Marino Achille, Tirelli Francesca, Giani Teresa, Cimaz Rolando
Department of Pediatrics, Desio Hospital, ASST Monza, Desio, MB, Italy.
Biomedical Sciences, University of Florence, Florence, Italy.
J Transl Autoimmun. 2019 Dec 17;3:100031. doi: 10.1016/j.jtauto.2019.100031. eCollection 2020.
Innate immune system represents the ancestral defense against infectious agents preserved along the evolution and species; it is phylogenetically older than the adaptive immune system, which exists only in the vertebrates. Cells with phagocytic activity such as neutrophils, macrophages, and natural killer (NK) cells play a key role in innate immunity. In 1999 Kastner et al. first introduced the term "autoinflammation" describing two diseases characterized by recurrent episodes of systemic inflammation without any identifiable infectious trigger: Familial Mediterranean Fever (FMF) and TNF Receptor Associated Periodic Syndrome (TRAPS). Autoinflammatory diseases (AIDs) are caused by self-directed inflammation due to an alteration of innate immunity leading to systemic inflammatory attacks typically in an on/off mode. In addition to inflammasomopathies, nuclear factor (NF)-κB-mediated disorders (also known as Rhelopathies) and type 1 interferonopathies are subjects of more recent studies. This review aims to provide an overview of the field with the most recent updates (see "Most recent developments in.." paragraphs) and a description of the newly identified AIDs.
先天免疫系统是在进化过程中以及不同物种间保留下来的针对感染因子的原始防御机制；从系统发生学角度来看，它比仅存在于脊椎动物中的适应性免疫系统更为古老。具有吞噬活性的细胞，如中性粒细胞、巨噬细胞和自然杀伤（NK）细胞，在先天免疫中发挥关键作用。1999年，卡斯特纳等人首次引入了“自身炎症”一词，用于描述两种以反复出现全身性炎症发作为特征、且无任何可识别感染诱因的疾病：家族性地中海热（FMF）和肿瘤坏死因子受体相关周期性综合征（TRAPS）。自身炎症性疾病（AIDs）是由于先天免疫改变导致的自我导向性炎症引起的，通常以发作/缓解模式引发全身性炎症攻击。除了炎性小体病，核因子（NF）-κB介导的疾病（也称为Rhelopathies）和1型干扰素病也是最近研究的对象。本综述旨在提供该领域的最新进展概述（见“最新进展”段落）以及对新发现的自身炎症性疾病的描述。
