Primary Sclerosing Cholangitis (PSC)

What is the illness?

Description

It is an inflammation of the major and middle bile ducts that may lead to liver cirrhosis and bile duct tumours.

Spread

It is considered a rare disease. There are no epidemiological data for Switzerland; it is estimated that there are about 800 patients. 60% of patients are male. Children are also affected; the disease in children is known as autoimmune sclerosing cholangitis (ASC).

How is it contracted and recognised?

Causes/infection

The cause is unknown. The current hypothesis is that the disease is triggered by unknown environmental factors in a genetically predisposed individual.

Symptoms

About half of patients have no symptoms at the time of diagnosis. The disease is diagnosed owing to the presence of altered liver values in blood tests. The most frequent symptoms are abdominal pains, itching, jaundice (yellow colouring of the skin and eyes).

Diagnosis

The diagnosis is carried out by blood tests and by a special type of magnetic resonance imaging of the liver which studies the bile ducts. This examination is carried out without contrast agents. In about 10% of patients the bile ducts affected are too small to be seen by magnetic resonance imaging and a liver biopsy is necessary to locate the disease.

How is it treated?

Terapie

Unfortunately there are still no drugs with proven efficacy for the treatment of PSC. Ursodeoxycholic acid (UDCA) has been studied, a drug without side effects and also used in the treatment of gallstones which has shown a probable benefit.  For this reason, UDCA is almost always used.  It is extremely important to carry out regular tests in order to monitor the progress of the disease, check that tumours are not being formed and, if necessary, prepare the patient for a liver transplant.

Associated diseases

They are extremely significant, particularly inflammatory diseases of the intestine that affect 80% of individuals with PSC.  Inflammation of the intestine, which may not cause any discomfort, should always be looked for by endoscopic examinations.  In fact, if the intestine is inflamed the liver will also be inflamed.  On the other hand, if the inflammation of the intestine is controlled by the various drugs available, the inflammation of the liver is also reduced.