{"title":"‘Can we cure IgG4-related diseases?’","authors":"Kazuichi Okazaki , Tsukasa Ikeura , Kazushige Uchida","doi":"10.1016/j.coi.2025.102564","DOIUrl":null,"url":null,"abstract":"<div><div>IgG4-related disease (IgG4-RD), recognized as a novel clinical entity, is a rare, chronic, immune-mediated systemic fibroinflammatory disorder of unknown origin with either synchronous or metachronous multi-organ involvement. Although the pathogenic mechanism remains unclear, possible multipathogenic factors such as genetic backgrounds, disease-specific or related antigens, and abnormal innate or adaptive immunity may be involved. Many immunocytes, including neutrophil extracellular trap, M2 macrophage, plasmablast, B cells, and T-cells (Th2-CD4<sup>+</sup>T, follicular helper T cells, and CD4<sup>+</sup>SLAMF7<sup>+</sup>cytotoxic T cells) play important roles in the pathogenesis. Conventional therapies with glucocorticoid or rituximab in combination with/without immunomodulators are recommended in all symptomatic patients with active IgG4-RD. Because of a few of randomized clinical trials, the comprehensive management for IgG4-RD has not been established yet. Targeted treatment approaches against the plasmablast to B cell lineage and the CD4<sup>+</sup>SLAMF7<sup>+</sup>cytotoxic T cell seem to be promising for the future-directed treatment.</div></div>","PeriodicalId":11361,"journal":{"name":"Current Opinion in Immunology","volume":"95 ","pages":"Article 102564"},"PeriodicalIF":5.8000,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Current Opinion in Immunology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0952791525000408","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"IMMUNOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
IgG4-related disease (IgG4-RD), recognized as a novel clinical entity, is a rare, chronic, immune-mediated systemic fibroinflammatory disorder of unknown origin with either synchronous or metachronous multi-organ involvement. Although the pathogenic mechanism remains unclear, possible multipathogenic factors such as genetic backgrounds, disease-specific or related antigens, and abnormal innate or adaptive immunity may be involved. Many immunocytes, including neutrophil extracellular trap, M2 macrophage, plasmablast, B cells, and T-cells (Th2-CD4+T, follicular helper T cells, and CD4+SLAMF7+cytotoxic T cells) play important roles in the pathogenesis. Conventional therapies with glucocorticoid or rituximab in combination with/without immunomodulators are recommended in all symptomatic patients with active IgG4-RD. Because of a few of randomized clinical trials, the comprehensive management for IgG4-RD has not been established yet. Targeted treatment approaches against the plasmablast to B cell lineage and the CD4+SLAMF7+cytotoxic T cell seem to be promising for the future-directed treatment.
期刊介绍:
Current Opinion in Immunology aims to stimulate scientifically grounded, interdisciplinary, multi-scale debate and exchange of ideas. It contains polished, concise and timely reviews and opinions, with particular emphasis on those articles published in the past two years. In addition to describing recent trends, the authors are encouraged to give their subjective opinion of the topics discussed.
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Current Opinion in Immunology builds on Elsevier''s reputation for excellence in scientific publishing and long-standing commitment to communicating reproducible biomedical research targeted at improving human health. It is a companion to the new Gold Open Access journal Current Research in Immunology and is part of the Current Opinion and Research(CO+RE) suite of journals. All CO+RE journals leverage the Current Opinion legacy-of editorial excellence, high-impact, and global reach-to ensure they are a widely read resource that is integral to scientists'' workflow.