Department of Pathogen Biology, School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Immunol. 2021 Sep 1;12:738540. doi: 10.3389/fimmu.2021.738540. eCollection 2021.
Immunoglobin G-related disease (IgG-RD) is one of the newly discovered autoimmune diseases characterized by elevated serum IgG concentrations and multi-organ fibrosis. Despite considerable research and recent advances in the identification of underlying immunological processes, the etiology of this disease is still not clear. Adaptive immune cells, including different types of T and B cells, and cytokines secreted by these cells play a vital role in the pathogenesis of IgG-RD. Antigen-presenting cells are stimulated by pathogens and, thus, contribute to the activation of naïve T cells and differentiation of different T cell subtypes, including helper T cells (Th1 and Th2), regulatory T cells, and T follicular helper cells. B cells are activated and transformed to plasma cells by T cell-secreted cytokines. Moreover, macrophages, and some important factors (TGF-β, etc.) promote target organ fibrosis. Understanding the role of these cells and cytokines implicated in the pathogenesis of IgG-RD will aid in developing strategies for future disease treatment and drug development. Here, we review the most recent insights on IgG-RD, focusing on immune dysregulation involved in the pathogenesis of this autoimmune condition.
免疫球蛋白 G 相关疾病（IgG-RD）是一种新发现的自身免疫性疾病，其特征是血清 IgG 浓度升高和多器官纤维化。尽管在识别潜在免疫过程方面进行了大量研究和最近的进展，但该疾病的病因仍不清楚。适应性免疫细胞，包括不同类型的 T 和 B 细胞以及这些细胞分泌的细胞因子，在 IgG-RD 的发病机制中起着至关重要的作用。抗原呈递细胞被病原体刺激，从而有助于幼稚 T 细胞的激活和不同 T 细胞亚型的分化，包括辅助性 T 细胞（Th1 和 Th2）、调节性 T 细胞和滤泡辅助性 T 细胞。T 细胞分泌的细胞因子可激活 B 细胞并将其转化为浆细胞。此外，巨噬细胞和一些重要因素（TGF-β 等）可促进靶器官纤维化。了解 IgG-RD 发病机制中涉及的这些细胞和细胞因子的作用将有助于制定未来疾病治疗和药物开发的策略。在这里，我们综述了 IgG-RD 的最新研究进展，重点关注了该自身免疫疾病发病机制中的免疫失调。
