Primary Sclerosing Cholangitis (PSC)

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Primary sclerosing cholangitis (PSC) definition

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Primary sclerosing cholangitis describes a disease process in which the bile ducts in the liver become inflamed, narrow and prevent bile from flowing properly.

The liver produces bile to help digest food in the intestine. Bile from liver cells is transported through the bile ducts in the biliary tree, where it then enters the gallbladder. When food enters the small intestine, bile helps break down fat into fatty acids so that they can be absorbed and used by the body. This also helps in the absorption of fat soluble vitamins (A,D,E & K).

As the bile ducts become inflamed and narrow, bile cannot easily flow and begins to back up. This increases the pressure within the liver causing liver cells to become inflamed. Over time, this inflammation decreases blood flow within the liver, increasing the pressure in the portal vein. This eventually causes portal hypertension, a back up in the portal system causing veins that line the esophagus, stomach, and intestine to swell (varices) and the spleen to swell (splenomegaly).

As the disease progress, liver cells die and are replace by scar tissue. This is called cirrhosis and is associated with liver failure.

What causes primary sclerosing cholangitis?

The cause of PSC is unknown but it has an association with inflammatory bowel disease, especially ulcerative colitis. It is thought that there may be an autoimmune component to the disease, where the body's immune system attacks the bile ducts in the liver and causes them to become, inflamed and narrowed.

What are the risk factors for primary sclerosing cholangitis?

The cause of PSC is unknown but it may be an autoimmune disease.

PSC is more often seen in men in the 20-30 age range. There is an association with inflammatory bowel, disease, most often with ulcerative colitis and less commonly with Crohn's Disease.

People with PSC who do not have inflammatory bowel disease, are more likely to be female and older.

Medically Reviewed by a Doctor on 4/23/2014

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