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Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic, progressive disease of the bile duct system. The bile duct system carries bile from the liver and gallbladder into the first part of the small intestine, called the duodenum.

Primary Sclerosing Cholangitis Symptoms

Patients with PSC may not have any symptoms. PSC is usually diagnosed in these patients when a routine blood test shows abnormal liver function. Even as the disease progresses, there still may not be any symptoms. When symptoms do develop, it is a result of an obstruction to the bile flow. Symptoms include:

  • Jaundice (yellowing of the skin and whites of the eyes)

  • Itching

  • Pain in the right upper part of the abdomen

  • Fever, chills

  • Unexplained weight loss

  • Fatigue

Primary Sclerosing Cholangitis Diagnosis at Johns Hopkins

A diagnosis of PSC begins with a comprehensive physical exam during which you describe your symptoms and medical history. Other diagnostic procedures include:

Laboratory Tests

Blood tests will be ordered to evaluate your liver function. Patients with PSC almost always have abnormal results. Usually a simple blood test can determine if you have abnormally elevated levels of certain serums.

Liver Biopsy

A biopsy offers a definitive diagnosis. Your doctor may perform a biopsy to confirm PSC and stage it (determine the level of severity). During a liver biopsy, tissue is removed from your liver and sent to a pathology lab for analysis.

Endoscopic Retrograde Cholangiopancreatography (ERCP)

An ERCP is an endoscopic technique that allows visualization of the bile and pancreatic ducts. An endoscope is a thin, flexible, lighted tube that is inserted into your mouth to provide access to your upper gastrointestinal system.

During this procedure:

  1. A special side-viewing endoscope to help place the endoscopic tools into the bile and pancreatic ducts is used.

  2. A dye is injected into the ducts to highlight any abnormalities.

  3. An X-ray is taken to see the ducts.

ERCP is the preferred method for visualizing the biliary tree (the network of biliary ducts). Your doctor will find many strictures (narrowing) and dilations (opening), which gives the duct its characteristic beaded appearance.

A: Intrahepatic and extrahepatic strictures in a patient with primary sclerosing cholangitis; B: corresponding ERCP cholangiogram.
A: Intrahepatic and extrahepatic strictures in a patient with primary sclerosing cholangitis; B: corresponding ERCP cholangiogram. (Click to Enlarge)

Percutaneous Transhepatic Cholangiography (PTC)

This method is used in patients when ERCP is unsuccessful. Depending on the type of strictures in your biliary system, your doctor may not be able to see the biliary tree well with ERCP. A percutaneous (through the skin) approach is more effective.

A percutaneous transhepatic cholangiography is a sophisticated X-ray procedure. Because the livers drainage system does not normally show up on an X-ray, your doctor will need to insert a special dye into the area. During a PTC:

  1. A needle is inserted in between two of your ribs and X-ray dye is introduced into the area.

  2. The X-ray images appear on a nearby video screen.

  3. We obtain all the necessary images, which may take some time.

  4. The images show if there is a blockage in your liver or bile ducts. If the dye does not fill the entire drainage system that means there is a blockage.

Magnetic Resonance Cholangiography (MRC)

Magnetic resonance imaging (MRI) may be useful in detecting blockages. An MRI uses powerful magnetic waves to create a detailed image of the inside of your body. An MRC is a specialized MRI used to gather images of the bile ducts.

Primary Sclerosing Cholangitis Treatment at Johns Hopkins

There are a number of different treatment options for PCS, ranging from medication to surgery. Learn more about treatment for primary sclerosing cholangitis at Johns Hopkins.

See illustration: Technique of endoscopic biliary sphincterotomy illustration.

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