• Autoimmunity reviews · Apr 2014

    Review

    Diagnosis and classification of autoimmune pancreatitis.

    • Kazuichi Okazaki, Takashi Tomiyama, Toshiyuki Mitsuyama, Kimi Sumimoto, and Kazushige Uchida.
    • The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan. Electronic address: okazaki@hirakata.kmu.ac.jp.
    • Autoimmun Rev. 2014 Apr 1; 13 (4-5): 451-8.

    AbstractRecent 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. Copyright © 2014 Elsevier B.V. All rights reserved.

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