title: Diagnosis and classification of autoimmune pancreatitis.
abstract: Recent studies suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 related with IgG4 as the pancreatic manifestation of IgG4-related disease (IgG4-RD), and type 2 related with a granulocytic epithelial lesion. Apart from type 2 AIP, the characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmacytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extra-pancreatic manifestations of IgG4-RD (e.g. sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and steroid responsiveness. Although the way how to diagnose IgG4-RD has not been established yet, the Comprehensive Diagnostic Criteria (CDC) for IgG4-RD for general use, and several organ specific criteria for AIP have been proposed; the International Consensus Diagnostic Criteria (ICDC) and the revised clinical diagnostic criteria in 2011 by Japan Pancreas Society (JPS-2011) for type1 AIP. In cases of probable or possible IgG4-RD diagnosed by the CDC, organ specific diagnostic criteria should be concurrently used according to an algorithm of diagnosis for IgG4-RD with reference to the specialist.
authors.affiliation: The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan. Electronic address: okazaki@hirakata.kmu.ac.jp.
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
authors.affiliation: The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Tomiyama
authors.affiliation: The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Mitsuyama
authors.affiliation: The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
authors.forename: Kimi
authors.initials: K
authors.lastname: Sumimoto
authors.affiliation: The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.
authors.forename: Kazushige
authors.initials: K
authors.lastname: Uchida
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D005355:Fibrosis; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D010195:Pancreatitis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D017410:Practice Guidelines as Topic
chemical_list: D007074:Immunoglobulin G
keywords: Autoimmune pancreatitis; IgG4; IgG4-related disease; Mikulicz disease; Regulatory T cell (Treg)