Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA.
Department of Medicine, Veterans Affairs Palo Alto Health Care System, Palo Alto, CA, USA.
Nat Immunol. 2021 Jan;22(1):10-18. doi: 10.1038/s41590-020-00816-x. Epub 2020 Nov 30.
The immunopathogenesis of rheumatoid arthritis (RA) spans decades, beginning with the production of autoantibodies against post-translationally modified proteins (checkpoint 1). After years of asymptomatic autoimmunity and progressive immune system remodeling, tissue tolerance erodes and joint inflammation ensues as tissue-invasive effector T cells emerge and protective joint-resident macrophages fail (checkpoint 2). The transition of synovial stromal cells into autoaggressive effector cells converts synovitis from acute to chronic destructive (checkpoint 3). The loss of T cell tolerance derives from defective DNA repair, causing abnormal cell cycle dynamics, telomere fragility and instability of mitochondrial DNA. Mitochondrial and lysosomal anomalies culminate in the generation of short-lived tissue-invasive effector T cells. This differentiation defect builds on a metabolic platform that shunts glucose away from energy generation toward the cell building and motility programs. The next frontier in RA is the development of curative interventions, for example, reprogramming T cell defects during the period of asymptomatic autoimmunity.
类风湿关节炎(RA)的免疫发病机制跨越数十年,始于针对翻译后修饰蛋白的自身抗体的产生(检查点 1)。在多年无症状自身免疫和进行性免疫系统重塑之后,组织耐受性丧失,并且由于出现组织侵袭性效应 T 细胞和保护性关节驻留巨噬细胞失败,关节炎症随之发生(检查点 2)。滑膜基质细胞向自身攻击性效应细胞的转化将滑膜炎从急性转化为慢性破坏性(检查点 3)。T 细胞耐受性的丧失源于 DNA 修复缺陷,导致异常的细胞周期动态、端粒脆弱性和线粒体 DNA 不稳定性。线粒体和溶酶体异常最终导致短暂的组织侵袭性效应 T 细胞的产生。这种分化缺陷建立在一个代谢平台上,该平台将葡萄糖从能量产生转向细胞构建和运动程序。RA 的下一个前沿领域是开发治愈性干预措施,例如,在无症状自身免疫期间重编程 T 细胞缺陷。