Autoimmune Hepatitis (AIH)
Autoimmune Liver Diseases (AILD)
Autoimmune hepatitis (AIH) is a rare autoimmune liver disease. The immune system of patients with AIH mistakenly targets the body’s own liver cells causing damage and inflammation. AIH can be diagnosed at any age and affects more females than males. The causes are still unknown. Research suggests that some people have a genetic predisposition to AIH and that the onset of the disease has a trigger, but it is not something that the patient has done.
AIH can develop slowly in some individuals, with no symptoms or very mild, vague symptoms. In a few people, AIH develops rapidly and hospitalisation is required. In around a third of patients, severe liver damage (liver cirrhosis) may have already developed by the time the patient is diagnosed. If the colour of your skin or the white area of your eye turns yellow, you should immediately see your doctor because this a warning sign that the function of your liver is reduced.
There are not any specific tests that tell a doctor you have AIH. It is actually a matter of piecing together results (both positive and negative) from a range of tests (including blood tests and liver biopsy). If you have AIH, you are likely to have higher levels of the following in your blood (all indicating liver inflammation)
- Aspartate aminotransferase (AST) / Glutamate-oxaloacetate transaminase (GOT)
- Alanine aminotransferase (ALT) / Glutamate-pyruvate transaminase (GPT)
- Immunoglobulin G (IgG)
In addition, you may test positive for autoantibodies, especially antinuclear antibodies (ANA), anti-smooth muscle antibodies (anti-SMA), anti-soluble liver antigen/liver pancreas antigen antibodies (anti-SLA/LP) and/or anti-liver-kidney microsome antibodies (anti-LKM-1).
Liver biopsy is important to confirm the diagnosis, to determine the severity of liver damage, and to rule out other liver diseases. It involves a needle puncture through the skin with removal of a tiny piece of the liver to be examined under a microscope. Most hospitals perform liver biopsy using ultrasound to identify the exact location of the liver. Local anaesthetic is injected in the area of the lower, right ribs, and for children and teenagers, general anaesthesia/sedation is offered. There is a small risk of bleeding after any liver biopsy and your doctor will discuss the benefits and risks of this procedure with you.
Your doctor will put all the pieces together (blood test results, liver biopsy, exclusion of other liver diseases) and will tell you whether you have AIH or not.
To control inflammation in the liver, the standard treatment for AIH consists of a combination of medications which include corticosteroids (e.g. prednisolone) and immunosuppressants (e.g. azathioprine). Corticosteroids work quickly and help to control liver inflammation immediately. Azathioprine allows the reduction of steroid doses, potentially allowing steroids to be stopped all together in some patients and helps to suppress the inflammation of the liver in the long term. Your doctor will adjust your medication doses over time to minimise side effects and maximise long term treatment success.
AIH medications are effective if used correctly, but can cause side effects, for example, nausea. It is important that you take all medications as prescribed and that you discuss any side effects with your doctor immediately so that different treatments or doses can be considered. It is important not to stop or alter the dose of your medications without consulting your doctor. This can cause disease reactivation with additional scar forming in the liver (liver cirrhosis).
Autoimmune hepatitis in children and teenagers
AIH can occur in any age group, even in babies and it differs in some ways from adult AIH. In childhood and adolescence it often responds well to therapy and in most cases, AIH should not limit the future possibilities for your child. However, AIH can be aggressive in children and that is why a child with AIH should always be treated by doctors at a specialised liver centre.
The diagnosis of AIH in children and teenagers can be challenging. One in five young patients also have another autoimmune disease such as inflammatory bowel disease, celiac, thyroid, and rheumatic disease. Occasionally, the bile ducts can also be affected, and your child’s doctor should check them with a special type of MRI scan called an MRCP. Both the pattern of the antibodies (called autoantibodies) in the blood and the aspect of liver inflammation seen on the liver biopsy may be different than in adults.
Children and teenagers will be given general anaesthesia/sedation when having liver biopsies. Young children (usually up to 6 or 7) are offered anaesthesia or sedation when having MRI scans.
Treatment of AIH in children and teenagers
- AIH in children and teenagers responds well to treatment. Treatment strategies consider the fact that the child is growing, and the dose or medication may change over time.
- Both the disease itself and side effects of the treatment with corticosteroids might affect the child’s growth and puberty development. Growth and puberty development should be evaluated at regular intervals by the treating physician.
- Treatment with corticosteroids might cause weight gain and aesthetic complications such as striae and (worsening of) acne in teenagers. This can be particularly distressing for the patient and requires special attention.
- Due to their young age, children are more prone to develop infections compared to adults. The treatment for AIH can lower the normal immune response of the child. When a child treated for AIH spikes a temperature or gets ill, seek a doctor’s advice quickly.
- It is important that patients continue to take their prescribed medicines to avoid relapse, especially during adolescence. In adolescents, this can be a challenge, requiring a multidisciplinary approach involving medical doctors, paramedics and psychologists familiar with the care of teenagers and young adults.
- The long-term risk factors include the amount of scarring in the liver, the liver’s ability to regenerate during treatment and the overall response to treatment. Liver transplantation is an option for some patients who have severe scarring in the liver. Talk to your paediatrician about the long-term risk for your child and how it will be monitored.
- Vaccinations: Your child’s vaccination programme should be reviewed by your treating physician. This is because special recommendations exist when it comes to immunisation in children and teenagers taking immunosuppressive treatment. Ask your child’s doctor how discomfort from injections (such as anxiety and pain) can be minimised.
Lay Summary of the ERN RARE-LIVER Position Paper: Second-line and Third-line Therapy for Autoimmune Hepatitis, available in EN, DE and HU here