Primary biliary cirrhosis

Carlo Selmi, Christopher Bowlus, M. Eric Gershwin, Ross L. Coppel

Research output: Contribution to journalArticle

173 Citations (Scopus)

Abstract

Primary biliary cirrhosis is a chronic liver disease characterised by intrahepatic bile-duct destruction, cholestasis, and, in some cases, cirrhosis. Evidence supporting the autoimmune nature of this disorder includes the appearance of highly specific antimitochondrial antibodies (AMAs) and autoreactive T cells. Concordance rates in monozygotic twins, familial prevalence, and genetic associations underscore the importance of genetic factors, whereas findings of epidemiological studies and murine models suggest a possible role for exogenous chemicals and infectious agents through molecular mimicry. The incidence of primary biliary cirrhosis has increased over recent decades, possibly attributable to augmented testing of liver biochemistry rather than a rise in disease incidence. AMAs remain the hallmark of diagnosis in most cases and allow detection of asymptomatic patients. Symptomatic individuals usually present with either pruritus or fatigue and, more rarely, with either jaundice or complications of cirrhosis. The prognosis of primary biliary cirrhosis has improved because of early diagnosis and use of ursodeoxycholic acid, the only established medical treatment for this disorder. Although not a cure, treatment can slow disease progression and delay the need for liver transplantation. However, some patients do not respond adequately to ursodeoxycholic acid and might need alternative therapeutic approaches.

Original languageEnglish (US)
Pages (from-to)1600-1609
Number of pages10
JournalThe Lancet
Volume377
Issue number9777
DOIs
StatePublished - May 7 2011

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Biliary Liver Cirrhosis
Ursodeoxycholic Acid
Fibrosis
Molecular Mimicry
Intrahepatic Bile Ducts
Monozygotic Twins
Antibodies
Cholestasis
Incidence
Pruritus
Jaundice
Biochemistry
Liver Transplantation
Fatigue
Disease Progression
Liver Diseases
Epidemiologic Studies
Early Diagnosis
Chronic Disease
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Primary biliary cirrhosis. / Selmi, Carlo; Bowlus, Christopher; Gershwin, M. Eric; Coppel, Ross L.

In: The Lancet, Vol. 377, No. 9777, 07.05.2011, p. 1600-1609.

Research output: Contribution to journalArticle

Selmi, Carlo ; Bowlus, Christopher ; Gershwin, M. Eric ; Coppel, Ross L. / Primary biliary cirrhosis. In: The Lancet. 2011 ; Vol. 377, No. 9777. pp. 1600-1609.
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