目的:甲膜下镜检查(NVC)在区分系统性硬化症(SSc)引起的原发性和继发性雷诺现象方面发挥着公认的作用。然而,NVC与SSc新型严重器官受累/进展的关系从未在多中心进行过评估,跨国研究,这是我们现在第一次表演。
方法:2008年11月至2016年1月,由EUSTAR数据库中的七个三级中心注册的334名SSc患者(265名女性;18名LSSc/203LcSSc/113DcSSc)的随访数据,进行了分析。新的严重器官受累/进展被定义为外周血管系统的新的/进行性受累。肺,心,皮肤,胃肠道,肾脏,肌肉骨骼系统,或死亡,在12或24个月的随访。根据风湿性疾病微循环EULAR研究组的标准化定义,对入选时的NVC图像进行定量和定性评估。单变量和多变量逻辑回归模型(ULR,进行MLR)。
结果:257/334(76.9%)患者出现新的整体严重器官受累/进展。在MLR之后,正常毛细血管密度与较低频率的新型整体严重器官受累/进展(OR=0.77,p<0.001)和新型外周血管受累(OR=0.79,p=0.043)相关;微出血与较低频率的新型肺动脉高压相关(OR=0.47,p=0.029);“严重”(活跃/晚期)NVC模式与新型整体严重器官受累/进展相关(OR=2.14,p=0.0049,
结论:我们的结果表明,NVC可能是SSc的有希望的生物标志物,当然值得进一步调查。尽管有高等教育中心的参与,以标准化的方式跟随他们的病人,我们在检测与罕见严重器官受累/进展的相关性方面的能力不足.
OBJECTIVE: Nailfold videocapillaroscopy (NVC) plays a well-established role in differentiating primary from secondary Raynaud\'s phenomenon due to systemic sclerosis (SSc). However, the association of NVC with novel severe organ involvement/progression in SSc has never been evaluated in a multicentre, multinational study, which we now perform for the first time.
METHODS: Follow-up data from 334 SSc patients (265 women; 18 LSSc/203 LcSSc/113 DcSSc) registered between November 2008 and January 2016 by seven tertiary centres in the
EUSTAR-database, were analysed. Novel severe organ involvement/progression was defined as new/progressive involvement of the peripheral vasculature, lungs, heart, skin, gastrointestinal tract, kidneys, musculoskeletal system, or death, at 12- or 24-month follow-up. NVC images at enrolment were quantitatively and qualitatively evaluated according to the standardised definitions of the EULAR Study Group on Microcirculation in Rheumatic Diseases. Uni- and multivariable logistic regression modelling (ULR, MLR) was performed.
RESULTS: 257/334 (76.9%) patients developed novel overall severe organ involvement/progression. Following MLR, normal capillary density was associated with less frequent novel overall severe organ involvement/progression (OR = 0.77, p < 0.001) and novel peripheral vascular involvement (OR = 0.79, p = 0.043); microhaemorrhages were associated with less novel pulmonary hypertension (OR = 0.47, p = 0.029); and a \"severe\" (active/late) NVC pattern was associated with novel overall severe organ involvement/progression (OR = 2.14, p = 0.002) and skin progression (OR = 1.70, p = 0.049).
CONCLUSIONS: Our results suggest that NVC may be a promising biomarker in SSc, certainly warranting further investigation. Despite the participation of tertiary centres, which follow their patients in a standardised way, we were underpowered to detect associations with infrequent severe organ involvement/progression.