Objectives Idiopathic inflammatory myopathies (IIM) are a diverse group of muscle diseases often complicated by interstitial lung disease (ILD), which significantly impacts morbidity and mortality. Krebs von den Lungen-6 (sKL-6) has been proposed as a biomarker for ILD severity. Nailfold videocapillaroscopy (NVC) detects microvascular changes, but its diagnostic and prognostic value in IIM remains unclear. This study aimed to assess the relationship between NVC abnormalities, sKL-6 levels, and pulmonary outcomes in IIM patients. Methods A retrospective analysis was conducted in IIM patients from a reference center, comparing those with and without ILD. Data included epidemiological, clinical, and immunological features, pulmonary function tests, sKL-6 levels, and NVC findings. Statistical analyses included Spearman’s rank correlation coefficient to assess the relationships between sKL-6 levels, pulmonary function tests, and NVC parameters. Multiple logistic regression modelling to identify to identify predictors of IIM-ILD. Results Among 95 patients (34% male, median age 55.3 ± 24 years, disease duration 6.8 ± 7 years), ILD was associated with avascular zones (p= 0.004), capillary loss (p= 0.04), and microhemorrhages (p= 0.04). Negative correlations were observed between capillary loss and enlarged capillaries with %FVC (rs=-0.46, p= 0.001; rs=-0.57, p< 0.0001) and %DLCO (rs=-0.32, p= 0.04; rs=-0.23, p= 0.03). sKL-6 levels correlated positively with ILD (rs = 0.77, p= 0.0004), microhemorrhages (rs = 0.21, p= 0.04), and avascular areas (rs = 0.64, p= 0.03) and negatively with %FVC (rs=-0.47, p= 0.001) and %DLCO (rs=-0.59, p= 0.005). Predictors of ILD included male sex, respiratory symptoms, %FVC, %DLCO, sKL-6, anti-Jo1 positivity, and NVC abnormalities. Conclusions NVC findings, sKL-6 levels, and autoantibodies are valuable in identifying and monitoring ILD in IIM, highlighting their role in early diagnosis and management.

中文翻译：

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甲襞视频毛细血管镜检查结果与特发性炎症性肌病相关间质性肺病的肺部结局相关


目的特发性炎症性肌病 （IIM） 是一组多样化的肌肉疾病，经常并发间质性肺病 （ILD），显著影响发病率和死亡率。Krebs von den Lungen-6 （sKL-6） 已被提议作为 ILD 严重程度的生物标志物。甲襞视频毛细血管镜检查 （NVC） 可检测微血管变化，但其在 IIM 中的诊断和预后价值仍不清楚。本研究旨在评估 IIM 患者 NVC 异常、sKL-6 水平和肺部结局之间的关系。方法 对来自参考中心的 IIM 患者进行回顾性分析，比较有和没有 ILD 的患者。数据包括流行病学、临床和免疫学特征、肺功能测试、sKL-6 水平和 NVC 结果。统计分析包括 Spearman 秩相关系数，以评估 sKL-6 水平、肺功能测试和 NVC 参数之间的关系。多元 logistic 回归模型来识别 IIM-ILD 的预测因子。结果 在 95 例患者 （34% 为男性，中位年龄 55.3 ± 24 岁，病程 6.8 ± 7 年）中，ILD 与无血管区 （p= 0.004） 、毛细血管丢失 （p= 0.04） 和微出血 （p= 0.04） 相关。观察到毛细血管丢失和毛细血管增大与 %FVC （rs=-0.46，p= 0.001;rs=-0.57，p< 0.0001） 和 %DLCO （rs=-0.32，p= 0.04;rs=-0.23，p= 0.03）呈负相关。sKL-6 水平与 ILD （rs = 0.77，p = 0.0004）、微出血 （rs = 0.21，p = 0.04） 和无血管区域 （rs = 0.64，p = 0.03） 呈正相关，与 %FVC （rs=-0.47，p= 0.001） 和 %DLCO （rs=-0.59，p= 0.005）呈负相关。ILD 的预测因素包括男性、呼吸道症状、%FVC、%DLCO、sKL-6、抗 Jo1 阳性和 NVC 异常。 结论 NVC 检查结果、 sKL-6 水平和自身抗体对识别和监测 IIM 中的 ILD 很有价值，突出了它们在早期诊断和管理中的作用。