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Pediatric autoimmune hepatitis (AIH)

Pediatric autoimmune hepatitis (AIH) (au·​to·​im·​mune hep·​a·​ti·​tis) or AIH occurs when the body’s immune system mistakenly attacks and damages the liver, causing inflammation that can lead to other conditions.

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What is pediatric autoimmune hepatitis (AIH)?

Autoimmune hepatitis is a chronic (long-lasting) liver disease that can occur at any age and typically affects more girls than boys.

Without treatment, this condition can have lasting impacts, including liver complications and additional autoimmune diseases such as:

  • Celiac disease - An autoimmune disorder that attacks the small intestine when a child eats gluten
  • Cirrhosis - A condition that causes the liver to develop scar tissue in place of healthy liver tissue
  • Inflammatory bowel disease - A gastrointestinal condition that causes ulcerative colitis (sores and swelling in the colon and rectum)
  • Liver cancer - When cancerous cells grow in the liver due to liver damage
  • Liver failure - When the liver loses its function.
  • Primary biliary cholangitis (PBC) - A chronic liver disease caused by slow, continual and permanent damage to the bile ducts (tubes that carry digestive liquid from the liver to small intestine)
  • Primary sclerosing cholangitis (PSC) - A chronic liver disease caused by the bile ducts narrowing and hardening over time
  • Rheumatoid arthritis - When the immune system mistakenly attacks the joints, causing pain and inflammation
  • Thyroid conditions - When the thyroid (regulates hormones for growth and development) is attacked by autoimmune disorders, it can cause conditions like Graves’ disease and Hashimoto’s disease
  • Type 1 diabetes - When the pancreas is destroyed by an autoimmune response, it can make it difficult for the body to regulate blood sugar with insulin and convert food into energy
  • Vitiligo - When pigment in the skin is destroyed, causing white patches to appear

What are the different types of pediatric autoimmune hepatitis (AIH)?

There are two types of autoimmune hepatitis:

  1. Type 1 this is the most common type of the disease, and can occur at any age and more often in girls than boys (about 4 to 1).
  2. Type 2 this is more severe and difficult to control, most often occurring in children.

What are the signs and symptoms of pediatric autoimmune hepatitis (AIH)?

Symptoms of autoimmune hepatitis range from mild to severe, depending on the type.

  • Constantly feeling tired
  • Dark-colored urine
  • Jaundice (yellowish color to the skin or eyes)
  • Joint pain
  • Nausea and vomiting
  • Pain in the abdomen, specifically near the liver (upper right area)
  • Pale-colored stool
  • Poor or no appetite
  • Skin rashes or vitiligo

What are the causes of pediatric autoimmune hepatitis (AIH)?

The exact cause of autoimmune hepatitis is unknown. A healthy immune system makes antibodies and lymphocytes (white blood cells) to battle infections. But with an autoimmune condition, something changes the normal response pattern – causing the antibodies to see healthy tissues and organs as dangerous infections.

Possible triggers that can lead to an autoimmune response include:

  • Genetics - Autoimmune diseases can run in families; researchers are looking into what causes this genetic response and how it’s inherited
  • Medication - Antibiotics that are used to treat acne (minocycline) and urinary tract infections (nitrofurantoin) can cause liver damage that resembles autoimmune hepatitis
  • Viruses - Experts are researching how a body’s initial response to fighting a common virus can lead to a larger autoimmune response

Pediatric autoimmune hepatitis (AIH) doctors and providers