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The 2016 diagnostic criteria for primary sclerosing cholangitis

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

Background

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown cause that is characterized pathologically by an inflammatory and fibrotic process centered on the epithelium, leading to diffuse biliary stenosis and increased wall thickness throughout the intra- and extra-hepatic biliary trees. A nationwide survey in Japan revealed several differences in the clinical aspects of PSC between Japan and Western countries. PSC was recently designated an intractable hepatobiliary disease in Japan. The aim of this study was to establish Japanese diagnostic criteria for PSC according to the current Japanese conditions.

Methods

The Intractable Hepato-Biliary Diseases Study Group in Japan of the Committee of Research on Measures for Intractable Diseases established a working group consisting of researchers specializing in PSC and proposed the 2016 Japanese diagnostic criteria for PSC.

Results

The diagnostic criteria consisted of the major and minor items. Major items are directly related to bile duct changes based on biliary tract imaging and an increased alkaline phosphatase level. Minor items consist of an association with inflammatory bowel disease and liver histology.

Conclusion

The diagnostic criteria for PSC are useful for general physicians in practice.

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Acknowledgements

This study was conducted and supported by Health Labor Science Research Grants from Research on Measures for Intractable Diseases, the Intractable Hepato-Biliary Diseases Study Group in Japan.

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Authors and Affiliations

  1. Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, 2-9 Myoken-cho Showa-ku, Nagoya, 466-8650, Japan

    Takahiro Nakazawa

  2. Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan

    Kenji Notohara

  3. Department of General Internal Medicine, Hiroshima University Hospital and Graduate School of Biomedical and Health Science, Hiroshima, Japan

    Susumu Tazuma

  4. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan

    Atsushi Tanaka & Hajime Takikawa

  5. Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

    Hiroyuki Isayama

  6. Department of Gastroenterology and Nephrology, Graduate School of Medicine, Chiba University, Chiba, Japan

    Toshio Tsuyuguchi

  7. Department of Surgery, Kyorin University School of Medicine, Tokyo, Japan

    Toshiyuki Mori

Authors
  1. Takahiro Nakazawa
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  2. Kenji Notohara
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  3. Susumu Tazuma
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  4. Atsushi Tanaka
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  5. Hiroyuki Isayama
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  6. Toshio Tsuyuguchi
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  7. Toshiyuki Mori
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  8. Hajime Takikawa
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Corresponding author

Correspondence to Takahiro Nakazawa.

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The authors declare that they have no conflict of interest.

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Nakazawa, T., Notohara, K., Tazuma, S. et al. The 2016 diagnostic criteria for primary sclerosing cholangitis. J Gastroenterol 52, 838–844 (2017). https://doi.org/10.1007/s00535-016-1286-x

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  • DOI: https://doi.org/10.1007/s00535-016-1286-x

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