What is autoimmune hepatitis?

Autoimmune hepatitis is when your body’s infection-fighting system (immune system) attacks your liver cells. This causes redness and swelling (inflammation) and liver damage.

It is a long-term or chronic inflammatory liver disease.

There are 2 types of autoimmune hepatitis: type 1 (classic) or type 2.

What causes autoimmune hepatitis?

Experts don’t know what causes autoimmune hepatitis.

It is linked to a disorder called hypergammaglobulinemia. This disorder occurs when you have too many protein antibodies in your blood. It may be caused by a long-term (chronic) infection or certain blood diseases.

Type 1 autoimmune hepatitis is linked to other disorders where the body attacks itself (autoimmune disorders). These may include:

• Thyroiditis

• Grave's disease

• Type 1 diabetes

• Rheumatoid arthritis

• Hemolytic anemia

• Immune thrombocytopenia

• Celiac disease

• Ulcerative colitis

What are the symptoms of autoimmune hepatitis?

Each person’s symptoms may vary. Some people may have no symptoms or very mild ones, while others can have severe disease and symptoms. Some of the most common symptoms may include:

• Extreme tiredness (fatigue)

• Yellowing of the skin and eyes (jaundice)

• Belly (abdominal) pain

• Joint pain or swelling

• Mild flu-like symptoms

• Itching

• Large abdomen due to large liver and spleen

• Spiderlike blood vessels in the skin

Other autoimmune hepatitis symptoms may include:

• Dark urine

• Pale or gray-colored stools

• Stop in menstrual periods in women

• Loss of appetite

• Fluid buildup in the belly (ascites)

• Confusion

• Rectal bleeding or vomiting blood

The symptoms of autoimmune hepatitis may look like other health problems. Always see your healthcare provider for a diagnosis.

How is autoimmune hepatitis diagnosed?

Your healthcare provider will look at your health history and give you a physical exam.

Some lab blood tests used to diagnose autoimmune hepatitis include:

• Liver function tests. These check for any redness and swelling (inflammation) or damage to your liver.

• Complete blood count or CBC. This test looks at the number and types of cells in your blood.

• Coagulation panel. This test looks at how well the clotting proteins are working.

• Electrolyte panel. This test checks to see if you have too many or too few minerals (electrolyte imbalance) in your blood.

• Autoimmune antibodies. These are used to see if you have autoimmune hepatitis or another liver disease with similar symptoms.

• Other liver tests. These are done to check for other possible types of liver disease. 

• Tests for other chemicals in your body.

You may also have imaging tests, such as:

• CT scan. This test is more detailed than a standard X-ray. It can show detailed images of any part of the body, including the bones, muscles, fat, and organs. It uses both X-rays and computer technology to make horizontal images (often called slices) of the body.

• MRI. This test makes detailed pictures of organs and structures inside your body. It uses a magnetic field and pulses of radio wave energy. A dye may be shot or injected into your vein. The dye helps the liver and other organs in the belly to be seen more clearly on the scan.

• Ultrasound. This test uses high frequency sound waves to create a picture of the organs. It can also check blood flow in blood vessels.

• Liver biopsy. Small tissue samples are taken from your liver with a needle. These samples are checked under a microscope to find out the type of liver disease you have.

How is autoimmune hepatitis treated?

Treatment works best when autoimmune hepatitis is found early. The goal of treatment is to control the disease and to reduce or get rid of any symptoms (be in remission).

To do this, medicines (corticosteroids and immune system suppressors) are used to help slow down or suppress your overactive immune system. They also stop your body from attacking your liver.

Once you have started treatment, it can take 6 months to a few years for the disease to go into remission. Some people can stop taking medicine, but often the disease comes back. You may need treatment now and then for the rest of your life. Some people need to remain on treatment if they have relapsed many times or if their disease is severe.

In some cases, autoimmune hepatitis may go away without taking any medicines. But for most people, autoimmune hepatitis is a chronic disease.

It can lead to scarring of the liver (cirrhosis). The liver can become so badly damaged that it no longer works. This is called liver failure.

If you have liver failure, a liver transplant may be needed.

Be sure to ask your healthcare provider about recommended vaccines. These include vaccines for viruses that can cause liver disease.

Key points about autoimmune hepatitis

• Autoimmune hepatitis is when your body's infection-fighting system (immune system) attacks your liver cells.

• It is a long-term chronic liver disease that causes redness and swelling (inflammation) and liver damage.

• Experts don’t know what causes it.

• It affects more women than men.

• Medicines are often used to control the disease.

• It can lead to scarring of the liver (cirrhosis) and liver failure.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions.