Autoimmune Hepatitis

Autoimmune hepatitis in children is a rare and serious disease of the liver. Most cases of autoimmune hepatitis can be controlled with medicine.

What Is Autoimmune Hepatitis in Children?

Autoimmune hepatitis in children is a rare and serious disease of the liver. It happens when something goes wrong with the body’s immune system. 

The immune system uses several types of special cells to help fight off disease. They fight bacteria, fungi, parasites, viruses, foreign cells, or cancer cells. The cells of the immune system can either attack directly, or they can make special substances called antibodies to help them fight. In autoimmune disorders, the immune system becomes confused. It mistakes the body’s own healthy cells for foreign, harmful ones, then attacks and kills them.

In autoimmune hepatitis, the immune system attacks normal liver cells. This causes damage that makes it hard for the liver to work properly.

There are two types of autoimmune hepatitis in children. Both types have similar symptoms. The main difference is which types of antibodies are being produced. 

  • Type I is by far the most common type of autoimmune hepatitis. In children, it most commonly presents in schoolchildren and teenagers. 
  • Type II is very rare in the United States. This type of autoimmune hepatitis tends to be more severe and difficult to treat. It may appear at a younger age than Type I. Both types are only very rarely seen in infants.

What Causes Autoimmune Hepatitis in Children?

It is not known exactly why the immune system begins attacking liver cells in children with autoimmune hepatitis. 

Experts are looking at a number of possible causes, including:

  • Genetics. Physical traits passed down from parents
  • Environment. Causes of disease from outside the body, such as toxic substances, certain medicines, or germs (mostly viruses)
  • Problems with the immune system. For example, in patients with autoimmune hepatitis, it seems that some cells that regulate (settle down) the immune system are fewer or weaker, while other cells that make the immune system attack are more frequent or more active.

Inside the Liver Center: Meet Dr. Weymann

Dr. Weymann leads a team of highly skilled specialists dedicated to caring for children suffering from a wide range of liver diseases. Named to the Best Doctors in America list, Dr. Weymann understands that liver problems can be life-threatening and life-changing. Quick evaluation, correct diagnosis and early treatment can impact long-term health.

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How Is Autoimmune Hepatitis in Children Diagnosed?

Your child’s health care provider will do several tests to look for autoimmune hepatitis and other related diseases. These tests may include:

  • Blood tests
  • Liver biopsy (under anesthesia – while the child is asleep – remove a tiny piece of the liver to look at under a microscope)
  • Special scans (pictures) of the liver, such as ultrasound and magnetic resonance cholangiopancreatography (MRCP)
  • Exam of the inside of the intestines (endoscopy), under anesthesia

What Children Are at Risk for Autoimmune Hepatitis?

Autoimmune hepatitis is far more common in girls than in boys. Also, autoimmune hepatitis occurs more often in children who:

  • Have other immune system diseases
  • Have close family members with an autoimmune disease
  • Take certain medications
  • Have had certain infections recently

What Are the Symptoms of Autoimmune Hepatitis in a Child?

Both Type I and Type II autoimmune hepatitis have similar symptoms. Autoimmune hepatitis symptoms can come on suddenly or may develop over time. Some children with autoimmune hepatitis do not show any obvious symptoms; such children may only be diagnosed because of abnormal blood test results. When symptoms do appear, they may include:

  • Fatigue (feeling tired all the time)
  • Pale or gray stool
  • Nausea
  • Vomiting
  • Enlarged liver or spleen
  • Itching or rash
  • Jaundice (yellowish skin and eyes) 
  • Dark urine
  • Loss of appetite
  • Pain in the abdomen (belly)
  • Aches in joints

How Is Autoimmune Hepatitis Treated in a Child?

Autoimmune hepatitis is a serious, long-lasting (chronic) disease. Right now, there is no cure for autoimmune hepatitis. Fortunately, most children with autoimmune hepatitis respond well to treatment. 

The goal of autoimmune hepatitis treatment is remission. This means symptoms become less severe and liver damage slows or stops. Some children are able to stop taking medicine after two or three years. These children will still need to be watched for a return of autoimmune hepatitis symptoms and other health issues.

Two main types of medicine are used to help control autoimmune hepatitis in children:

  • Corticosteroids (“Steroids”). Prednisone (or prednisolone) helps stop the immune system from attacking the liver. It also reduces liver inflammation. Budesonide is another corticosteroid that is sometimes used; it has less side effects, but it is mostly given later on when the disease is under control already.
  • Immunosuppressants. Azathioprine or mercaptopurine are often added to the treatment; they work together with prednisone to get the immune system under control. There are other medications to suppress the immune system that can be used if the standard treatment is not working well.

Rarely does autoimmune hepatitis damage the liver so much that it stops working. This can either happen right away, or after months or years of treatment when the medications do not work. In those cases, the child will need a liver transplant. This is very complex, major surgery. The diseased liver is removed and replaced with a new one. It may be a whole liver or part of a liver from a deceased donor. It is sometimes possible to use part of a liver from a compatible living donor (who may or may not be related to the child). After a liver transplant, special medicines and procedures (anti-rejection therapies) help the body accept and adjust to the new organ.

What Are the Complications of Autoimmune Hepatitis in a Child?

Autoimmune hepatitis can cause scar tissue to start forming on the damaged liver (fibrosis). This makes it harder for the liver to work properly. Over time, lots of scar tissue can build up in the liver (cirrhosis). This can block the blood flowing through the liver and may lead to problems such as bleeding in the esophagus (food pipe) or stomach, or water in the belly (ascites). Cirrhosis also can cause the liver to fail; signs may be jaundice (yellow eyes and skin), bleeding/bruising, or confusion.

Should I Call My Child’s Health Care Provider?

If your child has been diagnosed with autoimmune hepatitis, call your health care provider right away if:

  • Your child’s symptoms get worse
  • New symptoms appear
  • You have any questions or concerns about your child’s condition

Key Points about Autoimmune Hepatitis in Children

  • Autoimmune hepatitis is a rare disease of the liver.
  • Autoimmune hepatitis affects girls more often than boys.
  • Most cases of autoimmune hepatitis can be controlled with medicine.
  • Autoimmune hepatitis sometimes causes serious complications such as cirrhosis or liver failure.
  • In rare cases, a child with severe autoimmune hepatitis may need a liver transplant.

Next Steps

If your child has autoimmune hepatitis, see his or her health care provider on a regular basis. A health care provider will work with you to keep your child’s liver as healthy as possible. Some other helpful things you can do include:

  • Make your child take all medicines regularly and on time.
  • Learn about possible side effects of your child’s medicines.
  • Ask if and how diet and exercise may help your child.
  • Find out if there are any activities your child should not do.
  • Always ask your child’s health care provider before trying any home remedies or over-the-counter medicines, such as herbal supplements.