Primary Sclerosing Cholangitis

Reviewed/Revised Jul 2023
VIEW PROFESSIONAL VERSION
Get the full details

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis is a long-term disease that causes 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 a procedure to open your bile duct or a 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

  • Itchy skin

  • 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 (magnetic resonance imaging) called MRCP (magnetic resonance cholangiopancreatography) 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

  • Liver transplant

Where can I get more information about primary sclerosing cholangitis?

quizzes_lightbulb_red
Test your KnowledgeTake a Quiz!
Download the free Merck Manual App iOS ANDROID
Download the free Merck Manual App iOS ANDROID
Download the free Merck Manual App iOS ANDROID