Primary sclerosing cholangitis is a disease of the bile ducts, which carry the digestive liquid bile from the liver to the small intestine. In the disease process in which the bile ducts in the liver become inflamed, narrow, scarred and prevent bile from flowing properly. Over time, this inflammation decreases blood flow within, increasing the pressure in the portal vein. The scars make the ducts hard and narrow and gradually cause serious liver damage. In most people with PSC, the disease progresses slowly and can lead to liver failure, repeated infections and tumors.

 

Symptoms

In its early stages, PSC is asymptomatic. 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. Thus, before they have symptoms, many people continue to feel generally well for several years, but there is no reliable way to predict how quickly or slowly the disease will progress. Early symptoms often include fatigue and itching.  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

 

Causes

It is not clear what causes PSC. It is thought that there may be an autoimmune component to the disease, where the body's immune system attacks the bile ducts in the liver and causes them to become, inflamed and narrowed. A large proportion of people with PSC also have inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn's disease. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. And rarely, people with primary sclerosing cholangitis develop inflammatory bowel disease only after having a liver transplant.

 

Risk Factors

The following categories of people are considered to e at greater risk of suffering from primary sclerosing cholangitis:

  • People at the age between 30 and 50.
  • Men rather than women.
  • People with inflammatory bowel disease.
  • People in the United States and Northern Europe have a higher risk of primary sclerosing cholangitis than do people in Asia and Southern Europe.

 

Complications

PSC is a progressive disease, damaging the bile ducts and reducing the bile flow, ultimately leading to portal hypertension, cirrhosis, and liver failure. Complications of primary sclerosing cholangitis may include:

  • Liver disease and failure;
  • Repeated infections;
  • Portal hypertension;
  • Thinning bones;
  • Bile duct cancer;
  • Colon cancer

 

Prevention

The specific cause of PSC is still unknown and therefore it seems not to be preventable. It is appropriate for patients with inflammatory bowel disease and their health care professional to be aware of the relationship with PSC, in case symptoms suggestive of the disease arise.