Definition & Facts

In this section:

• What is autoimmune hepatitis?
• What are the types of autoimmune hepatitis?
• How common is autoimmune hepatitis?
• Who is more likely to have autoimmune hepatitis?
• What other conditions do people with autoimmune hepatitis have?
• What are the complications of autoimmune hepatitis?

What is autoimmune hepatitis?

Autoimmune hepatitis is a chronic disease in which your body’s immune system attacks the liver and causes inflammation and liver damage. Without treatment, autoimmune hepatitis may get worse and lead to complications, such as cirrhosis and liver failure.

Autoimmune hepatitis is an autoimmune disease. Your immune system normally makes large numbers of antibodies and lymphocytes that help fight off infections. The normal immune system does not attack healthy cells in a person’s body. In autoimmune diseases, your immune system makes certain types of antibodies—called autoantibodies—and lymphocytes that attack your body’s own cells and organs.

What are the types of autoimmune hepatitis?

Experts have identified two types of autoimmune hepatitis: type 1 and type 2. The immune system makes different autoantibodies in each type. Type 1 autoimmune hepatitis is more common than type 2, in both adults and children. Only about 5% to 10% of people with autoimmune hepatitis have type 2, and type 2 most often develops during childhood. ^1,2

How common is autoimmune hepatitis?

Researchers aren’t sure how common autoimmune hepatitis is in the United States. Rates of autoimmune hepatitis vary in different parts of the world and in different age groups. Studies have found that about 4 to 43 out of 100,000 adults and about 2 to 10 out of 100,000 children have autoimmune hepatitis.³

Who is more likely to have autoimmune hepatitis?

Autoimmune hepatitis can occur at any age and affects people of all racial and ethnic groups.

The disease is more common in females than in males. Studies have found that 71% to 95% of adults with autoimmune hepatitis are women, and 60% to 76% of children with the disease are girls.³

What other conditions do people with autoimmune hepatitis have?

Some people who have autoimmune hepatitis may also have features of liver diseases that affect the bile ducts—the tubes that carry bile out of the liver—such as

• primary biliary cholangitis (PBC)
• primary sclerosing cholangitis (PSC)
• bile duct problems that can’t be classified as PBC or PSC

People with autoimmune hepatitis are at risk of having other autoimmune diseases. Examples include

• celiac disease
• thyroid conditions, such as Graves’ disease or Hashimoto’s disease
• rheumatoid arthritis
• type 1 diabetes
• inflammatory bowel disease, such as ulcerative colitis
• vitiligo
• lupus

What are the complications of autoimmune hepatitis?

Autoimmune hepatitis may lead to complications, but early diagnosis and treatment can lower your chances of developing them.

Early diagnosis and treatment of autoimmune hepatitis can lower your chances of developing complications.

Acute liver failure

Very rarely, autoimmune hepatitis can cause acute liver failure, a condition in which your liver fails rapidly without warning.

Cirrhosis

Many people have cirrhosis when they are first diagnosed with autoimmune hepatitis. In cirrhosis, scar tissue replaces healthy liver tissue and prevents your liver from working normally. Scar tissue also partly blocks the flow of blood through the liver. As cirrhosis gets worse, the liver begins to fail.

Liver failure

Cirrhosis may eventually lead to liver failure, also called end-stage liver disease. With liver failure, your liver is badly damaged and stops working. People with liver failure may require a liver transplant.

Liver cancer

Cirrhosis increases your chance of getting liver cancer. Your doctor may suggest blood tests and an ultrasound or another type of imaging test to check for liver cancer. Finding cancer at an early stage improves the chance of curing the cancer.

References

Symptoms & Causes

What are the symptoms of autoimmune hepatitis?

