Primary Biliary Cirrhosis

What is the Treatment for Primary Biliary Cirrhosis?

Because primary biliary cirrhosis (PBC) is not curable, the main focus of treatment is to relieve symptoms, slow progression of the disease and prevent complications.

Methods to relieve symptoms of primary biliary cirrhosis:

For intense itching experienced by those with PBC the following methods are used:

  • Cholestyramine and Colestipol – Two cholesterol lowering drugs that are extremely effective in controlling the itching commonly experienced by people who have PBC, Cholestyramine and Colestipol unfortunately, have certain side effects for some people. Both drugs may interact with other medications and both drugs interfere with the body’s metabolism of UDCA (ursodeoxycholic acid, a bile acid that helps move bile through the liver), hormones, some other drugs and vitamins.
  • Rifampin – This medication is usually prescribed for people who can’t tolerate the side effects of Cholestyramine and Colestipol. This slow acting drug doesn’t work for everyone and can take up to a month to be effective.
  • Opioid antagonists – Studies are being done to determine whether opioid antagonists are effective in relieving severe itching. Although some of these drugs appear to be effective, it is necessary for them to be administered intravenously and may cause withdrawal symptoms.
  • Liver transplant – A last resort to relieve the intense itching experienced by some people with PBC for whom medications do not work.

With the goal of slowing progression of PBC and prolonging survival, the following treatments are used:

  • Ursodeoxycholic acid (UDCA) – Commonly considered the first line of therapy, UDCA, also known as ursodiol (Actigall), is a bile acid that improves the livers ability to function by moving bile through the liver. When administered early in the disease, it may prolong life and delay the need for a liver transplant. Some, not all people may experience side effects from UDCA which include diarrhea, weight gain and hair loss.
  • Other drugs – Many drugs have been studied for use in the treatment of primary biliary cirrhosis but because of severe side effects, lack of effectiveness or proof of long term benefits, UDCA is the only accepted treatment for primary biliary cirrhosis at this time.
  • Liver transplant – When the liver begins to fail because treatment no longer works, liver transplant has been proven to prolong life. Individuals who have PBC often do very well after a liver transplant, although the disease can return.

Because people who have PBC are at risk of developing complications, certain tests, precautionary measures, and lifestyle changes may be necessary. For example, a bone mineral density scan to detect osteoporosis (thinning of the bones) may be performed. In the case where osteoporosis has been detected, treatment with bisphosphonates may be necessary. Bisphosphonates are a group of drugs that can inhibit bone breakdown and even increase bone mass. Taking calcium supplements and vitamin D supplements, as well as eliminating smoking and getting weight bearing exercise may be beneficial.

Vitamin deficiencies are another complication of primary biliary cirrhosis, so injections of vitamin K and water-based oral supplements of vitamins A, D and E may be recommended.

American Liver Foundation, Primary Biliary Cirrhosis” http://www.liverfoundation.org/abouttheliver/info/pbc/  Retrieved September 7, 2011

Mayo Clinic, “Primary Biliary Cirrhosis” http://www.mayoclinic.com/health/primary-biliary-cirrhosis/DS00604  Retrieved September 7, 2011

MedicineNet.com, “Primary Biliary Cirrhosis” http://www.medicinenet.com/primary_biliary_cirrhosis/article.htm  Retrieved September 7, 2011

Medscape Reference, “Primary Biliary Cirrhosis” http://emedicine.medscape.com/article/171117-overview   Retrieved September 7, 2011<//p>

PubMed Health, “Primary Biliary Cirrhosis” http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001327/ Retrieved September 7, 2011

World Journal of Gastroenterology, “Clinical Features and Management of Primary Biliary Cirrhosis” http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2716586/?tool=pmcentrez Retrieved September 7, 2011