Primary biliary cirrhosis

Elizabeth J. Carey, Ahmad H. Ali, Keith Lindor

Research output: Contribution to journalArticle

179 Citations (Scopus)

Abstract

Primary biliary cirrhosis is a chronic cholestatic liver disease characterised by destruction of small intrahepatic bile ducts, leading to fibrosis and potential cirrhosis through resulting complications. The serological hallmark of primary biliary cirrhosis is the antimitochondrial antibody, a highly disease-specific antibody identified in about 95% of patients with primary biliary cirrhosis. These patients usually have fatigue and pruritus, both of which occur independently of disease severity. The typical course of primary biliary cirrhosis has changed substantially with the introduöction of ursodeoxycholic acid (UDCA). Several randomised placebo-controlled studies have shown that UDCA improves transplant-free survival in primary biliary cirrhosis. However, about 40% of patients do not have a biochemical response to UDCA and would benefit from new therapies. Liver transplantation is a life-saving surgery with excellent outcomes for those with decompensated cirrhosis. Meanwhile, research on nuclear receptor hormones has led to the development of exciting new potential treatments. This Seminar will review the current understanding of the epidemiology, pathogenesis, and natural history of primary biliary cirrhosis, discuss management of the disease and its sequelae, and introduce research on new therapeutic options.

Original languageEnglish (US)
Pages (from-to)1565-1575
Number of pages11
JournalThe Lancet
Volume386
Issue number10003
DOIs
StatePublished - Oct 17 2015

Fingerprint

Biliary Liver Cirrhosis
Ursodeoxycholic Acid
Fibrosis
Intrahepatic Bile Ducts
Antibodies
Pruritus
Cytoplasmic and Nuclear Receptors
Disease Management
Research
Liver Transplantation
Fatigue
Liver Diseases
Epidemiology
Therapeutics
Placebos
Transplants
Survival

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Primary biliary cirrhosis. / Carey, Elizabeth J.; Ali, Ahmad H.; Lindor, Keith.

In: The Lancet, Vol. 386, No. 10003, 17.10.2015, p. 1565-1575.

Research output: Contribution to journalArticle

Carey, EJ, Ali, AH & Lindor, K 2015, 'Primary biliary cirrhosis', The Lancet, vol. 386, no. 10003, pp. 1565-1575. https://doi.org/10.1016/S0140-6736(15)00154-3
Carey, Elizabeth J. ; Ali, Ahmad H. ; Lindor, Keith. / Primary biliary cirrhosis. In: The Lancet. 2015 ; Vol. 386, No. 10003. pp. 1565-1575.
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