Last reviewed:September 2019
Last updated:April  2019

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
  • fever
  • spider angiomata
  • gastrointestinal bleeding
  • splenomegaly
  • ascites

Risk factors

  • female gender
  • 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-GT
  • alkaline phosphatase
  • serum globulin
  • serum albumin
  • prothrombin time
Full details

Investigations to consider

  • anti-nuclear 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)
  • 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
Full details

Emerging tests

  • anti-actin antibodies (AAAs)
  • antibodies against asialoglycoprotein receptor (anti-ASGP-R)
Full details

Treatment algorithm

Contributors

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 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.

Roopjeet K. Bath

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 reviewersVIEW ALL

Assistant Professor

Division of Gastroenterology

Department of Medicine

Brown University

RI

Disclosures

KP declares that he has no competing interests.

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