PDF(Pubmed)
Abstract：
IgG4-related disease (IgG4-RD) is a recently described autoimmune disorder characterized by elevated serum IgG4 levels and tissue infiltration of IgG4+ plasma cells in multiple organ systems. Recent advancements have significantly enhanced our understanding of the pathological mechanism underlying this immune-mediated disease. T cell immunity plays a crucial role in the pathogenesis of IgG4-RD, and follicular helper T cells (Tfh) are particularly important in germinal center (GC) formation, plasmablast differentiation, and IgG4 class-switching. Apart from serum IgG4 concentrations, the expansion of circulating Tfh2 cells and plasmablasts may also serve as novel biomarkers for disease diagnosis and activity monitoring in IgG4-RD. Further exploration into the pathogenic roles of Tfh in IgG4-RD could potentially lead to identifying new therapeutic targets that offer more effective alternatives for treating this condition. In this review, we will focus on the current knowledge regarding the pathogenic roles Tfh cells play in IgG4-RD and outline potential therapeutic targets for future clinical intervention.
摘要：
IgG4相关疾病(IgG4-RD)是最近描述的自身免疫性疾病,其特征在于血清IgG4水平升高和多个器官系统中IgG4+浆细胞的组织浸润。最近的进展显着增强了我们对这种免疫介导的疾病的病理机制的理解。T细胞免疫在IgG4-RD的发病机制中起着至关重要的作用。滤泡辅助性T细胞（Tfh）在生发中心（GC）形成中尤为重要，浆细胞分化,和IgG4类切换。除了血清IgG4浓度,循环Tfh2细胞和浆母细胞的扩增也可作为IgG4-RD疾病诊断和活性监测的新型生物标志物.对Tfh在IgG4-RD中的致病作用的进一步探索可能潜在地导致鉴定新的治疗靶标,其提供用于治疗该病症的更有效的替代方案。在这次审查中,我们将重点介绍目前关于Tfh细胞在IgG4-RD中的致病作用的知识,并概述未来临床干预的潜在治疗靶点.
