Auto-immune hepatitis (AIH) and sclerosing cholangitis are auto-immune diseases, which means that the body attacks its own cells.
In other words, antibodies produced by the body attack the liver.
How is it diagnosed?
Auto-immune hepatitis is more common in girls. The diagnosis is determined through a biopsy of the liver, which shows organ damage.
Blood test results are disrupted and can detect the presence of certain antibodies in the blood.Sclerosing cholangitis (PSC or primary sclerosing cholangitis) is more common in boys.
It can be associated with inflammatory bowel diseases (Crohn’s disease, ulcerative colitis).
The diagnosis is also determined through a liver biopsy and blood tests (presence of auto-antibodies). Imaging is also used to look for dilation of the bile ducts, which is typical of this disease (a so-called “rosary” image).
Unlike auto-immune hepatitis, sclerosing cholangitis does not respond to immunosuppressants (steroids, azathioprine).
So a wait-and-see attitude is adopted, during which it is important to avoid cholestasis complications: fat-soluble vitamin deficiency and bone fragility.
Due to an increased risk of cancer of the bile ducts, sclerosing cholangitis is often an indication for transplantation.
What are the treatments?
For auto-immune hepatitis, treatment consists of slowing down the patient's immune system in order to reduce inflammation of the liver, which will then minimize the risk of cirrhosis over the long term.
Usually, prednisone and azathioprine (Imurek®) are used as immunosuppressive drugs. Unlike sclerosing cholangitis, auto-immune hepatitis does not respond to immunosuppressants (steroids, azathioprine).
A strategy to monitor the complications from cholestasis is adopted: fat-soluble vitamin deficiency and bone fragility. Due to an increased risk of cancer of the bile ducts, sclerosing cholangitis is often an indication for liver transplantation.
In some cases, the child may have an overlap syndrome.
In the biopsy, one of two images are shown: the biopsy shows signs of AIH on the one hand and PSC on the other. It is important to monitor these children closely to limit the side effects of treatment if the medication does not work.