Mayo Clinic Health Manager
Get free personalized health guidance for you and your family.
Get Started
close window
Housecall Archives
About our e-newsletters
- Free e-newsletters
- Mayo Clinic expertise
- We do not share your e-mail address
Housecall, our weekly general-interest e-newsletter, keeps you up to date on a wide variety of health topics with timely, reliable, practical information, recipes, blogs, questions and answers with Mayo Clinic experts and more. Our biweekly topic-specific e-newsletters also include blogs, questions and answers with Mayo Clinic experts, and other useful information that will help you manage your health.
Housecall Archives
close window
Mayo Clinic Housecall
Stay up to date on the latest health information.
What you get
- Free weekly e-newsletter
- Mayo Clinic expertise
- Recipes, tools and other helpful information
- We do not share your e-mail address
Treatments and drugs
By Mayo Clinic staffBecause 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), 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.
- Other drugs. Sometimes other drugs are used off-label or in clinical trials to treat primary biliary cirrhosis, but many have proved to have serious side effects or haven't been effective. For example, some studies show that the drug methotrexate, which is normally used to treat arthritis, psoriasis and some types of cancer, isn't helpful in primary biliary cirrhosis, whereas others show it to be somewhat effective.
- Liver transplant. When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. People with primary biliary cirrhosis who have liver transplants often do very well, although the disease may recur in the new liver.
Treating the symptoms
The only medication that may help treat the fatigue that often accompanies primary biliary cirrhosis is modafinil (Provigil). Early research on this medication has shown promise in treating fatigue, but more research is needed to determine its role in primary biliary cirrhosis.
Several therapies may be used to help control the intense itching that often occurs in primary biliary cirrhosis, including:
- Cholestyramine and colestipol. These cholesterol-lowering drugs can provide marked relief of itching in some people, but they have several drawbacks. Cholestyramine is an unpleasant-tasting powder that must be mixed with food or liquids. Both drugs interfere with how your body metabolizes UDCA and certain other medications, as well as hormones and some vitamins. And, they may interact with other medications you are taking. Check with your doctor before combining medications, hormones or vitamins.
- Rifampin. This drug may be prescribed for people who can't tolerate the side effects of cholestyramine. Rifampin doesn't work for everyone, and it's slow acting, sometimes taking about a month before it starts working.
- Opioid antagonists. Researchers are studying whether this class of drugs relieves severe itching in people with primary biliary cirrhosis. Although some of these medications seem very effective, they must be administered intravenously and may cause withdrawal symptoms.
- Liver transplant. This may be the only option for some people with unrelieved and intolerable itching.
Preventing complications
Working together, you and your doctor can help prevent some of the complications that can occur with primary biliary cirrhosis:
- 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. You'll need more frequent screening if enlarged veins are found, if you have cirrhosis or if you've had gastrointestinal bleeding.
- Weak bones (osteoporosis). Although it's not certain that osteoporosis can always be prevented in cases of primary biliary cirrhosis, treatment with bisphosphonates may help increase bone mineral density. Bisphosphonates are a group of drugs that can inhibit bone breakdown and even increase bone mass. Taking at least 1,500 milligrams of calcium, along with a vitamin D supplement every day, may be of benefit. Ask your doctor what amount of vitamin D you need. Equally important are lifestyle measures such as stopping smoking and getting regular weight-bearing exercise.
- Vitamin deficiencies. Your doctor may recommend injections of vitamin K and water-based oral supplements of vitamins A, D and E.
- Raynaud's phenomenon. Although drug and even surgical options exist for treating Raynaud's, self-care measures are often highly successful and are usually tried first. These measures include keeping your body's core temperature elevated and protecting your hands and feet from cold, both indoors and out. Biofeedback, a technique that teaches you to control certain body responses, may help. Autogenic training, a type of progressive muscle relaxation and hypnosis, also may help improve blood flow to the extremities. If these methods fail, your doctor may prescribe a vasodilator such as a long-acting calcium channel blocker, a drug that can open small blood vessels and increase circulation.
References
- Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.htm. Accessed Aug. 14, 2009.
- Primary biliary cirrhosis. American Liver Foundation. http://www.liverfoundation.org/education/info/pbc/. Accessed Aug. 14, 2009.
- Cirrhosis. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/sec03/ch026/ch026c.html. Accessed Aug. 14, 2009.
- Friedman LS. Liver, biliary tract and pancreas disorders. In: McPhee SJ, et al. Current Medical Diagnosis & Treatment. 48th ed. Los Altos, Calif.: Lange Medical Publications; 2009. http://www.accessmedicine.com/content.aspx?aID=7993. Accessed Aug. 14, 2009.
- Kaplan M, et al. Primary biliary cirrhosis. New England Journal of Medicine. 2005;353:1261.
- Kaplan MM. Clinical manifestations, diagnosis, and natural history of primary biliary cirrhosis. http://www.uptodate.com/home/index.html. Accessed July 30, 2009.
- Kaplan MM. Overview of the treatment of primary biliary cirrhosis. http://www.uptodate.com/home/index.html. Accessed July 30, 2009.
- Hirschfeld GM, et al. Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. New England Journal of Medicine. 2009;360:2544.
- Taouli B, et al. Advanced MRI methods for assessment of chronic liver disease. American Journal of Roentgenology. 2009;193:14.
- Lazaridis K, et al. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis. Hepatology. 2007;46:785.
- Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. New England Journal of Medicine. 2007;357:1254.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Aug. 31, 2009.
- Hay JE. Bone disease in cholestatic liver disease. Gastroenterology. 1995;108:278.
