| IgG4相关性疾病(IgG4-RD)发病机制的研究进展 |
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| 引用本文： | 纪宗斐,(综述),马玲瑛,姜林娣,(审校). IgG4相关性疾病(IgG4-RD)发病机制的研究进展[J]. 复旦学报(医学版), 2019, 46(1): 114-118. DOI: 10.3969/j.issn.1672-8467.2019.01.020 |
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| 作者姓名： | 纪宗斐 (综述) 马玲瑛 姜林娣 (审校) |
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| 作者单位： | 1复旦大学附属中山医院风湿免疫科 上海 200032; 2复旦大学风湿免疫过敏中心 上海 200032 |
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| 基金项目： | 复旦大学附属中山医院青年基金(2014ZSQN13) |
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| 摘 要： | IgG4 相关性疾病(IgG4-related disease,IgG4-RD)是以多发脏器肿大、血清IgG4升高为特点的一类自身免疫性疾病,发病机制仍不明确。病原体感染导致抗原呈递细胞表面模式识别受体激活,通过抗原呈递促进CD4+T细胞激活,分化为Th1、Th2、Treg和Tfh细胞,产生一系列T细胞因子,进一步促进B细胞活化并转化为浆细胞,产生IgG4。而巨噬细胞和Treg细胞产生的转化生长因子β (transforming growth factor β,TGF-β)和血小板源性生长因子等进一步促进了纤维化的发生。以上细胞因子和免疫细胞参与IgG4-RD发病,为今后的治疗策略提供了新的思路。
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| 关 键 词： | IgG4相关性疾病(IgG4-RD) 发病机制 浆细胞 |
Research progress on the pathogenesis of IgG4-related disease (IgG4-RD) |
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| JI Zong-fei,,MA Ling-ying,,JIANG Lin-di,. Research progress on the pathogenesis of IgG4-related disease (IgG4-RD)[J]. Fudan University Journal of Medical Sciences, 2019, 46(1): 114-118. DOI: 10.3969/j.issn.1672-8467.2019.01.020 |
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| Authors: | JI Zong-fei MA Ling-ying JIANG Lin-di |
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| Affiliation: | 1Department of Rheumatology,Zhongshan Hospital,Fudan University,Shanghai 200032,China;2Institute of Rheumatology,Immunology and Allergy,Fudan University,Shanghai 200032,China |
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| Abstract: | IgG4-related disease (IgG4-RD) is a kind of autoimmune disease which is characterized by multi-organomegaly and elevated serum IgG4.Till now,the pathogenesis of IgG4-RD is unknown.The pattern recognition receptor of antigen-presenting cell is activated by pathogen infection,then CD4+ T cell is activated by antigen presentation and transform into Th1,Th2,Treg and Tfh cell.The cytokines secreted by Th1,Th2,Treg and Tfh cell will promote the activation of B cells and transformation to plasma cells and production of IgG4.Futhermore,transforming growth factor β (TGF-β) and platelet-derived growth factor secreted by macrophage and Treg will promote the fibrosis.The above cytokines and immune cells play important roles in the pathogenesis of IgG4-RD,which provide new strategy for treatment in the future. |
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| Keywords: | IgG4-related disease (IgG4-RD) pathogenesis plasma cell |
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