幼年性硬皮病是一组与硬化性皮肤病变相关的异质性疾病,分为青少年系统性硬化症系统性硬化症)和青少年局限性硬皮病。本研究旨在测量幼年硬皮病患者参与干扰素信号传导的细胞因子和趋化因子水平,并确定其与疾病严重程度的相关性。
方法:29例少年局限性硬皮病5例少年系统性硬化症,9名健康对照者被纳入研究.根据LoSAI(LoSCAT活动指数)对青少年局限性硬皮病患者进行评分,LoSDI(LoSCAT损伤指数),和PGA-A(医师全球评估活动)指数。参与干扰素基因信号传导的细胞因子和趋化因子(IL-1,IL-6,IL-8,IP-10,MCP1,TNF-α,CXCL-11,IFN-α,IFN-β,通过ELISA和RT-PCR方法分别检测IFN-γ)和干扰素刺激基因(ISG),包括IFI27,IFI44,ISIG15,IFIT1,OAS1,RSAD2。
结果:IFN-α的显着增加,IFN-β,IFN-γ,TNF-α,与健康对照组比较,观察幼年系统性硬化症患者的IL-1,IL-6,IL-8,IP-10和MCP1水平。此外,与健康对照组相比,青少年局部硬皮病和青少年系统性硬化症的IFN-α和IP-10升高。IFN-γ和IFN-α与LoSAI和LoSDI水平呈正相关,分别。根据PGA-A分析,IFN-β,IFN-γ,TNF-α,IL-8、IP10、MCP1和CXCL11在活动性疾病中显著高于非活动性疾病组。
结论:结果表明,幼年硬皮病患者的干扰素信号可能受损。在细胞因子和与IFN信号相关的基因中观察到显着变化,这可能在监测疾病活动中起着至关重要的作用。此外,我们已经获得了使用IFN-α和IFN-γ作为监测青少年硬皮病活动和损害的生物标志物的可能性的重要见解。
Juvenile scleroderma is a heterogeneous group of diseases associated with sclerotic skin lesions, grouped as juvenile systemic sclerosis systemic sclerosis) and juvenile localized scleroderma. This study aims to measure the cytokine and chemokine levels involved in interferon signaling in patients with juvenile scleroderma and determine their correlation with disease severity.
METHODS: Twenty-nine juvenile localized scleroderma five juvenile systemic sclerosis, and nine healthy controls were included in the study. Patients with juvenile localized scleroderma were scored according to the LoSAI (LoSCAT activity index), LoSDI (LoSCAT damage index), and PGA-A (physician global assessment-activity) indices. Cytokines and chemokines involved in interferon gene signaling (IL-1, IL-6, IL-8, IP-10, MCP1, TNF-α, CXCL-11, IFN-α, IFN-β, IFN-γ) and interferon-stimulated genes (ISGs) including IFI27, IFI44, ISIG15, IFIT1, OAS1, RSAD2 were measured by ELISA and RT-PCR method respectively.
RESULTS: A significant increase in IFN-α, IFN-β, IFN-γ, TNF-α, IL -1, IL -6 IL -8, IP-10, and MCP1 levels was observed in patients with juvenile systemic sclerosis compared with the healthy control group. Furthermore, IFN- α and IP-10 were elevated in both juvenile localized scleroderma and juvenile systemic sclerosis compared to the healthy control group. IFN-γ and IFN-α positively correlated with LoSAI and LoSDI levels, respectively. According to PGA-A analysis, IFN-β, IFN-γ, TNF-α, IL -8, IP10, MCP1, and CXCL11 were significantly higher in active disease than in the inactive state in both groups.
CONCLUSIONS: The results suggest that interferon signaling may be impaired in patients with juvenile scleroderma. Significant changes were observed in cytokines and genes related to IFN signaling, which may have a crucial role in monitoring disease activity. In addition, we have gained important insights into the possibility of using IFN-α and IFN-γ as biomarkers for monitoring juvenile scleroderma activity and damage.