Primary biliary cholangitis (primary biliary cirrhosis)
Overview
Primary biliary cholangitis (PBC, often referred to as primary biliary cirrhosis) is a type of liver disease that can get gradually worse over time. Without treatment, it may eventually lead to liver failure.
Symptoms
PBC does not always cause symptoms, but some people may experience: bone and joint achesfatigue (extreme tiredness)itchy skindry eyes and mouthpain or discomfort in the upper right side of their tummy Read more about the symptoms of PBC
Causes
Bile is a liquid produced inside the liver that's used to help digest fats, absorb certain vitamins and remove waste products from the body. It passes out of the liver through small tubes called bile ducts. In PBC, the immune system (the body's natural defence against infection and illness) mistakenly attacks the bile ducts. It's not clear why this happens, but it's thought to be caused by a combination of subtle differences in how the immune system works. The bile ducts become damaged and injured, causing bile to build up in the liver. This further damages the liver and may lead to scarring (cirrhosis).
Treatment
Ursodeoxycholic acid (UDCA) to prevent liver damage; Obeticholic acid. Liver transplant.
Prevention
Stop smoking; Limit alcohol (to protect liver).
When to see a doctor
See GP if: persistent itch; severe fatigue.