甲襞视频毛细血管显微镜检查结果与特发性炎性肌病相关间质性肺疾病的肺部预后相关。

Nailfold videocapillaroscopy findings correlate with lung outcomes in idiopathic inflammatory myopathies-related interstitial lung disease.

作者信息

Sieiro Santos Cristiana, Tandaipan Jose Luis, Castillo Diego, Codes Helena, Martínez-Martínez Laura, Magallares Berta, Moya Patricia, Mariscal Anais, Park Hye Sang, Díaz-Torné Cesar, Fernandez-Sanchez Susana P, Bernardez Julia, Corominas Hector, Diez Alvarez Elvira, Castellví Ivan

机构信息

Rheumatology, Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain.

Rheumatology, Complejo Asistencial Universitario de León, León, Spain.

出版信息

Rheumatology (Oxford). 2024 Dec 10. doi: 10.1093/rheumatology/keae669.

DOI:10.1093/rheumatology/keae669

PMID:39658251

Abstract

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.

摘要

目的

特发性炎性肌病（IIM）是一组多样的肌肉疾病，常并发间质性肺疾病（ILD），这对发病率和死亡率有显著影响。克雷伯氏肺炎-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明确。本研究旨在评估IIM患者中NVC异常、sKL-6水平与肺部结局之间的关系。

方法

对来自一个参考中心的IIM患者进行回顾性分析，比较有ILD和无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检查结果。统计分析包括Spearman等级相关系数，以评估sKL-6水平、肺功能测试和NVC参数之间的关系。采用多元逻辑回归模型来确定IIM-ILD的预测因素。

结果

在95例患者中（男性占34%，中位年龄55.3±24岁，病程6.8±7年），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扩张的毛细血管与%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（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。