How Does a Child Get Autoimmune Hepatitis?

Oct 8, 2025 | Autoimmune Hepatitis Education, Pediatric

How Does a Child Get Autoimmune Hepatitis?

What Causes Autoimmune Hepatitis in Children?

By Craig Lammert, M.D., AIHA Executive Director

Autoimmune hepatitis (AIH) is much less common in children than in adults, but it does occur. For families, the question often arises: how does a child develop this disease? The honest answer is that we don’t fully know, but research has given us some important clues.

Autoimmune hepatitis is a rare and chronic disease where the body’s own immune system mistakenly attacks the liver. This ongoing attack causes inflammation and, if untreated, can lead to scarring of the liver (fibrosis) or even liver failure.

How Common Is Autoimmune Hepatitis in Children?

AIH is rare overall:

  • A worldwide review estimated that about 1.37 people per 100,000 develop AIH each year, with about 17.44 per 100,000 living with it (BMJ, 2023).
  • In children, the numbers are smaller. A Canadian study found about 0.23 new cases per 100,000 children each year (Pediatrics, 2015).
  • Most affected children are girls, and in up to half of pediatric cases, AIH begins suddenly, with acute symptoms (Canadian pediatric study).

While rare, AIH is crucial to recognize early in children, as timely treatment can help protect the liver.

Why We Don’t Fully Know How It Starts

Autoimmune diseases like AIH can usually happen because of a mix of genetic predisposition and possibly environmental triggers.

Genetic Risk: A Piece of the Puzzle

The strongest genetic links involve HLA genes, which guide how the immune system recognizes proteins:

  • Certain HLA gene variants, such as DRB103:01 and DRB104:01, are more common in patients with AIH in Europe and North America (Human Genomics, 2020).
  • Still, many people with these genes are never diagnosed with AIH, and some patients don’t carry these variants at all.

Researchers are also studying non-HLA genes that may affect how immune cells work or how the body prevents “self-attack.” But these findings are less consistent, especially in children.

Environmental Factors: Possible Triggers

Scientists believe that environmental exposures could “set off” the disease in genetically susceptible children. Some areas of focus include:

  • Viral infections — Viruses like Epstein–Barr virus or hepatitis viruses may confuse the immune system through a process called molecular mimicry (Human Genomics review).
  • Medications — Certain drugs, such as nitrofurantoin or minocycline, have been linked to AIH-like reactions (Clinical Liver Disease).
  • Environmental exposures — Chemicals, pollutants, and occupational exposures are under study but not yet proven (Liver International, 2023).
  • Microbiome and vitamin D — Changes in the gut microbiome or low vitamin D levels have also been suggested as possible contributors (Human Genomics review).

Because these factors are so varied and often subtle, proving cause and effect is extremely difficult.

How Doctors Confirm the Diagnosis in Children

Diagnosing autoimmune hepatitis in children is complex, as its symptoms can mimic other liver diseases. Doctors typically use a combination of:

  • Blood tests: Elevated liver enzymes (ALT, AST) often prompt further evaluation. Doctors also look for specific autoantibodies (ANA, SMA, or anti-LKM1) and immunoglobulin G (IgG) levels, which are frequently high in AIH.
  • Excluding other causes: Viral hepatitis, metabolic diseases, and drug-induced liver injury must be ruled out.
  • Imaging: Dedicated liver imaging may be done to assess the liver and rule out other conditions.
  • Liver biopsy: This is often the gold standard. By examining a small piece of liver tissue under a microscope, doctors can see the characteristic inflammation and damage or scar caused by AIH.

Because AIH can be aggressive in children, making an accurate and quick diagnosis is essential for starting treatment.

Symptoms and Signs in Children

Children with AIH may present in different ways, ranging from mild and silent to severe and sudden. Common signs and symptoms include:

  • Fatigue and lack of energy
  • Jaundice (yellowing of skin and eyes)
  • Abdominal pain or swelling
  • Dark urine and pale stools
  • Nausea, loss of appetite, or weight loss
  • Joint pain or muscle aches
  • Itching (pruritus)
  • Enlarged liver and/or spleen, sometimes detected on exam
  • Acute hepatitis presentation, with sudden jaundice and illness (seen in many pediatric cases)

Some children may have few or no symptoms early on, which makes regular monitoring important once suspicion is raised.

Complexity and the Need for More Research

Autoimmune hepatitis shows just how complex autoimmune diseases can be:

  • Many factors interact — genetics, infections, drugs, environment, and even gut bacteria.
  • Children may differ from adults, but most studies have been done in adult populations.
  • Rare disease challenges — Because AIH is uncommon, especially in children, it is hard to run large studies.

What Families Should Know

  • If your child has been diagnosed with autoimmune hepatitis, it is not your fault. The disease arises from factors outside of anyone’s control.
  • Having “risk genes” does not mean a child will definitely be diagnosed with AIH.
  • Families can contribute to progress by staying engaged with research efforts, registries, and advocacy groups.
  • Ongoing research is essential for developing more effective treatments and, hopefully, finding ways to prevent AIH in the future. Healthcare providers play a crucial role in this ongoing effort.
  • Learn more about the life expectancy of a child with autoimmune hepatitis and what current research shows.

Key Takeaways:

  • AIH can affect children, though it is rare.
  • Genetics and environmental factors both play roles, but we don’t fully understand how.
  • Diagnosis relies on blood work, the exclusion of other diseases, and often a liver biopsy.
  • Symptoms vary, but jaundice, fatigue, and abdominal pain are common in children.
  • Seek support networks and advocate for your family with the AIHA Pediatric Patient Tool Kit.

Join the Autoimmune Hepatitis Association

Learn more about topics like what causes autoimmune hepatitis in children and other aspects of living with AIH. Become a member of the Autoimmune Hepatitis Association to connect with others who understand your journey. Stay informed with the latest research updates and gain early access to educational content and event tickets.