Autoimmune hepatitis is a disease in which your immune system attacks your own liver cells and causes your liver to become inflamed. The disease may be acute or chronic, meaning it lasts many years. If untreated, it can lead to liver failure, cirrhosis, and/or death.

There are two forms of this rare disease. Type 1, or classic, autoimmune hepatitis is the more common form. Type 1 autoimmune hepatitis mostly affects young or middle-aged women and is often associated with other autoimmune diseases. Type 2 autoimmune hepatitis is uncommon and generally affects girls between the ages of 2 and 14 years old.

Facts at-a-Glance

Autoimmune Hepatitis is a serious condition that may progress over time if not treated.
Autoimmune Hepatitis can lead to cirrhosis and liver failure.
Autoimmune Hepatitis is a rare disorder that affects females 4 times as often as males.
Those with any autoimmune condition have a 25-50% chance of developing another one and thus a higher risk for developing Autoimmune Hepatitis.

Symptoms and Causes

Your immune system normally attacks bacteria, viruses and other invading organisms. It is not supposed to attack your own healthy cells; if it does, the response is called autoimmunity. In autoimmune hepatitis, your immune system attacks your liver cells, causing long-term inflammation and liver damage. Scientists don't know what triggers the immune system to attack its own liver, although heredity and prior infections may play a role.

Often, the initial symptoms of autoimmune hepatitis are minor or even non-existent. When symptoms do occur, the most common are fatigue, abdominal discomfort, aching joints, itching, jaundice (yellowing of the skin and whites of the eyes), enlarged liver, nausea and spider-like angiomas (blood vessels) on the skin.

Other symptoms may include dark urine, loss of appetite, pale stool and absence of menstruation. Cirrhosis may result from chronic liver inflammation if chronic autoimmune hepatitis is left untreated. In 10%-20% of cases, autoimmune hepatitis may present with sudden onset of severe symptoms of liver failure such as ascites (fluid in the abdomen), mental confusion, jaundice, and gastrointestinal bleeding. Some of these latter cases may have underlying cirrhosis from previously non-symptomatic chronic autoimmune hepatitis, but many are due to acute autoimmune hepatitis.

Diagnosis and Tests

Autoimmune hepatitis often occurs suddenly. Initially, you may feel like you have a mild case of the flu. In others, routine blood tests may reveal a liver problem. To confirm a diagnosis of autoimmune hepatitis, your doctor will take a careful medical history and use specific blood tests, liver imaging, and possibly a liver biopsy, in which a sample of liver tissue is removed with a needle for examination in a laboratory.

Management and Treatment

The goal of treatment is to stop the body's attack on itself by suppressing the immune system, regardless of the type of autoimmune hepatitis. This is accomplished with a medicine called prednisone, a type of steroid. Often, a second drug, azathioprine is also used. Treatment starts with a high dose of prednisone. As blood test results improve, the dosage is gradually lowered, and azathioprine may be added. Within months, treatment with azathioprine alone may be possible.

Prevention

Why the body’s immune system attacks itself is unclear, therefore prevention can be a challenge. About 70 percent of people with autoimmune hepatitis are women, usually between the ages of 15 and 40 years old at the time of diagnosis. Many people with this disease also have a history of other autoimmune diseases, including type 1 diabetes, autoimmune thyroiditis (inflammation of the thyroid gland), ulcerative colitis (inflammation of the colon), vitiligo (patchy loss of skin pigmentation), or Sjogren's syndrome (dry eyes and dry mouth).

Outlook / Prognosis

Most of the time, you will need to stay on medication for about two years before considering stopping treatment, and then observing closely for relapse over the years. Life-long therapy is recommended for those who relapse. For some, chronic treatment with steroids may be necessary. Long-term prednisone use can cause serious side effects including type 2 diabetes, osteoporosis, high blood pressure, glaucoma, weight gain and decreased resistance to infection. Addition of other medications may be needed to manage any treatment side effects. To reduce prednisone related side effects, azathioprine only and/or budesonide therapy is typically used to maintain remission of autoimmune hepatitis. If medical therapy fails, liver transplantation surgery is an option that has a high success rate with long-term survival.

Living With

Maintaining a healthy lifestyle of diet and exercise along with following a medication regimen are key factors to living well with AIH.

Questions to Ask Your Doctor

Are my symptoms that I am experiencing (if you are) such as fatigue and lethargy due to AIH?
Which type of Autoimmune Hepatitis do I have? Type 1 or Type 2?
What is the status of my liver?
Do I have irreversible liver damage?
Will I need a liver transplant?
Is it possible for me to have AIH along with other autoimmune diseases?
Will a liver biopsy be needed?
What kind of medications might be possible to treat AIH?
What are the potential side effects of treatment for AIH?
Are these medications safe to take during pregnancy and/or breast feeding?
Do my blood tests show that my liver enzyme levels and immunoglobulin levels are responding to therapy?
Will I be tapered off (dose reduced) steroid therapy over time if I respond well to therapy?
When may I safely try to stop treatment?

Will there be routine imaging/screening to check for other kinds of liver diseases such as liver cancer?
Will I need a booster vaccine of for Hepatitis A and Hepatitis B?

Patient Stories

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Medically reviewed July 2025

Last updated on July 25th, 2025 at 09:19 am

Autoimmune Hepatitis (AIH)