Last reviewed: 16 Sep 2021
Last updated: 18 Aug 2021

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

Contributors

Authors

George Y. Wu, MD, PhD
George Y. Wu

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
Roopjeet K. Bath

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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