What is Primary Sclerosing Cholangitis (PSC)?

Primary sclerosing cholangitis or PSC is inflammation and scarring of the bile ducts inside and outside the liver. As the ducts become scarred, they become blocked and bile can not pass through. Bile is essential for the digesting fat in food; when the ducts become blocked, the bile backs up in the liver and can eventually cause liver failure.

What are the symptoms of PSC?

The symptoms of PSC include itching, fatigue and yellow skin (also called jaundice). Having fever and chills is a sign of an infection in the bile ducts in a patient who has PSC. The disease progresses slowly, therefore, you may have the disease prior to symptoms being present.

What causes PSC?

The cause of primary sclerosing cholangitis (PSC) is unknown.  It may be related to bacteria, a virus and/or immune system problems.  

Who gets PSC?

PSC typically occurs in individuals aged 30 to 60 years old, but can also be present in children.  More men than women get PSC.

How is PSC related to Inflammatory Bowel Disease?

PSC is associated with ulcerative colitis.

How is PSC diagnosed and treated?

PSC is diagnosed through blood tests, a cholangiography, which consists of injecting dye into the bile ducts and taking an x-ray, or an ERCP or MRCP. 

• An MRCP is a special MRI of the bile ducts. 
• An ERCP is a procedure that allows the physician to diagnose and treat problems in the liver, gallbladder, bile ducts, and pancreas. The procedure combines x-ray and the use of an endoscope — a long, flexible, lighted tube. The scope is guided through the patient’s mouth and throat, then through the esophagus, stomach and duodenum. The physician can examine the inside of these organs and detect any abnormalities. A tube is then passed through the scope and a dye is injected that will allow the internal organs to appear on an x-ray. 

There are no curative treatments for PSC. The symptoms or problems related to PSC are treated with antibiotics (that treat bile duct infections resulting from blockages), vitamin supplements to replenish the body and medications to relieve itching. Depending on the severity of the condition, surgery may be necessary to open blockages in the bile ducts. In extreme cases, a liver transplant may be needed if the liver begins to fail due to blockages.