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序贯甲襞毛细血管镜检查对系统性硬化症器官进展的检测具有反应性。

Sequential nailfold videocapillaroscopy examinations have responsiveness to detect organ progression in systemic sclerosis.

机构信息

Rheumatology A department, Cochin Hospital, Paris Descartes University, Sorbonne Paris Cité, 27 rue du faubourg Saint Jacques, 75014 Paris, France.

Rheumatology A department, Cochin Hospital, Paris Descartes University, Sorbonne Paris Cité, 27 rue du faubourg Saint Jacques, 75014 Paris, France; Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.

出版信息

Semin Arthritis Rheum. 2017 Aug;47(1):86-94. doi: 10.1016/j.semarthrit.2017.02.006. Epub 2017 Feb 10.

Abstract

OBJECTIVE

To determine the merit of nailfold videocapillaroscopy (NVC) to detect meaningful microvascular changes over time in patients with systemic sclerosis (SSc) and whether these changes are associated with overall disease progression and organ involvements.

METHODS

A prospective cohort of 140 SSc patients was recruited over a 12-month period and was followed up on an annual basis for 3 years. Detailed NVC analysis was performed at inclusion and repeated annually. Disease progression and organ damage were defined according to validated definitions.

RESULTS

Significant NVC changes were detected in 72 SSc patients (51%) during the follow-up period. Patients with incident or increased number of giant capillaries were less at risk to develop new digital ulcers (DU) [hazard ratio (HR) = 0.53, 95% confidence interval (CI): 0.07-0.93]. Loss of capillaries over time was confirmed as a robust and independent marker of organ progression. The reduction of the number of capillaries was associated with overall disease progression (HR = 4.35, 95% CI: 1.87-10.12), occurrence of new DU (HR = 5.33, 95% CI: 1.69-16.71), lung vascular progression (HR = 18.53, 95% CI: 1.28-78.33), progression of skin fibrosis (HR = 4.22, 95% CI: 1.24-14.36), and worsening of the Medsger severity score (HR = 5.26, 95% CI: 1.78-15.52).

CONCLUSION

Significant NVC changes are observed in almost half of the patients with SSc during a follow-up of 3 years. Sequential NVC examinations have responsiveness to detect disease progression. Sequential NVC is confirmed of value to monitor SSc, as well as progressive loss of capillaries over time as a potential surrogate marker for disease progression.

摘要

目的

确定甲襞毛细血管显微镜检查(NVC)在系统性硬化症(SSc)患者中随时间检测有意义的微血管变化的优势,以及这些变化是否与整体疾病进展和器官受累有关。

方法

在 12 个月的时间内招募了 140 名 SSc 患者的前瞻性队列,并在接下来的 3 年内每年进行随访。在纳入时和每年重复进行详细的 NVC 分析。根据验证的定义定义疾病进展和器官损伤。

结果

在随访期间,72 名 SSc 患者(51%)检测到明显的 NVC 变化。发生或巨毛细血管数量增加的患者发生新的手指溃疡(DU)的风险较低[风险比(HR)=0.53,95%置信区间(CI):0.07-0.93]。随着时间的推移,毛细血管的丢失被确认为器官进展的可靠且独立的标志物。毛细血管数量的减少与整体疾病进展相关(HR=4.35,95%CI:1.87-10.12),新 DU 的发生(HR=5.33,95%CI:1.69-16.71),肺血管进展(HR=18.53,95%CI:1.28-78.33),皮肤纤维化进展(HR=4.22,95%CI:1.24-14.36),Medsger 严重程度评分恶化(HR=5.26,95%CI:1.78-15.52)。

结论

在 3 年的随访中,几乎一半的 SSc 患者观察到明显的 NVC 变化。连续的 NVC 检查具有检测疾病进展的反应性。连续的 NVC 被证实对监测 SSc 有价值,随着时间的推移,毛细血管逐渐丧失也可以作为疾病进展的潜在替代标志物。

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