Autoimmune hepatitis

The underlying cause of autoimmune hepatitis is not known. It is possible that any of several genetic or environmental factors (such as medications or viral infections) may trigger the disease. People with another autoimmune disease or a family history of autoimmune disease may be more likely to develop autoimmune hepatitis.

Symptoms of autoimmune diseases in general are caused by the body's immune system mistakenly attacking healthy tissues and cells. In the case of autoimmune hepatitis, the body's immune system attacks healthy liver tissue, ultimately damaging the liver.

The diagnosis of autoimmune hepatitis is typically made based on a person's signs and symptoms, medical history, various blood tests, and a liver biopsy. Blood tests may be able to tell autoimmune hepatitis apart from viral hepatitis, or from other health problems that cause similar symptoms. A liver biopsy can confirm the diagnosis and determine the extent of liver damage. Sometimes a person with no symptoms is suspected of having the disease after a routine health exam or when being evaluated for another health problem.

Treatment for autoimmune hepatitis should be managed by a hepatologist, which is a doctor who specializes in liver disease. Treatment aims to suppress the immune system, which is overactive in people with this disease. In many cases, especially when the disease is diagnosed early, treatment can slow the progression of the disease and may reverse some of the liver damage that has already occurred. People who have no symptoms or have a very mild form of the disease may not need treatment. A hepatologist may evaluate symptoms and use various laboratory tests to determine when a person should begin treatment.

Medications that may be used initially ("induction therapy") include :

• Corticosteroids (such as prednisone).
• Azathioprine (often in combination with corticosteroids).
• Other immune system suppressants - particularly when treatment with corticosteroids and azathioprine is not effective or causes severe side effects. Examples include mycophenolate mofetil, cyclosporine, or tacrolimus.

Most people go into remission with initial treatment within two to three years. This means that their symptoms improve, and laboratory tests show that liver function is improving. In some cases, people who achieve remission can taper off medications for a period of time. However, relapses are common, and many people need long-term management ("maintenance therapy") to keep the disease under control. Maintenance therapy may involve long-term use of much lower doses of prednisone or azathioprine, which are effective in controlling the disease is most people.

Some people do not respond to treatment or cannot continue treatment due to side effects. If the disease progresses and causes severe cirrhosis and liver failure, a liver transplant may be needed. About 10-20% of people with autoimmune hepatitis eventually need a liver transplant.