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February 9, 2012

Primary Sclerosing Cholangitis

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What is primary sclerosing cholangitis (PSC)?

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 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.

Picture of the Liver and Hepatic Ducts

How common is primary sclerosing cholangitis?

Primary sclerosing cholangitis is a rare disease with an estimated prevalence in the United States of 6 per 100,000 people. It is more common in men then in women; approximately 70% of primary sclerosing cholangitis patients are men. The mean age at diagnosis of primary sclerosing cholangitis is around 40 years of age.

There is a strong association between primary sclerosing cholangitis and chronic ulcerative colitis. Primary sclerosing cholangitis can also occur alone or in association with Crohn's disease, a disease of the intestines that is related to ulcerative colitis.




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Primary Sclerosing Cholangitis (PSC)

What is Ulcerative Colitis?

Ulcerative colitis is a chronic inflammation of the large intestine (colon). The colon is the part of the digestive system where waste material is stored. The rectum is the end of the colon adjacent to the anus. In patients with ulcerative colitis, ulcers and inflammation of the inner lining of the colon lead to symptoms of abdominal pain, diarrhea, and rectal bleeding.

Ulcerative colitis is closely related to another condition of inflammation of the intestines called Crohn's disease. Together, they are frequently referred to as inflammatory bowel disease (IBD). Ulcerative colitis and Crohn's diseases are chronic conditions that can last years to decades. They affect approximately 500,000 to 2 million people In the United States. Men and women are affected equally. They most commonly begin during adolescence and early adulthood, but they also can begin during childhood and later in life.

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Read the Ulcerative Colitis article »







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