Retrospective Study
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 21, 2018; 24(3): 387-396
Published online Jan 21, 2018. doi: 10.3748/wjg.v24.i3.387
Autoimmune liver disease-related autoantibodies in patients with biliary atresia
Shu-Yin Pang, Yu-Mei Dai, Rui-Zhong Zhang, Yi-Hao Chen, Xiao-Fang Peng, Jie Fu, Zheng-Rong Chen, Yun-Feng Liu, Li-Yuan Yang, Zhe Wen, Jia-Kang Yu, Hai-Ying Liu
Shu-Yin Pang, Yu-Mei Dai, Yi-Hao Chen, Yun-Feng Liu, Li-Yuan Yang, Hai-Ying Liu, Clinical Laboratory, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou 510623, Guangdong Province, China
Rui-Zhong Zhang, Xiao-Fang Peng, Jie Fu, Guangzhou Institute of Pediatrics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou 510623, Guangdong Province, China
Zheng-Rong Chen, Department of Pathology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou 510623, Guangdong Province, China
Zhe Wen, Jia-Kang Yu, Department of Neonatal Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou 510623, Guangdong Province, China
Author contributions: Pang SY, Chen YH, Peng XF and Fu J performed the majority of experiments; Chen ZR reviewed the liver sections; Liu YF and Yang LY analyzed the data and contributed to editing of the manuscript; Wen Z and Yu JK collected all the clinical information; Pang SY, Dai YM, Zhang RZ and Liu HY designed the study and wrote the manuscript.
Supported by the Guangdong Provincial Science and Technology Planning Project, No. 2014A020212520; and the Guangzhou Science and Technology Project, No. 201707010014.
Institutional review board statement: The study protocol was approved by the Ethics Committee of Guangzhou Women and Children’s Medical Center, No. 2015090117.
Informed consent statement: Informed consent was obtained from the legal guardian of all patients prior to study enrolment.
Conflict-of-interest statement: The authors declare no conflict of interests related to this study.
Data sharing statement: No additional data are available.
Open-Access: 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/
Correspondence to: Hai-Ying Liu, PhD, Chief Technician, Clinical Laboratory, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, No. 9, Jinsui Road, Guangzhou 510623, Guangdong Province, China. xiangliuhaiying@aliyun.com
Telephone: +86-20-38076255 Fax: +86-20-38076255
Received: November 9, 2017
Peer-review started: November 9, 2017
First decision: November 30, 2017
Revised: December 14, 2017
Accepted: December 20, 2017
Article in press: December 20, 2017
Published online: January 21, 2018
Processing time: 71 Days and 5 Hours
ARTICLE HIGHLIGHTS
Research background

Accumulating evidence supports the biliary atresia (BA) pathogenesis theory of a primary perinatal viral trigger that is followed by an aberrant autoimmune-mediated attack on bile duct epithelia, resulting in inflammatory and fibrosing obstruction of the bile ducts. Similarly, both primary biliary cholangitis and autoimmune hepatitis are characterized by autoimmune-mediated injury to liver cells and biliary ducts, and autoimmune liver disease (ALD)-related autoantibodies play a crucial role in the accurate classification for such. The authors hypothesized that ALD-related autoantibodies would be also associated with BA.

Research motivation

No previous study has comprehensively evaluated the prevalence of ALD-related autoantibodies and their roles in prognosis of BA. The authors attempted to explore the ALD-related autoantibodies in BA sera.

Research objectives

The authors tried to search appropriate non-invasive biomarker for diagnosis and prognosis of BA.

Research methods

The authors collected the sera of BA children and evaluated the prevalence of ALD-related autoantibodies using multiple methods, including the autoimmune liver diseases profile and specific anti-nuclear antibodies (ANAs) by line-blot assay; ANA and anti-neutrophil cytoplasmic antibody (ANCA) by indirect immunofluorescence assay; specific ANCAs and anti-M2-3E by enzyme linked immunosorbent assay. Simultaneously, associations of these autoantibodies with the clinical features of followed BA children were evaluated by Spearman’s correlation coefficient.

Research results

The overall positivity of serum ALD-related autoantibodies in BA patients was 56.5%. The prevalence of anti-M2-3E, ANA, and ANCA was significantly increased in a large cohort of infants with BA. In addition, the authors found that the ANCA showed a positive correlation to the occurrence of postoperative cholangitis.

Research conclusions

High prevalence of ALD-related autoantibodies in the BA developmental process strongly reveals the autoimmune-mediated pathogenesis. ANCA positivity may be a useful serum biomarker to predict postoperative cholangitis of BA.

Research perspectives

Our future research will focus on identifying potentially pathogenic, bile duct-specific autoantibodies and the titer and source of autoantibodies in BA.