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Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012

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Journal of Hepato-Biliary-Pancreatic Sciences

Abstract

Background

IgG4-sclerosing cholangitis (IgG4-SC) patients have an increased level of serum IgG4, dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall, and a good response to steroid therapy. However, it is not easy to distinguish IgG4-SC from primary sclerosing cholangitis, pancreatic cancer, and cholangiocarcinoma on the basis of cholangiographic findings alone because various cholangiographic features of IgG4-SC are similar to those of the above progressive or malignant diseases.

Methods

The Research Committee of IgG4-related Diseases and the Research Committee of Intractable Diseases of Liver and Biliary Tract in association with the Ministry of Health, Labor and Welfare, Japan and the Japan Biliary Association have set up a working group consisting of researchers specializing in IgG4-SC, and established the new clinical diagnostic criteria of IgG4-SC 2012.

Results

The diagnosis of IgG4-SC is based on the combination of the following 4 criteria: (1) characteristic biliary imaging findings, (2) elevation of serum IgG4 concentrations, (3) the coexistence of IgG4-related diseases except those of the biliary tract, and (4) characteristic histopathological features. Furthermore, the effectiveness of steroid therapy is an optional extra diagnostic criterion to confirm accurate diagnosis of IgG4-SC.

Conclusion

These diagnostic criteria for IgG4-SC are useful in practice for general physicians and other nonspecialists.

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Author information

Authors and Affiliations

  1. Department of Community-based Medical Education, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, 467-8601, Japan

    Hirotaka Ohara

  2. The Third Department of Internal Medicine, Kansai Medical University, Hirakata, Japan

    Kazuichi Okazaki & Kazushige Uchida

  3. Digestive and Lifestyle Diseases, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan

    Hirohito Tsubouchi

  4. Department of Internal Medicine, Second Teaching Hospital, Fujita Health University, Nagoya, Japan

    Kazuo Inui

  5. Center for Health, Safety and Environmental Management, Shinshu University, Matsumoto, Japan

    Shigeyuki Kawa

  6. Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo, Japan

    Terumi Kamisawa

  7. Department of General Medicine, Hiroshima University Graduate School of Medical Science, Programs of Applied Medicine, Clinical Pharmacotherapy, Hiroshima, Japan

    Susumu Tazuma

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

    Kenji Hirano

  9. Division of Gastroenterology, Department of Medicine, Showa University School of Medicine, Tokyo, Japan

    Hitoshi Yoshida

  10. Department of Gastroenterology, Tokyo Women’s Medical University Yachiyo Medical Center, Tokyo, Japan

    Takayoshi Nishino

  11. Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Shigeru B. H. Ko

  12. Department of Gastroenterology, Aichi Cancer Center Hospital, Nagoya, Japan

    Nobumasa Mizuno

  13. Division of Medical Informatics, Department of Internal Medicine, Gastroenterology, Shinshu University Hospital, Matsumoto, Japan

    Hideaki Hamano

  14. Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan

    Atsushi Kanno

  15. Department of Pathology, Kurashiki Central Hospital, Kurashiki, Japan

    Kenji Notohara

  16. Department of Gastroenterology, Nagano Municipal Hospital, Nagano, Japan

    Osamu Hasebe

  17. Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan

    Takahiro Nakazawa

  18. Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Yasuni Nakanuma

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

    Hajime Takikawa

Authors
  1. Hirotaka Ohara
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  2. Kazuichi Okazaki
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  17. Osamu Hasebe
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  18. Takahiro Nakazawa
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  19. Yasuni Nakanuma
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  20. Hajime Takikawa
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Corresponding author

Correspondence to Hirotaka Ohara.

Additional information

This article is a secondary publication based on a study first reported in the JBA (Journal of Japan Biliary Association), 2012;26:59–63.

Appendix: members of the working group for the clinical diagnostic criteria of IgG4-SC

Appendix: members of the working group for the clinical diagnostic criteria of IgG4-SC

The Research Committee of IgG4-related Diseases in association with the Ministry of Health, Labor, and Welfare of Japan (Chairman, Kazuichi Okazaki): K. Okazaki, K. Inui, S. Kawa, T. Kamisawa, S. Tazuma, K. Uchida, K. Hirano, H. Yoshida, T. Nishino, S.B.H. Ko, N. Mizuno, H. Hamano, A. Kanno, K. Notohara, O. Hasebe, T. Nakazawa, and H. Ohara.

The Research Committee of Intractable Diseases of Liver and Biliary Tract in association with the Ministry of Health, Labor, and Welfare of Japan (Chairman, Hirohito Tsubouchi): H. Tsubouchi, S. Tazuma, Y. Nakanuma, and H. Takikawa.

The Japan Biliary Association (Chairman, Kazuo Inui): K. Inui.

This work was supported partially by the Research Program of Intractable Disease provided by the Ministry of Health, Labor, and Welfare of Japan.

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Ohara, H., Okazaki, K., Tsubouchi, H. et al. Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012. J Hepatobiliary Pancreat Sci 19, 536–542 (2012). https://doi.org/10.1007/s00534-012-0521-y

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  • DOI: https://doi.org/10.1007/s00534-012-0521-y

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