Autoimmune Hepatitis

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Definition & Facts

In this section:

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 much more common than type 2, which mainly affects children.

How common is autoimmune hepatitis?

Researchers aren’t sure how common autoimmune hepatitis is in the United States. Studies conducted in northern European countries have found that between 10 and 24 of every 100,000 people in that region have autoimmune hepatitis. Researchers have found that the disease is more common among Alaska Natives, affecting about 43 of every 100,000 people.1

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 girls and women than in boys and men.

Type 1 autoimmune hepatitis occurs in people of all ages, while type 2 more often affects children.

What other conditions do people with autoimmune hepatitis have?

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

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

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.

African senior medical doctor with patient
Early diagnosis and treatment 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

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. With liver failure, your liver is badly damaged and stops working. Liver failure is also called end-stage liver disease. This may require a liver transplant.

Liver cancer

Cirrhosis increases your chance of getting liver cancer. Your doctor may suggest 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

When symptoms of autoimmune hepatitis are present, they can range from mild to severe.

Some people with autoimmune hepatitis have no symptoms. In such cases, doctors may find evidence of liver problems during routine blood tests that leads 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
  • bloating from a buildup of fluid in the abdomen, called ascites
  • swelling of the lower legs, ankles, or feet, called edema
  • itchy skin
  • jaundice

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. The most common medicines that cause liver injury that resembles autoimmune hepatitis are minocycline—an antibiotic used to treat acne—and nitrofurantoin—an antibiotic used to treat urinary tract infections. 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 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 that you might have, such as inflammatory bowel disease or thyroid conditions.

Physical exam

During a physical exam, your 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
  • 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.

Blood tests

Your doctor may order one or more blood tests to help diagnose autoimmune hepatitis. A health care professional will take a blood sample from you and send the sample to a lab. Your doctor will use blood tests to look for evidence of autoimmune hepatitis.

Blood tests include tests that check levels of the liver enzymes alanine transaminase (ALT) and aspartate transaminase (AST) and check for autoantibodies such as antinuclear antibody (ANA) and anti-smooth muscle antibody (SMA). 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 the response to treatment.

Doctors will 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.

A health care professional taking a blood sample from a patient.
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. The most commonly used test is ultrasound. Ultrasound uses a device called a transducer, which 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.

Doctors sometimes order a computed tomography (CT) scan, which 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 is evidence of cirrhosis. Doctors may also order magnetic resonance imaging (MRI), which uses radio waves and magnets to produce detailed images of organs and soft tissues without using x-rays. MRI can show the shape and size of the liver and detect evidence of cirrhosis.

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 injury 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 corticosteroidsprednisone or prednisolone—with or without another medicine called azathioprine.

Doctors typically start with a relatively high dose of corticosteroids and then gradually lower the dose. Your doctor will try to find the lowest dose that works for you. Your doctor will use blood tests to find out how you are responding to the treatment. A decrease in levels of the liver enzymes alanine transaminase (ALT) and aspartate transaminase (AST) shows a response to treatment. ALT and AST falling to normal levels shows a full response. In some cases, a doctor may repeat a liver biopsy to confirm the response to treatment and find out whether the damage has resolved.

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 who have no symptoms or only a mild form of the disease may or may not need medicines.

Remission

With treatment, you may go into 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.

If you are in remission, your doctor may gradually lower the dose of medicines again and may stop the medicines. 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.

Many people with autoimmune hepatitis go into remission. If people don’t go into remission after taking the medicines discussed above, doctors may prescribe other medicines to try to achieve remission.

Relapse

After a period of remission, you may relapse if your medicines are withdrawn. When you relapse, blood tests show a rise in ALT and AST, and the autoimmune hepatitis begins causing symptoms or damaging your liver again. Most people who have medicines withdrawn during remission relapse. If you relapse, your doctor will restart or adjust your medicines to treat the disease.

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.

Incomplete or failed response to treatment

Some people with autoimmune hepatitis have an incomplete response to treatment, meaning that treatment helps but does not lead to remission. If you have an incomplete response to treatment, you may need to take different medicines to help prevent liver damage.

Some people may fail to respond to treatment, meaning that the inflammation and liver damage of autoimmune hepatitis keep getting worse. Your doctor may recommend additional blood tests and higher doses of medicines. If liver damage leads to complications, you may need treatment for complications.

Doctor talking with patient who is holding a prescription pill bottle.
Doctors treat autoimmune hepatitis with medicines that suppress your immune system.

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 corticosteroids or azathioprine if you have severe side effects.

Side effects of corticosteroids may include

Side effects of azathioprine may include

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

Corticosteroids and azathioprine 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.

How do doctors treat the complications of autoimmune hepatitis?

If autoimmune hepatitis leads to cirrhosis, doctors can treat health problems and complications related to cirrhosis with medicines, surgery, and other medical procedures. If you have cirrhosis, you have a greater chance of developing liver cancer. Your doctor may order an ultrasound or other types of imaging tests to check for liver cancer.

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

Eating, Diet, & Nutrition

What should I eat if I have autoimmune hepatitis?

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

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

Clinical Trials

The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and other components of the National Institutes of Health (NIH) conduct and support research into many diseases and conditions.

What are clinical trials, and are they right for you?

Clinical trials are part of clinical research and at the heart of all medical advances. Clinical trials look at new ways to prevent, detect, or treat disease. Researchers also use clinical trials to look at other aspects of care, such as improving the quality of life for people with chronic illnesses. Find out if clinical trials are right for you.

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

What clinical trials are open?

Clinical trials that are currently open and are recruiting can be viewed at www.ClinicalTrials.gov.

May 2018
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This content is provided as a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), part of the National Institutes of Health. The NIDDK translates and disseminates research findings through its clearinghouses and education programs to increase knowledge and understanding about health and disease among patients, health professionals, and the public. Content produced by the NIDDK is carefully reviewed by NIDDK scientists and other experts.

The NIDDK would like to thank John Moore Vierling, M.D., Baylor College of Medicine