Autoimmune hepatitis (AIH) is a chronic liver disease that occurs when your body’s immune system attacks liver cells. AIH can result in cirrhosis (scarring) of the liver, and even lead to liver failure.

Most types of hepatitis are caused by viruses, but this is not true of the autoimmune variety. There are two primary types of AIH. It is not contagious.

This article breaks down how autoimmune hepatitis works, what treatment looks like, and when you should see a doctor.

AIH occurs when your immune system mistakes your liver cells for a foreign threat, and creates antibodies to attack them. Doctors don’t know why this happens.

However, certain risk factors for developing AIH have been identified, including:

Other autoimmune conditions can cause symptoms of liver disease and are also associated with the development of AIH.

These diseases include:

There are two types of AIH, which can be identified by the unique antibodies each type produces. AIH is more likely to affect women, and people who were assigned female at birth.

  • Type I is the most common type of AIH. It can affect people of any age, but mostly women.
  • Type II primarily affects children, in particular girls.

Symptoms of AIH range from mild to severe. In the early stages, you may have no symptoms (asymptomatic). Symptoms may come on slowly as your liver becomes scarred, and sometimes can present suddenly in later stages.

More advanced symptoms of AIH during cirrhosis can look like:

  • fatigue and weakness
  • unexplained weight loss
  • ascites (abdominal bloating due to buildup of fluid)
  • edema (swelling in limbs or feet)
  • jaundice
  • itchy skin

AIH can be confused with other illnesses. Its symptoms are very similar to those of viral hepatitis. To make a proper diagnosis, blood testing is required.

Blood tests can:

  • rule out viral hepatitis
  • determine the type of AIH you have
  • check your liver function

Blood tests are also used to measure the levels of specific antibodies in your blood. Antibodies associated with AIH include:

Blood tests can also measure the amount of immunoglobulin G (IgG) antibodies in your blood. IgG antibodies help the body fight infection and inflammation. This blood test can also help catch AIH even if other antibodies didn’t show up in your blood.

A liver biopsy may sometimes be necessary to diagnose AIH. A biopsy can reveal the type and severity of your liver damage and inflammation. The procedure involves removing a small piece of your liver tissue with a long needle and sending it to a laboratory for testing.

Autoimmune hepatitis is a chronic disease. It has no cure, but can be managed, and treated into remission. Remission means the disease is not active, or presenting symptoms.

Treatment can slow down, stop, and sometimes reverse liver damage. Approximately 90 percent of treated AIH patients achieve remission. However if left untreated, the 5-year survival rate of AIH is around 50 percent.

Multiple approaches are usually used together to treat AIH.

Immunosuppressant drugs

These drugs can lessen or stop your immune system’s attack on your body. Common immunosuppressants used for AIH include 6-mercaptopurine and azathioprine.

The risk of immunosuppressant drugs is that they compromise your body’s ability to fight other infections. During this type of treatment, opportunistic infections can occur.

Corticosteroids

Corticosteroid medications lower inflammation in the body, and can reduce immune system activity at high doses. They come in oral, topical, injectable, and inhaled forms.

The most common corticosteroid used for AIH is oral prednisone. In order to reduce liver inflammation, prednisone is often prescribed for a minimum of 18 to 24 months. Others may need to be on the drug indefinitely in order to prevent AIH from recurring.

Prednisone may cause serious side effects including:

Liver transplant

In very severe cases of AIH, a liver transplant is necessary. During this procedure, your entire liver will be removed and replaced with the liver of a donor (who may be alive or deceased).

However, the disease can sometimes recur even after a successful transplant.

Getting any organ transplant is a major medical procedure, so this option is only pursued as a last resort. According to the National Institute of Diabetes and Digestive and Kidney Diseases, there’s an 86 percent 1-year survival rate for people who have a liver transplant. The 5-year survival rate is about 72 percent.

It’s essential to treat AIH as soon as possible. While early symptoms may not be noticeable or only slightly uncomfortable, AIH can quickly become life-threatening.

Potential complications of untreated AIH include:

  • scarring of the liver (cirrhosis)
  • liver cancer
  • liver failure (which can lead to coma and death)
  • increased blood pressure in the portal vein, which supplies blood to the liver
  • enlarged veins in your stomach and esophagus (esophageal varices)
  • fluid accumulation in the abdomen (ascites)

AIH is a serious health condition that needs timely medical treatment. If you notice any symptoms associated with AIH, or suspect your liver function is impaired, talk with a doctor.

If your condition is acute, you may need to contact emergency medical services. Nausea and vomiting, jaundice, and abdominal bloating should be evaluated right away.

A doctor who specializes in treating liver disorders and diseases is called a hepatologist.

While most types of hepatitis are caused by a virus, autoimmune hepatitis is not. AIH causes your immune system to mistakenly attack your liver cells, causing damage to the organ. It’s still unclear why this happens.

Over time, AIH creates scarring in the liver and can lead to liver failure. There are two main types of AIH, which are more likely to occur in women and girls. Blood tests can identify if you have AIH, and which type.

AIH can be treated with medication, and in severe instances, a liver transplant. Talk with your doctor about your risk factors, and seek evaluation for any troubling symptoms you may be experiencing.