IgG4-Related Disease: Emerging Roles of Novel Genetic Variants, Immune Cell Subsets and Therapeutic Targets
Date
2025
Authors
Tedesco, L.
Ali, S.B.
Erman, B.
Baris, S.
Pant, H.
Hissaria, P.
Bilginer, Y.
Tumes, D.J.
Cildir, G.
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Journal article
Citation
Allergy. European Journal of Allergy and Clinical Immunology, 2025; 80(10):2738-2754
Statement of Responsibility
Louisa Tedesco, Syed B. Ali, Baran Erman, Safa Baris, Harshita Pant, Pravin Hissaria, Yelda Bilginer, Damon J. Tumes, Gökhan Cildir
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Abstract
IgG4, the least abundant IgG subclass in humans, is increasingly recognised for its involvement in allergic and autoimmune pathologies. Its unique properties, such as the tendency to form half-molecules (one heavy chain and one light chain) and its generally non-inflammatory nature, distinguish it from other IgG subclasses. Its role in immune dysregulation is further underscored by a distinct disease entity called IgG4-related disease (IgG4-RD), a systemic fibroinflammatory disorder characterised by IgG4⁺ plasma cell tissue infiltration, storiform fibrosis and obliterative phlebitis, reflecting a dysregulated immune response affecting multiple organs. This review examines the current understanding of IgG4, its emerging roles in immune dysregulation and IgG4-RD, including clinical manifestations and treatment options. We also discuss the recent advances in understanding the genetic underpinnings of IgG4-RD, highlighting the significance of germline gene variants and implicated immune cell types. Finally, we explore future research directions, emphasising the need for deeper insights into pathogenesis, specific biomarkers and optimised treatment strategies.
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© 2025 The Author(s). Allergy published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.