Primary biliary cirrhosis (PBC) is a chronic disease that causes the bile ducts in the liver to become inflamed, damaged and ultimately disappear. Bile is a liquid produced in the liver that travels through the bile ducts to the gallbladder and then the small intestine, where it helps digest fats and fat-soluble vitamins. When the bile ducts become damaged from chronic inflammation, bile builds up in the liver, injuring liver tissue. Injured liver tissue from chronic inflammation and the buildup of bile leads to cirrhosis, a condition in which the liver slowly deteriorates and malfunctions.
Primary biliary cirrhosis develops over time and may ultimately cause the liver to stop working completely. 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. Early treatment delays—but does not stop—the eventual onset of cirrhosis and liver failure. When a person has end-stage liver disease, a liver transplant is necessary for survival.
In PBC, the immune system attacks the bile ducts. Genetic factors may make a person prone to develop the disease. Also, the disease is more common in people who have a parent or sibling—particularly an identical twin—with the disease.
A specific treatment that stops or reverses the progression of primary biliary cirrhosis has not been found. However, medication prescribed during the early stage of the disease may slow liver damage. Initial treatment for primary biliary cirrhosis is aimed at relieving symptoms. Vitamin replacement therapy, calcium supplements, and drugs to treat itching are usually prescribed.