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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Gastroenterology

Treatment Strategies: Controlling the Immune Attack

At a Glance

The primary goal of autoimmune hepatitis (AIH) treatment is to achieve biochemical remission and stop liver inflammation. Treatment typically starts with corticosteroids, followed by long-term maintenance therapy with immunosuppressants like azathioprine to prevent permanent liver damage.

The primary goal of treating Autoimmune Hepatitis (AIH) is to achieve biochemical remission. This means calming the immune system enough that your liver enzymes (ALT and AST) and your IgG levels return to a normal range [1][2]. When the inflammation is stopped, the liver has a chance to heal, and the risk of long-term scarring (cirrhosis) is significantly reduced [1].

The Standard Treatment Journey

Treatment typically happens in two main phases: Induction and Maintenance.

1. Induction: Calming the Fire

The first step is to quickly stop the active immune attack. The induction phase usually lasts for several weeks to a few months.

  • Corticosteroids: Most patients start with Prednisone or Prednisolone [3]. These are powerful anti-inflammatory drugs that act quickly to “put out the fire” in the liver [4].
  • Starting Azathioprine: To avoid the long-term side effects of high-dose steroids, doctors often add Azathioprine (an immunosuppressant and steroid-sparing medication) shortly after or alongside the steroids [3][5].

Important Safety Step: Before starting Azathioprine, your doctor should run a blood test for TPMT (thiopurine methyltransferase). This test checks your genetic ability to process the drug safely. If your TPMT levels are very low, azathioprine could be dangerous, and your doctor will choose a different medication [4].

2. Maintenance: Keeping the Peace

Once your liver tests normalize and your steroids have been safely tapered down, you enter the maintenance phase.

  • Most patients move to taking azathioprine alone or alongside a very small “maintenance dose” of prednisone [1].
  • Duration: Maintenance therapy is usually required for at least two years of perfect laboratory results before a doctor will even consider a trial of stopping the medication [6][7].

Understanding Medication Side Effects

These medications are life-saving, but they carry significant side effects that require careful management.

Corticosteroids (Prednisone)

  • Common immediate effects: Weight gain, mood changes (irritability, anxiety), difficulty sleeping, and “moon face” (swelling of the face).
  • Long-term risks: Bone loss (osteoporosis), elevated blood sugar (diabetes risk), and high blood pressure.

Immunosuppressants (Azathioprine / MMF)

  • Infection Risk: Because these drugs suppress your immune system, you are at a much higher risk for infections. If you develop a fever, you must seek medical care immediately.
  • Cancer Risk: Azathioprine increases your risk of certain skin cancers.
  • Vaccines: Never receive a “live” vaccine (such as the measles, mumps, and rubella (MMR) or the nasal flu vaccine) while on immunosuppressants, as this can cause a severe, life-threatening infection. Always ensure your non-live vaccines are up to date before starting induction therapy.

Second-Line Options: When Standard Care Isn’t Enough

If you cannot tolerate azathioprine or if your liver doesn’t respond, other options are available:

  • Mycophenolate Mofetil (MMF): This is the most common “second-line” therapy [8]. It is highly effective for people who had to stop azathioprine due to side effects [9][10]. Crucial Warning: MMF can cause severe birth defects and must never be taken during pregnancy.
  • Tacrolimus: Sometimes used for cases where the disease doesn’t respond to steroids [11][12].

Understanding Relapse

It is very common for AIH to “flare up” again if medication is stopped or reduced too quickly. Because the underlying genetic tendency toward AIH doesn’t go away, many patients require some form of maintenance therapy for the rest of their lives to prevent permanent liver damage [7][1].

When is a Transplant Necessary?

For the vast majority of patients, medication successfully controls AIH. However, a liver transplant may be necessary if the initial attack is extremely severe (Acute Liver Failure) or if years of inflammation have caused enough scarring that the liver can no longer function (End-Stage Liver Disease) [13][14][15]. The success rate for liver transplants in AIH patients is excellent, with long-term survival rates around 80% [16][17].

