Autoimmune liver disease

What are autoimmune diseases?

Autoimmune diseases are caused by the body’s immune system attacking itself.

An antibody is a protein, made by the immune system, which identifies and removes bacteria and viruses from the body. Sometimes, the antibody attacks the body’s own tissues instead of a bacteria or a virus. These are called autoantibodies.

It is not know exactly what causes the autoantibodies to act in this way. It is thought that there may be a number of causes which include:

  • A problem with the immune system
  • An individual’s genes
  • Environmental factors

In children there are two liver conditions caused by autoimmune disorders:

Autoimmune hepatitis (AIH)

and

AIH/sclerosing cholangitis overlap syndrome known as autoimmune sclerosing cholangitis (ASC)

These are autoimmune liver diseases. They are similar conditions and are treated similarly.

What is autoimmune hepatitis?

Hepatitis means inflammation of the liver. In autoimmune hepatitis the inflammation is caused by the immune system.

What is autoimmune sclerosing cholangitis?

Autoimmune sclerosing cholangitis is similar to autoimmune hepatitis but as well as hepatitis there is also damage to the bile ducts.

 

What are the symptoms of autoimmune liver disease?

It can be difficult for childhood autoimmune liver disease to be diagnosed because the symptoms are similar to many other liver disorders and they can vary greatly.

Some children and young people with autoimmune liver disease may appear well and active whilst others can be very ill and have signs of liver failure. Generally autoimmune liver disease in children and young people is more aggressive than it is in adults but treatment can be very successful.

The most common symptoms are:

  • Tiredness and generally feeling unwell
  • Loss of appetite

Other symptoms are:

  • Nausea (feeling sick) or being sick
  • Abdominal pain
  • Jaundice with dark urine and pale stools (poo)
  • Joint and muscle pain
  • Weight loss
  • Itching
  • Fever
  • Nose bleeds, bleeding gums and bruising easily
  • Amenorrhoea (delayed starting of periods or they stop once started)
  • Diarrhoea (bowel symptoms are more common in autoimmune sclerosing cholangitis)

Later symptoms which can appear as the condition progresses include:

  • A swollen abdomen (ascites)
  • Swelling, especially in the lower legs (oedema)
  • Irritability and confusion

 

How is autoimmune liver disease diagnosed?

If a child or young person is displaying the symptoms of autoimmune hepatitis there are a number of tests which are used to confirm a diagnosis:

  • Blood tests
  • Liver biopsy
  • Ultrasound scan
  • MRI-MRCP scan
  • Upper and lower gastrointestinal endoscopy if there are bowel symptoms

Find more information on liver tests and investigations.

Children with autoimmune liver disease usually have high immunoglobulin G levels (a type of antibody). They also usually have positive autoantibodies which help define the type of autoimmune liver disease they have.

 

What are the different types of autoimmune hepatitis?

There are two types of autoimmune hepatitis. Each type has different types of autoantibody present:

Type 1 = Anti-nuclear (ANA) and/or smooth muscle (SMA) antibodies.

Type 1 makes up two out of three of all cases of autoimmune hepatitis. It normally starts around puberty.

Type 2 = Liver kidney microsomal (LKM) antibodies.

Type 2 is mainly found in younger children.

Children who have autoimmune hepatitis are usually positive for ANA and/or SMA autoantibodies. Very few children will have LKM autoantibodies.

 

How is autoimmune liver disease treated?

Steriods

High doses of steroids are required when treatment starts. The dose will gradually be reduced depending on blood results and symptoms. The overall aim of treatment is to ensure the disease is treated using as little medicine as possible.

Side effects of steroids include:

  • Being more likely to pick up an infection
  • Increased appetite and weight gain
  • Acne
  • A risk of bone weakness
  • Behavioural changes
  • Slower growth
  • Diabetes in some individuals

 

Other medications

Other medicines can be prescribed for different conditions and side effects. Click here to download our Autoimmune Liver Disease leaflet for information on these.

 

It’s important to note that most children and young people respond well to treatment.

For young people the side effects of treatment can be distressing especially cosmetic changes such as weight gain. CLDF’s young people’s team  is here to support young people aged 11-24.

The psychology teams at the liver centres can also provide help and support. Get in touch to find out about the support available.

 

How long does treatment continue for?

This can depend on a variety of things but treatment usually has to be continued for a long time, normally years.

Remission is the aim of treatment. This means blood tests measuring liver function and inflammation are within a normal range.

 

Relapse

A relapse is where the disease becomes active again after remission. Relapses can occur suddenly, particularly during puberty. This may be picked up on blood tests or may be noticeable due to symptoms reappearing. Relapse can also happen when medicines have not been taken as prescribed.

It is really important that young people understand the importance of taking their medication properly, even if they feel ok.

During a relapse medicine dosages may need to be increased to bring the disease back under control.

Considering stopping treatment can only be done if there have been at least two years of completely normal blood tests and often only if a repeat liver biopsy shows that the inflammation in the liver has disappeared. If treatment is decreased, it is done very slowly.

About 20% of children/young people with type 1 autoimmune hepatitis who respond well to medication can eventually stop treatment.

Very few children/young people with type 2 autoimmune hepatitis can ever stop treatment.

 

How is autoimmune liver disease monitored?

Those with autoimmune hepatitis or autoimmune sclerosing cholangitis require regular blood tests.

Monitoring the condition is really important. Regular blood tests are taken to measure AST (aspartate aminotransferase) and ALT (alanine aminotransferase). These are enzymes which are normally present in the liver and their levels can be used to monitor the health of the liver.

When first diagnosed, testing may be done weekly to see how quickly the level of steroids can be reduced. As the condition stabilises, blood tests will be needed less often (every 3-6 months).

 

What happens if the disease progresses?

Complications of chronic liver disease include:

  • Weight loss/poor weight gain
  • Ascites (swelling of the tummy)
  • Portal hypertension (high blood pressure in the blood vessels around the liver)
  • Jaundice

If the function of the liver is affected by the disease then other medicines may be given. For example vitamin K may be given which helps the blood to clot.

Liver transplantation is considered in cases that don’t respond to medicine or when the liver is damaged beyond repair and the complications are life threatening. Rate of liver transplantation is higher in cases of autoimmune sclerosing cholangitis and recurrence of autoimmune liver disease may occur following liver transplantation, especially in cases with autoimmune sclerosing cholangitis.

Most children can lead a good quality of life whilst taking medication.

Find out more about liver transplantation.

Download CLDF’s leaflet on autoimmune liver disease

 

This website provides general information but does not replace medical advice. It is important to contact your/your child’s medical team if you have any worries or concerns