Respiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese , Siena , Italy.
Department of Medical and Surgical Sciences & Neurosciences, University of Siena , Siena , Italy.
Expert Rev Respir Med. 2019 Oct;13(10):981-991. doi: 10.1080/17476348.2019.1655401. Epub 2019 Aug 23.
: The pathogenesis of sarcoidosis is not yet completely understood, although in recent years our knowledge has made considerable progress. : This review aims to highlight the latest findings, identified from PubMed, EMBASE, and Web of Science, on the pathogenetic mechanisms of sarcoidosis, considering the studies on potential environmental antigens, genetic background and host immune responses. Particular emphasis has been on recent studies on antigens, as it now seems clear that it is not a single, but various antigens of microbial and non-microbial origin that share the ability to induce the series of immune-inflammatory events that lead to granuloma formation, activating host genetically influenced immune responses that involve innate and even more adaptive immunity. The dysregulation of Th17, Th17.1 cells and Tregs, and their role in the resolution and maintenance of granulomatous inflammation has been reported. : The considerable amount of data that has been accumulated on sarcoidosis pathogenesis will have to be carefully interpreted, particularly to discover which pathways lead to severe forms with organ damage. There is an urgent need for a panel of biomarkers indicating the involvement of the various pathways, to be used for better characterizing patient phenotypes and developing targeted therapies.
: 尽管近年来我们对结节病的发病机制有了相当大的了解，但仍不完全清楚。本综述旨在强调从PubMed、EMBASE 和 Web of Science 中确定的关于结节病发病机制的最新发现，同时考虑到对潜在环境抗原、遗传背景和宿主免疫反应的研究。特别强调了最近对抗原的研究，因为现在似乎很清楚，导致肉芽肿形成的一系列免疫炎症事件的诱导能力并非来自单一抗原，而是来自微生物和非微生物来源的各种抗原，从而激活了宿主具有遗传影响的免疫反应，涉及先天甚至更适应性免疫。已经报道了 Th17、Th17.1 细胞和 Tregs 的失调及其在肉芽肿性炎症的消退和维持中的作用。已经积累了大量关于结节病发病机制的资料，必须仔细加以解释，特别是要发现哪些途径会导致有器官损伤的严重形式。迫切需要一组生物标志物来指示各种途径的参与，以便更好地对患者表型进行特征描述并开发靶向治疗方法。
