Autoimmune Hepatitis

What does the liver do?

• Storing glycogen (fuel for the body) which is made from sugars. When required, glycogen is broken down into glucose which is released into the bloodstream.
• Helping to process fats and proteins from digested food.
• Making proteins that are essential for blood to clot (clotting factors).
• Processing many medicines which you may take.
• Helping to remove or process alcohol, poisons and toxins from the body.
• Making bile which passes from the liver to the gut down the bile duct. Bile breaks down the fats in food so that they can be absorbed from the bowel.

What is autoimmune hepatitis?

Hepatitis means inflammation of the liver. There are many causes of hepatitis. For example, alcohol excess and infections with various viruses are the common causes of hepatitis.

Autoimmune hepatitis is an uncommon cause of chronic hepatitis. Chronic means that the inflammation is persistent or long-term. The chronic inflammation gradually damages the liver cells which can result in serious problems.

What causes autoimmune hepatitis?

The cause is not clear. It is thought to be an autoimmune disease. Our immune system normally defends us against infection from bacteria, viruses and other foreign 'germs'. In people with autoimmune diseases the immune system attacks part or parts of the body as if it were 'foreign'. This causes inflammation and may damage the affected part or parts of the body.

The immune system includes white blood cells and antibodies (special proteins) which attack and destroy foreign germs and materials. There are various different autoimmune diseases. The symptoms of each disease depend on which part or parts of the body are attacked by the immune system. In some autoimmune diseases it is antibodies which do the damage. In autoimmune hepatitis, it is white blood cells called lymphocytes which are mainly involved. The lymphocytes attack the liver cells (hepatocytes) which causes inflammation and damage.

It is not known why autoimmune hepatitis or other autoimmune diseases occur. Some factor may trigger the immune system to attack the body's own tissues. There are various theories as to possible triggers. For example, a virus, a chemical or some other factor may trigger the disease. There is also an inherited factor which makes some people more prone to autoimmune diseases. (That is, you may need to be 'genetically susceptible' for a 'trigger' to set off the disease.)

Other autoimmune diseases include rheumatoid arthritis, myasthenia gravis and some forms of diabetes.

Who gets autoimmune hepatitis?

Autoimmune hepatitis is uncommon. It affects around 1 in 10,000 people in the UK. About 7 in 10 cases are in women. It most commonly develops in women aged around 45 years but it can affect people at all ages (including children and the elderly). People with autoimmune hepatitis have a greater chance than normal of also having one or more other autoimmune diseases.

What are the symptoms of autoimmune hepatitis?

In many cases the symptoms develop gradually over weeks or months. At the beginning of the disease, many people have no symptoms at all. The most common early symptoms include feeling more tired than usual, feeling generally unwell and also joint and muscle pains which are usually worse in the mornings.

Jaundice can develop when the liver inflammation becomes worse. Jaundice is when you 'go yellow'. You tend to notice it first when the whites of the eyes become yellow. This is due to a build-up of the chemical bilirubin which is made in the liver and spills into the blood in some liver conditions. (With jaundice due to hepatitis your urine goes dark, your faeces (stools) may go pale and and you tend to itch.)

Without treatment, in time the persistent inflammation causes liver damage and can lead to cirrhosis. Cirrhosis is like a 'scarring' of the liver which can cause serious problems and 'liver failure' when it is severe. See separate leaflet called 'Cirrhosis' for more detail.

In some cases, the symptoms develop quickly over a few days with an 'acute hepatitis'. (Acute means sudden onset or 'short duration'.) This can cause a fairly sudden onset of fever, abdominal pain, jaundice, feeling sick, vomiting and feeling unwell. In some of these cases, the 'acute' hepatitis settles down to the more common type of chronic hepatitis. In some cases the 'acute hepatitis' becomes severe and rapidly leads to liver failure.

If left untreated, the time it takes from the onset of the condition until severe cirrhosis develops varies from person to person. However, with treatment, the inflammation can usually be controlled and the outlook is very good.

How is autoimmune hepatitis diagnosed?

