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Primary Sclerosing Cholangitis


The Manual's Editorial Staff

Last full review/revision Oct 2019| Content last modified Oct 2019
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What is primary sclerosing cholangitis?

"Sclerosing" means scarring and hardening. "-Itis" means inflammation or swelling. "Cholang-" means bile ducts. So cholangitis is inflammation of your bile ducts. Primary sclerosing cholangitis is a disease with swelling, scarring, and narrowing of the bile ducts.

Your bile ducts are tubes that carry bile from your liver to your intestines. Bile is a greenish thick fluid that helps in digestion. Inside your liver are small bile ducts. Outside your liver are larger bile ducts that connect your liver to your intestines.

  • If you have primary sclerosing cholangitis, your bile ducts become blocked and eventually your liver stops working

  • Primary sclerosing cholangitis is most common in people who have inflammatory bowel disease, particularly men who are in their 40s

  • The first symptoms are weakness, tiredness, and itchy skin

  • Blocked bile ducts can cause bile duct cancer and cirrhosis

  • Doctors treat primary sclerosing cholangitis with medicine for itching and sometimes liver transplant

What causes primary sclerosing cholangitis?

Doctors aren't sure exactly what causes primary sclerosing cholangitis, but it's likely an autoimmune disease (a disease that causes your body’s immune system to attack its own tissues).

You're more likely to get it if you have:

  • Inflammatory bowel disease, especially ulcerative colitis

  • People in your family with the disease

What are the symptoms of primary sclerosing cholangitis?

Symptoms of primary sclerosing cholangitis include:

  • Feeling weak and tired

  • Yellow eyes and skin (jaundice)

  • Sometimes, pain in your upper belly

Later you may have:

Liver failure usually happens about 12 years after diagnosis.

How can doctors tell if I have primary sclerosing cholangitis?

Doctors suspect you have primary sclerosing cholangitis from your symptoms and abnormalities they see on liver blood tests. To tell for sure they'll do:

  • Ultrasound of your bile ducts

  • A special kind of MRI called MRCP that focuses on your bile ducts

  • Special blood tests

How do doctors treat primary sclerosing cholangitis?

There's no cure for primary sclerosing cholangitis.

If you don’t have any symptoms, doctors will do blood tests twice a year to check how well your liver is working.

If you have symptoms, doctors treat primary sclerosing cholangitis using:

  • Medicine to lessen itching and treat infection

  • A procedure to open your blocked bile ducts

Where can I get more information about primary sclerosing cholangitis?

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version


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