National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Autoimmune hepatitis


Other Names:
Autoimmune chronic hepatitis; AIH
Categories:
Autoimmune hepatitis is a disease in which the body’s immune system attacks liver cells. This immune response causes inflammation of the liver, also called hepatitis. The disease can be quite serious and, if not treated, gets worse over time, leading to cirrhosis of the liver and/or liver failure. Autoimmune hepatitis likely results from a combination of autoimmunity, environmental triggers, and a genetic predisposition. It occurs more frequently in females. Treatment typically includes corticosteroids and medications that suppress the immune system. In severe cases, a liver transplant may be needed.[1][2]
Last updated: 10/1/2016
Signs and symptoms in people with autoimmune hepatitis range from mild to severe depending on the amount of liver damage present. Symptoms are generally due to scarring of liver tissue (cirrhosis). Some people have no symptoms at first and are diagnosed after being evaluated for another health problem.[3] Some of the most common signs and symptoms in people with autoimmune hepatitis may be nonspecific and include:[1][2][3][4]
  • Fatigue (the most common symptom reported).
  • Nausea.
  • Loss of appetite.
  • Diarrhea.
  • Jaundice (yellowing of the skin and whites of the eyes).

Other signs and symptoms that may develop as the disease progresses include:[1][2][3][4]

  • Loss of brain function (hepatic encephalopathy).
  • Fluid in the abdomen (ascites).
  • Swelling of the legs (edema).
  • Easy bruising and bleeding.
  • An enlarged spleen (splenomegaly).
  • Gallstones.
  • Itchy skin (pruritis) or skin rashes.
  • Joint pain.
  • Vomiting.
  • Dark urine.
  • Pale or gray-colored stools.
  • Absence of menstrual periods in women (amenorrhea).
Some symptoms a person experiences may be due to other underlying heath conditions or autoimmune diseases that are associated with autoimmune hepatitis.[1][2][3]
Last updated: 1/8/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
100% of people have these symptoms
Increased circulating IgG level 0003237
80%-99% of people have these symptoms
Anti-liver cytosolic antigen type 1 antibody positivity 0030909
Antineutrophil antibody positivity 0003453
Antinuclear antibody positivity 0003493
Elevated hepatic transaminase
High liver enzymes
0002910
Liver kidney microsome type 1 antibody positivity 0030908
Smooth muscle antibody positivity 0003262
30%-79% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ] 		0002027
Arthralgia
Joint pain
0002829
Chronic fatigue
Chronic extreme exhaustion
0012432
Depressivity
Depression
0000716
Spider hemangioma 0012522
5%-29% of people have these symptoms
Acute hepatitis
Acute liver inflammation
0200119
Anxiety
Excessive, persistent worry and fear
0000739
Arthritis
Joint inflammation
0001369
Ascites
Accumulation of fluid in the abdomen
0001541
Cirrhosis
Scar tissue replaces healthy tissue in the liver
0001394
Diffuse hepatic steatosis 0006555
Gastrointestinal hemorrhage
Gastrointestinal bleeding
0002239
Glomerulonephritis 0000099
Increased total bilirubin
High bili total
0003573
Jaundice
Yellow skin
Yellowing of the skin
[ more ] 		0000952
Sclerosing cholangitis 0030991
Splenomegaly
Increased spleen size
0001744
Thyroiditis
Thyroid gland inflammation
0100646
Ulcerative colitis 0100279
Vitiligo
Blotchy loss of skin color
0001045
1%-4% of people have these symptoms
Fulminant hepatitis 0004787
Hepatocellular carcinoma 0001402
Viral hepatitis 0006562
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Last updated: 7/1/2020
The underlying cause of autoimmune hepatitis is not known. It is possible that any of several genetic or environmental factors (such as medications or viral infections) may trigger the disease.[1][3] People with another autoimmune disease or a family history of autoimmune disease may be more likely to develop autoimmune hepatitis.[5]

Symptoms of autoimmune diseases in general are caused by the body's immune system mistakenly attacking healthy tissues and cells. In the case of autoimmune hepatitis, the body's immune system attacks healthy liver tissue, ultimately damaging the liver.
Last updated: 1/8/2018
The diagnosis of autoimmune hepatitis is typically made based on a person's signs and symptoms, medical history, various blood tests, and a liver biopsy.[1] Blood tests may be able to tell autoimmune hepatitis apart from viral hepatitis, or from other health problems that cause similar symptoms. A liver biopsy can confirm the diagnosis and determine the extent of liver damage.[5] Sometimes a person with no symptoms is suspected of having the disease after a routine health exam or when being evaluated for another health problem.[3]
Last updated: 1/8/2018
Treatment for autoimmune hepatitis should be managed by a hepatologist, which is a doctor who specializes in liver disease.[6] Treatment aims to suppress the immune system, which is overactive in people with this disease. In many cases, especially when the disease is diagnosed early, treatment can slow the progression of the disease and may reverse some of the liver damage that has already occurred. People who have no symptoms or have a very mild form of the disease may not need treatment.[1] A hepatologist may evaluate symptoms and use various laboratory tests to determine when a person should begin treatment.[6]

Medications that may be used initially ("induction therapy") include :[1][6]
  • Corticosteroids (such as prednisone).
  • Azathioprine (often in combination with corticosteroids).
  • Other immune system suppressants - particularly when treatment with corticosteroids and azathioprine is not effective or causes severe side effects. Examples include mycophenolate mofetil, cyclosporine, or tacrolimus.
Most people go into remission with initial treatment within two to three years. This means that their symptoms improve, and laboratory tests show that liver function is improving. In some cases, people who achieve remission can taper off medications for a period of time.[6] However, relapses are common, and many people need long-term management ("maintenance therapy") to keep the disease under control.[3][6] Maintenance therapy may involve long-term use of much lower doses of prednisone or azathioprine, which are effective in controlling the disease is most people.[6]

Some people do not respond to treatment or cannot continue treatment due to side effects.[6] If the disease progresses and causes severe cirrhosis and liver failure, a liver transplant may be needed.[1] About 10-20% of people with autoimmune hepatitis eventually need a liver transplant.[3]
Last updated: 1/9/2018

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Autoimmune hepatitis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Patient Registry

  • The Autoimmune Registry supports research for Autoimmune hepatitis by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • Mayo Clinic has an information page on Autoimmune hepatitis.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Digestive Diseases Information Clearinghouse (NIDDK) offers information on this condition. Click on the link to view information on this topic.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Autoimmune hepatitis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Autoimmune Hepatitis. National Digestive Diseases Information Clearinghouse (NDDIC). March 2014; https://www.niddk.nih.gov/health-information/health-topics/liver-disease/autoimmune-hepatitis/Pages/facts.aspx.
  2. Phillips MM. Autoimmune hepatitis. MedlinePlus. August, 2016; http://www.nlm.nih.gov/medlineplus/ency/article/000245.htm.
  3. Wolf DC. Autoimmune Hepatitis. Medscape Reference. September 25, 2017; http://emedicine.medscape.com/article/172356-overview.
  4. Symptoms of Liver Failure. American Liver Foundation. http://he123.liverfoundation.org/what-is-he/liver-failure-symptoms/. Accessed 1/9/2018.
  5. Autoimmune hepatitis. Mayo Clinic. August 4, 2017; https://www.mayoclinic.org/diseases-conditions/autoimmune-hepatitis/symptoms-causes/syc-20352153.
  6. Heneghan MA. Autoimmune hepatitis: Treatment. UpToDate. Waltham, MA: UpToDate; September 27, 2017; https://www.uptodate.com/contents/autoimmune-hepatitis-treatment.