Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis Facts

Primary sclerosing cholangitis is a chronic, progressive disease of the liver.
The cause of primary sclerosing cholangitis is not known though an immune cause is suspected in at least a minority of patients.
Primary sclerosing cholangitis has a strong association with ulcerative colitis and cancer of the bile ducts.
The primary symptoms of primary sclerosing cholangitis are due to obstruction of the bile ducts and cirrhosis of the liver.
Primary sclerosing cholangitis is diagnosed on the basis of abnormal blood tests and radiologic imaging of the bile ducts.
Primary sclerosing cholangitis is treated with medications, endoscopy, and liver transplantation.

Primary Sclerosing Cholangitis Overview

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts that channel bile from the liver into the intestines.

The liver performs many functions; one of them is manufacturing bile. Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine, particularly fat, and ridding the body of toxins. Liver cells secrete the bile they make into small canals within the liver. The bile flows through the canals and into larger collecting canals (ducts) within the liver (the intrahepatic bile ducts). The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts. From the extrahepatic bile ducts, the bile flows into the small intestine where the bile mixes with food.

In primary sclerosing cholangitis, the intrahepatic and extrahepatic bile ducts become inflamed, scarred and thickened (sclerotic), narrowed, and finally obstructed. Obstruction of the ducts can lead to abdominal pain, itching, jaundice, infection in the bile ducts (cholangitis), and liver scarring that leads to liver cirrhosis and liver failure.