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HepatologyBiliary tract

Primary biliary cirrhosis

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Please submit a new question (maximum 100 words) to Please note that this service is only for medically qualified professionals, and not for the general public. The questions should ask a technical question about a topic in gastroenterology, hepatology or endsocopy. You may nominate the expert from our Global Academic Faculty whom you would like us to contact for the first reply. Don't forget to include your full name, city, country and your email address.

Does hypercholestrolemia in patients with primary biliary cirrhosis confer the same cardiovascular risk as in non-PBC patients? If so what is the preferred statin drug?
Section Editor: Andrew Burroughs  13 October 2003

Hyperlipidemia occurs in the majority of patients with primary biliary cirrhosis. It is not uncommon for the diagnosis to be made as a consequence cholesterol screening tests.

Although serum cholesterol and triglycerides are both raised there is no evidence that these abnormalities are associated with an increased cardiovascular risk. One explanation for this paradox is that elevated HDL levels and reduced levels of lipoprotein A, both of which are characteristic of early stages of the disease, protect against atherosclerosis. Whereas reduced cholesterol synthesis and absorption in advanced PBC outweigh the effects of reduced biliary secretion.

An alternative explanation is that most cholesterol in PBC is localized within lipoprotein X, a unique lipoprotein associated with obstructive jaundice which does not confer the same cardiovascular risk as LDL.

The question then arises is it worth treating hyperlipidemia in PBC? Ursodeoxycholic acid and HMG CO-A reductase inhibitors are both well-tolerated in PBC and reduce serum cholesterol levels, but there are no data to show this leads to a reduction in cardiovascular risk. However, the ability of ursodeoxycholic acid therapy to reduce serum cholesterol and improve xanthelasma is a further indication for its use in PBC.

Although current data do not suggest a specific cardiovascular indication to treat hypercholesterolemia in PBC, given the safety of statins it would seem prudent to carefully assess other cardiovascular risk factors and to have a low threshold for introducing a statin in addition to UDCA. Simvastatin has been shown to be safe in PBC and is therefore a good first choice.

Finally it is important that further studies are carried out to determine exactly what the cardiovascular risk is in PBC and whether lipid-lowering therapy confers a benefit in this group of patients.


David H Adams, Liver Research Laboratories, University of Birmingham, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH, England


  1. Balan V, Dickson ER, Jorgensen RA, et al. Effect of ursodeoxycholic acid on serum lipids of patients with primary biliary cirrhosis. Mayo Clin Proc 1994; 69: 923-9.
  2. Del Puppo M, Galli KM, Crosignani A, et al. Cholesterol metabolism in primary biliary cirrhosis during simvastatin and UDCA administration. J Lipid Res 2001; 42: 437-41.
  3. Gregory WL, Game FL, Farrer M, et al. Reduced serum lipoprotein(a) levels in patients with primary biliary cirrhosis. Atherosclerosis 1994; 105: 43-50.
  4. Gylling H, Farkkila M, Vuoristo M, et al. Metabolism of cholesterol and low- and high-density lipoproteins in primary biliary cirrhosis: cholesterol absorption and synthesis related to lipoprotein levels and their kinetics. Hepatology 1995; 21: 89-95.
  5. Longo M, Crosignani A, Battezzati PM, et al. Hyperlipidaemic state and cardiovascular risk in primary biliary cirrhosis. Gut 2002; 51: 265-9.
  6. O'Kane MJ, Lynch PL, Callender ME, et al. Abnormalities of serum apo A1 containing lipoprotein particles in patients with primary biliary cirrhosis. Atherosclerosis 1997; 131: 203-10.
  7. Ritzel U, Leonhardt U, Nather M, et al. Simvastatin in primary biliary cirrhosis: effects on serum lipids and distinct disease markers. J Hepatol 2002; 36: 454-8.

  13 October 2003

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