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Medical Author:

Dennis Lee, MD

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.

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Medical Editor:

Jay W. Marks, MD

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.

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In this Article

• 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
• Find a local Gastroenterologist in your town
• Primary Sclerosing Cholangitis Index

Cirrhosis

As primary sclerosing cholangitis progresses, the disease causes cirrhosis of the liver (irreversible scarring of the liver) and liver failure; leading to the consideration of liver transplantation. Primary sclerosing cholangitis is, in fact, one of the more common reasons for liver transplantation. Patients with advanced cirrhosis may develop frequent infections, fluid in the ankles and the abdomen (ascites), internal bleeding from rupture of esophageal varices, and mental confusion with progression to coma (hepatic encephalopathy).

Cholangitis

The narrowing of the bile ducts predisposes the bile to bacterial infection (cholangitis). Cholangitis is a serious and potentially life-threatening infection with fever, shaking chills (rigors), jaundice, and upper abdominal pain. Cholangitis can result in bacterial infection spreading to the blood stream (a condition called sepsis). Sepsis can cause damage to kidneys and lungs and even cause shock.

Cholangiocarcinoma

Cholangiocarcinoma (cancer of the bile ducts) is more common among patients with primary sclerosing cholangitis. An estimated 9%-15% of patients with primary sclerosing cholangitis will develop cholangiocarcinoma, a very lethal type of cancer. Patients at highest risks for developing cholangiocarcinoma are primary sclerosing cholangitis patients with cirrhosis who also have long-standing ulcerative colitis.

Colon cancer

Long-standing chronic ulcerative colitis alone is a risk factor for colon cancer. Patients with both primary sclerosing cholangitis and ulcerative colitis have an even higher risk for developing colon cancer than patients with ulcerative colitis alone.