Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, and Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulations, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulations, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, and Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Clin Exp Rheumatol. 2024 Feb;42(2):367-376. doi: 10.55563/clinexprheumatol/l9gudh. Epub 2024 Mar 14.
To assess nailfold video capillaroscopic (NVC) abnormalities and their association with clinical features, myositis-specific autoantibodies (MSA), and myositis-associated antibodies (MAA) in a large multi-ethnic cohort of patients with idiopathic inflammatory myopathies (IIM).
We recruited 155 IIM patients from three centres in Mexico, Spain, and the USA. We evaluated the clinical and laboratory features of the patients and performed semiquantitative and quantitative analyses of the NVC. Each NVC study was defined as having a normal, non-specific, early systemic sclerosis (SSc), active SSc, or late SSc pattern. Twenty-three patients had at least one follow-up NVC when disease control was achieved. Quantitative variables were expressed as medians and interquartile range (IQR) and were compared with the Kruskal-Wallis, the Mann-Whitney U-test, and the Wilcoxon test for paired medians. Associations between qualitative variables were assessed with the χ2 test.
Most patients were women (68.3%), Hispanic (73.5%), and had dermatomyositis (DM) (61.2%). Fourteen patients (9%) had a normal NVC. A non-specific abnormality pattern was the most frequent (53.9%), and was associated with joint involvement, interstitial lung disease, Jo1 autoantibodies, anti-synthetase syndrome, and immune-mediated necrotising myopathy. The SSc pattern was observed mostly in DM and overlap myositis and was associated with cutaneous features and anti-TIF-1g autoantibodies. After treatment, there was a decrease in the capillaroscopic score, the capillary diameter, and the number of avascular areas, and an increase in capillary density and bushy capillary number.
NVC abnormalities are related to the diagnosis, clinical features, disease activity, and autoantibodies of patients with IIM.
评估甲襞视频毛细血管镜（NVC）异常及其与临床特征、肌炎特异性自身抗体（MSA）和肌炎相关自身抗体（MAA）的关系，该研究纳入了来自墨西哥、西班牙和美国的三个中心的 155 名特发性炎症性肌病（IIM）患者。
我们招募了来自墨西哥、西班牙和美国的三个中心的 155 名特发性炎症性肌病患者。我们评估了患者的临床和实验室特征，并对 NVC 进行了半定量和定量分析。每个 NVC 研究均定义为正常、非特异性、早期系统性硬化症（SSc）、活动期 SSc 或晚期 SSc 模式。23 名患者在疾病控制后至少进行了一次随访 NVC。定量变量用中位数和四分位距（IQR）表示，采用 Kruskal-Wallis 检验、Mann-Whitney U 检验和配对中位数 Wilcoxon 检验进行比较。采用 χ2 检验评估定性变量之间的关系。
大多数患者为女性（68.3%）、西班牙裔（73.5%）和皮肌炎（DM）（61.2%）。14 名患者（9%）的 NVC 正常。最常见的是无特异性异常模式（53.9%），与关节受累、间质性肺病、Jo1 自身抗体、抗合成酶综合征和免疫介导的坏死性肌病有关。SSc 模式主要见于 DM 和重叠性肌炎，与皮肤特征和抗 TIF-1g 自身抗体有关。治疗后，毛细血管镜评分、毛细血管直径和无血管区数量减少，毛细血管密度和丛状毛细血管数量增加。
NVC 异常与 IIM 患者的诊断、临床特征、疾病活动度和自身抗体有关。
