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Int. J. Mol. Sci. 2015, 16(3), 6432-6446; doi:10.3390/ijms16036432

Primary Biliary Cirrhosis Is a Generalized Autoimmune Epithelitis

1
Department of Geriatric Gastroenterology, the First Affiliated Hospital of China Medical University, Shenyang 110001, China
2
Storr Liver Centre, Westmead Millennium Institute for Medical Research, the University of Sydney at Westmead Hospital, Westmead, NSW 2145, Australia
*
Author to whom correspondence should be addressed.
Academic Editor: Johannes Haybaeck
Received: 15 December 2014 / Revised: 10 February 2015 / Accepted: 17 February 2015 / Published: 20 March 2015
(This article belongs to the Collection Molecular Mechanisms of Human Liver Diseases)
View Full-Text   |   Download PDF [786 KB, uploaded 20 March 2015]   |  

Abstract

Primary biliary cirrhosis (PBC) is a chronic progressive autoimmune cholestatic liver disease characterized by highly specific antimitochondrial antibodies (AMAs) and the specific immune-mediated injury of small intrahepatic bile ducts. Unique apoptotic feature of biliary epithelial cells (BECs) may contribute to apotope presentation to the immune system, causing unique tissue damage in PBC. Perpetuation of inflammation may result in senescence of BECs, contributing to irreversible loss of bile duct. In addition to the classic liver manifestations, focal inflammation and tissue damage are also seen in salivary glands and urinary tract in a significant proportion of PBC patients. These findings provide potent support to the idea that molecular mimicry may be involved in the breakdown of autoimmune tolerance and mucosal immunity may lead to a systematic epithelitis in PBC patients. Thus, PBC is considered a generalized epithelitis in clinical practice. View Full-Text
Keywords: primary biliary cirrhosis; biliary epithelial cell; salivary gland epithelial cell; uroepithelium; apoptosis; senescence; mucosal immunity; epithelitis; genetics; epigenetics primary biliary cirrhosis; biliary epithelial cell; salivary gland epithelial cell; uroepithelium; apoptosis; senescence; mucosal immunity; epithelitis; genetics; epigenetics
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Gao, J.; Qiao, L.; Wang, B. Primary Biliary Cirrhosis Is a Generalized Autoimmune Epithelitis. Int. J. Mol. Sci. 2015, 16, 6432-6446.

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