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Related Experiment Video

Updated: Jan 13, 2026

Induction of Drug-Induced, Autoimmune Hepatitis in BALB/c Mice for the Study of Its Pathogenic Mechanisms
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IgG4-Associated Autoimmune Hepatitis: a Systematic Review.

Chun-Hsun Liao1, Hsu-Hua Tseng2, Ting-An Shen3

  • 1Division of Gastroenterology and Hepatology, Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan.

Journal of Gastroenterology and Hepatology
|January 6, 2026
PubMed
Summary

IgG4-associated autoimmune hepatitis (IgG4-AIH) presents unique features overlapping with AIH and IgG4-RD. This review highlights its distinct profile, emphasizing the need for clear diagnostic criteria.

Keywords:
autoimmune hepatitisimmunoglobulin G4‐associated autoimmune hepatitisimmunoglobulin G4‐related diseasesystematic review

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Area of Science:

  • Hepatology
  • Immunology
  • Gastroenterology

Background:

  • IgG4-associated autoimmune hepatitis (IgG4-AIH) is an emerging condition with features of autoimmune hepatitis (AIH) and IgG4-related disease (IgG4-RD).
  • Diagnostic criteria for IgG4-AIH are inconsistent, hindering understanding of its prevalence and clinical significance.

Purpose of the Study:

  • To systematically review and characterize the clinical, serological, and histopathological features of IgG4-AIH.

Main Methods:

  • A systematic literature search was performed using PRISMA guidelines across PubMed, Embase, and Web of Science.
  • Data extraction and quality assessment were conducted by two independent reviewers using Joanna Briggs Institute (JBI) tools.
  • A narrative synthesis approach was employed for data analysis.

Main Results:

  • The review included 43 studies with 185 patients, predominantly Asian females.
  • Common findings included antinuclear antibody positivity (73%-78%), elevated serum IgG4 levels (>135 mg/dL), interface hepatitis, plasma cell infiltration, and advanced fibrosis in ~50% of patients.
  • Glucocorticoid therapy was effective, achieving biochemical remission in over 70% of patients within three months.

Conclusions:

  • IgG4-AIH represents a distinct clinical entity bridging AIH and IgG4-RD.
  • Consensus diagnostic criteria and further prospective studies are crucial for optimizing management and understanding long-term outcomes.