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 between the ages of 25 and 45

• 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

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

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:

• Osteoporosis (weak bones)

• Bruising and bleeding easily

• Greasy and bad-smelling stool

• Gallstones

• Liver problems, such as cirrhosis

Usually primary sclerosing cholangitis worsens gradually, resulting in liver failure.

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

• A procedure called endoscopic retrograde cholangiopancreatography (ERCP), where doctors take x-rays after a substance that shows up on x-rays is injected into your bile ducts

• Sometimes liver biopsy

• Special blood tests

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

• National Institute of Diabetes and Digestive and Kidney Diseases

• International Foundation for Gastrointestinal Disorders