Primary Sclerosing Cholangitis (cont.)

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What are the symptoms of primary sclerosing cholangitis?

Most patients with early primary sclerosing cholangitis have no symptoms, and the presence of primary sclerosing cholangitis is recognized only because of abnormally elevated blood levels of liver enzymes (particularly alkaline phosphatase levels) that often are performed along with a routine physical examination.

Early symptoms of primary sclerosing cholangitis include fatigue and bodily itching (pruritus). As the disease progresses, patients may developjaundice (yellowing of skin and darkening of urine). Jaundice is due to the accumulation of bilirubin in the body. The bilirubin accumulates because it is not able to be eliminated in the bile due to prolonged obstruction of the bile ducts. The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow. The reason for the pruritus is not entirely known. It may be due to accumulation of bile salts in the body, also as a result of obstruction of the bile ducts.

As primary sclerosing cholangitis progresses, patients typically develop right upper abdominal pain, fever, fatigue, pruritus, and jaundice. These patients also are at risk of developing primary sclerosing cholangitis complications.

The patients with the autoimmune form of primary sclerosing cholangitis have more rapid and early onset of symptoms of abdominal pain, jaundice and fever than the majority of patients with the more indolent form of primary sclerosing cholangitis.

What are the complications of primary sclerosing cholangitis?


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).


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 (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.

Gall bladder cancer

Primary sclerosing cholangitis causes gall stones and is a risk factor for gall bladder cancer.

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.

Medically Reviewed by a Doctor on 2/6/2014

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