What Is Autoimmune Hepatitis (AIH)?

If your doctor says you have autoimmune hepatitis (AIH), it means your immune system -- the body's main defense against germs -- starts to attack your liver cells. The result: a liver disease that you need to keep tabs on throughout your life.

There's no cure for autoimmune hepatitis, but the right treatment lets you manage your symptoms and prevent damage to your liver.

Who Gets AIH?

There are two major types of autoimmune hepatitis, and both are rare. Type 1 is the more common of the two. You're more likely to get it if you're a woman age 15 to 40, although people of any age or gender can get it. Type 2 usually happens to girls age 2 to 14.

If you have AIH, there's a good chance you also have another autoimmune disease, such as Crohn's disease, rheumatoid arthritis, lupus, or Sjogren's syndrome.

What Causes It?

Doctors aren't sure exactly what causes your immune system to turn against your liver. Your genes may have something to do with it, since AIH can run in families.

But genes aren't the whole story. Experts believe that something you come into contact with may prompt your genes to put AIH in motion.

Some possible triggers are:

Symptoms

If you have AIH, it's possible you may have no symptoms. If you do, they can be mild or severe.

Sometimes symptoms like fever, belly pain, and yellowing of the skin and eyes come on suddenly. More often, though, your symptoms show up gradually over weeks or months.

The most common sign of AIH is feeling tired. You may also notice problems like:

Sometimes, AIH can cause liver damage called cirrhosis. If this happens to you, you may also have some of these symptoms:

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Diagnosis

Your doctor will ask you about your symptoms and how long you've had them. They'll also want to know what medications you take and how much alcohol you drink. Both can damage your liver.

They'll order blood tests that can rule out other causes like viral hepatitis. Blood tests can also spot substances called autoantibodies, which suggest you've got an autoimmune disease. Other blood tests can tell if your liver has been damaged.

Your doctor will do a liver biopsy. They take out a small piece of your liver and look at the cells under the microscope.

Treatments

If you don't have any symptoms of AIH, your doctor may decide not to treat you right away. Instead, you'll get blood tests and liver biopsies now and then to make sure your liver is still healthy.

Treatment usually starts once you have symptoms or your doctor notices that your lab test results are getting worse. At first your doctor will likely suggest you take prednisone, a steroid that lessens inflammation. They might start you on a high dose, then lower it and add azathioprine (Imuran) or 6-mercaptopurine (Purinethol), which curbs your immune system.

Both drugs can have side effects. Steroids can make your bones weak, cause you to add pounds, and give you eye problems. Azathioprine and 6-mercaptopurine can lower the number of your white blood cells and raise your chances of getting cancer.

Sometimes doctors use the steroid budesonide instead of prednisone. Your doctor may suggest other drugs that curb your immune system instead of azathioprine, such as mycophenolate mofetil (CellCept).

After 3 years of treatment, 80% of people find their disease is under control. You may be able to stop treatment while your doctor keeps an eye on your health. If your symptoms come back, you'll start treatment again.

There are also lifestyle changes that can help keep you healthy. Eat healthy foods and plenty of fruits and vegetables, and stay away from alcohol. Don't take any drugs or supplements without checking first with your doctor.

Sometimes, if you get cirrhosis or liver failure, you may need a liver transplant. This is surgery to take out the diseased liver and replace it with a healthy one from a donor.

WebMD Medical Reference Reviewed by Neha Pathak, MD on September 21, 2017

Sources

SOURCES:      

Autoimmune Hepatitis Association: "What is Autoimmune Hepatitis? A brief overview," "What causes AIH?"

American Liver Foundation: "Autoimmune Hepatitis."

National Organization for Rare Diseases: "Autoimmune Hepatitis."

National Institute of Diabetes and Digestive and Kidney Disease: "Autoimmune Hepatitis."

World Health Organization: "What is hepatitis?"

UpToDate: "Patient education: Autoimmune hepatitis (Beyond the Basics)."

Medscape: "Autoimmune Hepatitis."

National Library of Medicine: "Acute Hepatitis."

British Liver Trust: "Autoimmune Hepatitis."

Genetic and Rare Disease Information Center: "Autoimmune hepatitis."

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