Many people with autoimmune hepatitis have no or only vague symptoms for quite some time in the early stages of the disease. Therefore, the diagnosis is often made when you have tests for an unrelated condition. When symptoms suggest that you have hepatitis, your doctor will normally arrange tests to confirm hepatitis and to find the cause of the hepatitis. Tests usually include:

• Blood tests called liver function tests. These measure the activity of enzymes (chemicals) and other substances made in the liver. This gives a general guide as to whether the liver is inflamed, and how well it is working. See separate leaflet called 'Blood Test - Liver Function Tests' for more detail. These tests can confirm that you have hepatitis but not usually the cause of the hepatitis.
• An ultrasound scan of the liver may be performed.
• Other blood tests can measure various auto-antibodies which occur in various autoimmune diseases. For example, antinuclear antibodies (ANAs) or smooth muscle antibodies (SMAs). Some of these auto-antibodies are raised in autoimmune hepatitis and indicate that this may be the cause of the hepatitis.
• A biopsy (small sample) of the liver may be taken to look at under the microscope. This can show inflammation and the extent of any cirrhosis in the liver. See separate leaflet called 'Biopsy - Liver' for more detail. The type of cells involved in the inflammation usually help to confirm the diagnosis of autoimmune hepatitis, and to rule out other causes of hepatitis.

Various blood tests and other tests may be done to rule out other liver disorders such as viral hepatitis.

What is the treatment for autoimmune hepatitis?

Treatment for autoimmune hepatitis is almost always needed. Early treatment can improve symptoms, reduce the risk of complications, and also greatly improve your outlook. Treatment aims to reduce inflammation and suppress the immune system with 'immunosuppressant' drugs:

• Steroid medication (usually prednisolone) is the usual first treatment. Steroids are good at reducing inflammation. A high dose is usually needed at first. The dose is then gradually reduced over a few weeks. The aim is to find the lowest dose needed to control the inflammation. The dose needed varies from person to person. See separate leaflet called 'Steroid Tablets' for more detail.
• Azothiaprine is an immunosuppressant drug that works in a different way to steroids. It is usually used in addition to the steroid. It is often not started until the inflammation has been brought under control with the steroids. A steroid plus azothiaprine tends to work better than either alone. Also, the dose of steroid needed is usually less if you also take azothiaprine. This means that any side-effects from steroids may be less severe.

Treatment works well in most cases. Usually, the inflammation settles and symptoms improve within a few months of starting treatment. However, it may take a year or more to get the disease totally under control. Azothiaprine is usually given for at least three years.

Side-effects from the treatment may be troublesome in some cases. The dose of treatment is kept as low as possible to keep the condition under control with the minimum of side-effects. If you cannot tolerate the azothiaprine and/or steroids then alternative treatment may be given. This may include ciclosporin or tacrolimus.

Once the condition has completely settled, your doctor may advise a trial without treatment. A liver biopsy is usually performed at the end of the course of treatment to ensure that the liver inflammation has fully resolved. However, only around one in five people can remain off treatment for long periods of time. This is because the hepatitis usually returns (relapses) which means you will have to then take medication again. In many cases, long-term 'maintenance' treatment is needed. This means that the majority of people with autoimmune hepatitis will need to take low doses of immunosuppressant treatment for long periods of time, or even indefinitely.

For the few people who do not respond to drug treatment, or who are diagnosed in the late stage of the disease with severe cirrhosis or liver failure, a liver transplant may be an option. The long-term outlook after liver transplantation is good.

Diet and alcohol

Most people with autoimmune hepatitis will be advised to eat a normal healthy diet. Ideally, anybody with inflammation of the liver should not drink alcohol, or only in very small amounts. If you already have liver inflammation, alcohol may increase the risk and speed of developing cirrhosis.

What is the outlook?

With treatment, most people with autoimmune hepatitis have a normal life expectancy and feel well most of the time. The treatment used for autoimmune hepatitis has improved the outlook tremendously. It is very important that you do not stop your treatment too early without your doctor's knowledge, as your hepatitis may return.

The treatment of autoimmune hepatitis is a developing area of medicine. New treatments continue to be developed and researched and the information above is very general. The specialist who knows your case can give more accurate information about the outlook for your particular situation.

Further help and information

British Liver Trust

2 Southampton Road, Ringwood BH24 1HY
Tel: 0800 652 7330 Web: www.britishlivertrust.org.uk

Autoimmune Hepatitis Support Group

Web: www.autoimmunehepatitis.co.uk