Autoimmune Hepatitis

By consultant Peter Ott, Aarhus University Hospital

What is an autoimmune disease?

The body has an immune defence which fights bacteria and viruses and keeps us healthy. Once in a while, the immune defence makes a mistake and attacks one of the body’s organs. That’s when you have what is called an autoimmune disease. Auto is Latin for “self”, so autoimmune means “immune to self”.

Autoimmune hepatitis

In the autoimmune hepatitis, the immune system mistakenly decides to try to combat the liver. Even though no virus is involved, the result is often hepatitis, which in many ways resemble the hepatitis contracted by virus infections. It may start with acute fever, joint pain, fatigue and jaundice. In other cases, it’ll be less obvious and the main symptom will be fatigue. Liver tests are high, especially transferases. There will also often be raised immune globulin levels. There are specific blood tests, which can add increased suspicion about autoimmune hepatitis, for example, the smooth muscle cell antibody. If these things are positive, and virus hepatitis and other possible causes of liver disease have been ruled out, the diagnosis autoimmune hepatitis is reached. In many cases, a liver biopsy is required though.

Are there other autoimmune diseases in the liver?

The immune system also plays a role in diseases such as primary biliary cirrhosis and primary sclerosing cholangitis, but blood samples and liver biopsies will look different. There are however things in between, so called transition stages, which can both have features of autoimmune hepatitis and one of the two other diseases. This may of course make a diagnosis and course of treatment more difficult to reach.

How is autoimmune hepatitis treated?

When it has been diagnosed, doctors will try to lower the immune system with medicine. To begin with, prednisolone is usually used in a relatively high dose, such as 40 mg per day. In most cases, the patient will see improvements in the matter of a few weeks. When the liver values start to normalise, the prednisolone dosage will gradually be reduced. Often, another medicine Imurel, which has other side effects than the prednisolone, is added in order to reach the most tolerable treatment. Prednisolone can cause a number of side effects that sound extreme. These are restlessness and insomnia, high appetite and weight gain, developing diabetes and increased blood pressure as well as increased loss of calcium in the bones. Not everyone gets these side effects though and medicine dosage is quickly reduced to amounts that hardly have any side effects. Few notice taking Imurel, but blood samples are required to test that kidney function and bone marrow are not affected. Overall, most patients feel healthy while treatment is ongoing, as soon as their liver values have dropped to lower levels.

How does the disease progress?

Once the blood samples have normalised, the patient will usually feel healthy. This might take 3-6 months. Changes to the liver take longer to normalise and you should be prepared for treatment lasting 2-3 years and often more. If blood tests have been normal for 2 years on a small dosage of medicine, doctors will try to slowly phase it out entirely, this is done by first slowly stopping prednisolone and then the Imurel treatment. It is worth it to take plenty of time and let the phase out take an extra year. Not all patients can be entirely free of medicine. Some relapse every time they try to phase out and for them, lifelong treatment is often best, since they can be stable on very low doses of medicine. Around 2/3 will relapse after phasing out. Relapse can happen several years after treatment has ceased. In most cases it will happen within the first six months, though. Patients with autoimmune hepatitis will need control check-ups, even after treatment has ended.

What are the prospects for the future with autoimmune hepatitis?

If the autoimmune hepatitis isn’t treated, a large group of patients will develop cirrhosis of the liver and become chronically and seriously ill from it. With medical treatment, most patients will recover completely and have a normal life expectancy. There are however some patients who, despite treatment, will experience a slow loss of liver function and who may eventually require a liver transplant.

Concurrent treatment

In some cases, treating diabetes, high blood pressure and loss of bone density, may be necessary.

What if the treatment doesn’t work?

In some patients, there isn’t a complete response after treatment with prednisolone and Imurel. In this case one can try newer drugs to inhibit the immune system. It’s a slightly more specialised treatment and the side effects of these drugs are different. In this way, it’s usually possible to control even the most stubborn cases of autoimmune hepatitis.

Who treats autoimmune hepatitis?

The treatment of autoimmune heptatitis is, in my opinion, best treated by doctors who have some experience in this type of treatment. In some cases, this will be the doctors at the local hospital, who are interested in the liver. According to the Danish Health Authority’s instructions on regional functions, it will be the two regional departments in Aarhus and Copenhagen that will handle the complicated cases where there is a combination of autoimmune heptatitis and other forms of hepatitis, or if Prednisolone and Imurel don’t work.