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Case Report
A challenging case report of IgG4-related sclerosing cholangitis with literature review
YANG Duo  LI Lixin  WANG Xiaotong  JIANG Yingying  BAI Yuping 

DOI:10.12015/issn.1674-8034.2026.02.020.


[Keywords] IgG4;IgG4-related sclerosing cholangitis;cholangiocarcinoma;mass-forming IgG4-related sclerosing cholangitis;magnetic resonance imaging

YANG Duo1, 2   LI Lixin1, 2   WANG Xiaotong1   JIANG Yingying3   BAI Yuping1, 2*  

1 Second Clinical School, Lanzhou University, Lanzhou 730030, China

2 Department of Magnetic Resonance, Lanzhou University Second Hospital, Lanzhou 730030, China

3 Gansu University of Chinese Medicine, Lanzhou 730030, China

Corresponding author: BAI Y P, E-mail: 309131762@qq.com

Conflicts of interest   None.

Received  2025-11-29
Accepted  2026-01-30
DOI: 10.12015/issn.1674-8034.2026.02.020
DOI:10.12015/issn.1674-8034.2026.02.020.

[1]
HERTA T, SCHRÖDER M, GEISEL D, et al. Management of IgG4-related cholangitis: diagnosis, therapy, and long-term surveillance[J/OL]. Gastroenterol Rep, 2025, 13: goaf032 [2025-11-28]. https://pubmed.ncbi.nlm.nih.gov/40191403/. DOI: 10.1093/gastro/goaf032.
[2]
BEUERS U, TRAMPERT D C. IgG4-related cholangitis[J]. Semin Liver Dis, 2025, 45(3): 381-396. DOI: 10.1055/a-2588-3875.
[3]
GHAZALE A, CHARI S T, ZHANG L Z, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy[J]. Gastroenterology, 2008, 134(3): 706-715. DOI: 10.1053/j.gastro.2007.12.009.
[4]
ZEN Y, FUJII T, SATO Y, et al. Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4-related disease[J]. Mod Pathol, 2007, 20(8): 884-894. DOI: 10.1038/modpathol.3800836.
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GHUMAN S S, BUXI T B S, JAIN K, et al. Imaging of benign biliary tract disease[J]. Indian J Radiol Imaging, 2024, 34(4): 726-739. DOI: 10.1055/s-0044-1786038.
[6]
TANAKA A, TAZUMA S, OKAZAKI K, et al. Nationwide survey for primary sclerosing cholangitis and IgG4-related sclerosing cholangitis in Japan[J]. J Hepatobiliary Pancreat Sci, 2014, 21(1): 43-50. DOI: 10.1002/jhbp.50.
[7]
FARRUKH L, AKHTAR M F, WAQAR H H, et al. Significant CA 19-9 elevation in IgG4-related autoimmune pancreatitis - A diagnostic dilemma[J]. Am J Med Sci, 2024, 367(1): 67-71. DOI: 10.1016/j.amjms.2023.09.016.
[8]
WANG N P, ZHU P, XIANG Y, et al. IgG4-related autoimmune pancreatitis and sclerosing cholangitis: A case report and literature review[J/OL]. Medicine, 2024, 103(17): e37922 [2025-11-28]. https://pubmed.ncbi.nlm.nih.gov/38669380/. DOI: 10.1097/MD.0000000000037922.
[9]
DOHERTY B, NAMBUDIRI V E, PALMER W C. Update on the diagnosis and treatment of cholangiocarcinoma[J/OL]. Curr Gastroenterol Rep, 2017, 19(1): 2 [2025-11-28]. https://pubmed.ncbi.nlm.nih.gov/28110453/. DOI: 10.1007/s11894-017-0542-4.
[10]
DRAZILOVA S, VESELINY E, LENARTOVA P D, et al. IgG4-related sclerosing cholangitis: rarely diagnosed, but not a rare disease[J/OL]. Can J Gastroenterol Hepatol, 2021, 2021: 1959832 [2025-11-28]. https://pubmed.ncbi.nlm.nih.gov/34970512/. DOI: 10.1155/2021/1959832.
[11]
MADHUSUDHAN K S, DAS P, GUNJAN D, et al. IgG4-related sclerosing cholangitis: a clinical and imaging review[J]. AJR Am J Roentgenol, 2019, 213(6): 1221-1231. DOI: 10.2214/AJR.19.21519.
[12]
EUROPEAN ASSOCIATION FOR THE STUDY OF THE LIVER. EASL Clinical Practice Guidelines on the management of extrahepatic cholangiocarcinoma[J]. J Hepatol, 2025, 83(1): 211-238. DOI: 10.1016/j.jhep.2025.03.007.
[13]
KAMISAWA T, NAKAZAWA T, TAZUMA S, et al. Clinical practice guidelines for IgG4-related sclerosing cholangitis[J]. J Hepatobiliary Pancreat Sci, 2019, 26(1): 9-42. DOI: 10.1002/jhbp.596.

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