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'Can we cure IgG4-related diseases?'
Kazuichi Okazaki1, Tsukasa Ikeura2, Kazushige Uchida3
1Department of Internal Medicine, Kansai Medical University Kori Hospital, Neyagawa, Osaka 572-8551, Japan.
Current Opinion in Immunology
|May 21, 2025
Summary
Immunoglobulin G4-related disease (IgG4-RD) is a rare, chronic, multi-organ disorder. Future treatments may target plasmablasts, B cells, and specific T cells involved in IgG4-RD pathogenesis.
Area of Science:
- Immunology
- Rheumatology
- Pathology
Background:
- IgG4-related disease (IgG4-RD) is a novel, rare, chronic, immune-mediated fibroinflammatory disorder.
- It affects multiple organs and its pathogenic mechanisms are not fully understood.
- Potential factors include genetics, antigens, and aberrant immune responses.
Purpose of the Study:
- To review the current understanding of IgG4-related disease (IgG4-RD) pathogenesis.
- To discuss current therapeutic strategies for IgG4-RD.
- To identify promising future treatment targets for IgG4-RD.
Main Methods:
- Literature review of IgG4-related disease (IgG4-RD).
- Analysis of immunocytes involved in IgG4-RD pathogenesis.
- Evaluation of current and potential future therapies for IgG4-RD.
Main Results:
- Multiple immunocytes, including neutrophil extracellular traps, M2 macrophages, plasmablasts, B cells, and various T cells (Th2-CD4+T, Tfh, CD4+SLAMF7+ cytotoxic T cells), are implicated in IgG4-RD.
- Conventional therapies involve glucocorticoids or rituximab, often with immunomodulators.
- Limited randomized clinical trials hinder comprehensive management establishment.
Conclusions:
- Current management of IgG4-RD relies on conventional therapies, but comprehensive strategies are lacking due to few clinical trials.
- Targeting plasmablasts, B cells, and CD4+SLAMF7+ cytotoxic T cells shows promise for future IgG4-RD treatments.
- Further research and clinical trials are needed to establish definitive management protocols for IgG4-RD.
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