Children's Hospital Colorado

Primary Sclerosing Cholangitis (PSC)

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a rare autoimmune disease that targets the bile ducts of the liver. PSC in children can lead to cirrhosis and an increased risk of biliary and colon cancers in adulthood.

What causes primary sclerosing cholangitis?

Genetics often play a part in making a person more likely to develop an autimmune disease. With autoimmune diseases, the person’s own immune system mistakenly sees a part of the body as a threat and attacks it. In many cases of autoimmune diseases, something triggers the immune system, such as an infection, to attack the body. In the case of PSC, an environmental trigger, such as a viral infection, may initiate a bile duct injury. This results in an immune-mediated attack on the bile ducts.

Who gets primary sclerosing cholangitis?

Pediatric PSC affects all ages, genders and ethnicities. It is more common in males and is usually associated with inflammatory bowel disease, another autoimmune disease.