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An atypical case of isolated immunoglobulin G4-related sclerosing cholangitis with a cholangiogram resembling primary sclerosing cholangitis

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Abstract

An asymptomatic 77-year-old man with intrahepatic bile duct dilation was referred to our hospital. Cholangiography revealed alternations between strictures and dilated segments from the right and left hepatic ducts to the lower bile ducts, with findings of a pruned tree, beaded, shaggy appearance, and diverticulum-like outpouching. Histopathology revealed abundant immunoglobulin G4 (IgG4)-positive plasma cells (> 10 per high-power field) with an IgG4/IgG-positive cell ratio of 40–50%. After 2 weeks of steroid therapy, the cholangiography markedly improved. Because the cholangiographic findings resembled those of primary sclerosing cholangitis, steroid therapy proved useful in differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.

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Authors and Affiliations

  1. Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine, 65, Tsuruma-Cho, Showa-Ku, Nagoya, 466-8560, Japan

    Yoshihisa Takada, Takuya Ishikawa, Yasuyuki Mizutani, Tadashi Iida, Kota Uetsuki & Hiroki Kawashima

  2. Department of Endoscopy, Nagoya University Hospital, Nagoya, Japan

    Kentaro Yamao

Authors
  1. Yoshihisa Takada
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  2. Takuya Ishikawa
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  4. Yasuyuki Mizutani
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  7. Hiroki Kawashima
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Correspondence to Takuya Ishikawa.

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Takada, Y., Ishikawa, T., Yamao, K. et al. An atypical case of isolated immunoglobulin G4-related sclerosing cholangitis with a cholangiogram resembling primary sclerosing cholangitis. Clin J Gastroenterol 17, 338–344 (2024). https://doi.org/10.1007/s12328-023-01903-w

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  • DOI: https://doi.org/10.1007/s12328-023-01903-w

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