What is Primary Biliary Cirrhosis?

By  ,  National Institute of Health
Jan 13, 2013

Primary biliary cirrhosis is a chronic disease that causes the bile ducts in the liver to become inflamed and 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 A, D, E, and K. 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. Scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. Scarring also impairs the liver’s ability to

  • control infections
  • remove bacteria and toxins from the blood
  • process nutrients, hormones, and drugs
  • make proteins that regulate blood clotting
  • produce bile to help absorb fats—including cholesterol—and fat-soluble vitamins
  • effectively replace its own cells when they become damaged

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. 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.

Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men.

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