Autoimmune hepatitis (AIH)

AIH is a rare, non-contagious liver disease. In AIH a person's liver cells are attacked by the immune system which leads to inflammation of the liver (hepatitis). AIH can lead to acute liver failure or, in the case of a chronic illness, to cirrhosis.

Causes of AIH

It is not known why the immune system attacks the body's own liver cells in cases of AIH. A genetic predisposition to AIH is suspected. However, this alone is not enough to cause the disease. Stress or damage to the body (e.g. toxins, drugs, infections or pregnancy) can trigger AIH, but these are not the cause of the disease.

Symptoms

AIH does not always present with symptoms. Possible identifying signs range from fatigue, mild flu-like symptoms, pale stools, dark urine, fever, rheumatoid-like joint pain or yellowing of the skin and the whites of the eyes to dry mucous membranes, hair loss or absence of menstruation.

These symptoms are not exclusive because AIH also occurs with other autoimmune diseases. These include, for example, thyroid conditions (Hashimoto's thyroiditis), primary biliary cholangitis and primary sclerosing cholangitis, type 1 diabetes, inflammatory bowel diseases, coeliac disease or dermatitis.

Problems may also be due to the side effects of the immunosuppressive therapy required for AIH.

Diagnosis

To diagnose AIH, other liver diseases must be ruled out.

The first sign of AIH is certain autoantibodies in the blood (for example, ANA, p-ANCA or SMA). These alone are not however proof of the verified presence of AIH. If these liver values are measured and found to be raised, this is also not concrete evidence of AIH. The same applies to a raised level of Immunoglobulin G levels. For this reason, a needle biopsy of the liver is important as tissue changes typical of AIH will be visible under the microscope. Distinguishing this from acute liver damage as a result of drugs remains difficult even with a liver biopsy. In this instance, AIH can be detected by taking immunosuppressive drugs to see if they have an effect. If they do, this indicates AIH.

Treatment options

To reduce the exaggerated immune response (the cause of AIH), immunosuppressant therapies are usually prescribed. These medicines include prednisolone in combination with azathioprine. The side effects and long-term effects of immunosuppressants include: weight gain, a bloated face, skin problems, blood count changes, an increased risk for diabetes and osteoporosis. To prevent osteoporosis, calcium and vitamin D3 supplements can be taken. Regular physical activity can also be very helpful. Alcohol must be avoided completely. Untreated AIH generally leads to liver cirrhosis over time.

In most cases, long-term therapy is essential to keep the immune system stable. As a result, life expectancy is not affected. Rarely, medications are not sufficient to keep the liver inflammation under control and the AIH develops into liver cirrhosis. In such cases, a liver transplant is the last resort.

No alternative medicine treatments are known to be effective against AIH.

Read more about AIH in the article: "A reasoned approach to the treatment of autoimmune hepatitis"
Co-author: PD Dr. med. Benedetta Terziroli Beretta-Piccoli

Informative Video from ERN RARE-LIVER Project about Autoimmune hepatitis: