Update on primary biliary cirrhosis

Pietro Invernizzi, Carlo Selmi, M. Eric Gershwin

Research output: Contribution to journalArticlepeer-review


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
Pages (from-to)401-408
Number of pages8
JournalDigestive and Liver Disease
Issue number6
Publication statusPublished - Jun 2010


  • Autoantibodies
  • Autoimmune cholangitis
  • Tolerance breakdown

ASJC Scopus subject areas

  • Gastroenterology
  • Hepatology
  • Medicine(all)


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