National Institute of Geriatrics Rheumatology and Rehabilitation, Warsaw, Poland.
Immunol Lett. 2018 Mar;195:45-54. doi: 10.1016/j.imlet.2017.10.011. Epub 2017 Oct 26.
Systemic Sclerosis (SSc) is an autoimmune disease characterised by vasculopathy, uncontrolled inflammation and enhanced fibrosis which can subsequently lead to the loss of organ function or even premature death. Interferons (IFNs) are pleiotropic cytokines that are critical not only in mounting an effective immune response against viral and bacterial infections but also strongly contribute to the pathogenesis of SSc. Furthermore, elevated levels of IFNs are found in SSc patients and correlate with skin thickness and disease activity suggesting potential role of IFNs as biomarkers. In this review, we summarise existing knowledge regarding all types of IFNs and IFN-inducible genes in the pathogenesis of SSc. We then argue why IFN-blocking strategies are promising therapeutic targets in SSc and other autoimmune diseases.
系统性硬化症(SSc)是一种自身免疫性疾病,其特征为血管病变、失控性炎症和纤维化增强,随后可导致器官功能丧失甚至过早死亡。干扰素(IFNs)是一种多功能细胞因子,不仅在对病毒和细菌感染产生有效免疫反应方面至关重要,而且还强烈促成 SSc 的发病机制。此外,在 SSc 患者中发现 IFN 水平升高,并且与皮肤厚度和疾病活动度相关,提示 IFN 可能作为生物标志物发挥作用。在这篇综述中,我们总结了在 SSc 发病机制中所有类型 IFN 和 IFN 诱导基因的现有知识。然后,我们论证了为什么 IFN 阻断策略是 SSc 和其他自身免疫性疾病有前途的治疗靶点。