Summary
Definition
History and exam
Key diagnostic factors
- fatigue/malaise
- anorexia
- abdominal discomfort
- hepatomegaly
- jaundice
- encephalopathy
Other diagnostic factors
- pruritus
- arthralgia
- nausea
- amenorrhea
- spider angiomata
- fever
- maculopapular skin rash
- gastrointestinal bleeding
- splenomegaly
- ascites
Risk factors
- female sex
- genetic predisposition
- immune dysregulation
- measles virus
- cytomegalovirus
- Epstein-Barr virus
- hepatitis viruses A, C, and D
- certain drugs
Diagnostic tests
1st tests to order
- aspartate transaminase
- alanine transaminase
- bilirubin
- gamma glutamyl transferase (gamma-GT)
- alkaline phosphatase
- serum globulin
- serum albumin
- prothrombin time
Tests to consider
- antinuclear antibodies (ANAs)
- smooth muscle antibodies (SMAs)
- anti-soluble liver antigens or liver/pancreas (anti-SLA/LP)
- perinuclear antineutrophil cytoplasmic antibodies (pANCA)
- antibodies against single-stranded DNA (anti-ssDNA)
- antibodies against double-stranded DNA (anti-dsDNA)
- antibodies to liver/kidney microsome type 1 antigen (anti-LKM-1)
- antibodies to liver/kidney microsome type 3 antigen (anti-LKM-3)
- antibodies to liver cytosol 1 antigen (anti-LC1)
- antimitochondrial antibodies (AMAs)
- antibodies against asialoglycoprotein receptor (anti-ASGP-R)
- IgM antibodies to hepatitis A virus (IgM anti-HAV)
- hepatitis B surface antigen (HBsAg)
- antibodies to hepatitis B core antigen (anti-HBc)
- hepatitis C antibodies and viral RNA
- ceruloplasmin
- alpha-1 antitrypsin
- serum iron and total iron binding capacity
- thiopurine methyltransferase (TPMT) activity
- abdominal ultrasound
- liver biopsy
Emerging tests
- Transient elastography (TE)
Treatment algorithm
acute severe disease
active disease
inactive disease or minimally active disease with comorbidities
AIH-primary biliary cirrhosis overlap syndrome
inadequate response to initial therapy or single relapse: without hepatic decompensation
multiple previous relapses: without hepatic decompensation
decompensated liver disease
Contributors
Authors
George Y. Wu, MD, PhD
Professor Emeritus
Division of Gastroenterology - Hepatology
University of Connecticut School of Medicine
Farmington
CT
Disclosures
GYW presents lectures on autoimmune hepatitis nationally and internationally. He has acted as an expert witness for legal firms, is comprehensive editor in chief of the Journal of Clinical and Translational Hepatology, associate editor in chief of Medjaden, associate editor at the Journal of Digestive Diseases, and a book series editor for Springer-Nature, and a consultant in mitochondrial diseases at Mitrix Bio, Inc.
Roopjeet K. Bath, MBBS
Assistant Professor of Medicine
University of Connecticut School of Medicine
Farmington
CT
Disclosures
RKB declares that she has no competing interests.
Acknowledgements
Dr George Y. Wu and Dr Roopjeet K. Bath would like to gratefully acknowledge Dr Micheal Tadros, Dr Lucija Virovic Jukic, Dr Marcy L. Coash, and Dr Deepika Devuni, previous contributors to this topic. Dr Wu would also like to acknowledge the assistance of Dr Nathan Selky in the 2011 update.
Disclosures
MT, LVJ, MLC, and DD declare that they have no competing interests.
Peer reviewers
Kittichai Promrat, MD
Assistant Professor
Division of Gastroenterology
Department of Medicine
Brown University
RI
Disclosures
KP declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
Mack CL, Adams D, Assis DN, et al. Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the Study of Liver Diseases. Hepatology. 2020 Aug;72(2):671-722.Full text Abstract
European Association for the Study of the Liver. EASL clinical practice guidelines: autoimmune hepatitis. J Hepatol. 2015 Oct;63(4):971-1004. Abstract
Gleeson D, Heneghan MA; British Society of Gastroenterology. British Society of Gastroenterology (BSG) guidelines for management of autoimmune hepatitis. Gut. 2011 Dec;60(12):1611-29.Full text Abstract
Strassburg CP, Manns MP. Treatment of autoimmune hepatitis. Semin Liver Dis. 2009 Aug;29(3):273-85. Abstract
Selvarajah V, Montano-Loza AJ, Czaja AJ. Systematic review: managing suboptimal treatment responses in autoimmune hepatitis with conventional and nonstandard drugs. Aliment Pharmacol Ther. 2012 Oct;36(8):691-707.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Chronic hepatitis B
More DifferentialsGuidelines
- ACR appropriateness criteria: abnormal liver function tests
- Clinical practice guidelines: non-invasive tests for evaluation of liver disease severity and prognosis
More Guidelines
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