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About the education tab | All about the liver and liver disease | Liver diseases | Education | Autoimmune Liver Disease (including sclerosing cholangitis)
Autoimmune Liver Disease (including sclerosing cholangitis)
Autoimmune Liver Disease (including sclerosing cholangitis)
Autoimmune diseases are caused by the body's defence mechanism, the immune system, malfunctioning and attacking part of itself. There are two types of autoimmune liver disease: autoimmune hepatitis and autoimmune sclerosing cholangitis, both of which probably belong to the same disease group and are therefore treated similarly. It may be helpful to describe either condition as autoimmune liver disease to family and friends, as the word hepatitis is often misunderstood. Autoimmune liver disease is rare and represents about 2% of the liver disorders seen in specialised paediatric liver centres. What is Autoimmune Hepatitis (AIH)? The name of this condition, when broken down, describes it well. Hepatitis: Inflammation of the liver cells (which are damaged and eventually destroyed)
Auto-immune: Caused by the body's defence mechanism, the immune system, malfunctioning and attacking part of itself - in this case, primarily the liver. The continuing cell destruction results in scarring of the liver and can lead to cirrhosis. It is not fully understood why the immune system malfunctions in this way but it is known that there is a genetically inherited tendency to develop autoimmune disorders (others include thyroiditis, diabetes, arthritis and colitis). Some unknown factor triggers the malfunction of the immune system, which then attacks the organs. Research suggests that the trigger of autoimmunity may be a viral infection, which may have happened a long time, even years, before the liver disease manifests itself. 70% of cases of autoimmune hepatitis (AIH) in children are girls. The condition can occur from the age of 6 months, is rare before 6 years, most common between 10 and 30 years.
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