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甲襞视频毛细血管显微镜检查结果与IIM亚型及IFN激活相关。

Nailfold videocapillaroscopy findings are associated with IIM subtypes and IFN activation.

作者信息

Tang Mingwei, Shi Jia, Pang Yiyun, Zhou Shuang, Liu Jinjing, Wu Chanyuan, Li Mengtao, Zeng Xiaofeng, Wang Qian

机构信息

Department of Rheumatology and Clinical Immunology, Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases Key Laboratory of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences, Ministry of Education, Peking Union Medical College Hospital, Peking Union Medical College Hospital, Beijing, 100730, China.

Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, 100730, China.

出版信息

Arthritis Res Ther. 2025 Mar 20;27(1):62. doi: 10.1186/s13075-025-03532-9.

DOI:10.1186/s13075-025-03532-9
PMID:40114239
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11924856/
Abstract

OBJECTIVE

This study aimed to characterize nailfold videocapillaroscopy (NVC) features in patients with different subtypes of Idiopathic inflammatory myopathy (IIM) and to investigate the correlations between NVC findings, myositis-specific antibody (MSA) subtypes, disease activity, cytokine profiles, and interferon-stimulated gene (ISG) expression levels.

METHODS

This cross-sectional observational single-center study included 55 IIM patients, categorized into MDA5 (+), anti-aminoacyl-tRNA-synthetase antibodies (ARS) (+), and MSA(-) groups based on their MSA profiles. Demographic data, laboratory tests, and NVC assessments were systematically collected and analyzed. The relative expression of type I ISGs in whole blood, as well as serum cytokine and chemokine profiles, were measured. Statistical analyses were performed to explore correlations between NVC scores and clinical parameters, including serum biomarkers.

RESULTS

NVC abnormalities were observed in most IIM patients, with significant differences in NVC features among the MSA subgroups. The MDA5(+) group exhibited significantly higher scores for capillary dilation (P < 0.01), giant capillaries (P < 0.05), microhemorrhages (P < 0.01), and abnormal capillary morphology (P < 0.05) compared to the ARS (+) group. ISG expression and cytokine levels were upregulated in IIM patients, with active disease patients showing significantly higher levels of certain ISGs and cytokines compared to clinically stable patients. Notably, specific NVC score dimensions were positively correlated with the levels of certain ISGs and cytokines. For example, microhemorrhage, capillary dilation, and capillary density all had significantly positive correlations with MX1, IFI27, IP-10, RANTES, and GROα (P < 0.05). And giant capillary is also related to levels of IFI27, SDF-1α, IP-10, RANTES, and GROα (P < 0.05).

CONCLUSION

IIM patients exhibit distinct NVC abnormalities, which vary across different MSA subtypes. NVC findings have potential clinical value in screening disease activity and interferon pathway activation in IIM patients.

摘要

目的

本研究旨在描述特发性炎性肌病(IIM)不同亚型患者的甲襞视频毛细血管镜检查(NVC)特征,并探讨NVC检查结果、肌炎特异性抗体(MSA)亚型、疾病活动度、细胞因子谱和干扰素刺激基因(ISG)表达水平之间的相关性。

方法

这项横断面观察性单中心研究纳入了55例IIM患者,根据其MSA谱分为MDA5(+)、抗氨酰tRNA合成酶抗体(ARS)(+)和MSA(-)组。系统收集并分析人口统计学数据、实验室检查和NVC评估结果。检测全血中I型ISG的相对表达以及血清细胞因子和趋化因子谱。进行统计分析以探讨NVC评分与临床参数(包括血清生物标志物)之间的相关性。

结果

大多数IIM患者观察到NVC异常,MSA亚组之间的NVC特征存在显著差异。与ARS(+)组相比,MDA5(+)组在毛细血管扩张(P<0.01)、巨大毛细血管(P<0.05)、微出血(P<0.01)和异常毛细血管形态(P<0.05)方面的评分显著更高。IIM患者的ISG表达和细胞因子水平上调,与临床稳定患者相比,活动期疾病患者的某些ISG和细胞因子水平显著更高。值得注意的是,特定的NVC评分维度与某些ISG和细胞因子水平呈正相关。例如,微出血、毛细血管扩张和毛细血管密度均与MX1、IFI27、IP-10、RANTES和GROα显著正相关(P<0.05)。并且巨大毛细血管也与IFI27、SDF-1α、IP-10、RANTES和GROα水平相关(P<0.05)。

结论

IIM患者表现出明显的NVC异常,不同MSA亚型之间存在差异。NVC检查结果在筛查IIM患者的疾病活动度和干扰素通路激活方面具有潜在的临床价值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3374/11924856/22af234ed1d3/13075_2025_3532_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3374/11924856/3add62aa064b/13075_2025_3532_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3374/11924856/22af234ed1d3/13075_2025_3532_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3374/11924856/3add62aa064b/13075_2025_3532_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3374/11924856/22af234ed1d3/13075_2025_3532_Fig2_HTML.jpg

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本文引用的文献

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Nat Rev Rheumatol. 2023 Nov;19(11):695-712. doi: 10.1038/s41584-023-01033-0. Epub 2023 Oct 6.
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Nailfold capillaroscopy changes with disease activity in patients with inflammatory myositis including overlap myositis, pure dermatomyositis, and pure polymyositis.
在包括重叠性肌炎、单纯性皮肌炎和单纯性多发性肌炎在内的炎性肌病患者中,甲襞毛细血管镜检查结果随疾病活动度而变化。
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Standardised interpretation of capillaroscopy in autoimmune idiopathic inflammatory myopathies: A structured review on behalf of the EULAR study group on microcirculation in Rheumatic Diseases.自身免疫性特发性炎性肌病毛细血管镜检查的标准化解读:代表 EULAR 风湿病微循环研究小组的结构化综述。
Autoimmun Rev. 2022 Jun;21(6):103087. doi: 10.1016/j.autrev.2022.103087. Epub 2022 Apr 12.
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Detection of microvascular changes in systemic sclerosis and other rheumatic diseases.系统性硬化症和其他风湿性疾病的微血管变化检测。
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6
The comparison of nailfold videocapillaroscopy findings between anti-melanoma differentiation-associated gene 5 antibody and anti-aminoacyl tRNA synthetase antibody in patients with dermatomyositis complicated by interstitial lung disease.抗黑色素瘤分化相关基因 5 抗体与抗氨酰基 tRNA 合成酶抗体阳性的皮肌炎合并间质性肺病患者甲襞微循环的比较。
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