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.

中文翻译：

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抗黑色素瘤分化相关基因 5 抗体阳性皮肌炎中的甲襞毛细血管和肌炎特异性抗体

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，并评估与包括血清生物标志物在内的临床参数的关联。此外，我们旨在明确 DM 患者在免疫抑制治疗期间导致 NFC 异常缓解的时期。方法 进行了一项前瞻性观察研究，包括首次访问广岛大学医院并被诊断为 DM 或临床无肌病 DM 并使用抗 MDA5 抗体的患者（n = 10）。我们比较了使用 LEGENDplexTM 多分析物流动检测试剂盒测量的甲皱视频毛细血管镜 (NVC) 检测到的 NFC 异常、物理检查结果、血液检测、呼吸功能检测和血管相关生长因子。结果 在开始免疫抑制治疗后 2 至 17 周，所有患者的 NFC 异常均有改善。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。M-CSF 和干细胞因子的基线值与抗 MDA-5 滴度相关。结论 我们的研究表明，治疗前 NVC 评分和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。NVC评分与用力肺活量呈正相关。M-CSF 和干细胞因子的基线值与抗 MDA-5 滴度相关。结论 我们的研究表明，治疗前 NVC 评分和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。NVC评分与用力肺活量呈正相关。M-CSF 和干细胞因子的基线值与抗 MDA-5 滴度相关。结论 我们的研究表明，治疗前 NVC 评分和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。