Update on primary biliary cirrhosis

Pietro Invernizzi, Carlo F Selmi, M. Eric Gershwin

Research output: Contribution to journalArticle

57 Scopus citations

Abstract

Primary biliary cirrhosis is an autoimmune chronic liver disease characterized by progressive bile duct destruction eventually leading to cirrhosis, liver failure, and death. The autoimmune pathogenesis is supported by a plethora of experimental and clinical data, such as the presence of autoreactive T cells and serum autoantibodies. The aetiology remains unknown, although evidence suggests a role for both genetic susceptibility and environmental factors that remain to be determined. In fact, a number of chemicals and infectious agents have been proposed to induce the disease in predisposed individuals. The recent availability of several murine models will significantly help in understanding pathophysiology mechanisms. In this review, we critically summarize the most recent data on the aetiopathogenesis of primary biliary cirrhosis, discuss the latest theories and developments, and suggest directions for future research.

Original languageEnglish (US)
Pages (from-to)401-408
Number of pages8
JournalDigestive and Liver Disease
Volume42
Issue number6
DOIs
StatePublished - Jun 2010

Keywords

  • Autoantibodies
  • Autoimmune cholangitis
  • Tolerance breakdown

ASJC Scopus subject areas

  • Gastroenterology
  • Hepatology
  • Medicine(all)

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