Innate immunity in Sjögren's syndrome.

Innate immunity in Sjögren's syndrome.
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DOI:
10.1016/j.clim.2017.04.003
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发表时间:
2017-09
期刊:
Clinical immunology (Orlando, Fla.)
影响因子:
--
通讯作者:
Kramer JM
Kramer JM
中科院分区:
其他
文献类型:
--
作者:
Kiripolsky J;McCabe LG;Kramer JM

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干燥综合征(SS)是一种主要影响女性的外分泌组织自身免疫性疾病。虽然患者通常会出现口干和干眼症,但也会出现许多全身性疾病的表现。包括SS在内的许多自身免疫性疾病都与先天免疫功能亢进有关。SS小鼠模型的结果表明,先天性免疫失调导致疾病,这是SS发病机制中的一个重要事件。在SS患者中的发现证实了在小鼠模型中的结果,因为先天性免疫细胞和途径在外分泌组织和外周血液中都是失调的。我们将就先天性免疫系统在SS发病机制中的作用作一综述。我们将讨论SS的病因学,重点是先天性免疫功能障碍。此外,我们将回顾在SS中介导炎症的固有细胞,与疾病有关的途径,以及控制其失调的潜在机制。最后,我们将讨论针对SS调节失调的先天免疫信号的新的治疗方法。
Sjögren’s syndrome (SS) is an autoimmune disease of exocrine tissue that primarily affects women. Although patients typically experience xerostomia and xerophthalmia, numerous systemic disease manifestations are seen. Innate immune hyperactivity is integral to many autoimmune diseases, including SS. Results from SS mouse models suggest that innate immune dysregulation drives disease and this is a seminal event in SS pathogenesis. Findings in SS patients corroborate those in mouse models, as innate immune cells and pathways are dysregulated both in exocrine tissue and in peripheral blood. We will review the role of the innate immune system in SS pathogenesis. We will discuss the etiology of SS with an emphasis on innate immune dysfunction. Moreover, we will review the innate cells that mediate inflammation in SS, the pathways implicated in disease, and the potential mechanisms governing their dysregulation. Finally, we will discuss emerging therapeutic approaches to target dysregulated innate immune signaling in SS.
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