Primary Sclerosing Cholangitis
Dennis Lee, MD
Dr. Lee was born in Shanghai, China, and received his college and medical training in the United States. He is fluent in English and three Chinese dialects. He graduated with chemistry departmental honors from Harvey Mudd College. He was appointed president of AOA society at UCLA School of Medicine. He underwent internal medicine residency and gastroenterology fellowship training at Cedars Sinai Medical Center.
Jay W. Marks, MD
Jay W. Marks, MD, is a board-certified internist and gastroenterologist. He graduated from Yale University School of Medicine and trained in internal medicine and gastroenterology at UCLA/Cedars-Sinai Medical Center in Los Angeles.
- What is primary sclerosing cholangitis (PSC)?
- How common is primary sclerosing cholangitis?
- What causes primary sclerosing cholangitis?
- What are the symptoms of primary sclerosing cholangitis?
- What are the complications of primary sclerosing cholangitis?
- How is the diagnosis of primary sclerosing cholangitis made?
- How is primary sclerosing cholangitis treated?
- Primary Sclerosing Cholangitis At A Glance
- Patient Comments: Primary Sclerosing Cholangitis (PSC) - Symptoms
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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.
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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