Digestive System Home > Primary Biliary Cirrhosis

Primary biliary cirrhosis is a disease that slowly destroys the liver's bile ducts and can even make the liver stop working. The cause of the disease is unknown. However, some research suggests that it may be linked to a problem in the immune system. Treatment can include lifestyle changes, medications, or even a liver transplant.

What Is Primary Biliary Cirrhosis?

Primary biliary cirrhosis is a liver disease that slowly destroys the bile ducts in the liver. When the ducts are damaged, bile (which is a substance that helps digest fat) builds up in the liver and damages the liver tissue. Over time, the primary biliary cirrhosis can progress and even make the liver stop working.

Understanding the Liver and Cirrhosis

The liver is an organ that performs many important functions, such as filtering waste and poisons, processing nutrients, storing fuel, and producing bile. Because the liver does so many important things for your body, you can't live without one.
But, similar to other organs, the liver can become damaged. When the liver cells are first damaged, the immune system sends in special white blood cells in response to the injury. This often causes the liver cells to swell, and this process is called inflammation.
As the liver tries to repair itself, it can form scar tissue. Primary biliary cirrhosis means that large areas of the liver have become very badly scarred -- usually permanently. This causes the liver to shrink and harden.
Scar tissue cannot do what healthy liver tissue does -- make protein, help fight infections, clean the blood, help digest food, and store energy for when you need it. Scar tissue also blocks the normal flow of blood through the liver.
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Last reviewed by: Arthur Schoenstadt, MD
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