Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 26, 2024; 12(6): 1174-1181
Published online Feb 26, 2024. doi: 10.12998/wjcc.v12.i6.1174
Autoimmune hepatitis-primary biliary cholangitis overlap syndrome complicated by various autoimmune diseases: A case report
Yu-Jie Qin, Ting Gao, Xing-Nian Zhou, Ming-Liang Cheng, Hong Li
Yu-Jie Qin, Xing-Nian Zhou, Clinical Medicine School of Guizhou Medical University, Guiyang 550025, Guizhou Province, China
Ting Gao, Department of Pathology, The Affiliated Hospital of Guizhou Medical University, Guiyang 550025, Guizhou Province, China
Ming-Liang Cheng, Department of Infectious Diseases, Guizhou Medical University, Guiyang 550025, Guizhou Province, China
Hong Li, Department of Infectious Diseases, Guizhou Provincial People's Hospital, Guiyang 550025, Guizhou Province, China
Co-first authors: Yu-Jie Qin and Ting Gao.
Author contributions: Qin YJ and Gao T contributed to manuscript writing and editing, and data collection; Zhou XN and Cheng ML contributed to data analysis; Li H contributed to conceptualization and supervision; all authors have read and approved the final manuscript.
Supported by National Natural Science Foundation of China, No. 82060123; and National Health Commission of Guizhou Province, No. gzwjk2019-1-082.
Informed consent statement: Informed consent was obtained from the patient before the publication of this case report.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read CARE Checklist (2016), and the manuscript was prepared and revised according to CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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:
Corresponding author: Hong Li, MD, Associate Chief Physician, Associate Professor, Department of Infectious Diseases, Guizhou Provincial People's Hospital, No. 83 Zhongshan East Road, Guiyang 550025, Guizhou Province, China.
Received: November 25, 2023
Peer-review started: November 25, 2023
First decision: December 27, 2023
Revised: January 28, 2024
Accepted: February 2, 2024
Article in press: February 2, 2024
Published online: February 26, 2024

Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two common clinical autoimmune liver diseases, and some patients have both diseases; this feature is called AIH-PBC overlap syndrome. Autoimmune thyroid disease (AITD) is the most frequently overlapping extrahepatic autoimmune disease. Immunoglobulin (IgG) 4-related disease is an autoimmune disease recognized in recent years, characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues.


A 68-year-old female patient was admitted with a history of right upper quadrant pain, anorexia, and jaundice on physical examination. Laboratory examination revealed elevated liver enzymes, multiple positive autoantibodies associated with liver and thyroid disease, and imaging and biopsy suggestive of pancreatitis, hepatitis, and PBC. A diagnosis was made of a rare and complex overlap syndrome of AIH, PBC, AITD, and IgG4-related disease. Laboratory features improved on treatment with ursodeoxycholic acid, methylprednisolone, and azathioprine.


This case highlights the importance of screening patients with autoimmune diseases for related conditions.

Keywords: Overlap syndrome, Autoimmune hepatitis, Primary biliary cholangitis, Primary sclerosing cholangitis, Autoimmune thyroid disease, Case report

Core Tip: Autoimmune hepatitis-primary biliary cholangitis overlap syndrome is relatively rare in clinical practice. In this report, we describe the case of a patient presenting with autoimmune hepatitis, primary biliary cholangitis, thyroid disease, and autoimmune pancreatitis overlap syndrome. The report underscores the significance of vigilance and routine screening for related autoimmune diseases to prevent missed diagnoses and treatment opportunities, which can significantly impact the disease course and the patient’s quality of life. Additionally, it highlights the importance of tailoring treatment to individual patients to optimize effectiveness and enhance patient adherence.