Chronic inflammation of the bile ducts (primary sclerosing cholangitis)

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What is it about ?

Primary sclerosing cholangitis (PSC) is an inflammation of the bile ducts that carry bile from the liver to the gallbladder and then to the intestine. Inflammation of these ducts can be caused by narrowing or obstruction (for example by gallstones) or bacteria.

But sometimes there is no clear cause, and it is called primary sclerosing cholangitis. This disease progresses slowly, and inflammation of the bile ducts causes scar tissue to form, which can prevent bile from flowing. As the bile cannot be evacuated, problems appear in the liver with the formation of scars in the liver.

In some people, changes in the bile ducts can cause bile duct cancer to develop.

PSC is frequently associated with inflammatory bowel disease (Crohn’s disease and ulcerative colitis), and, according to some indications, it is an autoimmune disease.

What is its frequency?

Primary sclerosing cholangitis (PSC) affects 5 to 10 in 100,000 people. Men are more often affected than women. PUC is found more frequently in some countries, for example in the Scandinavian countries.

It usually starts between the ages of 25 and 45, but can also start in childhood.

In every 100 people with this disease, 10 to 15 develop cancer of the bile ducts.

How to recognize it?

Usually there are no symptoms, and PSC is noticeable in blood test results that show abnormal liver tests.

Inflammation of the bile ducts, fever, weightloss, from itching and an jaundice are late signs of the disease. Often the person also experiences tired.

How is the diagnosis made?

The best exam is cholangiography and endoscopic retrograde pancreatography (ERCP). To carry out this examination, we use a camera equipped with a tube which is slipped through the mouth to the level of the opening of the bile duct in the intestine. There, the doctor can inject a contrast medium to visualize the course of the bile ducts. If necessary, a stent can be placed to allow bile to flow into the bile duct.

Often, an MRI of the abdomen as well as a colonoscopy (endoscopic examination of the large intestine). Sometimes a liver biopsy (a piece of tissue from the liver) is done.

What can you do ?

Do not drink alcohol if the liver is inflamed. Alcohol may accelerate the progression to liver cirrhosis. Cirrhosis of the liver is characterized by irreversible replacement of normal liver tissue with scar tissue.

Make sure you eat a healthy, varied diet rich in fiber. When the absence of bile leads to malabsorption of fats which are then evacuated with the stool (fatty stools), the doctor may suggest a specific diet, low in fat and more digestible.

What can your doctor do?

There is no cure for the disease. A number of medicines can slow it down or dissolve cholesterol stones (which are formed by the build-up of hardened cholesterol). Inflammation and other complications must be treated.

Primary sclerosing cholangitis (PSC) sometimes requires a liver transplant. It is used if there are signs of complications (recurring) or rapid progression of the disease.

People with PSC are closely followed every 3 to 6 months and need regular imaging tests, depending on the situation. They must also pass a bowel examination.

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