Definition & Facts for Autoimmune Hepatitis

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.

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