Department of Medical Biochemistry and Biophysics, Medical Inflammation Research, Karolinska Institutet, Stockholm, Sweden.
Eur J Immunol. 2014 Jun;44(6):1593-9. doi: 10.1002/eji.201444486. Epub 2014 May 3.
Extensive genome-wide association studies have recently shed some light on the causes of chronic autoimmune diseases and have confirmed a central role of the adaptive immune system. Moreover, better diagnostics using disease-associated autoantibodies have been developed, and treatment has improved through the development of biologicals with precise molecular targets. Here, we use rheumatoid arthritis (RA) as a prototype for chronic autoimmune disease to propose that the pathogenesis of autoimmune diseases could be divided into three discrete stages. First, yet unknown environmental challenges seem to activate innate immunity thereby providing an adjuvant signal for the induction of adaptive immune responses that lead to the production of autoantibodies and determine the subsequent disease development. Second, a joint-specific inflammatory reaction occurs. This inflammatory reaction might be clinically diagnosed as the earliest signs of the disease. Third, inflammation is converted to a chronic process leading to tissue destruction and remodeling. In this review, we discuss the stages involved in RA pathogenesis and the experimental approaches, mainly involving animal models that can be used to investigate each disease stage. Although we focus on RA, it is possible that a similar stepwise development of disease also occurs in other chronic autoimmune settings such as multiple sclerosis (MS), type 1 diabetes, and systemic lupus erythematosus.
最近,广泛的全基因组关联研究揭示了慢性自身免疫性疾病的病因,并证实了适应性免疫系统的核心作用。此外,利用与疾病相关的自身抗体开发了更好的诊断方法,通过开发针对精确分子靶点的生物制剂,治疗也得到了改善。在这里,我们以类风湿关节炎 (RA) 为例,提出自身免疫性疾病的发病机制可以分为三个不同的阶段。首先,未知的环境挑战似乎激活了先天免疫,从而为诱导导致自身抗体产生并决定随后疾病发展的适应性免疫反应提供了辅助信号。其次,会发生关节特异性炎症反应。这种炎症反应可能在临床上被诊断为疾病的最早迹象。第三,炎症转变为慢性过程,导致组织破坏和重塑。在这篇综述中,我们讨论了 RA 发病机制中的各个阶段,以及主要涉及动物模型的实验方法,这些模型可用于研究每个疾病阶段。虽然我们重点讨论 RA,但在多发性硬化症 (MS)、1 型糖尿病和系统性红斑狼疮等其他慢性自身免疫性疾病中,也可能存在类似的逐步发病过程。