Primary sclerosing cholangitis (PSC) is a chronic liver disease caused by progressive inflammation and scarring of the bile ducts of the liver. This results in impaired flow of bile which damages the liver cells and may lead to cirrhosis, liver failure, and cancer. 

Symptoms of PSC 

Primary sclerosing cholangitis (PSC) progresses slowly, and many children and adolescents do not experience symptoms for several years. Common initial symptoms include:

  • Fatigue
  • Itching of the skin
  • Jaundice
  • Diarrhea
  • Weight loss / poor growth
  • In more advanced disease, if the bile ducts become infected, patients may experience fever, chills and abdominal pain (cholangitis).

Causes of PSC

While the cause of PSC is not fully understood, it is believed that it may be caused by an immune reaction to an infectious trigger, and that genetic susceptibilities also may play a role. People with inflammatory bowel disease and certain other conditions are more likely to have PSC, although the relationship between these diseases is not yet clear.

Treatments for PSC

There is currently no established cure for PSC. However, medical therapy may help to alleviate symptoms such as itching, infection, and malnutrition. Surgery or endoscopic procedures may be performed to address blockage in the bile ducts and improve bile flow.

If a patient’s condition advances to liver failure, liver transplantation may be recommended. Although PSC can recur after transplant, a liver transplant is curative in the majority of cases.