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系统性硬化症(SSc)患者甲襞毛细血管镜检查发现的毛细血管密度降低与主动脉根部扩张有关。

Aortic root dilation in associated with the reduction in capillary density observed at nailfold capillaroscopy in SSc patients.

作者信息

Colaci Michele, Dal Bosco Ylenia, Schinocca Claudia, Ronsivalle Giuseppe, Guggino Giuliana, De Andres Ilenia, Russo Alessandra A, Sambataro Domenico, Sambataro Gianluca, Malatino Lorenzo

机构信息

Rheumatology Centre, Internal Medicine Unit, Department of Clinical and Experimental Medicine, c/o Azienda Ospedaliera per l'Emergenza Cannizzaro, University of Catania, Catania, Italy.

Cardiology Unit, Ospedale "S. Vincenzo", Taormina, Italy.

出版信息

Clin Rheumatol. 2021 Mar;40(3):1185-1189. doi: 10.1007/s10067-020-05201-5. Epub 2020 Jun 6.

DOI:10.1007/s10067-020-05201-5
PMID:32506312
Abstract

Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction and fibroblasts activation. Microvascular disease may be easily observed by means of nailfold capillaroscopy. Recent evidences emphasized also the involvement of large-medium arteries in SSc, mainly in terms of increased stiffness of the vessel wall. The study aims to measure aortic root diameter in a cohort of SSc patients and to correlate echocardiographic findings with the capillaroscopic pictures. We analyzed the clinical records of 125 consecutive SSc patients (M/F 14/111, mean age 55 ± 12.7 years, median disease duration 11 years) referring in 3 second-level rheumatology centers. All subjects underwent to heart ultrasound evaluation and videocapillaroscopic evaluation. At capillaroscopy, the patients with early SSc pattern belonged to the subgroup 1, while those with the active/late patterns (characterized by the reduction of capillary density) belonged to the subgroup 2. We found aortic root dilation in 8 (6.4%) SSc patients, with a mean value of 37.8 ± 1.2 mm (range 37-40 mm). Aortic root dilation was observed in only one patient in the subgroup 1 (1/62, 1.6%) and in 7 cases of the subgroup 2 (7/63, 11.1%; p = 0.03). Our study found a significant association between aortic root dilation and impairment of capillary density at nailfold videocapillaroscopy in SSc patients. We hypothesize that SSc-related microangiopathy revealed by nailfold videocapillaroscopy could mimic that of aortic vasa vasorum, contributing to deteriorate the aortic wall structure and favoring aortic root dilation and stiffening.

摘要

系统性硬化症(SSc)是一种自身免疫性疾病,其特征为内皮功能障碍和成纤维细胞活化。通过甲襞毛细血管镜检查可以很容易地观察到微血管疾病。最近的证据还强调了中大型动脉在SSc中的累及情况,主要表现为血管壁硬度增加。本研究旨在测量一组SSc患者的主动脉根部直径,并将超声心动图结果与毛细血管镜图像相关联。我们分析了在3个二级风湿病中心就诊的125例连续的SSc患者(男/女14/111,平均年龄55±12.7岁,疾病中位数病程11年)的临床记录。所有受试者均接受了心脏超声评估和视频毛细血管镜评估。在毛细血管镜检查中,具有早期SSc模式的患者属于亚组1,而具有活动期/晚期模式(以毛细血管密度降低为特征)的患者属于亚组2。我们在8例(6.4%)SSc患者中发现了主动脉根部扩张,平均值为37.8±1.2mm(范围37 - 40mm)。在亚组1中仅1例患者(1/62,1.6%)观察到主动脉根部扩张,在亚组2中有7例(7/63,11.1%;p = 0.03)。我们的研究发现SSc患者的主动脉根部扩张与甲襞视频毛细血管镜检查时毛细血管密度受损之间存在显著关联。我们推测,甲襞视频毛细血管镜检查所揭示的与SSc相关的微血管病变可能类似于主动脉滋养血管的病变,导致主动脉壁结构恶化,并促进主动脉根部扩张和僵硬。

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New Insights into Profibrotic Myofibroblast Formation in Systemic Sclerosis: When the Vascular Wall Becomes the Enemy.系统性硬化症中促纤维化肌成纤维细胞形成的新见解:当血管壁成为敌人时。
Life (Basel). 2021 Jun 24;11(7):610. doi: 10.3390/life11070610.

本文引用的文献

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J Rheumatol. 2001 Jul;28(7):1573-6.
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Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis.系统性硬化症微血管损伤的甲襞视频毛细血管镜评估
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Cardiac involvement in progressive systemic sclerosis (P.S.S.)--an echocardiographic study.进行性系统性硬化症(P.S.S.)的心脏受累——一项超声心动图研究。
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