Common questions in this guide

What are the phases of autoimmune hepatitis treatment?
Treatment usually happens in two main phases. The induction phase uses powerful steroids to quickly stop the active immune attack on your liver. The maintenance phase uses long-term immunosuppressants to keep the disease controlled with fewer side effects.
Why do I need a TPMT test before starting azathioprine?
A TPMT blood test checks your genetic ability to process azathioprine safely. If your levels of the TPMT enzyme are too low, the medication can cause dangerous side effects, and your doctor will need to prescribe an alternative treatment.
Can I stop taking my AIH medication once my liver enzymes are normal?
You should never stop medication without consulting your doctor. Many patients require lifelong maintenance therapy to prevent permanent liver damage, as the disease frequently flares up if medications are reduced or stopped too quickly.
What should I do if I get a fever while taking AIH immunosuppressants?
Because immunosuppressant medications lower your body's ability to fight off infections, you are at a much higher risk for getting sick. If you develop a fever while on these drugs, you must seek medical care immediately.
Are there alternative treatments if I cannot tolerate azathioprine?
Yes, if azathioprine causes unmanageable side effects or doesn't work well enough, your doctor may recommend second-line options. Mycophenolate Mofetil (MMF) is the most common alternative, though it cannot be taken during pregnancy.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Will you test my TPMT levels before I start azathioprine to make sure my body can process it safely?
  2. 2.What is my specific timeline for the 'induction' phase, and when do we plan to start tapering my steroids?
  3. 3.If I develop a fever or signs of infection while on immunosuppressants, what exact steps should I take?
  4. 4.What are my options if I cannot tolerate azathioprine due to side effects?
  5. 5.Are my immunizations up to date, and which vaccines (especially live vaccines) do I need to avoid while on treatment?

Questions For You

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References

References (17)
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    Inequity of care provision and outcome disparity in autoimmune hepatitis in the United Kingdom.

    Dyson JK, Wong LL, Bigirumurame T, et al.

    Alimentary pharmacology & therapeutics 2018; (48(9)):951-960 doi:10.1111/apt.14968.

    PMID: 30226274
  2. 2

    Aminotransferases During Treatment Predict Long-Term Survival in Patients With Autoimmune Hepatitis Type 1: A Landmark Analysis.

    Biewenga M, Verhelst X, Baven-Pronk M, et al.

    Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 2022; (20(8)):1776-1783.e4 doi:10.1016/j.cgh.2021.05.024.

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    Systematic review with meta-analysis: mycophenolate mofetil as a second-line therapy for autoimmune hepatitis.

    Santiago P, Schwartz I, Tamariz L, Levy C

    Alimentary pharmacology & therapeutics 2019; (49(7)):830-839 doi:10.1111/apt.15157.

    PMID: 30761563
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    Biochemical efficacy of tioguanine in autoimmune hepatitis: a retrospective review of practice in the Netherlands.

    van den Brand FF, van Nieuwkerk CMJ, Verwer BJ, et al.

    Alimentary pharmacology & therapeutics 2018; (48(7)):761-767 doi:10.1111/apt.14939.

    PMID: 30109891
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    High discontinuation rate of azathioprine in autoimmune hepatitis, independent of time of treatment initiation.

    Pape S, Gevers TJG, Vrolijk JM, et al.

    Liver international : official journal of the International Association for the Study of the Liver 2020; (40(9)):2164-2171 doi:10.1111/liv.14513.

    PMID: 32410363
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    Well-controlled autoimmune hepatitis treatment withdrawal may be safely accomplished without liver-biopsy guidance.

    Guirguis J, Alonso Y, Lopez R, Carey W

    Gastroenterology report 2018; (6(4)):284-290 doi:10.1093/gastro/goy020.

    PMID: 30430017
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    Stopping immunosuppressive treatment in autoimmune hepatitis (AIH): Is it justified (and in whom and when)?

    Harrison L, Gleeson D

    Liver international : official journal of the International Association for the Study of the Liver 2019; (39(4)):610-620 doi:10.1111/liv.14051.

    PMID: 30667576
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    A reasoned approach to the treatment of autoimmune hepatitis.

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    Mycophenolate mofetil treatment in patients with autoimmune hepatitis failing standard therapy with prednisolone and azathioprine.

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    Efficacy and Safety of Mycophenolate Mofetil in Patients With Autoimmune Hepatitis and Suboptimal Outcomes After Standard Therapy.

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    PMID: 29050991
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    Comparative Effectiveness of Mycophenolate Mofetil and Tacrolimus as a Second-Line Therapy for Autoimmune Hepatitis: A Systematic Review and Meta-Analysis.

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    Efficacy and Safety of Mycophenolate Mofetil and Tacrolimus as Second-line Therapy for Patients With Autoimmune Hepatitis.

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    Long-Term Observation of Cyclosporine as Second-Line Therapy in Adults for Severe Acute Autoimmune Hepatitis.

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This page explains autoimmune hepatitis treatments and medications for educational purposes only. Always consult your hepatologist or gastroenterologist before changing your medication regimen or stopping any prescribed treatments.

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