People with autoimmune hepatitis may have some of the following symptoms

• feeling tired
• joint pain
• nausea
• poor appetite
• pain over the liver, in the upper part of the abdomen
• yellowish color of the whites of the eyes and skin, called jaundice
• darkening of the color of urine
• lightening of the color of stools
• skin conditions, such as rash, psoriasis, vitiligo, or acne

If you have symptoms of autoimmune hepatitis, they can range from mild to severe.

Many people with autoimmune hepatitis have no symptoms. In such cases, doctors may find you have signs of liver problems during routine blood tests, and this may lead to a diagnosis of autoimmune hepatitis. People without symptoms at diagnosis may develop symptoms later.

Some people with autoimmune hepatitis don’t have symptoms until they develop complications due to cirrhosis. These symptoms include

• feeling tired or weak
• losing weight without trying
• swelling of the abdomen from a buildup of fluid, called ascites
• swelling of the lower legs, ankles, or feet, called edema
• itchy skin
• jaundice
• vomiting blood if cirrhosis leads to enlarged veins in the esophagus, called esophageal varices, and those varices burst
• confusion or difficulty thinking if cirrhosis leads to a buildup of toxins in the brain, called hepatic encephalopathy

What causes autoimmune hepatitis?

Experts aren’t sure what causes autoimmune hepatitis. Studies suggest that certain genes make some people more likely to develop autoimmune diseases. In people with these genes, factors in the environment may trigger an autoimmune reaction that causes their immune system to attack the liver.

Researchers are still studying the environmental triggers that play a role in autoimmune hepatitis. These triggers may include certain viruses and medicines.

Some medicines can cause liver injury that resembles autoimmune hepatitis. In most cases, the liver injury goes away when the medicine is stopped. Telling your doctor the names of all the medicines you take, even over-the-counter medicines or herbal or botanical products, is important.

Diagnosis

How do doctors diagnose autoimmune hepatitis?

Doctors diagnose autoimmune hepatitis based on a combination of information from your medical history, a physical exam, and tests.

Medical history

Your doctor will ask about your symptoms and other factors that could be damaging your liver. For example, your doctor may ask about any medicines and herbal or botanical products you take and how much alcohol you drink. Your doctor will ask you about other autoimmune diseases you might have, such as inflammatory bowel disease or thyroid conditions.

Physical exam

During a physical exam, the doctor will check for signs of liver damage, such as

• yellowish color of the whites of the eyes
• changes in the skin
• enlargement of the liver or spleen
• tenderness or swelling in the abdomen, or belly
• swelling in the lower legs, feet, or ankles, called edema

What tests do doctors use to diagnose autoimmune hepatitis?

Your doctor may order blood tests, imaging tests, and a liver biopsy to diagnose autoimmune hepatitis. No single test can diagnose autoimmune hepatitis. In most cases, doctors order a combination of tests, including a liver biopsy, to make a diagnosis.

Blood tests

Your doctor may order one or more blood tests to look for signs of autoimmune hepatitis or other liver diseases. A health care professional will take a blood sample from you and send the sample to a lab.

Liver tests

Liver tests can check levels of the liver enzymes alanine aminotransferase (ALT) and aspartate aminotransferase (AST). ALT and AST are particularly important because these liver enzymes are highly elevated in people with autoimmune hepatitis. Doctors check ALT and AST levels to follow the progress of the disease and how it responds to treatment.

Antibody tests

Doctors order antibody tests to check for autoantibodies—antibodies that attack your healthy tissues and cells by mistake—such as antinuclear antibody (ANA) and anti-smooth muscle antibody (SMA). Doctors may also order blood tests to check levels of a type of protein called immunoglobulin G (IgG).

Other blood tests

Doctors order additional blood tests to look for other liver diseases that have symptoms similar to autoimmune hepatitis, such as viral hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, nonalcoholic steatohepatitis (NASH), or Wilson disease.

Doctors use blood tests to look for signs of autoimmune hepatitis or other liver diseases.

Imaging tests

Your doctor may order imaging tests of your abdomen and liver.

