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Japanese Experience

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Autoimmune (IgG4-related) Pancreatitis and Cholangitis

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Abstract

Based on recent studies, the international consensus of diagnostic criteria (ICDC) for autoimmune pancreatitis (AIP) proposed two subtypes of AIP, type 1 related with IgG4 (lymphoplasmacytic sclerosing pancreatitis; LPSP) and type 2 related with a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis; IDCP). The Japanese nationwide study estimated the incidence of AIP at 2.87 cases in 100,000 population (about 2,800 patients in Japan) with type 1 AIP, but only 7 cases of type 2 AIP have been histologically confirmed. The mean age at diagnosis was 62.6 years and the ratio of male to female was 4 to 1. The rates for specific sites of OOI were 52.9 % for sclerosing cholangitis, 13.9 % for sclerosing sialadenitis, 6.6 % for lachrymal gland involvement, and 8.1 % for retroperitoneal fibrosis. For diagnosis, the Japan Pancreas Society (JPS)-2006 and Asian criteria have been available, and most recently the JPS-2011 for type 1 AIP were proposed in response to the ICDC in Japan. Steroids are the first-line therapy for the induction of remission and relapse, and a low-dose maintenance steroid treatment is recommended. Japanese patients with AIP frequently (<6 M,32 %; <1 year,56 %; <3 year,92 %) show relapse of the disease after or during the steroid therapy, but long-term outcomes remain unclear.

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Acknowledgments

This study was partially supported by (1) Grant-in-Aid for Scientific Research (C) of the Ministry of Culture and Science of Japan (23591017, 24591020), (2) Research Program Intractable Diseases, from Minister of Labor and Welfare of Japan, and (3) grants-in-aid from the Ministry of Education, Culture, Sports, Science and Technology of Japan, from CREST Japan Science and Technology Agency.

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Authors and Affiliations

  1. Department of Gastroenterology and Hepatology, Kansai Medical University, 2-3-1, Shinmachi Hirakata, Osaka, Japan, 573-1191

    Kazuichi Okazaki M.D., Ph.D. & Kazushige Uchida M.D., Ph.D.

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  1. Kazuichi Okazaki M.D., Ph.D.
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  2. Kazushige Uchida M.D., Ph.D.
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Correspondence to Kazuichi Okazaki M.D., Ph.D. .

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Editors and Affiliations

  1. and Hepatology, Mayo Clinic Division of Gastroenterology, Rochester, Minnesota, USA

    Michael J. Levy

  2. and Hepatology, Mayo Clinic Division of Gastroenterology, Rochester, Minnesota, USA

    Suresh T. Chari

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Okazaki, K., Uchida, K. (2013). Japanese Experience. In: Levy, M., Chari, S. (eds) Autoimmune (IgG4-related) Pancreatitis and Cholangitis. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6430-4_22

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  • DOI: https://doi.org/10.1007/978-1-4419-6430-4_22

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