Primary sclerosing cholangitis
DEFINITION
Primary sclerosing (skluh-ROHS-ing) cholangitis (koh-lan-JIE-tis) is a disease of the bile ducts, which carry the digestive liquid bile from your liver to your small intestine. In primary sclerosing cholangitis, inflammation causes scars within the bile ducts. These scars make the ducts hard and narrow and gradually cause serious liver damage.
In most people with primary sclerosing cholangitis, the disease progresses slowly and can lead to liver failure, repeated infections, and tumors of the bile duct or liver. Liver transplant is the only known cure for primary sclerosing cholangitis.
The search for other treatments to slow or stop primary sclerosing cholangitis is ongoing, and scientists have turned up many promising leads. Until better treatments are proved safe and effective, though, care for primary sclerosing cholangitis focuses on monitoring liver function, managing symptoms and, when possible, doing procedures that temporarily open blocked bile ducts.
CAUSES
It’s not clear what causes primary sclerosing cholangitis. An immune system reaction to an infection or toxin may trigger the disease in people who are genetically predisposed to it.
A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn’s disease. Primary sclerosing cholangitis and inflammatory bowel disease don’t always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. Somewhat less often, people being treated for inflammatory bowel disease turn out to have primary sclerosing cholangitis as well. And rarely, people with primary sclerosing cholangitis develop inflammatory bowel disease only after having a liver transplant.
SYMPTOMS
Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities.
Early symptoms often include:
- Fatigue
- Itching
Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years, but there’s no reliable way to predict how quickly or slowly the disease will progress for any individual. Signs and symptoms that may appear as the disease progresses include:
- Pain in the upper right part of the abdomen
- Fever
- Chills
- Night sweats
- Enlarged liver
- Weight loss
- Yellow eyes and skin (jaundice)
When to see a doctor
Make an appointment with your doctor if you have severe, unexplained itching on much of your body — itching that persists no matter how much you scratch. Also see your doctor if you feel extremely tired all the time, no matter what you do.
It’s particularly important to bring unexplained fatigue and itching to your doctor’s attention if you have ulcerative colitis or Crohn’s disease, both of which are types of inflammatory bowel disease. A majority of people with primary sclerosing cholangitis also have one of these diseases.
