What is primary biliary cholangitis (PBC)?

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a progressive autoimmune disease (when the immune system attacks the body) that damages or destroys the bile ducts in the liver. When the ducts are destroyed, bile builds up in the liver contributing to inflammation and scarring (fibrosis). Eventually this can lead to cirrhosis and associated complications, as scar tissue replaces healthy liver tissue and liver function becomes increasingly impaired.

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 gall bladder 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 due to chronic inflammation, bile builds up in the liver and injures the liver tissue.

The injured liver tissue 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 do the following:

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.

PBC 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.

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

This answer is based on source information from the National Institute of Diabetes and Digestive and Kidney Diseases

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