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Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
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Abstract

Background

IgG4-related sclerosing cholangitis (IgG4-SC) needs to be differentiated from pancreatic cancer (PCa), primary sclerosing cholangitis (PSC), and cholangiocarcinoma (CC). We attempted to establish diagnostic criteria for IgG4-SC based on cholangiographic classification by comparison with several diagnostic modalities.

Methods

We classified 62 IgG4-SC patients into three groups on the basis of cholangiographic findings to allow differentiation from PCa, PSC, and CC: Group A IgG4-SC showed features similar to PCa (Type 1, n = 32), Group B showed similarity to PSC (Type 2, n = 15), and Group C showed similarity to CC (Type 3, 4, n = 15). Thirty-five patients with PCa, 40 with PSC, and 32 CC were enrolled as controls. We retrospectively compared the clinical, imaging, serological, and histopathological features and involvement of other organs between Group A and PCa, Group B and PSC, and Group C and CC.

Results

Association with autoimmune pancreatitis (AIP) (P < 0.001) and involvements with other organs (specificity 100%) were common useful diagnostic parameters in all three IgG4-SC groups. A high serum IgG4 level was a useful parameter in Groups A and B (P < 0.001). Discriminant analysis of cholangiograms (P < 0.001), liver biopsy (specificity 100%), and exclusion of inflammatory bowel disease (specificity 100%) were useful parameters in Group B. Intraductal ultrasonography findings (P < 0.001) and exclusion of malignancy by bile duct biopsy (specificity 100%) were useful parameters in Group C. We established diagnostic criteria for IgG4-SC (sensitivity 100%, specificity 96.3%) by incorporating parameters that showed P < 0.001 or 100% specificity.

Conclusions

Diagnostic criteria for IgG4-SC based on cholangiographic classification are useful for distinguishing it from PCa, PSC, and CC.

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Acknowledgments

This study was partly supported by a Grant-in-Aid for Scientific Research from the Ministry of Culture and Science of Japan (23591015, 23790803) and a Grant-in-Aid for the “Research for Intractable Disease” Program from the Ministry of Health, Labor and Welfare of Japan.

Conflict of interest

The authors declare that they have no conflicts of interest.

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Authors and Affiliations

  1. Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, 467-8601, Japan

    Takahiro Nakazawa, Itaru Naitoh, Kazuki Hayashi, Fumihiro Okumura, Katsuyuki Miyabe, Michihiro Yoshida, Hiroaki Yamashita & Takashi Joh

  2. Department of Community-Based Medical Education, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan

    Hirotaka Ohara

Authors
  1. Takahiro Nakazawa
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  2. Itaru Naitoh
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  3. Kazuki Hayashi
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  4. Fumihiro Okumura
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  5. Katsuyuki Miyabe
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  6. Michihiro Yoshida
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  7. Hiroaki Yamashita
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  8. Hirotaka Ohara
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  9. Takashi Joh
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Corresponding author

Correspondence to Takahiro Nakazawa.

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Nakazawa, T., Naitoh, I., Hayashi, K. et al. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol 47, 79–87 (2012). https://doi.org/10.1007/s00535-011-0465-z

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  • DOI: https://doi.org/10.1007/s00535-011-0465-z

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