Primary sclerosing cholangitis (PSC) – What you should know

People with inflammatory bowel disease (IBD) are at increased risk for a condition called primary sclerosing cholangitis (PSC). We’ll help you understand PSC and why regular screenings for this disease are essential if you have IBD.

What is primary sclerosing cholangitis? 

PSC is a chronic condition in which the body’s immune system attacks and damages the bile ducts and liver. PSC progresses slowly and gets worse over time. This rare condition affects about five to six people per 100,000 in the U.S., but it is much more common in patients with IBD. The average age at diagnosis is about 40.

How does PSC affect the body?

PSC affects the bile duct system within the liver. The bile duct system carries digestive liquid (bile) from the liver and gallbladder to the small intestine where it aids in digestion. PSC is caused by inflammation in the bile ducts (cholangitis), eventually leading to scarring (sclerosis). The scars make the ducts hard and narrow and restrict bile flow through the ducts. The liver becomes damaged and stops working correctly. Over time, the liver fails.

Treatment can help manage the symptoms and complications of PSC. But currently, there are no treatments to slow down or stop the disease. When PSC advances to late-stage liver disease, it may be time to consider a liver transplant.

What’s the connection between IBD and PSC?

IBD and PSC are closely linked:

• Between 70% and 90% of people with PSC have IBD.
• It is less common for people with IBD to have PSC. Only about 5% of people with IBD also have PSC.
• It is more common in patients with ulcerative colitis, but can occur in patients with Croh'ns disease as well.

People who have both IBD and PSC are at increased risk for several types of cancer, including:

• Bile duct cancer
• Colon cancer 
• Gallbladder cancer

Researchers aren’t sure why IBD and PSC sometimes affect the same person. Genetics, the immune system, the gut microbiome and environmental factors may play a role.

Signs and symptoms

Most people discover they have PSC after other imaging or blood tests are obtained for an unrelated reason. These tests can reveal liver abnormalities that may point to a PSC diagnosis. Additional tests can confirm the diagnosis.

About half of people with PSC have no symptoms at the time of diagnosis. As the disease progresses, early symptoms include:

• Fatigue
• Itching (sometimes severe)
• Pain in the right upper area of the abdomen
• Yellow skin and eyes (jaundice)

Symptoms that may appear later include:

• An enlarged liver or spleen
• Fever, chills and night sweats
•  Unexplained weight loss

If you experience any of these symptoms, see your IBD specialist. Diagnostic tests can help your doctor understand the cause. If you have PSC, your care team will monitor the disease and help you manage your symptoms.

Risk and screening recommendations

The exact cause of PSC is unknown, so you can’t take steps to prevent it. But you can be proactive and get routine screenings for PSC. An annual blood test to monitor liver enzymes is the most common way to screen for PSC. Talk to your doctor about these screening tests and how often you need them.

We are here to help.

The Crohn’s & Colitis Foundation is available to support you. Contact their IBD Help Center for personal answers from knowledgeable information specialists. 

• Call the free helpline (1-888-MY-GUT-PAIN). 
• Send an email at [email protected].
• Text chat with a live agent (subject to availability).

Foundation agents can answer your questions and connect you to other helpful resources. The service is available in more than 170 languages.

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