IgG4-Associated Autoimmune Hepatitis: a Systematic Review

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.