Primary biliary cirrhosis

Jayant A. Talwalkar, Keith Lindor

Research output: Contribution to journalArticle

271 Citations (Scopus)

Abstract

Primary biliary cirrhosis is a chronic cholestatic liver disease of adults. This disorder is characterised histologically by chronic non-suppurative destruction of interlobular bile ducts leading to advanced fibrosis, cirrhosis, and liver failure. The precise aetiopathogenesis of primary biliary cirrhosis remains unknown, although dysregulation of the immune system and genetic susceptibility both seem to be important. Affected patients are typically middle-aged women with abnormal serum concentrations of alkaline phosphatase. Presence of antimitochondrial antibody in serum is almost diagnostic of the disorder. Identification of primary biliary cirrhosis is important, because effective treatment with ursodeoxycholic acid has been shown to halt disease progression and improve survival without need for liver transplantation. However, therapeutic options for disease-related complications - including fatigue and metabolic bone disease - remain unavailable. Mathematical models have been developed that accurately predict the natural history of primary biliary cirrhosis in individuals. Despite advances in understanding of the disease, it remains one of the major indications for liver transplantation worldwide.

Original languageEnglish (US)
Pages (from-to)53-61
Number of pages9
JournalLancet
Volume362
Issue number9377
DOIs
StatePublished - Jul 5 2003
Externally publishedYes

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Biliary Liver Cirrhosis
Liver Transplantation
Fibrosis
Ursodeoxycholic Acid
Metabolic Bone Diseases
Liver Failure
Genetic Predisposition to Disease
Bile Ducts
Serum
Fatigue
Alkaline Phosphatase
Disease Progression
Liver Diseases
Immune System
Theoretical Models
Survival
Antibodies
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Primary biliary cirrhosis. / Talwalkar, Jayant A.; Lindor, Keith.

In: Lancet, Vol. 362, No. 9377, 05.07.2003, p. 53-61.

Research output: Contribution to journalArticle

Talwalkar, Jayant A. ; Lindor, Keith. / Primary biliary cirrhosis. In: Lancet. 2003 ; Vol. 362, No. 9377. pp. 53-61.
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