Primary Sclerosing Cholangitis (cont.)

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How is the diagnosis of primary sclerosing cholangitis made?

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The diagnosis of primary sclerosing cholangitis is suspected from the symptoms (such as fatigue, itching, and jaundice) and abnormal laboratory tests (such as abnormally elevated blood levels of alkaline phosphatase and other liver enzymes); and is confirmed by demonstration of abnormally thickened bile ducts using special radiological tests. It is also important to exclude other diseases that can mimic primary sclerosing cholangitis. These diseases include primary biliary cirrhosis (PBC), gallstones in the bile ducts, bile duct cancers and strictures.

Blood tests

The blood level of alkaline phosphates is usually elevated in primary sclerosing cholangitis. The blood levels of other liver enzymes (AST and ALT) may also be mildly elevated. Except in those patients with the autoimmune hepatitis overlap of primary sclerosing cholangitis, the bilirubin usually is normal but gradually increases as the disease progress. Antimitochondrial antibody (AMA), which is elevated in patients with PBC, is usually normal in patients with primary sclerosing cholangitis.

Radiologic tests

Endoscopic retrograde cholangio-pancreatography (ERCP) and magnetic resonance cholangio-pancreatography (MRCP) are commonly performed to visualize the intrahepatic and extrahepatic ducts. These ducts are typically normal in appearance in patients with PBC, but in primary sclerosing cholangitis patients, these ducts have a beaded appearance (multiple narrowings along the ducts with areas of widening in between).

MRCP is noninvasive and safe. ERCP is more invasive and carries a 5%-6% chance of causing an attack of acute pancreatitis. However, ERCP has the advantage of obtaining cell samples (a process called brush cytology) from the bile ducts. Brush cytology is not very accurate, but sometimes can help to diagnose cholangiocarcinoma. Also, during ERCP, doctor can also insert balloons and stents across major areas of narrowing (dominant strictures) to relieve obstruction and treat infection.


Patients with primary sclerosing cholangitis have a high likelihood of also having ulcerative colitis, and, as mentioned previously, patients with primary sclerosing cholangitis and ulcerative colitis have higher risks of developing colon cancer. Thus, colonoscopy is important to both diagnose ulcerative colitis and for early detection of cancer or precancerous conditions.

Medically Reviewed by a Doctor on 2/6/2014

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