Xia Y, Zhu JD, Su T, Shi L, Zhao SL, Xu HW, Xu CQ. Immunoglobulin G4-related sclerosing cholangitis: A case report. World J Gastrointest Endosc 2026; 18(4): 118485 [DOI: 10.4253/wjge.v18.i4.118485]
Corresponding Author of This Article
Chang-Qin Xu, Department of Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324 Jingwu Weiqi Road, Jinan 250000, Shandong Province, China. 64791275@qq.com
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Gastroenterology & Hepatology
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Case Report
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This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Apr 16, 2026 (publication date) through Apr 16, 2026
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World Journal of Gastrointestinal Endoscopy
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1948-5190
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Xia Y, Zhu JD, Su T, Shi L, Zhao SL, Xu HW, Xu CQ. Immunoglobulin G4-related sclerosing cholangitis: A case report. World J Gastrointest Endosc 2026; 18(4): 118485 [DOI: 10.4253/wjge.v18.i4.118485]
World J Gastrointest Endosc. Apr 16, 2026; 18(4): 118485 Published online Apr 16, 2026. doi: 10.4253/wjge.v18.i4.118485
Immunoglobulin G4-related sclerosing cholangitis: A case report
Yu Xia, Jun-Da Zhu, Tong Su, Lei Shi, Shu-Lei Zhao, Hong-Wei Xu, Chang-Qin Xu
Yu Xia, Jun-Da Zhu, Tong Su, Lei Shi, Shu-Lei Zhao, Hong-Wei Xu, Chang-Qin Xu, Department of Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250000, Shandong Province, China
Author contributions: Xia Y contributed to manuscript writing and editing, and data collection; Zhu JD and Su T contributed to data analysis; Shi L, Zhao SL, and Xu HW contributed to revising the manuscript as well as providing professional technical consultation; Xu CQ contributed to conceptualization and supervision; all authors have read and approved the final manuscript.
Supported by the Young Elite Sponsorship Program of Shandong Provincial Medical Association, No. 2024-GJ-0131.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Chang-Qin Xu, Department of Gastroenterology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, No. 324 Jingwu Weiqi Road, Jinan 250000, Shandong Province, China. 64791275@qq.com
Received: January 4, 2026 Revised: January 29, 2026 Accepted: March 13, 2026 Published online: April 16, 2026 Processing time: 99 Days and 22 Hours
Abstract
BACKGROUND
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a rare autoimmune disorder commonly associated with autoimmune pancreatitis. Because its clinical manifestations are nonspecific, the condition is frequently misdiagnosed as primary sclerosing cholangitis or cholangiocarcinoma. Early recognition and timely management are essential for improving patient outcomes.
CASE SUMMARY
An 88-year-old male presented with jaundice, epigastric pain, and biochemical evidence of cholestasis. Laboratory tests revealed markedly elevated serum IgG4 (2460 mg/dL), and imaging studies demonstrated bile duct wall thickening with multiple strictures. Histopathological examination showed minimal fibrosis accompanied by chronic inflammatory infiltration. After excluding malignancy, a diagnosis of isolated IgG4-SC was established. Steroid therapy with prednisone (40 mg/day) led to significant improvement in symptoms and biochemical markers. Follow-up imaging confirmed regression of the biliary strictures.
CONCLUSION
Isolated IgG4-SC mimics primary sclerosing cholangitis or cholangiocarcinoma, making early, accurate differentiation crucial to guide therapy and avoid unnecessary surgery.
Core Tip: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a relatively rare autoimmune disease that typically presents with bile duct stenosis and cholestasis. It is frequently associated with autoimmune pancreatitis; however, a small subset of patients develop IgG4-SC without any extra-biliary involvement from IgG4-related disease. This form, known as isolated IgG4-SC, is particularly challenging to diagnose. This report describes a case of isolated IgG4-SC and suggests that clinicians begin considering this condition in patients who present with unexplained biliary strictures and cholestasis, especially when serum IgG4 levels are elevated.
