Kids Health Info

Auto-immune hepatitis

  • 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 starts to work too much and gets things wrong. It sees a part of the body as foreign and starts to destroy it. This is called an auto-immune disease.

    Auto-immune hepatitis (AIH) is when the immune system attacks the liver. The liver cells become damaged and inflamed (hepatitis). It is a long term (chronic) 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 better the outcome for the patient. 

    AIH used to be called lupoid hepatitis.

    Types of Auto Immune Hepatitis

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

    Type 1 Auto Immune Hepatitis

    • Is more common.
    • Can occur at any age.
    • Is more common in girls than boys.
    • Can be associated with other conditions such as arthritis or ulcerative colitis.

    Type 2 Auto Immune Hepatitis

    • Type 2 auto immune hepatitis can be more severe.

    Signs and symptoms

    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
    • aching and 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 or grey coloured stools (poo)

    Causes

    There is no known cause for AIH. Many autoimmune diseases seem to be triggered by a viral infection, as well as drugs.

    How is it diagnosed?

    Blood tests are done to look at how the liver and the immune system are working. A small piece of liver can be taken and examined under a microscope (a liver biopsy). It may take sometime for AIH to be diagnosed as several other diseases have the same signs and symptoms as AIH, such as viral hepatitis.

    Treatment

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

    • The first is a corticosteroid called prednisolone. It is often given in high doses at first and reduced over a period of time, the aim being to use the lowest dose that keeps the disease under control.
    • The second is azathioprine. This also works on slowing the immune system and can help to decrease the amount of medication that needs to be used.
    • There are several other medications that can be used if these two do not work.
    • A small number of people can get severe liver disease which means they may need a liver transplant.

    Side effects

    Prednisolone has several side affects that need to be watched for, 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

    Follow-up

    Ongoing follow up with a gastroenterologist will be needed for several years. Blood tests that look at how the liver is working will also need to be done long term.

    Key points to remember

    • AIH happens when the body attacks the liver cells and causes damage to the liver.
    • It can be treated with a mixture of medicines.
    • The earlier it is found the better the treatment works.
    • 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.

    More information

     

    Developed in consultation with the RCH Department of Gastroenterology. First published in March 2007. Updated May 2014.


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.