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Int J Mol Sci. 2015 Mar 20;16(3):6432-46. doi: 10.3390/ijms16036432.

Primary biliary cirrhosis is a generalized autoimmune epithelitis.

Author information

1
Department of Geriatric Gastroenterology, the First Affiliated Hospital of China Medical University, Shenyang 110001, China. gaojunmxr@163.com.
2
Storr Liver Centre, Westmead Millennium Institute for Medical Research, the University of Sydney at Westmead Hospital, Westmead, NSW 2145, Australia. liang.qiao@sydney.edu.au.
3
Department of Geriatric Gastroenterology, the First Affiliated Hospital of China Medical University, Shenyang 110001, China. wangby0908@163.com.

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.

PMID:
25803105
PMCID:
PMC4394541
DOI:
10.3390/ijms16036432
[Indexed for MEDLINE]
Free PMC Article

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