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 sex
- 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 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 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)
- antibodies against asialoglycoprotein receptor (anti-ASGP-R)
- 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
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 of Medicine
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 of Medjaden, and is a book series editor for Nature-Springer.
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.
Differentials
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Chronic hepatitis B
More DifferentialsGuidelines
- Diagnosis and management of autoimmune hepatitis in adults and children
- EASL clinical practice guidelines: autoimmune hepatitis
More Guidelines
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