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 intestines where it helps digest food. When a child has PSC, the bile ducts become narrow, making it difficult for bile to flow smoothly out of the liver. The abnormal flow of bile and build up of bile in the liver can cause chronic liver problems. Over time, PSC can cause serious liver damage, leading to cirrhosis liver failure. In severe cases, a person with PSC may need a liver transplant.

PSC is a rare condition, estimated to affect between 6 and 16 people out of every 100,000.

 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:

  • 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

 What are the symptoms of primary sclerosing cholangitis?

Children and adults can have PSC for 20 years or more with no noticeable symptoms. For those who do, symptoms include:

  • fatigue (the most common symptom of PSC)
  • itchy skin
  • unexpected weight loss
  • abdominal pain
  • unexplained fever

As the condition progresses, a person with PSC may develop symptoms of liver failure:

  • jaundice
  • pain or tenderness in the upper right abdomen
  • weakness
  • dark urine
  • confusion or disorientation
  • easy bleeding and bruising

 What causes primary sclerosing cholangitis?

Researchers have not determined what causes PSC. It may be an autoimmune disease – a disorder that causes the body’s immune system to attack healthy tissues – though that has not yet been proven.

Many people with PSC also have ulcerative colitis. PSC raises the risk for cancer of the gallbladder, bile duct, liver or colon.

The following factors may increase a child’s risk for PSC:

  • family history
  • male gender
  • autoimmune disease

 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.