Autoimmune Hepatitis

Overview

What is autoimmune hepatitis?

Autoimmune hepatitis is a rare but serious disease of the liver. Autoimmune means that the body attacks itself; in this case, it mistakes healthy tissue and cells for infected tissue and cells and sends out antibodies (which usually rid the body of infections) to attack the healthy liver cells.

Hepatitis occurs when the healthy tissue of the liver become inflamed. If not treated, the problem may advance to cirrhosis (scarring) of the liver, and ultimately to liver failure. If the liver becomes severely damaged, a liver transplant may be necessary.

Autoimmune hepatitis is categorized as Type 1 or Type 2:

  • Type 1 is diagnosed in adolescents or adults. This type is more common and is usually found in North America.
  • Type 2 is diagnosed mostly in children and can be more difficult to treat.

Symptoms and Causes

What causes autoimmune hepatitis?

Autoimmune hepatitis may appear suddenly or develop slowly over time. The cause of the disease is not known, but in some cases may be related to other systemic (throughout the body) diseases or exposure to certain medications. Autoimmune hepatitis can also be inherited from a parent or a grandparent who also has the disease.

What are the symptoms of autoimmune hepatitis?

The disease affects each patient differently. Some patients do not have any symptoms, while others may have such symptoms as:

  • Tiredness/low energy
  • Nausea
  • Itchy skin or rash
  • Yellow-colored skin or whites of the eyes (jaundice)

As scarring in the liver continues and liver function gets worse, the patient may notice:

Diagnosis and Tests

How is autoimmune hepatitis diagnosed?

The symptoms of autoimmune hepatitis may be mild at first and are similar to the flu, so it may not be diagnosed immediately. The doctor will perform a physical examination of the patient and ask about the family medical history. The doctor may also order certain tests, including:

  • A blood test to check for inflammation in the liver, to evaluate the patient’s liver function, and to check for antibodies associated with autoimmune hepatitis.
  • Ultrasound of the liver. Ultrasound is a procedure that transmits high-frequency sound waves through body tissues. The echoes are recorded and transformed into video or photographic images of the inside of the body.
  • If necessary, the doctor may also order a liver biopsy, the removal of a small piece of tissue from the liver to be analyzed in the lab for disease.

Management and Treatment

How is autoimmune hepatitis treated?

Once autoimmune hepatitis is diagnosed, a doctor will prescribe medications to stop the attack of the antibodies and to heal the inflamed liver. The treatment for autoimmune hepatitis is usually a high dose of a steroid (prednisone or prednisolone) to suppress the immune system and keep it from attacking the liver. The doctor may also prescribe azathioprine (Imuran®) to suppress the immune system.

As the condition improves, the doctor may lower the dosage of the medications. If the condition does not improve, other immune-suppressing medications may be needed.

Patients may need to be treated for several years before the disease goes into remission (lessens or goes away), at which point they may be taken off the medications. If they have a relapse (return of the disease), they may need to take the medications for a long time.

If the medications do not adequately treat the autoimmune hepatitis and the patient develops cirrhosis or liver failure, he or she may need a liver transplant.

Prevention

Can autoimmune hepatitis be prevented?

Autoimmune hepatitis cannot be prevented.

Who is at risk for autoimmune hepatitis?

Autoimmune hepatitis affects one in every 100,000 people. The disease is more common in women than in men, and women are typically diagnosed in their 40s or 50s. Girls between two and 14 years old may also get the disease.

Those who may suffer from other autoimmune conditions, such as diabetes, rheumatoid arthritis, thyroid disease and celiac disease, are also at risk to develop autoimmune hepatitis (just as those with autoimmune hepatitis are also at higher risk to develop other autoimmune diseases).

A person who has a family history of autoimmune hepatitis also has a higher risk of developing the disease.

Outlook / Prognosis

What is the prognosis (outlook) for patients who have autoimmune hepatitis?

If autoimmune hepatitis is diagnosed early, and treated with the proper medication, the liver may begin to heal and will be able to make healthy cells again to replace the inflamed and scarred cells. The patient’s symptoms will ease and the liver may begin to work normally.

The patient will need to be monitored and managed for the rest of his or her life, even if he or she is feeling better and liver function has improved. In many cases, the patient will need to be on medication for the rest of his or her life.

Last reviewed by a Cleveland Clinic medical professional on 06/21/2018.

References

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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy