From inflammatory initiation to fibrotic remodeling: mechanisms and precision therapeutic strategies in otorhinolaryngologic involvement of IgG4-related disease - Summary - MDSpire

From inflammatory initiation to fibrotic remodeling: mechanisms and precision therapeutic strategies in otorhinolaryngologic involvement of IgG4-related disease

  • By

  • Minfang Wang

  • Yongcai Weng

  • Jun Ma

  • Yong Wu

  • June 25, 2026

  • 0 min

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Objective:

To integrate current understanding of the immunopathological mechanisms underlying IgG4-RD with organ-specific clinical features and therapeutic considerations in the head and neck.

Approach:
  • Immunopathological Mechanisms: The review discusses the role of clonally expanded CD4+ cytotoxic T lymphocytes and activated follicular helper T cells in driving IgG4 class-switch recombination and plasmablast expansion.
  • Therapeutic Considerations: It evaluates glucocorticoids as first-line treatment, the efficacy of rituximab in refractory cases, and the potential of ineibilizumab and dupilumab.
Key Findings:
  • IgG4-RD is characterized by a Th2-skewed cytokine environment promoting IgG4 class-switching.
  • Different head and neck subtypes of IgG4-RD exhibit varying balances of inflammatory and fibrotic features.
  • Glucocorticoids are standard for remission induction but have high relapse rates.
  • Rituximab is effective for refractory and relapsing disease.
  • Inebilizumab has shown significant benefit in clinical trials, while dupilumab requires further validation.
Interpretation:

The review aims to support a more individualized, biologically informed framework for managing IgG4-RD in the head and neck by mapping immunopathological differences to therapeutic approaches.

Limitations:
  • Accurate prevalence estimates of IgG4-RD remain limited due to low recognition rates.
  • The precise mechanisms underlying organ-specific differences in IgG4-RD are not fully established.
Conclusion:

The findings highlight the complexity of IgG4-RD and the need for tailored treatment strategies based on immunopathological insights.

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