Primary Biliary Cirrhosis (PBC)

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Primary biliary cirrhosis (PBC) definition

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Primary biliary cirrhosis (PBC) is a progressive disease of the liver caused by a buildup of bile within the liver (cholestasis) that results in damage to the small bile ducts that drain bile from the liver. Over time, this pressure build-up destroys the bile ducts leading to liver cell damage. As the disease progresses and enough liver cells die, cirrhosis and liver failure occur.

Bile is manufactured in the liver and then transported through the bile ducts to the gallbladder and intestine where it helps digest fats and fat soluble vitamins (A,D,E,K). When bile cannot be drained from the liver, it causes inflammation that leads to cell death. Scar tissue gradually replaces the areas of damaged liver and then the body cannot perform necessary functions.

What are the causes of primary biliary cirrhosis?

The cause of PBC is unknown. It is most likely an autoimmune disease, where the body's immune system attacks its own cells. In this disease, the bile ducts are under attack and are destroyed.

What are the risk factors for primary biliary cirrhosis?

  • There may be a genetic component to the development of PBC since a person with the disease is likely to have a family member also afflicted.
  • Women are nine times more likely than men to develop PBC. It most often develops in the middle age between the ages of 40 to 60.
  • The disease is seen more often in white, northern Europeans, compared to African-Americans.
Medically Reviewed by a Doctor on 4/23/2014

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Cirrhosis in the Liver Signs and Symptoms

Some of the more common symptoms and signs of cirrhosis include:

  • Yellowing of the skin (jaundice) due to the accumulation of bilirubin in the blood
  • Fatigue
  • Weakness
  • Loss of appetite
  • Itching
  • Easy bruising from decreased production of blood clotting factors by the diseased liver.