Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
Adv Exp Med Biol. 2024;1448:33-42. doi: 10.1007/978-3-031-59815-9_4.
Cytokine storm syndrome (CSS) is a severe life-threatening condition characterized by a clinical phenotype of overwhelming systemic inflammation, hyperferritinemia, hemodynamic instability, and multiple organ failure (MOF), and, if untreated, it can potentially lead to death. The hallmark of CSS is an uncontrolled and dysfunctional immune response involving the continual activation and expansion of lymphocytes and macrophages, which secrete large amounts of cytokines, causing a cytokine storm. Many clinical features of CSS can be explained by the effects of pro-inflammatory cytokines, such as interferon (IFN)-γ, tumor necrosis factor (TNF), interleukin (IL)-1, IL-6, and IL-18 [1-7]. These cytokines are elevated in most patients with CSS as well as in animal models of CSS [8, 9]. A constellation of symptoms, signs, and laboratory abnormalities occurs that depends on the severity of the syndrome, the underlying predisposing conditions, and the triggering agent.
细胞因子风暴综合征(CSS)是一种严重的危及生命的病症,其临床特征为全身炎症失控、高血铁蛋白血症、血流动力学不稳定和多器官衰竭(MOF),如果不治疗,可能会导致死亡。CSS 的标志是涉及淋巴细胞和巨噬细胞持续激活和扩增的失控和功能障碍的免疫反应,这些细胞会分泌大量细胞因子,导致细胞因子风暴。CSS 的许多临床特征可以用促炎细胞因子(如干扰素(IFN)-γ、肿瘤坏死因子(TNF)、白细胞介素(IL)-1、IL-6 和 IL-18)的作用来解释[1-7]。大多数 CSS 患者以及 CSS 的动物模型中这些细胞因子的水平均升高[8,9]。一系列的症状、体征和实验室异常发生,其取决于综合征的严重程度、潜在的诱发条件和触发因素。