Autoimmune Hepatitis in Children

Autoimmune hepatitis occurs when the body’s immune system attacks the liver. This causes inflammation in the liver, leading to swelling of the liver tissue and damage to liver cells.

There are two major forms of autoimmune hepatitis. Type 1, the more common form, tends to affect older children and adults. Type 2 is less common and affects younger children. Blood testing can usually distinguish between the two types.

Autoimmune hepatitis may occur either suddenly or gradually. If untreated, however, autoimmune hepatitis can lead to permanent liver scarring, called cirrhosis, or liver damage. In severe cases, autoimmune hepatitis can cause liver failure and require a liver transplantation.

Autoimmune hepatitis is caused by several factors, including genetic and environmental factors.

Symptoms of autoimmune hepatitis are often nonspecific. Patients can vary from having no symptoms (asymptomatic) to having symptoms of advanced liver disease.

Frequent symptoms of autoimmune hepatitis include:

• Fatigue (tiredness)
• Abdominal discomfort
• Itchiness
• Yellowing of the skin and whites of the eyes (jaundice)
• Enlarged liver
• Skin rashes
• Joint pains
• Loss of menstrual periods

If advanced liver disease develops, symptoms may include:

• Abnormal blood vessels on the skin (spider angiomas)
• Yellowing of the skin and whites of the eyes (jaundice)
• Abdominal swelling
• Poor growth
• Easy bruising and bleeding
• Confusion, loss of alertness, disorientation

If your health provider suspects your child has autoimmune hepatitis, they will collect a sample of your child’s blood for testing. Patients with autoimmune hepatitis have elevated liver enzymes and often elevated serum globulins (antibodies).

A definitive diagnosis is usually made by collecting a liver biopsy, or a small sample of liver tissue that can be examined under a microscope. 

Autoimmune hepatitis is treated with medications called immunosuppressants to stop the immune system from attacking the liver. This often includes initial treatment with steroids (prednisone) followed by long-term treatment with immunosuppressants (azathioprine or mycophenolate).

Treatment inhibits the immune system and requires monitoring of your child’s response to the medications, but it is generally well-tolerated.

Children with autoimmune hepatitis generally have an excellent response to treatment. In most cases, however, the disease is controlled but not cured.

The type of medications and length of treatment can vary. Most patients stay on one or more medications for several years, while other patients may need treatment for their lifetime. Rarely, the inflammation can lead to severe liver injury in some patients and require a liver transplant.

Having a new diagnosis is challenging, especially when the condition is chronic. Talk to your health provider or caregivers about what challenges your child encounters and if they have missed taking their medications. Not taking the medications can significantly impact your child’s health and put them at risk of developing complications from autoimmune hepatitis.

Encourage your child to share their feelings about the diagnosis with you and with your health provider. If your child often feels sad or anxious, lacks concentration or interest in school or hobbies, or has difficulty sleeping, talk to your health provider. They can provide advice and resources to help you and your child cope with this condition.

Authors: Johanna Ferreira, MD and Mercedes Martinez, MD
Editor: Athos Bousvaros, MD

October 2021