Breach of tolerance: primary biliary cirrhosis.

Authors

Lifeng Wang, Research Center for Biological Therapy, Institute of Translational Hepatology, Beijing 302 Hospital, Beijing, China
Fu-Sheng Wang, Research Center for Biological Therapy, Institute of Translational Hepatology, Beijing 302 Hospital, Beijing, China
Christopher Chang, Department of Pediatrics, Thomas Jefferson University, Nemours/A.I. DuPont Hospital for Children, Wilmington, DE, United StatesFollow
M Eric Gershwin, Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis School of Medicine, Davis, CA, United States

Document Type

Article

Publication Date

8-1-2014

Comments

This article has been peer reviewed. It was published in: Seminars in Liver Disease.

Volume 34, Issue 3, August 2014, Pages 297-317.

The published version is available at DOI: 10.1055/s-0034-1383729

Copyright © 2014 by Thieme Medical Publishers, Inc.

Abstract

In primary biliary cirrhosis (PBC), the breach of tolerance that leads to active disease involves a disruption in several layers of control, including central tolerance, peripheral anergy, a "liver tolerance effect," and the action of T regulatory cells and their related cytokines. Each of these control mechanisms plays a role in preventing an immune response against self, but all of them act in concert to generate effective protection against autoimmunity without compromising the ability of the host immune system to mount an effective response to pathogens. At the same time, genetic susceptibility, environmental factors, including infection agents and xenobiotics, play important roles in breach of tolerance in the development of PBC.

Recommended Citation

Wang, Lifeng; Wang, Fu-Sheng; Chang, Christopher; and Gershwin, M Eric, "Breach of tolerance: primary biliary cirrhosis." (2014). Department of Medical Genetics Faculty Papers. Paper 9.
https://jdc.jefferson.edu/medgenfp/9

PubMed ID

25057953