William S. Rowe Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Curr Opin Pediatr. 2011 Dec;23(6):640-6. doi: 10.1097/MOP.0b013e32834cba24.
Systemic juvenile idiopathic arthritis (SJIA) is an inflammatory condition characterized by fever, lymphadenopathy, rash, arthritis, and serositis. Although the ultimate cause of this disorder remains elusive, recent work defining cytokine effector mechanisms has led to a new treatment paradigm for this condition. In this review, we describe the recent immunological reclassification of SJIA as an autoinflammatory disorder as well as detailing the dramatic changes in its treatment.
SJIA is an autoinflammatory disorder in which defects of innate immune system pathways lead to significant inflammation. Recent studies of the pathophysiology, as well as successful treatment trials, have established interleukin-1β (IL-1β) and IL-6 as key cytokines in the pathogenesis of this condition. As a result, their inhibition has become the centerpiece of the current SJIA treatment paradigm.
There has been a major shift away from the traditional treatments of SJIA towards therapeutics that inhibit IL-1β and IL-6. In fact, the IL-1 blocker anakinra is now regarded as standard of care for SJIA patients with systemic symptoms, while the IL-6 inhibitor tocilizumab shows great potential. Future research holds promise for the development of more efficient cytokine inhibition as well a more comprehensive knowledge of the innate cytokine networks in this disease.
全身性幼年特发性关节炎（SJIA）是一种炎症性疾病，其特征为发热、淋巴结病、皮疹、关节炎和浆膜炎。尽管这种疾病的根本原因仍不清楚，但最近对细胞因子效应机制的定义工作为这种疾病的治疗带来了新的范例。在这篇综述中，我们描述了 SJIA 最近被重新归类为自身炎症性疾病，以及其治疗方式的显著变化。
SJIA 是一种自身炎症性疾病，其中先天免疫系统途径的缺陷导致显著的炎症。对其病理生理学的最近研究以及成功的治疗试验已经确立了白细胞介素-1β（IL-1β）和 IL-6 作为该疾病发病机制中的关键细胞因子。因此，它们的抑制已成为当前 SJIA 治疗范例的核心。
SJIA 的传统治疗方法已经发生了重大转变，转向了抑制 IL-1β 和 IL-6 的治疗方法。事实上，IL-1 阻滞剂 anakinra 现在被认为是 SJIA 有全身症状患者的标准治疗方法，而 IL-6 抑制剂tocilizumab 显示出巨大的潜力。未来的研究有望开发更有效的细胞因子抑制以及更全面地了解这种疾病中的先天细胞因子网络。
