Primary biliary cirrhosis

Douglas L. Nguyen, Brian D. Juran, Konstantinos N. Lazaridis

Research output: Contribution to journalArticle

27 Scopus citations

Abstract

Primary biliary cirrhosis (PBC) is an idiopathic chronic autoimmune liver disease that primarily affects women. It is believed that the aetiology for PBC is a combination between environmental triggers in genetically vulnerable persons. The diagnosis for PBC is made when two of the three criteria are fulfilled and they are: (1) biochemical evidence of cholestatic liver disease for at least 6 month's duration; (2) anti-mitochondrial antibody (AMA) positivity; and (3) histologic features of PBC on liver biopsy. Ursodeoxycholic acid (UDCA) is the only FDA-approved medical treatment for PBC and should be administered at a recommended dose of 13-15 mg/kg/day. Unfortunately despite adequate dosing of UDCA, approximately one-third of patients does not respond adequately and may require liver transplantation. Future studies are necessary to elucidate the role of environmental exposures and overall genetic impact not only in the development of PBC, but on disease progression and variable clinical response to therapy.

Original languageEnglish (US)
Pages (from-to)647-654
Number of pages8
JournalBest Practice and Research: Clinical Gastroenterology
Volume24
Issue number5
DOIs
StatePublished - Oct 2010

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Keywords

  • Bile ducts
  • Cholestasis
  • Genetics
  • Natural history

ASJC Scopus subject areas

  • Gastroenterology

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