目的:标准评估和描述儿童和青少年风湿性和肌肉骨骼疾病(jRMD)与健康对照(HC)的甲叠视频眼镜检查(NVC)评估。
方法:在来自全球13个中心的连续jRMD儿童和匹配的HC中,根据EULAR风湿性疾病微循环研究组的国际共识标准评估,每位患者在本地获取16张NVC图像,并集中阅读。95例幼年特发性关节炎(JIA),22患有皮肌炎(JDM),20与系统性红斑狼疮(cSLE),13患有系统性硬化症(jSSc),21患有局限性硬皮病(lSc),包括18例混合性结缔组织病(MCTD)和20例原发性雷诺现象(PRP)。通过多元回归分析计算青少年亚组和HC之间的NVC差异。
结果:从413名受试者(平均年龄12.1岁,70.9%女性)。与HC相比,以下亚组的定量NVC特征显着降低()或更高():对于密度:jSSc中的,JDM,MCTD,cSLE和lSc;对于扩张:^在jSSc中,MCTDandJDM;Fornormalnallyshape:^JDMandMCTD;Forborhymages:^injSSc,MCTD,JDM和cSLE。JIA的定性NVC评估,lSc和PRP与HC没有区别,而cSLE和jSSc,MCTD,JDM,cSLE亚组分别显示更多的非特异性和硬皮病模式。
结论:此分析来自jRMD中NVC的开创性注册。jRMD中的NVC评估与HC显着不同。未来的前瞻性随访将进一步阐明NVC在jRMD中的作用。
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.