Autoimmune hepatitis (AIH) is a chronic, or long-lasting, disease in which the body’s immune system attacks the normal components, or cells, of the liver and causes inflammation and liver damage. The immune system normally protects people from infection by identifying and destroying bacteria, viruses and other potentially harmful foreign substances.
Autoimmune hepatitis is a serious condition that may worsen over time if not treated. Autoimmune hepatitis can lead to cirrhosis and liver failure. Cirrhosis occurs when scar tissue replaces healthy liver tissue and blocks the normal flow of blood through the liver. Liver failure occurs when the liver stops working properly. People with autoimmune hepatitis and cirrhosis are also at risk of developing liver cancer.
Type 1 autoimmune hepatitis is the most common form in North America. Type 1 can occur at any age; however, it most often starts in adolescence or young adulthood. About 70 percent of people with type 1 autoimmune hepatitis are female. People with type 1 autoimmune hepatitis commonly have other autoimmune disorders. Type 2 autoimmune hepatitis is less common and occurs more often in children than adults. People with type 2 may also have other autoimmune disorders.
About 10 percent of people with typical features of primary biliary cholangitis (PBC)—a chronic disease that causes the small bile ducts in the liver to become inflamed and damaged and ultimately disappear—will have additional features of autoimmune hepatitis. A subset, however, have no such features but go on to develop an AIH overlap syndrome.
What causes AIH?
Autoimmune diseases are disorders in which the body’s immune system attacks the body’s own cells and organs with proteins called autoantibodies; this process is called autoimmunity.
The body’s immune system normally makes large numbers of proteins called antibodies to help the body fight off infections. In some cases, however, the body makes autoantibodies. Certain environmental triggers can lead to autoimmunity. Environmental triggers are things originating outside the body, such as bacteria, viruses, toxins and medications.
A combination of autoimmunity, environmental triggers and a genetic predisposition can lead to autoimmune hepatitis.
What are the symptoms of AIH?
The most common symptoms of autoimmune hepatitis are: fatigue, joint pain, nausea, loss of appetite, pain or discomfort over the liver, skin rashes, dark yellow urine, light-colored stools and jaundice (yellowing of the skin and whites of the eyes).
Symptoms of autoimmune hepatitis range from mild to severe. Some people may feel as if they have a mild case of the flu. Others may have no symptoms when a health care provider diagnoses the disease; however, they can develop symptoms later.
How is AIH diagnosed?
A health care provider will make a diagnosis of autoimmune hepatitis based on symptoms, a physical exam, blood tests and a liver biopsy.
A health care provider performs a physical exam and reviews the person’s health history, including the use of alcohol and medications that can harm the liver. A person usually needs blood tests for an exact diagnosis because a person with autoimmune hepatitis can have the same symptoms as those of other liver diseases or metabolic disorders.
How is AIH treated?
Treatment for autoimmune hepatitis includes medication to suppress, or slow down, an overactive immune system. Treatment may also include a liver transplant.
Treatment works best when autoimmune hepatitis is diagnosed early. People with autoimmune hepatitis generally respond to standard treatment, and the disease can be controlled in most cases. Long-term response to treatment can stop the disease from getting worse and may even reverse some damage to the liver.
People with autoimmune hepatitis who have no symptoms or a mild form of the disease may or may not need to take medication. A health care provider will determine if a person needs treatment. In some people with mild autoimmune hepatitis, the disease may go into remission. Remission is a period when a person is symptom-free and blood tests and liver biopsy show improvement in liver function.
Corticosteroids are medications that decrease swelling and reduce the activity of the immune system. Health care providers treat both types of autoimmune hepatitis with a daily dose of a corticosteroid called prednisone. Treatment may begin with a high dose that is gradually lowered as the disease is controlled. The treatment goal is to find the lowest possible dose that helps control the disease.
Medications that suppress the immune system prevent the body from making autoantibodies and block the immune reaction that contributes to inflammation. In most cases, health care providers use azathioprine (Azasan, Imuran) in conjunction with prednisone to treat autoimmune hepatitis. When using azathioprine, a health care provider can use a lower dose of prednisone, which may reduce prednisone’s side effects.
Last Reviewed: March 5, 2019