Autoimmune diseases

 

Autoimmune hepatitis (AIH) and sclerosing cholangitis are autoimmune diseases, that means that the body attacks its own cells. In other words, the liver is attacked by antibodies produced by the body.

Autoimmune hepatitis is more common in girls. The diagnosis is made based on a liver biopsy and on blood analyses that will reveal abnormal liver counts and the presence of certain antibodies in the blood. Treatment consists in slowing down the patient’s immune system in order to reduce the inflammation of the liver and thus to minimise the risks of cirrhosis in the long term. In general, immunosuppressive drugs such as prednisone and azathioprine (Imurek®) are administered.

Sclerosing cholangitis (PSC-primary sclerosing cholangitis) is more frequent in boys. It can be associated with inflammatory diseases of the intestine (Crohn’s disease, rectocolitis). The diagnosis is also established based on a liver biopsy and blood analyses (liver tests and tests for the presence of auto-antibodies). Imaging techniques are used as well in order to look for a dilation of the bile ducts (beading). In contrast to autoimmune hepatitis, sclerosing cholangitis does not respond to immunosuppressive drugs (steroids, azathioprine). So a wait-and-see attitude is adopted trying to avoid the complications of cholestasis: deficiency in fat-soluble vitamins and bone fragility. Due to an increased risk of cancer of the bile ducts, sclerosing cholangitis often is an indication for transplant.

In some cases, the child may present an ‘overlap syndrome’. The biopsy shows a picture comprised between the two diseases: the biopsy shows signs of AIH on the one hand and of PSC on the other hand. These children must then be followed up closely in order to limit the side effects of treatment when the drugs fail.

Dr. Valérie McLin

2011