Last reviewed: 20 Sep 2020
Last updated: 18 Apr 2019



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 gender
  • 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-GT
  • alkaline phosphatase
  • serum globulin
  • serum albumin
  • prothrombin time
More 1st investigations to order

Investigations to consider

  • antinuclear antibodies (ANAs)
  • smooth muscle antibodies (SMAs)
  • antisoluble 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)
  • 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
More investigations to consider

Emerging tests

  • antiactin antibodies (AAAs)
  • antibodies against asialoglycoprotein receptor (anti-ASGP-R)
More emerging tests

Treatment algorithm


George Y. Wu

Professor of Medicine

University of Connecticut School of Medicine




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




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



KP declares that he has no competing interests.

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