Autoinflammatory syndromes comprise a spectrum of clinical disorders characterised by recurrent, inflammatory episodes, many of which result from the release of the pro-inflammatory cytokine, interleukin-1β (IL-1β). Inflammation and programmed cell death are tightly linked, and lytic forms of cell death, such as necroptosis and pyroptosis, are considered to be inflammatory due to the release of damage-associated molecular patterns (DAMPs). In contrast, apoptosis is traditionally regarded as immunologically silent. Recent studies, however, have uncovered a high degree of crosstalk between cell death and inflammatory signalling pathways, and effectively consolidated them into one interconnected network that converges on NLRP3 inflammasome-mediated activation of IL-1β. The receptor-interacting protein kinases (RIPK) 1 and 3 are central to this network, as highlighted by the fact that mutations in genes encoding repressors of RIPK1 and/or RIPK3 activity can lead to heightened inflammation, particularly via NLRP3 inflammasome activation. In this review, we give an overview of extrinsic cell death and inflammatory signalling pathways, and then highlight the growing number of autoinflammatory diseases that are associated with aberrant cell death and inflammasome activation.

自身炎症综合征包括一系列以复发性炎症发作为特征的临床疾病，其中许多是由促炎细胞因子白细胞介素 1β (IL-1β) 的释放引起的。炎症和程序性细胞死亡密切相关，细胞死亡的裂解形式，如坏死性凋亡和细胞焦亡，被认为是由于释放损伤相关分子模式 (DAMP) 而引起的炎症。相比之下，细胞凋亡传统上被认为是免疫沉默的。然而，最近的研究发现细胞死亡和炎症信号通路之间存在高度的串扰，并有效地将它们整合到一个相互连接的网络中，该网络汇聚了 NLRP3 炎症小体介导的 IL-1β 激活。通过NLRP3 炎症小体激活。在这篇综述中，我们概述了外源性细胞死亡和炎症信号通路，然后强调了与异常细胞死亡和炎症小体激活相关的越来越多的自身炎症性疾病。