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PubMed This is a summary of 25 peer-reviewed journal articles Updated
Hepatology

Understanding Your Diagnosis: What is Autoimmune Hepatitis?

At a Glance

Autoimmune hepatitis (AIH) is a chronic but treatable condition where the immune system mistakenly attacks the liver, causing inflammation. With proper medication, most patients achieve remission, meaning liver enzymes return to normal and further liver damage is prevented.

Receiving a diagnosis of Autoimmune Hepatitis (AIH) can feel overwhelming, but it is important to know that you are not alone and this condition is not your fault. AIH is a chronic, progressive condition where your immune system, which normally protects you from germs, mistakenly identifies your liver cells as “foreign” and attacks them [1][2]. This leads to inflammation (swelling and irritation) of the liver, which can cause damage over time if left untreated [3].

While the diagnosis may be new to you, AIH is becoming more commonly recognized. Recent studies show that the number of people living with AIH has doubled over the last decade in some areas [4][5]. It is much more common in women than in men, though it can affect anyone at any age [6][7].

Why the Immune System Attacks

In a healthy body, the immune system has “tolerance”—it knows which cells are yours and which are invaders. In AIH, this tolerance is lost [1]. Scientists believe this happens because of an imbalance between two types of immune cells:

  • Effector T Cells: These are the “soldier” cells that cause inflammation to fight off threats [8].
  • Regulatory T Cells (Tregs): These are the “peacekeeper” cells that are supposed to turn off the immune response once the threat is gone [8][9].

In people with AIH, the peacekeepers (Tregs) are often weak or outnumbered, allowing the soldier cells (Effector T cells) to cause ongoing, unnecessary inflammation in the liver [8][10].

Types of Autoimmune Hepatitis

Doctors generally divide AIH into two main types based on the specific autoantibodies (proteins the immune system makes to attack its own tissues) found in your blood:

Feature Type 1 AIH Type 2 AIH
Common Antibodies ANA (Antinuclear Antibody) or SMA (Anti-smooth Muscle Antibody) [11] LKM-1 (Liver Kidney Microsomal type 1) or LC-1 (Liver Cytosol type 1) [11]
Who it affects Children and adults of any age [12] Predominantly diagnosed in children and young adults [12]
Frequency The most common form worldwide [13] Much less common than Type 1 [11]

Both types share similar symptoms and are treated with medications that calm the immune system [14][15].

The Role of Genetics and Environment

You might wonder why this happened to you. Research suggests that certain people are born with a genetic susceptibility, particularly in a group of genes called the HLA region (Human Leukocyte Antigen) [2][16]. This region helps your immune system tell the difference between your own proteins and those of a virus or bacteria.

However, genes are only part of the story. It usually takes an environmental “trigger”—such as a virus, a certain medication, or even changes in the gut microbiome—to start the immune attack in someone who is already genetically predisposed [17][18][19].

Associated Conditions

It is common for AIH to “travel” with other autoimmune issues. Between 14% and 44% of people with AIH have at least one other autoimmune condition [20][21]. These can include:

  • Autoimmune Thyroid Disease (e.g., Graves’ disease or Hashimoto’s) [22]
  • Celiac Disease (gluten intolerance) [23]
  • Type 1 Diabetes [24]
  • Inflammatory Bowel Disease (Ulcerative Colitis or Crohn’s disease) [22]

A Manageable Future

While AIH is a lifelong (chronic) condition, it is highly treatable. The goal of treatment is to reach biochemical remission, which means your liver enzymes (tested via blood work) return to normal levels [3][25]. Most patients achieve this using standard medications that suppress the overactive immune response, allowing the liver to heal and preventing long-term damage [3][15].

Common questions in this guide

What is the difference between Type 1 and Type 2 autoimmune hepatitis?
Type 1 is the most common form, affects people of any age, and involves ANA or SMA antibodies. Type 2 is much less common, usually affects children and young adults, and involves LKM-1 or LC-1 antibodies.
Why did I get autoimmune hepatitis?
AIH is caused by a loss of immune system tolerance, where 'peacekeeper' cells fail to stop 'soldier' cells from attacking the liver. This usually happens due to a combination of genetic susceptibility and an environmental trigger like a virus or medication.
Does having AIH put me at risk for other autoimmune diseases?
Yes, having AIH increases your chances of developing other autoimmune issues. Up to 44% of people with AIH also have conditions like autoimmune thyroid disease, celiac disease, type 1 diabetes, or inflammatory bowel disease.
Can autoimmune hepatitis be cured?
While AIH is a chronic, lifelong condition, it is highly manageable. Treatments that suppress the overactive immune response can stop the liver inflammation, allowing your liver enzymes to return to normal levels and preventing long-term damage.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Which type of autoimmune hepatitis (Type 1 or Type 2) do I have based on my antibody tests?
  2. 2.What do my baseline IgG levels and antibody titers (like ANA or SMA) tell you about how active my disease is right now?
  3. 3.Does my genetic profile, specifically my HLA type, give any clues about how my disease might progress?
  4. 4.Should I be screened for other autoimmune conditions, like thyroid disease or celiac disease, since I have AIH?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (25)
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    Failure of thymic deletion and instability of autoreactive Tregs drive autoimmunity in immune-privileged liver.

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    Genetic and Environmental Risk Factors for Autoimmune Hepatitis.

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    Inequity of care provision and outcome disparity in autoimmune hepatitis in the United Kingdom.

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This page provides an educational overview of autoimmune hepatitis (AIH). It is not a substitute for professional medical advice, diagnosis, or treatment from your hepatologist or primary care provider.

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