Primary Sclerosing Cholangitis

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a chronic condition in which the liver’s bile ducts become inflamed and scarred. Bile ducts carry bile, a liquid produced by the liver, to the stomach and intestines. Bile helps digest food and carries waste out of the body. When a child has PSC, bile backs up in the liver.

Over time, PSC can cause serious liver damage such as cirrhosis or liver failure. In severe cases, a person with PSC needs a liver transplant.

PSC is a rare condition that affects between 6 and 16 people out of every 100,000. Men between the ages of 30 and 50 are at greatest risk, however, an increasing number of children are being diagnosed with PSC.

What is the liver and what does it do?

The liver is the second largest organ in the body, located in the abdominal cavity. The liver helps the body in many ways. It:

  • produces proteins that allow blood to clot normally, transport oxygen and support the immune system
  • produces bile, a substance that helps digest food
  • stores extra nutrients
  • helps clean the bloodstream of harmful substances
  • helps control blood sugar and cholesterol levels

How we care for primary sclerosing cholangitis

The Center for Childhood Liver Disease at Boston Children’s Hospital specializes in helping infants, children, adolescents and young adults with a wide variety of liver, gallbladder and bile duct disorders (otherwise known as hepatobiliary). Doctors refer children with liver disease to our program at Boston Children’s Hospital from all over the world.