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首页 > 医学词汇大全 > systemic sclerosis
systemic sclerosis

免疫

关键词免疫 疾病 系统性硬化症

词汇介绍

拓展阅读

解析

systemic 英 [sɪˈstiːmɪk; sɪˈstemɪk] 美 [sɪˈstemɪk]

释    义    adj. 系统的;全身的;体系的

例    句    Without a resolution of this global debt burden, systemic risk will fester and grow.没有这个全球债务负担的解决,系统性风险将恶化和生长。

 

Sclerosis 英 [skləˈrəʊsɪs] 美 [skləˈroʊsɪs]

释    义    n. [病理] 硬化,[医] 硬化症;细胞壁硬化

例    句    Labs are using such cells to study amyotrophic lateral sclerosis, Parkinson's disease, Huntington's disease, and even autism.实验室通过这些细胞来研究肌萎缩性脊髓侧索硬化、帕金森氏症、亨廷顿氏病,甚至自闭症。

概述

系统性硬化症是一类病因不明的自身免疫性疾病,以广泛纤维化、血管病变以及针对多种细胞抗原的自身抗体为其主要特点。除常见的皮肤肿胀,继以变厚、变硬,最终萎缩外,还累及血管、肺、消化道、肾脏、心脏等多种内脏器官造成内脏受损的表现,严重影响预后。但其患病率偏低。   发病机制 1.遗传因素:有研究表明,系统性硬化症具有遗传性。家族中有系统性硬化症或其他自身免疫性疾病的患者,其亲属患系统性硬化症的风险增加,若为一级亲属,则风险更高

Plasmacytoid dendritic cells promote systemic sclerosis with a key role for TLR8复制标题

浆细胞样树突状细胞促进系统性硬化症与TLR8的关键作用

发表时间:2018-01-10

影响因子:17.2

作者: Ah Kioon MD

期刊:Sci Transl Med

Systemic sclerosis (SSc) is a multisystem life-threatening fibrosing disorder that lacks effective treatment. The link between the inflammation observed in organs such as the skin and profibrotic mechanisms is not well understood. The plasmacytoid dendritic cell (pDC) is a key cell type mediating Toll-like receptor (TLR)–induced inflammation in autoimmune disease patients, including lupus and skin diseases with interface dermatitis. However, the role of pDCs in fibrosis is less clear. We show that pDCs infiltrate the skin of SSc patients and are chronically activated, leading to secretion of interferon-α (IFN-α) and CXCL4, which are both hallmarks of the disease. We demonstrate that the secretion of CXCL4 is under the control of phosphatidylinositol 3-kinase δ and is due to the aberrant presence of TLR8 on pDCs of SSc patients, which is not seen in healthy donors or in lupus pDCs, and that CXCL4 primarily acts by potentiating TLR8- but also TLR9-induced IFN production by pDCs.

译文

系统性硬化(SSc)是一种多系统威胁生命的纤维化疾病,缺乏有效的治疗。在皮肤等器官中观察到的炎症与促纤维化机制之间的联系尚不清楚。浆细胞样树突状细胞(pDC)是介导Toll样受体(TLR)诱导的自身免疫性疾病(包括狼疮和伴有界面皮炎的皮肤病)炎症的关键细胞类型。然而,pDCs在纤维化中的作用尚不清楚。我们发现pDCs浸润SSc患者的皮肤,并被慢性激活,导致分泌干扰素-α(IFN-α)和CXCL4,这两种都是该病的特征。我们证明CXCL4的分泌受磷脂酰肌醇3-激酶δ的控制,这是由于在SSc患者的pdc上TLR8的异常存在所致,而在健康供体和狼疮pdc中均未见TLR8的异常存在,CXCL4主要通过增强TLR8-和TLR9诱导的pdc产生IFN而起作用。