Primary Sclerosing Cholangitis

Signs and Symptoms

In the early stages of primary sclerosing cholangitis (PSC), you may not experience any symptoms at all. As the disease progresses, symptoms may come and go and are caused by the bile not being drained properly. This can affect liver function and cause the bile to seep into your bloodstream. Symptoms may include:

• Chronic fatigue
• Jaundice, yellowing of the skin and eyes
• Loss of appetite
• Weight loss
• Chronic fatigue
• Chills
• Fever
• Upper abdominal tenderness

Diagnosis

Your doctor will first ask you about your medical history, which can suggest a likelihood of primary sclerosing cholangitis (PSC), especially if there is a history of inflammatory bowel disease and abnormal blood test results.

PSC is diagnosed through a procedure called cholangiography, which involves injecting dye into the bile ducts and taking an X-ray. Cholangiography can be performed as an endoscopic procedure, called endoscopic retrograde cholangiopancreatography (ERCP), through radiology or surgery, or with magnetic resonance imaging (MRI) scans. Cholangiography is performed under sedation. A lighted, flexible endoscope is inserted through the mouth, stomach and then into the small intestine. A thin tube is place through the scope into the bile ducts and dye is injected to highlight the bile ducts on the X-ray. If there is a narrowing of the bile ducts, the diagnosis of PSC is confirmed.

As the disease progresses, a liver biopsy is usually needed to confirm PSC, and determine how much damage has occurred. Under local anesthesia, a slender needle is inserted through the right lower chest to extract a small piece of liver for microscopic analysis.

Treatment

Treatment for primary sclerosing cholangitis (PSC) includes medication to reduce itching and jaundice, antibiotics to treat infections and vitamin supplements since people with PSC are often deficient in vitamins A, D and K. In some cases, surgery to open major blockages in the common bile duct also is necessary. In addition, liver transplantation may be required and in some cases, can cure PSC.