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Kids Health Info

Autoimmune hepatitis (AIH)

  • The immune system's job is to fight and kill things that the body sees as foreign, such as bacteria and viruses. Sometimes, for an unknown reason, the immune system mistakes a part of the body as foreign and starts to attack it. This is called an autoimmune disease.

    Autoimmune hepatitis (AIH) is when the immune system attacks the liver. The liver cells become damaged and inflamed (hepatitis). It is a chronic (long-term) disease that can last for years. It can lead to cirrhosis (hardening and scarring) of the liver, which can get worse over time. 

    There are medications that can treat AIH, but there is no cure. The earlier it is diagnosed and treated, the more effective the treatment. AIH used to be called lupoid hepatitis.

    Types of autoimmune hepatitis

    There are two main types of AIH: type 1 and type 2.

    • Type 1 AIH is more common, can occur at any age, and is more common in girls than boys. It can be associated with other conditions such as arthritis, ulcerative colitis and sclerosing cholangitis.
    • Type 2 AIH can be more severe but is less common.

    Signs and symptoms of autoimmune hepatitis

    AIH symptoms resemble those of other forms of hepatitis, and sometimes are mistaken for the flu. Symptoms of AIH include:

    • fatigue
    • abdominal discomfort and an enlarged liver
    • jaundice (a yellowish colouring of the skin and eyes)
    • aching, painful joints
    • skin rashes and itching
    • abnormal looking blood vessels on the skin (spider naevi)
    • nausea, vomiting and loss of appetite
    • dark coloured urine and pale coloured stools (poo).

    When to see a doctor

    If your child has any symptoms of AIH, take them to the GP. Your child will be referred to a paediatric gastroenterologist (liver specialist).

    To diagnose AIH, blood tests are done to look at how the liver and the immune system are working. A liver biopsy (small sample of the liver taken with a fine needle) is often performed during a general anaesthetic (where your child is put to sleep) and examined under a microscope. It may take some time for AIH to be diagnosed as several other diseases have the same signs and symptoms as AIH, such as viral hepatitis.

    Treatment for autoimmune hepatitis

    Treatment is based on trying to slow down the immune system. There are two main types of medications that are used to do this:

    • Corticosteroids (prednisolone): this is often given in high doses at first and reduced over a period of time, the aim being to use the lowest dose possible that keeps the disease under control.
    • Azathioprine: this medicine can help to decrease the amount of steroid medication that needs to be used.

    There are several other medications that can be used and your child's gastroenterologist will discuss these with you. A small number of children can get severe liver disease, and they may need a liver transplant.

    Medication side effects

    Your child's gastroenterologist will also talk to you about any side effects of the medications. Prednisolone has several possible side effects that need to be monitored, including:

    • weight gain
    • anxiety and confusion
    • thinning of the bones (osteoporosis), hair and skin
    • diabetes and high blood pressure
    • cataracts and glaucoma.

    Azathioprine also has some side effects including:

    • poor appetite and nausea
    • decreasing the number of white blood cells, reducing a person's ability to fight infections.

    Follow-up

    Ongoing follow-up with a gastroenterologist will be needed for several years, and your child will need regular blood tests to check their liver function.

    What causes autoimmune hepatitis?

    There is no known cause for AIH. Many autoimmune diseases seem to be triggered by a viral infection or certain medications.

    Key points to remember

    • AIH happens when the body attacks the liver cells and causes damage to the liver.
    • The earlier it is diagnosed, the better the treatment works.
    • AIH can be treated with medication that slows down the immune system.
    • It is a chronic disease needing long-term specialist follow up.
    • There is no cure and a small number of people will need a liver transplant.

    For more information

    Common questions our doctors are asked

    What are the chances my child will need a liver transplant?

    This depends on how far advanced the disease is at diagnosis and the response to treatment. Overall the chances of needing a liver transplant in children with autoimmune hepatitis is very low.

    Is there any special diet that can improve my child's symptoms?

    Unfortunately, as diet is not involved in causing the disease or making it worse, only a healthy diet is recommended. There is no evidence to support a “liver cleansing” diet. 


    Developed by The Royal Children's Hospital Gastroenterology department. We acknowledge the input of RCH consumers and carers.

    Reviewed August 2018.

    This information is awaiting routine review. Please always seek the most recent advice from a registered and practising clinician.

    Kids Health Info is supported by The Royal Children’s Hospital Foundation. To donate, visit www.rchfoundation.org.au.


Disclaimer

This information is intended to support, not replace, discussion with your doctor or healthcare professionals. The authors of these consumer health information handouts have made a considerable effort to ensure the information is accurate, up to date and easy to understand. The Royal Children's Hospital Melbourne accepts no responsibility for any inaccuracies, information perceived as misleading, or the success of any treatment regimen detailed in these handouts. Information contained in the handouts is updated regularly and therefore you should always check you are referring to the most recent version of the handout. The onus is on you, the user, to ensure that you have downloaded the most up-to-date version of a consumer health information handout.