Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
Department of Internal Medicine, Ghent University, Ghent, Belgium.
Rheumatology (Oxford). 2023 Apr 3;62(4):1605-1615. doi: 10.1093/rheumatology/keac487.
To standardly assess and describe nailfold videocapillaroscopy (NVC) assessment in children and adolescents with juvenile rheumatic and musculoskeletal diseases (jRMD) vs healthy controls (HCs).
In consecutive jRMD children and matched HCs from 13 centres worldwide, 16 NVC images per patient were acquired locally and read centrally per international consensus standard evaluation of the EULAR Study Group on Microcirculation in Rheumatic Diseases. A total of 95 patients with JIA, 22 with JDM, 20 with childhood-onset SLE (cSLE), 13 with juvenile SSc (jSSc), 21 with localized scleroderma (lSc), 18 with MCTD and 20 with primary RP (PRP) were included. NVC differences between juvenile subgroups and HCs were calculated through multivariable regression analysis.
A total of 6474 images were assessed from 413 subjects (mean age 12.1 years, 70.9% female). The quantitative NVC characteristics were significantly lower or higher in the following subgroups compared with HCs: for density: lower in jSSc, JDM, MCTD, cSLE and lSc; for dilations: higher in jSSc, MCTD and JDM; for abnormal shapes: higher in JDM and MCTD; for haemorrhages: higher in jSSc, MCTD, JDM and cSLE. The qualitative NVC assessment of JIA, lSc and PRP did not differ from HCs, whereas the cSLE and jSSc, MCTD, JDM and cSLE subgroups showed more non-specific and scleroderma patterns, respectively.
This analysis resulted from a pioneering registry of NVC in jRMD. The NVC assessment in jRMD differed significantly from HCs. Future prospective follow-up will further elucidate the role of NVC in jRMD.
标准化评估和描述儿童和青少年幼年特发性关节炎和肌肉骨骼疾病（jRMD）与健康对照（HCs）的甲褶毛细血管镜（NVC）评估。
在来自全球 13 个中心的连续 jRMD 儿童和匹配的 HCs 中，根据国际 EULAR 微血管研究组的共识标准，对每位患者进行了 16 次 NVC 图像采集和局部读取。共纳入 95 例 JIA、22 例 JDM、20 例儿童发病的系统性红斑狼疮（cSLE）、13 例幼年硬皮病（jSSc）、21 例局限性硬皮病（lSc）、18 例混合性结缔组织病（MCTD）和 20 例原发性重叠综合征（PRP）患者。通过多变量回归分析计算幼年亚组与 HCs 之间的 NVC 差异。
共评估了 413 名受试者的 6474 张图像（平均年龄 12.1 岁，70.9%为女性）。与 HCs 相比，以下亚组的定量 NVC 特征明显降低或升高：密度：jSSc、JDM、MCTD、cSLE 和 lSc 较低；扩张：jSSc、MCTD 和 JDM 较高；异常形状：JDM 和 MCTD 较高；出血：jSSc、MCTD、JDM 和 cSLE 较高。JIA、lSc 和 PRP 的定性 NVC 评估与 HCs 无差异，而 cSLE 和 jSSc、MCTD、JDM 和 cSLE 亚组分别显示出更多的非特异性和硬皮病模式。
这是一项对 jRMD 中 NVC 的开创性研究。jRMD 的 NVC 评估与 HCs 有显著差异。未来的前瞻性随访将进一步阐明 NVC 在 jRMD 中的作用。
