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World J Gastrointest Endosc. Apr 16, 2026; 18(4): 118485
Published online Apr 16, 2026. doi: 10.4253/wjge.v18.i4.118485
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, 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
Revised: January 29, 2026
Accepted: March 13, 2026
Published online: April 16, 2026
Processing time: 99 Days and 22 Hours
Core Tip
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.