About autoimmune liver disease
There are two types of autoimmune liver disease: autoimmune hepatitis and autoimmune sclerosing cholangitis. Autoimmune liver disease is rare and represents about 2% of the liver disorders seen in specialised paediatric liver centres.
What is autoimmune hepatitis?
Autoimmune hepatitis (AIH) is a condition in which the body’s defence or immune system malfunctions and attacks the liver, causing inflammation. 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. 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.
What are the signs and symptoms of autoimmune liver disease?
Children and young people vary greatly in the symptoms they have, ranging between those who are outwardly well and active through to those who are acutely ill and may also have signs of liver failure.
The most common symptoms are:
- Tiredness and generally feeling unwell
- Loss of appetite
- Deterioration of school performance
Other symptoms are
- A large liver or spleen being found on routine medical
- An abnormal liver function noted on blood tests taken for another reason
- The appearance or persistence of the symptoms
A diagnosis is often made by excluding all other causes of liver disease and the doctors finding:
- Abnormal liver function on blood tests and scans
- High level of immunoglobulins (a particular type of protein) in the blood and, in most cases, autoantibodies (antibodies to parts of the body). Both demonstrate malfunction of the immune system.
- Characteristic features on liver biopsy
- Normal bile ducts when examined by a special X-ray test.
What are the treatments for autoimmune hepatitis?
The main treatment is by immunosuppressive medicines, which make the immune system less active. They are used in an attempt to control the disease process. In some cases the malfunction eventually stops and treatment can be stopped.
The future for people with autoimmune hepatitis
Treatment length varies but it usually needs to be continued for a long time, often many years, with medication doses decreased over time in order to avoid side effects.
Want to know more?
Download CLDF's leaflet on autoimmune liver disease.
This is very similar to AIH, the only difference being that the immune system attacks not only the liver cells, but also the bile ducts inside and/or outside the liver. It is often referred to as simply sclerosing cholangitis.
Signs and symptoms to look for
Similar to AIH, with the exception that inflammation of the bowel (colitis) and diarrhoea are more common. A major symptom may be pruritus (itching).
How is sclerosing cholangitis diagnosed?
The diagnosis is made with blood tests, physical examination, liver biopsy and a special x-ray performed under general anaesthetic.
Treatment of sclerosing cholangitis
As with AIH, treatment is by immunosuppression medication, alongside medicine to improve bile flow if appropriate. If colitis is present, this will be treated with special medicines.
Want to know more?Download CLDF's leaflet on autoimmune liver disease.
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