Primary Biliary Cirrhosis

Treatment

While PBC can be life-threatening, many people live active, productive lives after its diagnosis. Those who show no symptoms when diagnosed may remain symptom-free for years. PBC is first treated with medication; even patients with cirrhosis may be treated to control the disease. If medical treatment no longer controls PBC and severe liver failure occurs, a liver transplant may be necessary.

Medication

The only drug currently approved by the Food and Drug Administration to treat PBC is a bile salt called ursodeoxycholic acid. It does not cure the disease, but slows its progress. The medication has been associated with reduction in liver enzymes typically elevated in PBC, a slower rate of disease progression and improved survival. This medication is well-tolerated but may have side effects.

Other medications help to lessen disease symptoms. These drugs include cholestyramine, diphenhydramine and rifampin to relieve the common symptoms of itching. Vitamin supplements such as calcium and vitamin D may also be prescribed to treat osteoporosis, which often accompanies PBC.

Transplantation

A liver transplant can extend or improve the quality of life for people with PBC. Transplant candidates should get on a liver transplant list; sometimes it may take a year or more before a liver becomes available. Mayo also has a living donor transplant program, which may shorten the wait. See information on liver transplant.

Signs and symptoms that indicate a patient has end-stage liver disease and may need a liver transplant include:

• Accumulation of fluid in the abdomen
• Changes in mental function
• Gastrointestinal bleeding
• Jaundice
• Severe weakness
• Loss of muscle mass
• Severe itching