Autoimmune hepatitis is a form of liver disease that occurs when the immune system of the body attacks the liver. This attack of immune cells on the liver causes liver enzymes to rise as liver cells are injured and burst. Decreased liver function may cause jaundice (yellowing of the skin).

Many patients with autoimmune hepatitis have no symptoms at all; their elevated liver enzymes are discovered accidentally when routine blood work is done. Other patients with autoimmune hepatitis, however, may suddenly develop acute liver disease with jaundice, abdominal pain and sometimes even liver failure.

Autoimmune hepatitis is classified into two types. The disease primarily affects girls and is usually associated with other autoimmune disorders. Type 1 is the most common and typically starts in adolescence or young adulthood. Type 2 is less common and usually affects girls aged 2 to 14.

Causes

When the immune system is working correctly, it protects the body from infections caused by bacteria and viruses. In the case of an autoimmune disease, the body does not recognize certain cells and body parts as part of itself, and begins damaging the body part it thinks is foreign.

When the immune system attacks the liver in this way, it is called autoimmune hepatitis. Autoimmune hepatitis is not caused by a virus or bacteria, so it is not a contagious disease. No one knows what triggers the immune system to react against the liver.

Symptoms

• Dark urine
• Loss of appetite
• Fatigue
• General discomfort, uneasiness, or ill feeling (malaise)
• Swollen abdomen (belly area)
• Generalized itching
• Pale or clay-colored stools
• Nausea and vomiting

Exams and Tests

Blood Tests

Your child's pediatric gastroenterologist will order blood tests to check for certain autoantibodies. An antibody is a protein that normally protects the body from foreign organisms, but sometimes the body's immune system mistakes its own cells, tissue and organs as invaders and produces autoantibodies that attack itself. Blood tests that show certain autoantibodies to be present will suggest a diagnosis of autoimmune hepatitis.

Liver Biopsy

Your child's pediatric GI specialist may want to do a liver biopsy to confirm chronic hepatitis. Your child will receive sedation medications or a general anesthetic before the biopsy. These medications will prevent your child from feeling pain or anxiety or remembering the procedure. An area on the right side of your child's abdomen or mid-upper abdomen will be cleansed with an antibacterial soap. The biopsy site will then be numbed with an injection of lidocaine (numbing medicine). The pediatric gastroenterologist will insert a needle through the skin and into the liver to obtain a very small piece of liver tissue. The sample will be sent to a lab and analyzed. The doctor will apply a small pressure dressing (bandage) to the site.

After the biopsy your child will stay in or return to his/her room for four hours, when a repeat blood count is checked. (Children less than one year are observed overnight.) He or she should lie on the right side or back, depending on the biopsy site, for two hours. Being in bed will reduce the chance of bleeding after the biopsy. Once your child is awake and alert, he/she will be allowed to drink fluids. If fluids are tolerated, a regular diet can be restarted. For the remainder of the day, your child will need to participate in quiet activity. The results of the liver biopsy are usually available two weeks after the procedure.

Treatment

Autoimmune hepatitis is treated by suppressing the immune system so the body cannot react to and injure the liver. High dose prednisone (corticosteroid) therapy is the most common treatment. Elevated liver enzymes typically return to normal some weeks after medication is started.

A drug named Imuran or azathioprine may help suppress the immune system so that less prednisone is needed. Many patients require lifelong immuno-suppressive therapy in order to keep the inflammation and injury to the liver under control. Sometimes patients can be weaned from the medications, but these patients should be followed closely to make sure their hepatitis does not relapse.

For More Information

American Liver Foundation liverfoundation.org

National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health - digestive.niddk.nih.gov/ddiseases/pubs/autoimmunehep/