Primary Sclerosing Cholangitis

Overview

Primary sclerosing cholangitis – or PSC – is a condition that affects the bile ducts. The bile ducts create bile, which is a liquid that helps with digestion and allows your body to get rid of cholesterol, toxins and old red blood cells.

In people with primary sclerosing cholangitis, inflammation (swelling) within the bile ducts leads to scarring. This scarring makes your bile ducts hard and narrow, leading to serious liver damage over time. Primary sclerosing cholangitis is a progressive condition, meaning that it usually comes on gradually and then gets worse over the course of months or years.

Primary sclerosing cholangitis causes

Experts haven’t yet pinpointed the exact cause of this condition, but they do believe that some infections or exposure to certain toxins can trigger the condition in people who are genetically predisposed to it. Many people with primary sclerosing cholangitis also have inflammatory bowel disease, which includes ulcerative colitis and Crohn’s disease.

Primary sclerosing cholangitis risk factors

Factors that put you at an increased risk for developing this condition include:

• Being male
• Being between the ages of 30 and 50
• Having inflammatory bowel disease
• Having Northern European ancestry

Primary sclerosing cholangitis symptoms

The only symptoms of primary sclerosing cholangitis in the early stages are fatigue and itching. As the disease progresses, you may also experience:

• Chills
• Enlarged liver
• Enlarged spleen
• Fever
• Night sweats
• Pain in the upper right area of your abdomen
• Unintentional weight loss
• Yellowing of the skin and eyes (jaundice)

Primary sclerosing cholangitis diagnosis

Your specialist will use one or more of the following tests to diagnose primary sclerosing cholangitis:

• Liver biopsy - during this test, your provider inserts a thin needle into your abdomen near your liver and then removes a small tissue sample (biopsy) for close analysis in the lab. Lab technicians can determine if your liver has been damaged.
• Liver function tests - your doctor may order liver function tests, which can check how well your liver is working and your liver enzyme levels.
• Magnetic resonance imaging (MRI) - your doctor may order an MRI of your bile ducts. This type of imaging uses sound waves to create detailed images of your liver and bile ducts. Your doctor can use this information to make an accurate diagnosis and determine the next steps of your treatment plan.
• Physical examination - your doctor will ask about your health history and symptoms specific to primary sclerosing cholangitis. For example, your doctor may feel your abdomen to check for an enlarged spleen or liver.
• X-ray - your doctor may order an X-ray of your bile ducts. X-ray creates detailed imaging of your liver and bile ducts. Your doctor can use this information to make an accurate diagnosis and determine the next steps of your treatment plan.

Primary sclerosing cholangitis treatment

Treatment for primary sclerosing cholangitis focuses on managing complications and reducing further liver damage. Your doctor may recommend one or more of the following treatment options for this condition:

• Antibiotic medications - bile that backs up in your liver and bile ducts can lead to infections. Therefore, you may need to take antibiotic medication to clear up any current infection and reduce your chances of developing new infections.
• Liver transplant - if primary sclerosing cholangitis has severely damaged your liver, you may need to undergo a liver transplant. During this procedure, your surgeon removes your damaged liver and replaces it with a partial or whole donor liver. The donor liver may come from a deceased liver donor, or a family member may be able to donate a partial liver.
• Treatment for blocked bile ducts - your doctor may recommend a procedure to open up blocked bile ducts. One option is known as balloon dilation, which involves your specialist opening up the blocked duct with a tiny balloon. Another option is stent placement, in which your specialist places a tiny, hollow tube (stent) made out of plastic or mesh permanently in your bile duct to keep it open.
• Treatment for itching - medications such as antihistamines, cholestyramine or rifampin can help you feel less itchy.
• Vitamin supplements - your doctor may recommend that you take regular vitamin supplements to make sure your body has enough of certain important nutrients. You may need to take calcium, vitamin D and other supplements.

When should I seek care?

If you experience any of these symptoms, start by voicing your concerns and symptoms to your primary care provider. From there, your doctor may suggest seeing a liver specialist for more specialized treatment.