Points to remember: Primary Biliary Cirrhosis

By  ,  National Institute of Health
Jan 13, 2013

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  • Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and damaged and, ultimately, disappear.
  • Injured liver tissue from chronic inflammation and the buildup of bile leads to cirrhosis, a condition in which the liver slowly deteriorates and malfunctions.
  • The cause of primary biliary cirrhosis is unknown. Most research suggests the disease is an autoimmune condition.
  • Primary biliary cirrhosis is more common in people who have a parent or sibling—articularly an identical twin—with the disease.
  • Most people are diagnosed early, before the disease progresses. The disease is often discovered when routine blood tests to check liver function are abnormal.
  • Many people with primary biliary cirrhosis do not have symptoms until after the disease is diagnosed.
  • The first and most common symptoms of the disease are itching, called pruritus, and fatigue. Other symptoms include dry eyes and mouth and jaundice.
  • The anti-mitochondrial antibody (AMA) blood test, the alkaline phosphatase blood test, and a liver biopsy may be necessary to confirm a diagnosis of primary biliary cirrhosis.
  • Some of the complications of primary biliary cirrhosis are osteoporosis and maldigestion.
  • Early treatment delays—but does not stop—the eventual onset of cirrhosis and liver failure.
  • Ursodiol (Actigall) is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.
  • Liver transplantation is the only treatment that will cure primary biliary cirrhosis. A liver transplant is considered when complications cannot be controlled by treatment.
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