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甲襞毛细血管和抗黑色素瘤分化相关基因 5 抗体阳性皮肌炎中的肌炎特异性抗体。

Nailfold capillaries and myositis-specific antibodies in anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis.

机构信息

Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Kasumi, Hiroshima, Japan.

出版信息

Rheumatology (Oxford). 2022 May 5;61(5):2006-2015. doi: 10.1093/rheumatology/keab681.

Abstract

OBJECTIVES

This study aimed to quantify nailfold capillary (NFC) abnormalities in anti-melanoma differentiation-associated gene 5 (MDA5) -positive DM patients and to evaluate the association with clinical parameters, including serum biomarkers. In addition, we aimed to clarify the period leading to remission of NFC abnormalities during immunosuppressive treatment in patients with DM.

METHODS

A prospective observational study was conducted including patients (n = 10) who first visited Hiroshima University Hospital and were diagnosed with DM or clinically amyopathic DM with anti-MDA5 antibodies. We compared the NFC abnormalities detected by nailfold-video capillaroscopy (NVC), physical findings, blood tests, respiratory function tests, and vascular-related growth factors measured using a LEGENDplexTM Multi-Analyte Flow Assay Kit.

RESULTS

NFC abnormalities improved in all patients from 2 to 17 weeks after the initiation of immunosuppressive treatment. The NVC scores were inversely correlated with anti-MDA5 antibody titres at baseline. NVC scores and forced vital capacity were positively correlated. Baseline values of M-CSF and stem cell factor were correlated with anti-MDA-5 titres.

CONCLUSION

Our study suggested that NVC scores and disease activity were inversely correlated before treatment. Vascular-related growth factors, such as M-CSF and stem cell factor, may be associated with the disease mechanism in patients with anti-MDA5 antibody-positive DM.

摘要

目的

本研究旨在量化抗黑色素瘤分化相关基因 5(MDA5)阳性 DM 患者的甲襞毛细血管(NFC)异常,并评估其与临床参数的相关性,包括血清生物标志物。此外,我们旨在阐明 DM 患者在免疫抑制治疗期间 NFC 异常缓解的时间。

方法

进行了一项前瞻性观察性研究,纳入了首次就诊于广岛大学医院并被诊断为 DM 或伴抗 MDA5 抗体的临床无肌病型 DM 的患者(n=10)。我们比较了通过甲襞视频毛细血管镜(NVC)检测到的 NFC 异常、体格检查、血液检查、呼吸功能检查以及使用 LEGENDplexTM 多分析物流式分析试剂盒测量的血管相关生长因子。

结果

所有患者在免疫抑制治疗开始后 2 至 17 周内 NFC 异常均得到改善。NVC 评分与基线时的抗 MDA5 抗体滴度呈负相关。NVC 评分与用力肺活量呈正相关。基线时的 M-CSF 和干细胞因子与抗 MDA-5 滴度相关。

结论

我们的研究表明,治疗前 NVC 评分与疾病活动度呈负相关。血管相关生长因子,如 M-CSF 和干细胞因子,可能与抗 MDA5 抗体阳性 DM 患者的疾病机制有关。

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