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World J Hepatol. Apr 27, 2026; 18(4): 117993
Published online Apr 27, 2026. doi: 10.4254/wjh.v18.i4.117993
Published online Apr 27, 2026. doi: 10.4254/wjh.v18.i4.117993
Tumor necrosis factor receptor superfamily member 13B mutation-associated chronic cholangitis with cirrhosis: A case report and review of literature
Ying Zhou, Qi-Jiao Cheng, Ming-Xing Liang, Su-Qun Li, Yu-Zhi Su, Yu-Xin Xie, Ying-Hua Chen, Yi-Huai He, Department of Infectious Diseases, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China
Yong-Ming Cai, Department of Infectious Diseases, People’s Hospital of Renhuai City, Zunyi 563000, Guizhou Province, China
Zong-Qin Pan, Department of Infectious Diseases, People’s Hospital Qiandongnan Miao and Dong Autonomous Prefecture, Kaili 556000, Guizhou Province, China
Co-first authors: Ying Zhou and Qi-Jiao Cheng.
Co-corresponding authors: Ying-Hua Chen and Yi-Huai He.
Author contributions: Zhou Y and Cheng QJ contributed equally to this work; Zhou Y, Cheng QJ, Liang MX, Li SQ, Su YZ, Cai YM, and Xie YX collected medical history; Zhou Y, Cheng QJ, and Pan ZQ summarized and analyzed the medical history data; He YH, Chen YH, and Zhou Y conceived and designed the content of the article; Zhou Y and Cheng QJ wrote the initial paper; He YH, Chen YH, Cai YM, Pan ZQ, and Xie YX revised the paper; He YH had primary responsibility for the final content; and all authors read and approved the final manuscript.
Supported by Science and Technology Planning Projects of Guizhou Province, No. QKHJC-MS(2025)384 and No. QKHJC-MS(2025)392; Health Research Project of Guizhou Province, No. gzwkj2024-103 and No. gzwkj2024-324; Scientific Research Project of the Guizhou Provincial Bureau of Traditional Chinese Medicine, No. QZYY-2023-021; and Beijing Liver and Gallbladder Mutual Aid Public Welfare Foundation Artificial Liver Special Fund, No. iGandanF-1082024-RGG018.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
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: Yi-Huai He, MD, Department of Infectious Diseases, Affiliated Hospital of Zunyi Medical University, No. 201 Dalian Street, Zunyi 563000, Guizhou Province, China. 993565989@qq.com
Received: December 22, 2025
Revised: January 19, 2026
Accepted: February 13, 2026
Published online: April 27, 2026
Processing time: 122 Days and 3.4 Hours
Revised: January 19, 2026
Accepted: February 13, 2026
Published online: April 27, 2026
Processing time: 122 Days and 3.4 Hours
Core Tip
Core Tip: Tumor necrosis factor receptor superfamily member 13B (TNFRSF13B) maintains immune homeostasis through bidirectional regulation of B cell function. This case report presents a case where a TNFRSF13B mutation that may impair TACI’s negative regulatory control over B cell activity, causing B cell hyperactivation, sustained local inflammatory responses, and chronic cholangitis progressing to cirrhosis. This finding provides a novel immunogenetic perspective for understanding certain autoimmune liver diseases of unknown etiology.