Primary Biliary Cirrhosis

Primary biliary cirrhosis is a disease that slowly destroys the liver’s bile ducts. Bile is a substance produced in the liver that helps digest fat in the small intestine and remove toxins from the body. When the ducts are damaged, bile builds up in the liver and damages liver tissue. Biliary cirrhosis can develop over time and may cause the liver to stop working. 

The cause of primary biliary cirrhosis is unknown. The disease affects women more often than men and usually occurs between the ages of 30 and 60 years. Some research suggests that the disease may be an autoimmune disorder. 


The first and most common symptoms of primary biliary cirrhosis are itchy skin and fatigue. Other symptoms may eventually develop, including: 

  • Jaundice, which leads to a yellowing of the eyes and skin 
  • Fatty deposits under the skin 
  • Fluid retention 
  • Dry eyes and mouth 

In the later stages of the disease, some people develop osteoporosis, arthritis, and thyroid problems. 

How is primary biliary cirrhosis diagnosed?

Primary biliary cirrhosis is diagnosed through laboratory tests, x-rays, and in some cases, a liver biopsy to help to determine the extent of disease progression. A liver biopsy uses a thin needle to remove a small sample of liver tissue. The sample is then examined with a microscope. 


Initial treatment is usually aimed at relieving symptoms. Vitamin replacement therapy, calcium supplements, and drugs to treat itching are usually prescribed. 

Some patients have also benefited from ursodeoxycholic acid (also known as ursodiol, and by the brand names Actigall and Urso Forte). Ursodiol enhances bile flow and is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis. Ursodiol does not cure the disease, but it can help delay its progression. If the liver becomes severely damaged, a transplant may be necessary.


For more information about this disease, check out the following sites: 

Learn more about Hepatology (Liver Disease) at The Oregon Clinic