Observational Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Aug 21, 2022; 28(31): 4417-4430
Published online Aug 21, 2022. doi: 10.3748/wjg.v28.i31.4417
Prevalence and clinical characteristics of autoimmune liver disease in hospitalized patients with cirrhosis and acute decompensation in China
Zi-Xuan Shen, Dan-Dan Wu, Jie Xia, Xian-Bo Wang, Xin Zheng, Yan Huang, Bei-Ling Li, Zhong-Ji Meng, Yan-Hang Gao, Zhi-Ping Qian, Feng Liu, Xiao-Bo Lu, Jia Shang, Hua-Dong Yan, Yu-Bao Zheng, Wen-Yi Gu, Yan Zhang, Jian-Yi Wei, Wen-Ting Tan, Yi-Xin Hou, Qun Zhang, Yan Xiong, Cong-Cong Zou, Jun Chen, Ze-Bing Huang, Xiu-Hua Jiang, Sen Luo, Yuan-Yuan Chen, Na Gao, Chun-Yan Liu, Wei Yuan, Xue Mei, Jing Li, Tao Li, Xin-Yi Zhou, Guo-Hong Deng, Jin-Jun Chen, Xiong Ma, Hai Li
Zi-Xuan Shen, Dan-Dan Wu, Wen-Yi Gu, Yan Zhang, Jian-Yi Wei, Xiong Ma, Hai Li, Department of Gastroenterology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200127, China
Zi-Xuan Shen, Dan-Dan Wu, Wen-Yi Gu, Yan Zhang, Jian-Yi Wei, Xiong Ma, Hai Li, Shanghai Institute of Digestive Disease, Key Laboratory of Gastroenterology and Hepatology, Chinese Ministry of Health, Shanghai Jiao Tong University, Shanghai 200001, China
Jie Xia, Wen-Ting Tan, Guo-Hong Deng, Department of Infectious Diseases, Southwest Hospital, The Third Military Medical University (Army Medical University), Chongqing 400038, China
Xian-Bo Wang, Yi-Xin Hou, Qun Zhang, Center of Integrative Medicine, Beijing Ditan Hospital, Capital Medical University, Beijing 100102, China
Xin Zheng, Yan Xiong, Cong-Cong Zou, Department of Infectious Diseases, Institute of Infection and Immunology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
Yan Huang, Jun Chen, Ze-Bing Huang, Department of Infectious Diseases, Hunan Key Laboratory of Viral Hepatitis, Xiangya Hospital, Central South University, Changsha 410008, Hunan Province, China
Bei-Ling Li, Jun Chen, Xiu-Hua Jiang, Jin-Jun Chen, Hepatology Unit, Department of Infectious Diseases, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong Province, China
Zhong-Ji Meng, Sen Luo, Yuan-Yuan Chen, Department of Infectious Disease, Taihe Hospital, Hubei University of Medicine, Shiyan 430418, Hubei Province, China
Yan-Hang Gao, Na Gao, Chun-Yan Liu, Department of Hepatology, The First Hospital of Jilin University, Changchun 130031, Jilin Province, China
Zhi-Ping Qian, Wei Yuan, Xue Mei, Department of Liver Intensive Care Unit, Shanghai Public Health Clinical Centre, Fudan University, Shanghai 201508, China
Feng Liu, Tianjin Institute of Hepatology, Nankai University Second People’s Hospital, Tianjin 300102, China
Feng Liu, Jing Li, Tao Li, Department of Infectious Diseases and Hepatology, The Second Hospital of Shandong University, Jinan 250033, Shandong Province, China
Xiao-Bo Lu, Xin-Yi Zhou, Infectious Disease Center, The First Affiliated Hospital of Xinjiang Medical University, Urumqi 830054, Xinjiang, China
Jia Shang, Department of Infectious Diseases, Henan Provincial People’s Hospital, Zhengzhou 450003, Henan Province, China
Hua-Dong Yan, Department of Infectious Diseases, Hwamei Hospital, The Second Hospital of Ningbo, University of Chinese Academy of Sciences, Ningbo 315153, Zhejiang Province, China
Yu-Bao Zheng, Department of Infectious Diseases, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, Guangdong Province, China
Author contributions: Shen ZX, Wu DD, Xia J, Wang XB, Zheng X, Huang Y and Li BL contributed equally to this work; Ma X and Li H contributed equally to this work; Li H generated the concept and designed the research; all authors acquired the data; Shen ZX, Wu DD performed statistical analysis; Shen ZX, Wu DD, Xia J, Wang XB generated the results; Shen ZX, Wu DD, Zheng X, Huang Y and Li BL interpreted the results; Shen ZX and Wu DD drafted the manuscript which was revised by Ma X and Li H for important intellectual content; Ma X made important contribution patient recruitment; Li H was responsible for administrative, technical, or material support and study supervision; and all authors have access to the data, approved this final version of the manuscript and are accountable for all aspects.
Supported by Shanghai Hospital Development Commission, No. SHDC2020CR1037B; the National Key R&D Program of China, No. 2017YFC0908100; the National Science and Technology Major Project, No. 2018ZX10302206, 2018ZX10723203 and 2017ZX10202202; Shanghai Municipal Education Commission-Guofeng Clinical Medicine Grant, No. 20152213; the National Natural Science Foundation of China, No. 82170629, 81930061, 81900579, 81970550, 82070613, 82070650, and 81972265; Chongqing Natural Science Foundation, No. CSTC2019jcyj-zdxmX0004; Beijing Municipal Science & Technology Commission, No. Z191100006619033; Local Innovative and Research Teams Project of Guangdong Pearl River Talents Program, No. 2017BT01S131; the Foundation for Innovative Research Groups of Hubei Provincial Natural Science Foundation, No. 2018CFA031; and Guangdong Basic and Applied Basic Research Foundation, No. 2020A1515010052.
Institutional review board statement: The study was conducted in accordance with the Declaration of Helsinki. The Medical Ethics Board of Shanghai Renji Hospital, Shanghai, China, approved the studies (ethics codes: [2014]148k and [2016]142k).
Informed consent statement: Informed consent was obtained from all patients for being included in the study.
Conflict-of-interest statement: All authors declare that they have no conflict of interest in this manuscript.
Data sharing statement: Data are available within 1 year after publication upon reasonable request via email: aclf_group@163.com.
STROBE statement: The authors have read the STROBE Statement-checklist of items, and the manuscript was prepared and revised according to the STROBE Statement-checklist of items.
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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Hai Li, MD, Professor, Department of Gastroenterology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, No. 1630 Dongfang Road, Shanghai 200127, China. hai_17@126.com
Received: March 3, 2022
Peer-review started: March 3, 2022
First decision: April 25, 2022
Revised: May 19, 2022
Accepted: July 25, 2022
Article in press: July 25, 2022
Published online: August 21, 2022
Abstract
BACKGROUND

