Because no cure exists for primary biliary cirrhosis, treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.
Treating the disease
Treatments aimed at slowing the disease and prolonging life include:
- Ursodeoxycholic acid (UDCA). Also known as ursodiol (Actigall, Urso), UDCA is a bile acid that helps move bile through your liver. Although UDCA doesn't cure primary biliary cirrhosis, it may prolong life if started early in the disease and is commonly considered the first line of therapy. It's less likely to help people with advanced liver damage. Side effects of UDCA may include weight gain, hair loss and diarrhea.
- Liver transplant. When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant is a procedure to remove your diseased liver and replace it with a healthy liver from a donor. Most donated livers come from people who have died. But in some cases, it may be possible to remove a portion of a liver from a living donor. Primary biliary cirrhosis often recurs in the transplanted liver, but it may take several years to develop.
Treating the symptoms
Your doctor may recommend treatments to control the signs and symptoms of primary biliary cirrhosis to make you more comfortable. Treatments may help control symptoms such as:
- Fatigue. Treatment for fatigue involves trying to determine what may contribute to your symptoms. Treating the underlying causes may help relieve fatigue. Medications have been studied for fatigue. One medication that has shown promise in studies is modafinil (Provigil). More research is needed to determine its role in primary biliary cirrhosis.
- Itching. One option for controlling itching is cholestyramine (Locholest, Prevalite), which is a powder that must be mixed with food or liquids. Though cholestyramine works for most people, the taste is unpleasant. Another option is rifampin (Rifadin), which is taken in pill form. Rifampin doesn't work for everyone. Itching that can't be controlled may be treated with a liver transplant.
Working together, you and your doctor can help prevent some of the complications that can occur with primary biliary cirrhosis:
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- Increased pressure in the portal vein (portal hypertension). Your doctor is likely to screen for portal hypertension and enlarged veins when you're first diagnosed and every few years thereafter. If you're diagnosed with portal hypertension or bleeding, treatment may involve medications or surgery.
- Weak bones (osteoporosis). Treatment for bone loss associated with primary biliary cirrhosis often involves calcium and vitamin D supplements. Your doctor may also recommend that you exercise most days of the week to help increase your bone density.
- Vitamin deficiencies. Your doctor may recommend supplements of vitamins A, D, E and K to counter vitamin deficiencies.
- Angulo P, et al. Primary biliary cirrhosis. In: Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2010. http://www.mdconsult.com/books/about.do?eid=4-u1.0-B978-1-4160-6189-2..X0001-7--TOP&isbn=978-1-4160-6189-2&about=true&uniqId=229935664-2192. Accessed Oct. 5, 2011.
- Liver biopsy. Alexandria, Va.: American Association for the Study of Liver Diseases. http://www.aasld.org/practiceguidelines/Documents/Bookmarked%20Practice%20Guidelines/PrimaryBillaryCirrhosis7-2009.pdf. Accessed Oct. 5, 2011.
- Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.aspx. Accessed Oct. 5, 2011.
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