国际眼科纵览 ›› 2023, Vol. 47 ›› Issue (2): 163-169.doi: 10.3760/cma.j.issn.1673-5803.2023.02.012

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IgG4相关性眼病的发病及恶变机制

闫海涵   马建民   

  1. 首都医科大学附属北京同仁医院 北京同仁眼科中心 眼科学与视觉科学北京市重点实验室 100730
  • 收稿日期:2022-11-06 出版日期:2023-04-22 发布日期:2023-04-22
  • 通讯作者: 马建民,Email:jmma@sina.com
  • 基金资助:
    北京市医院管理中心登峰计划专项基金(DFL20190201);北京市自然科学基金(7222025)

Pathogenesis and malignant transformation mechanism of IgG4-related ophthalmic diseases

Yan Haihan, Ma Jianmin   

  1. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital University; Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing 100730, China
  • Received:2022-11-06 Online:2023-04-22 Published:2023-04-22
  • Contact: Ma Jianmin, Email: jmma@sina.com
  • Supported by:
    Beijing Municipal Hospital Management Center Summit plan special fund(DFL20190201); Natural Science Foundation of Beijing (7222025)

摘要: IgG4相关性眼病(IgG4-related ophthalmic disease,IgG4-ROD)是一种隐匿性进展,表现为肿瘤样组织中以IgG4、浆细胞浸润、席纹状纤维化及闭塞性静脉炎的疾病。目前IgG4-ROD的发病机制及恶变机制尚不明确。病原体感染后,固有免疫识别种系编码的病原体识别受体激活B淋巴细胞,在Tfh细胞的辅助下促进B淋巴细胞的活化和同型转换造成了IgG4在组织中的浸润。B淋巴细胞激活细胞毒性T淋巴细胞,并与其共同促进纤维化。IgG4-ROD可恶变为黏膜相关淋巴组织淋巴瘤,免疫和炎症共同参与两者的发病过程。(国际眼科纵览,2023, 47:163-169)

关键词: IgG4相关性眼病, 纤维化, 黏膜相关淋巴组织淋巴瘤

Abstract: IgG4-related ophthalmic disease (IgG4-ROD) is an occult-progressive disease characterized by IgG4 and plasma cell infiltration, mattostriate fibrosis, and occlusive phlebitis in tumor-like tissues. At present, the pathogenesis and malignant mechanism of IgG4-ROD remain unclear. After pathogen infection, the pathogen recognition receptor encoded by the innate immune recognition germ line activates B cells, and promotes the activation and class switch to IgG4 of B cells with the assistance of Tfh cells, resulting in the infiltration of IgG4 in tissues. B cells activate cytotoxic T lymphocyte (CTL) cells, which together with CTL cells promote fibrosis. IgG4-ROD can malicious change into mucosa associated lymphoid tissue type (MALT),  immunity and inflammation may be involved in the pathogenesis.(Int Rev Ophthalmol, 2023, 47: 163-169)

Key words: IgG4-related ophthalmic disease, fibrous, mucosa associated lymphoid tissue type lymphoma