Ultrasound

Ultrasound uses a hand-held device, called a transducer, that bounces safe, painless sound waves off organs to create images of their structure. An ultrasound can show whether the liver is enlarged, has an abnormal shape or texture, or has blocked bile ducts.

Computed tomography (CT)

CT uses a combination of x-rays and computer technology to create images. A CT scan can show the size and shape of the liver and spleen and whether there are signs of cirrhosis.

Magnetic resonance imaging (MRI)

MRI uses radio waves and magnets to produce detailed images of organs and soft tissues without using x-rays. An MRI scan can show the shape and size of the liver and detect evidence of cirrhosis. Special MRI scans can estimate the amount of fat and scarring in the liver.

Liver biopsy

During a liver biopsy, a doctor will take a piece of tissue from your liver. A pathologist will examine the tissue under a microscope to look for the amount of damage and features of specific liver diseases. A doctor can use a liver biopsy to look for the features of autoimmune hepatitis and to check the amount of scarring to find out if you have cirrhosis.

Treatment

How do doctors treat autoimmune hepatitis?

Doctors treat autoimmune hepatitis with medicines that suppress—or decrease the activity of—your immune system, reducing your immune system’s attack on your liver. The medicines doctors most often prescribe are

• corticosteroids, also called steroids
• immunosuppressants

Doctors typically start with a relatively high dose of corticosteroids and then gradually lower the dose. The medicines used to treat autoimmune hepatitis can cause side effects, so your doctor will try to find the lowest dose that works for you.

Your doctor will use blood tests to check levels of the liver enzymes alanine aminotransferase (ALT) and aspartate aminotransferase (AST). If your ALT and AST levels drop, it means you are responding to the medicines.

Treatment can relieve symptoms and prevent or reverse liver damage in many people with autoimmune hepatitis. Early treatment of autoimmune hepatitis can lower the chances of developing cirrhosis and other complications. A minority of people with autoimmune hepatitis, including some people who have a mild form of the disease or increased risks related to the treatment, may not need medicines.

Remission

With treatment, you may experience remission. Remission is a period when you don’t have any symptoms and your test results show that your liver is working better and is no longer being damaged. Many people with autoimmune hepatitis go into remission. If the first medicines doctors prescribe don’t bring on remission, doctors may prescribe other medicines.

ALT and AST levels falling to normal is a sign of remission. If you are in remission, your doctor may gradually lower the dose of medicines again to help reduce the medicines’ side effects. After you stay in remission for at least 2 years, your doctor may try to stop your medicines to see if you remain in remission without them.⁴

Your doctor will continue to perform routine blood tests for ALT and AST and monitor your symptoms while you are in remission to check for a relapse. Your doctor may suggest a repeat liver biopsy to monitor liver damage and guide management.

Autoimmune hepatitis is often a long-term, if not lifelong, condition. Your doctor will need to watch your condition carefully, particularly when treatment is stopped, because the liver damage may return quickly and may be severe. Stopping treatment without your doctor’s guidance and monitoring may be very dangerous.

Relapse

Some people with autoimmune hepatitis stay in remission without medicines. However, most people relapse after stopping medicines and need to start taking medicines again. When you relapse, blood tests show a rise in ALT and AST levels, and autoimmune hepatitis begins causing symptoms or damaging your liver again.

If you relapse, your doctor will restart or adjust your medicines to treat autoimmune hepatitis.

Incomplete or failed response to treatment

Some people with autoimmune hepatitis have

• an incomplete response to treatment, meaning the treatment helped but did not lead to remission
• a failed response to treatment, meaning the inflammation and liver damage of autoimmune hepatitis keep getting worse despite treatment

If you have an incomplete or failed response to treatment, your doctor may change the medicines you take to treat autoimmune hepatitis. If the disease continues to damage your liver, you may develop complications and need additional treatments.

Do medicines used to treat autoimmune hepatitis have side effects?

