Autoimmune Hepatitis

Autoimmune hepatitis, or AIH, is a disease in which the body’s immune system attacks the liver and causes irritation (inflammation). Normally, the body’s immune system only attacks what it sees as foreign invaders, like viruses and bacteria. In AIH, the immune system makes a mistake and attacks the liver.

We don’t know what causes AIH. We do know that certain things can increase the likelihood of getting AIH. These include:

• Other diseases of the immune system
• Past infections
• Certain medicines
• Family history of autoimmune disease.

AIH can range from a silent disease, where a child has no signs or symptoms, to acute liver failure, where a child may have many signs and symptoms.

Symptoms that often appear first include:

• Tiredness and generally feeling unwell
• Loss of appetite

Other symptoms:

• Yellow coloring of the skin or eyes (jaundice)
• White or grey colored poop (stool)
• Very dark yellow pee (urine)
• Weight loss
• Nose bleeds that last a long time, bleeding gums, and bruising easily
• Blood in the stool
• In adolescent girls, periods may stop or become irregular

Your liver (hepatology) team will do tests to rule out other liver or gastrointestinal diseases, while also testing for AIH. These tests can include:

• Blood test
• Ultrasound scan
• Liver biopsy
• MRI-MRCP scan (special scan to look at the liver and pancreas)
• Upper and lower gastrointestinal endoscopy if there are intestine-related symptoms

AIH is a chronic illness, meaning your child may need life-long treatment. This disease can have flares, where AIH is very active, as well as remissions where AIH is under control or quiet for a long time. We treat AIH with medicines, regular blood testing, imaging (looking at pictures of the inside of the abdomen), and close monitoring.

Corticosteroids (also known as steroids)

Your child may start a steroid (prednisone) when they are diagnosed with AIH. Steroids help reduce the inflammation in the body very quickly. This helps get the liver healthy again. Often, we start this medicine at a higher dose and then decrease the dose as the liver labs return to normal. This helps lessen side effects. Some children might be able to be weaned off prednisone, but most stay on a low dose for a long period of time. Side effects of steroids can include:

Increased appetite and weight gain

• Behavioral changes
• Acne
• Sleep disturbances
• Increased risk of infection
• Impacts on eye and bone health (long-term)

Immune modulating medicines

AIH is also treated with medicine(s) that suppresses the immune system. These immune modulating medicines are not used to treat flare-ups but are helpful after inflammation is already under control with corticosteroids. By changing the immune system, they can help stop AIH from flaring up again. These medicines include:

• Azathioprine
• Mycophenolate Mofetil (MMF)
• Tacrolimus

The medicine used to treat your child’s AIH will depend on their body’s response to treatment, side effects, and how much they can tolerate.

Children with AIH usually do well on regular consistent treatment. If AIH is left untreated or progresses, some symptoms may include:

• Weight loss/poor weight gain
• Fluid in the stomach area
• Spleen enlargement
• Jaundice
• Gastrointestinal bleeding
• Liver failure, requiring a liver transplant

Regular visits with your healthcare team, taking medicine regularly, and doing required testing will help prevent disease progression.

Activity and eating:

• Have your child eat a balanced diet of proteins, fats and carbs from a variety of foods.
• Encourage regular exercise.
• Please talk to your doctor if your child plays contact sports. Ask about which sports are included.

General tips:

• Let us know if you start a new prescribed medicine like antibiotics or medicine for acne.
• It is especially important for your child to avoid illegal drugs or alcohol. These substances are processed by the liver and can cause more liver damage.
• Have your child wash their hands often and maintain good health hygiene.
• Get all recommended vaccinations, including a yearly flu shot.

Call your GI provider if your child has any of the following:

• Yellowing of the skin or eyes
• Pale (white or grey) colored stools
• More bruising than usual
• Bleeding from the gums or nosebleed that is hard to stop
• Severe stomach pain
• Vomiting blood or bile (green)
• Blood in the stool, or stool that is black in color

Seattle Children’s Family Resource Center
seattlechildrens.org/clinics/family-resource-center

American Liver Foundation
liverfoundation.org

Autoimmune Liver Disease Network for Kids
alink4hope.org

Liver UK
liveruk.org