Summary
Definition
History and exam
Key diagnostic factors
- presence of risk factors
- fatigue/malaise
- anorexia
- abdominal discomfort
- hepatomegaly
- jaundice
- encephalopathy
Other diagnostic factors
- pruritus
- arthralgia
- nausea
- amenorrhoea
- spider angiomata
- fever
- maculopapular skin rash
- gastrointestinal bleeding
- splenomegaly
- ascites
Risk factors
- female sex
- genetic pre-disposition
- 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 glutamyl transferase (gamma-GT)
- alkaline phosphatase
- serum globulin
- serum albumin
- prothrombin time
Investigations to consider
- antinuclear antibodies (ANAs)
- smooth muscle antibodies (SMAs)
- anti-soluble liver antigens or liver/pancreas (anti-SLA/LP)
- perinuclear anti-neutrophil cytoplasmic antibodies (pANCA)
- antibodies against single-stranded DNA (anti-ssDNA)
- antibodies against double-stranded DNA (anti-dsDNA antibodies)
- antibodies to liver/kidney microsome type 1 antigen (anti-LKM-1 antibodies)
- antibodies to liver/kidney microsome type 3 antigen (anti-LKM-3)
- antibodies to liver cytosol 1 antigen (anti-LC1)
- anti-mitochondrial 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
Treatment algorithm
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.
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