Primary biliary cirrhosis

Lancet. 2015 Oct 17;386(10003):1565-75. doi: 10.1016/S0140-6736(15)00154-3. Epub 2015 Sep 11.


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.

Publication types

  • Review

MeSH terms

  • Avitaminosis / complications
  • Bone Diseases, Metabolic / complications
  • Carcinoma, Hepatocellular / complications
  • Fatigue / etiology
  • Fatigue / therapy
  • Humans
  • Hyperlipidemias / complications
  • Hypertension, Portal / complications
  • Liver Cirrhosis, Biliary* / complications
  • Liver Cirrhosis, Biliary* / diagnosis
  • Liver Cirrhosis, Biliary* / drug therapy
  • Liver Neoplasms / complications
  • Prognosis
  • Pruritus / drug therapy
  • Pruritus / etiology
  • Sjogren's Syndrome / complications