Autoimmune liver disease (AILD) has been considered a relatively uncommon disease in China, epidemiological data for AILD in patients with cirrhosis and acute decompensation (AD) is sparse.

AIM

To investigate the prevalence, outcome and risk factors for AILD in cirrhotic patients complicated with AD in China.

METHODS

We collected data from patients with cirrhosis and AD from two prospective, multicenter cohorts in hepatitis B virus endemic areas. Patients were regularly followed up at the end of 28-d, 90-d and 365-d, or until death or liver transplantation (LT). The primary outcome in this study was 90-d LT-free mortality. Acute-on-chronic liver failure (ACLF) was assessed on admission and during 28-d hospitalization, according to the diagnostic criteria of the European Association for the Study of the Liver (EASL). Risk factors for death were analyzed with logistic regression model.

RESULTS

In patients with cirrhosis and AD, the overall prevalence of AILD was 9.3% (242/2597). Prevalence of ACLF was significantly lower in AILD cases (14%) than those with all etiology groups with cirrhosis and AD (22.8%) (P < 0.001). Among 242 enrolled AILD patients, the prevalence rates of primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and PBC-AIH overlap syndrome (PBC/AIH) were 50.8%, 28.5% and 12.0%, respectively. In ACLF patients, the proportions of PBC, AIH and PBC/AIH were 41.2%, 29.4% and 20.6%. 28-d and 90-d mortality were 43.8% and 80.0% in AILD-related ACLF. The etiology of AILD had no significant impact on 28-d, 90-d or 365-d LT-free mortality in patients with cirrhosis and AD in both univariate and multivariate analysis. Total bilirubin (TB), hepatic encephalopathy (HE) and blood urea nitrogen (BUN) were independent risk factors for 90-d LT-free mortality in multivariate analysis. The development of ACLF during hospitalization only independently correlated to TB and international normalized ratio.

CONCLUSION

AILD was not rare in hospitalized patients with cirrhosis and AD in China, among which PBC was the most common etiology. 90-d LT-free mortality were independently associated with TB, HE and BUN.

Keywords: Prevalence, Autoimmune liver disease, Cirrhosis and acute decompensation, Mortality, Acute-on-chronic liver failure

Core Tip: Autoimmune liver disease (AILD) has been regarded as a relatively rare disease in China. Our study reported that the overall prevalence of AILD was 9.3% hospitalized patients with cirrhosis and acute decompensation, among which primary biliary cirrhosis was the most prevalent type. In AILD patients with cirrhosis and acute decompensation, the etiology types of AILD had no significant effect on short-term mortality, total bilirubin, hepatic encephalopathy (HE) and blood urea nitrogen were independently associated with 90-d mortality in multivariate analysis. Strategies are needed to prevent presence of HE and closely monitor the changes of liver and renal function in clinical practice. These data will be a crucial complement to the public epidemiology research on AILD in Asian-Pacific regions.