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