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Autoimmune hepatitis

Last reviewed: 23 Oct 2024
Last updated: 22 Mar 2024

Summary

Definition

History and exam

Key diagnostic factors

  • fatigue/malaise
  • anorexia
  • abdominal discomfort
  • hepatomegaly
  • jaundice
  • encephalopathy
Full details

Other diagnostic factors

  • pruritus
  • arthralgia
  • nausea
  • amenorrhea
  • spider angiomata
  • fever
  • maculopapular skin rash
  • gastrointestinal bleeding
  • splenomegaly
  • ascites
Full details

Risk factors

  • female sex
  • genetic predisposition
  • immune dysregulation
  • measles virus
  • cytomegalovirus
  • Epstein-Barr virus
  • hepatitis viruses A, C, and D
  • certain drugs
Full details

Diagnostic tests

1st tests to order

  • aspartate transaminase
  • alanine transaminase
  • bilirubin
  • gamma glutamyl transferase (gamma-GT)
  • alkaline phosphatase
  • serum globulin
  • serum albumin
  • prothrombin time
Full details

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
Full details

Emerging tests

  • Transient elastography (TE)

Treatment algorithm

INITIAL

acute severe disease

ACUTE

active disease

inactive disease or minimally active disease with comorbidities

AIH-primary biliary cirrhosis overlap syndrome

ONGOING

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
George Y. Wu

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
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.

  • Differentials

    • Primary biliary cirrhosis
    • Primary sclerosing cholangitis
    • Chronic hepatitis B
    More Differentials
  • Guidelines

    • 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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