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Primary Biliary Cirrhosis

  • Definition

    There are two types of biliary cirrhosis: primary and secondary. Both types are characterized by cholestasis which impairs liver function. Secondary biliary cirrhosis results from prolonged bile duct obstruction or biliary atresia (absence or abnormality of the bile ducts from birth).


    The biliary duct consists of the ducts, within and leading from the liver, that deliver bile to the intestine.

    primary biliary cirrhosis is a rare form of biliary cirrhosis of unknown origin, occurring without obstruction or infection of the major bile ducts, sometimes developing after the administration of such drugs as chlorpromazine and arsenicals. Affecting chiefly middle-aged women, it is characterized by chronic cholestasis (accumulation of bile in the liver) with pruritis, jaundice, and hypercholesterolemia with xanthomas, and malabsorption.

    Primary biliary cirrhosis is characterized by chronic inflammation and scarring of the microscopic bile ducts within the liver. The result is that the liver is progressively destroyed and scarring (cirrhosis) develops.


    The cause is not known.


    Many people with primary biliary cirrhosis initially have no symptoms. Their condition is usually discovered during a routine physical examination that includes blood tests that relate to liver functions.

    When symptoms occur they include severe itching (pruritis) on the hands and feet and eventually the entire body. Jaundice, dark urine, and darkening of the skin exposed to the sun occur as the liver disease progresses. The slow progression results eventually in portal hypertension, the accumulation of fluids, and eventually liver failure and death. Small collections of fat are often seen in the area around the eyes, a reflection of the high cholesterol values in these persons.

    The diagnosis is usually suspected on the basis of the clinical history and appearance, and abnormal blood test results. A liver biopsy should be performed confirm the diagnosis. In addition, the physician may want to examine the bile ducts themselves by using x-rays, to exclude any obstruction in this area.


    There is no specific therapy for primary biliary cirrhosis, but the goal generally is to relieve the symptoms. The drug cholestyramine may be effective in reducing the itching. Increasingly, liver transplantation is being used for persons with severe liver failure from primary biliary cirrhosis.

    In spite of the progressive nature of the disease, asymptomatic patients may have survival rates equal to those in the normal population. Overall, a median 5-year survival rate of 63 percent has been noted.

    Numerous drugs, including prednisone, azathioprine, penicillamine (Cuprimine, Depen), chlorambucil (Leukeran), colchicine, cyclosporine (Sandimmune), and methotrexate, have been used in the treatment of primary biliary cirrhosis without consistent success; long-term drug toxicity is also a concern. Ursodeoxycholic acid (Actigall) has shown benefit in early studies, and may be the drug of choice in the future.


    Do any tests need to be done to rule out any other disease?

    Will a liver biopsy be required? What is the procedure?

    What are the complications?

    How severe is the liver damage?

    What treatment do you recommend? Will this slow down the progression of the disease?

    Will any medications be prescribed? What are the side effects?

    If the liver damage is severe is a liver transplant the only treatment?