Breach of tolerance: Primary biliary cirrhosis

Lifeng Wang, Fu Sheng Wang, Christopher Chang, M. Eric Gershwin

Research output: Contribution to journalArticle

34 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)297-317
Number of pages21
JournalSeminars in Liver Disease
Volume34
Issue number3
DOIs
StatePublished - 2014

Keywords

  • antimitochondrial antibodies
  • apoptosis
  • autoreactive T cells
  • liver-tolerance effect
  • pyruvate dehydrogenase complex

ASJC Scopus subject areas

  • Hepatology

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