Wekell Per, Berg Stefan, Karlsson Anna, Fasth Anders
Department of Pediatrics, NU-Hospital Group, Uddevalla, Sweden.
Department of Pediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden.
Front Immunol. 2017 Apr 27;8:497. doi: 10.3389/fimmu.2017.00497. eCollection 2017.
Autoinflammatory disease was introduced as a concept in 1999, demarcating an entirely new group of diseases in clinical, immunological, and conceptual terms. During recent years, the preconditions for the definition of autoinflammatory conditions have changed. This includes the recent discovery of a number of monogenic autoinflammatory conditions with complex phenotypes that combine autoinflammation with defects of the adaptive and/or innate immune system, resulting in the occurrence of infection, autoimmunity, and/or uncontrolled hyperinflammation in addition to autoinflammation. Further, there are strong indications that classical IL-1-driven autoinflammatory diseases are associated with activation of adaptive immunity. As suggested by this development, we are of the opinion that an all-encompassing definition of autoinflammatory diseases should regard autoinflammatory conditions and innate dysregulation as inseparable and integral parts of the immune system as a whole. Hence, in this article, we try to advance the conceptual understanding of autoinflammatory disease by, proposing a modification of the definition by Daniel Kastner et al., which allows for a congruent and precise description of conditions that expand the immunological spectrum of autoinflammatory disease.
自身炎症性疾病于1999年作为一个概念被提出，从临床、免疫学和概念层面划分出了全新的一类疾病。近年来，自身炎症性疾病的定义前提发生了变化。这包括最近发现了一些具有复杂表型的单基因自身炎症性疾病，这些疾病将自身炎症与适应性和/或先天性免疫系统缺陷相结合，除自身炎症外，还导致感染、自身免疫和/或不受控制的过度炎症的发生。此外，有强有力的迹象表明，经典的白细胞介素-1驱动的自身炎症性疾病与适应性免疫激活有关。鉴于这一发展趋势，我们认为，对自身炎症性疾病的全面定义应将自身炎症性疾病状态和先天性调节异常视为整个免疫系统不可分割的组成部分。因此，在本文中，我们试图通过对丹尼尔·卡斯特纳等人提出的定义进行修改，来推进对自身炎症性疾病的概念理解，从而能够一致且精确地描述那些扩展了自身炎症性疾病免疫谱的疾病状态。