Medicines for autoimmune hepatitis can cause side effects. Your doctor will monitor any side effects and help you manage them while you take these medicines. Your doctor also may adjust the doses or change the medicines you take. You may need to stop taking medicines if you have severe side effects.

Doctors treat autoimmune hepatitis with medicines that suppress your immune system.

Side effects of corticosteroids may include

• changes in how you look, which may include weight gain, a fuller face, acne, or more facial hair
• diabetes
• eye problems, such as cataracts or glaucoma
• high blood pressure
• loss of bone density, called osteopenia
• mental health problems, such as extreme changes in mood or psychosis
• pancreatitis

Side effects of azathioprine, an immunosuppressant that many doctors prescribe to treat autoimmune hepatitis, may include

• low white blood cell count
• nausea or vomiting
• skin rash
• liver damage
• pancreatitis

Corticosteroids and immunosuppressants suppress, or decrease the activity of, your immune system, which increases your risk for infections. These medicines can also increase your risk of developing cancers, especially skin cancers.

Before you start taking medicines to treat autoimmune hepatitis, your doctor may

• make sure you have had recommended vaccines, including vaccines for hepatitis A and hepatitis B.
• order tests to check for hepatitis B infection. In people with a current or past hepatitis B infection, medicines used to treat autoimmune hepatitis may cause hepatitis B reactivation. In some cases, doctors may recommend medicines to prevent hepatitis B reactivation.
• order a genetic test for thiopurine S-methyltransferase (TPMT) deficiency. When the TPMT enzyme doesn’t work the way it should, people have a higher risk for serious side effects from certain immunosuppressants.

How do doctors treat the complications of autoimmune hepatitis?

If autoimmune hepatitis leads to cirrhosis, doctors can treat the related health problems and complications with medicines, medical procedures, or surgery.

If you have cirrhosis, you have a greater chance of developing liver cancer. Your doctor may suggest blood tests and an ultrasound or another type of imaging test to check for liver cancer. Finding cancer at an early stage improves the chance of curing the cancer.

If autoimmune hepatitis causes acute liver failure, or if cirrhosis leads to liver cancer or liver failure, you may need a liver transplant.

Reference

Eating, Diet, & Nutrition

What should I eat if I have autoimmune hepatitis?

Researchers have not found that eating, diet, and nutrition play a role in causing or preventing autoimmune hepatitis.

If you have autoimmune hepatitis, you should eat a healthy, well-balanced diet. A healthy diet is also important if autoimmune hepatitis leads to cirrhosis. Find more tips on healthy eating when you have cirrhosis.

Using corticosteroids for a long time to treat autoimmune hepatitis can lead to a loss of bone density, called osteopenia, which can lead to osteoporosis. Doctors may recommend that people who take corticosteroids also take dietary supplements of calcium and vitamin D to help prevent osteoporosis. Follow your doctor’s instructions on the type and dose of supplements you should take.

Clinical Trials

The NIDDK conducts and supports clinical trials in many diseases and conditions, including liver diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.

What are clinical trials for autoimmune hepatitis?

Clinical trials—and other types of clinical studies—are part of medical research and involve people like you. When you volunteer to take part in a clinical study, you help doctors and researchers learn more about disease and improve health care for people in the future.

Researchers are studying many aspects of autoimmune hepatitis, including new treatments, especially for people who don’t respond to the current treatments.

Find out if clinical studies are right for you.

Watch a video of NIDDK Director Dr. Griffin P. Rodgers explaining the importance of participating in clinical trials.

What clinical studies for autoimmune hepatitis are looking for participants?

You can find clinical studies on autoimmune hepatitis at www.ClinicalTrials.gov. In addition to searching for federally funded studies, you can expand or narrow your search to include clinical studies from industry, universities, and individuals; however, the National Institutes of Health does not review these studies and cannot ensure they are safe. Always talk with your health care provider before you participate in a clinical study.