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Peer-reviewedCC BY-NC-NDInternal Medicine

A Case of Pancreaticobiliary Maljunction Associated with IgG4-related Sclerosing Cholangitis and Type 1 Autoimmune Pancreatitis

  • Tadashi Toyohara
  • Itaru Naitoh
  • Go Asano
  • Naruomi Jinno
  • Michihiro Yoshida
  • Yasuki Hori
  • Akihisa Kato
  • Kenta Kachi
  • Kayoko Oda
  • Kenta Saito
  • Yoichi Matsuo
  • Kenji Notohara
1 more authors
  • Hiromi Kataoka
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Abstract

We report a case of a 79-year-old woman with pancreaticobiliary maljunction (PBM) associated with IgG4-related sclerosing cholangitis (IgG4-SC) and type 1 autoimmune pancreatitis (AIP). Computed tomography revealed proximal bile duct dilatation and skip lesions in the head and tail of the pancreas. Endoscopic retrograde cholangiopancreatography revealed a long common channel and an intrapancreatic bile duct stricture. We preoperatively diagnosed distal bile duct cancer associated with PBM and AIP based on atypical biopsy findings and subsequently performed pancreaticoduodenectomy. The surgical specimen revealed IgG4-SC and AIP, without evidence of malignancy. To our knowledge, this is the first reported case of PBM associated with IgG4-SC and AIP in the literature.

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Cite

BibTeXRIS
BibTeX
@article{toyohara2026case,
  title = {{A Case of Pancreaticobiliary Maljunction Associated with IgG4-related Sclerosing Cholangitis and Type 1 Autoimmune Pancreatitis}},
  author = {Tadashi Toyohara and Itaru Naitoh and Go Asano and Naruomi Jinno and Michihiro Yoshida and Yasuki Hori and Akihisa Kato and Kenta Kachi and Kayoko Oda and Kenta Saito and Yoichi Matsuo and Kenji Notohara and Hiromi Kataoka},
  journal = {Internal Medicine},
  year = {2026},
  month = aug,
  doi = {10.2169/internalmedicine.7858-26},
  url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7858-26/_article},
  note = {CC BY-NC-ND},
}
RIS
TY  - JOUR
TI  - A Case of Pancreaticobiliary Maljunction Associated with IgG4-related Sclerosing Cholangitis and Type 1 Autoimmune Pancreatitis
AU  - Tadashi Toyohara
AU  - Itaru Naitoh
AU  - Go Asano
AU  - Naruomi Jinno
AU  - Michihiro Yoshida
AU  - Yasuki Hori
AU  - Akihisa Kato
AU  - Kenta Kachi
AU  - Kayoko Oda
AU  - Kenta Saito
AU  - Yoichi Matsuo
AU  - Kenji Notohara
AU  - Hiromi Kataoka
PY  - 2026
DA  - 2026/08/19
JO  - Internal Medicine
DO  - 10.2169/internalmedicine.7858-26
UR  - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7858-26/_article
AB  - We report a case of a 79-year-old woman with pancreaticobiliary maljunction (PBM) associated with IgG4-related sclerosing cholangitis (IgG4-SC) and type 1 autoimmune pancreatitis (AIP). Computed tomography revealed proximal bile duct dilatation and skip lesions in the head and tail of the pancreas. Endoscopic retrograde cholangiopancreatography revealed a long common channel and an intrapancreatic bile duct stricture. We preoperatively diagnosed distal bile duct cancer associated with PBM and AIP based on atypical biopsy findings and subsequently performed pancreaticoduodenectomy. The surgical specimen revealed IgG4-SC and AIP, without evidence of malignancy. To our knowledge, this is the first reported case of PBM associated with IgG4-SC and AIP in the literature.
SN  - 1349-7235
SN  - 0918-2918
ER  -