Summary
Definition
History and exam
Key diagnostic factors
- fatigue/malaise
- anorexia
- abdominal discomfort
- hepatomegaly
- jaundice
- encephalopathy
Other diagnostic factors
- pruritus
- arthralgia
- nausea
- fever
- spider angiomata
- gastrointestinal bleeding
- splenomegaly
- ascites
Risk factors
- female gender
- genetic predisposition
- immune dysregulation
- measles virus
- cytomegalovirus
- Epstein-Barr virus
- hepatitis viruses A, C, and D
- certain drugs
Diagnostic investigations
1st investigations to order
- aspartate transaminase
- alanine transaminase
- bilirubin
- gamma-GT
- alkaline phosphatase
- serum globulin
- serum albumin
- prothrombin time
Investigations to consider
- antinuclear antibodies (ANAs)
- smooth muscle antibodies (SMAs)
- antisoluble 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-KLM-1)
- antibodies to liver/kidney microsome type 3 antigen (anti-LKM-3)
- antibodies to liver cytosol 1 antigen (anti-LC1)
- antimitochondrial antibodies (AMAs)
- IgM antibodies to hepatitis A virus (IgM anti-HAV)
- 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
- antiactin antibodies (AAAs)
- antibodies against asialoglycoprotein receptor (anti-ASGP-R)
Treatment algorithm
Contributors
Authors

Professor of Medicine
University of Connecticut School of Medicine
Farmington
CT
Disclosures
GYW presents lectures on autoimmune hepatitis nationally and internationally. He is a member of the medical advisory boards for Gilead Sciences and Intercept Pharmaceuticals, and has acted as an expert witness for legal firms. GYW is comprehensive editor in chief of the Journal of Clinical and Translational Hepatology and associate editor of Medjaden.

Gastroenterology/Hepatology Fellow
University of Connecticut School of Medicine
Farmington
CT
Disclosures
RKB declares that she has no competing interests.
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. MT, LVJ, MLC, and DD declare that they have no competing interests.
Peer reviewers
Assistant Professor
Division of Gastroenterology
Department of Medicine
Brown University
RI
Disclosures
KP declares that he has no competing interests.
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