Primary biliary cirrhosis (PBC) is an autoimmune disease in which the body's immune cells attack the bile ducts of the liver. As a result, the bile ducts — through which bile, the substance that helps digest fat — are destroyed. The process results in scarring, which over time may progress to cirrhosis and, in severe cases, liver failure. Although primary biliary cirrhosis can affect all ages and sexes, middle-age women are most frequently diagnosed with the disease.
If the condition progresses and results in severe liver damage or liver failure, a liver transplant may be necessary. The UCSF Liver Transplant Program performs over 100 transplants each year, making it one of the leading programs in the United States. Survival statistics are among the very best in the country.
The initial signs and symptoms of PBC may be subtle, detectable only with blood testing. As the disease progresses over time, usually years, patients may suffer from itching and fatigue.
At an advanced stage, jaundice — a condition that gives a yellow tint to the skin and eyes — develops along with other signs of liver failure. These may include memory problems, fluid retention and internal bleeding.
During a liver biopsy, a small sample of tissue is removed from your liver using a thin needle. The tissue is prepared and stained in a laboratory for examination under a microscope.
The current treatment for PBC is a bile acid medication, called ursodeoxycholate. Although this medication does not cure the condition, it slows its progression. For advanced stage PBC, in which the liver is severely damaged, a liver transplant is the only option.
Liver transplant is recommended for people whose primary biliary cirrhosis causes severe liver damage or progresses to liver failure. The evaluation for a transplant is complex and generally requires several months. Therefore, even patients who feel well should be referred for a transplant at the first sign of liver failure, or if they have advanced liver disease diagnosed by X-ray studies or liver biopsy.
Reviewed by health care specialists at UCSF Medical Center.