (AIP) is characterized by a tumefactive inflammatory lesion resembling pancreatic carcinoma. Type 1 AIP is a pancreatic manifestation of IgG4-related disease characterized by unique histological features that can be identified on imaging. The capsule-like rim, which is a collar of hypertrophic lesion surrounding the pancreas, consists of lymphoplasmacytic infiltration and fibrosis, and storiform fibrosis is often identified. Hypertrophic lesions of various microscopic architectures such as the ducts, veins (obliterative phlebitis), arteries (periarteritis), and nerves are observed without parenchymal damage. The pancreatic lobules keep their contours, but the acinar cells are diminished and replaced by numerous inflammatory cells. These features provide clues to arrive at a diagnosis of type 1 AIP and to distinguish it from pancreatic carcinoma on imaging. In contrast, type 2 AIP is an epithelium-centered inflammation involving the ducts and lobules. Neutrophilic infiltration in the epithelium and/or lumens (granulocytic epithelial lesion) is a characteristic finding. Lobular swelling due to inflammation is the cause of pancreatic enlargement. IgG4-related sclerosing cholangitis is histologically similar to the hypertrophic ductal lesion in type 1 AIP and characterized by wall thickening due to inflammation and luminal stenosis. The epithelium is intact, which is different from bile duct carcinomas and primary sclerosing cholangitis, the latter of which is characterized by inflammation targeting the epithelium. Although the histological features of type 1 AIP and IgG4-related sclerosing cholangitis are unique, the biopsy diagnosis of these diseases has limitations, which should be recognized by clinicians.