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罗马尼亚硬皮病患者内皮功能障碍的临床表型

Clinical Phenotype of Endothelial Dysfunction in Romanian Scleroderma Patients.

作者信息

Groseanu Laura, Berghea Florian, Balanescu Andra, Predeteanu Denisa, Bojinca Violeta, Saulescu Ioana, Constantinescu Cosmin, Opris Daniela, Abobului Mihai, Borangiu Andreea, Negru Maria-Magdalena, Vlad Violeta, Ionescu Ruxandra

机构信息

"Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania.

出版信息

Maedica (Bucur). 2016 Mar;11(1):26-31.

PMID:28465747
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5394495/
Abstract

OBJECTIVE

To identify the particularities of the clinical phenotype of endothelial dysfunction in a lot of Romanian patients from a reference center and compare it to data reported by international registries.

MATERIAL AND METHODS

51 patients were included in a cross-sectional study. The patients were evaluated for the pattern of disease, main visceral involvement, serum markers of disease.

RESULTS

41.2% patients had history of digital ulcers, 27.45% had pulmonary arterial hypertension; cardiovascular involvement also included: diastolic dysfunction in 31.1% of the patients, global systolic dysfunction in 9.8%, rhythm and conduction disturbances in 19.6%, peripheral vascular disease in 19.6%. Scleroderma renal crisis was identified in 2 patients.

CONCLUSION

Vascular complications are a major cause of morbidity and mortality in systemic sclerosis. Earlier therapeutic intervention demands improved screening and diagnosis in all cases.

摘要

目的

确定来自一家参考中心的众多罗马尼亚患者内皮功能障碍的临床表型特点,并将其与国际登记处报告的数据进行比较。

材料与方法

51例患者纳入一项横断面研究。对患者进行疾病模式、主要内脏受累情况、疾病血清标志物的评估。

结果

41.2%的患者有指端溃疡病史,27.45%有肺动脉高压;心血管受累还包括:31.1%的患者有舒张功能障碍,9.8%有整体收缩功能障碍,19.6%有节律和传导紊乱,19.6%有外周血管疾病。2例患者被诊断为硬皮病肾危象。

结论

血管并发症是系统性硬化症发病和死亡的主要原因。早期治疗干预需要在所有病例中改进筛查和诊断。

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本文引用的文献

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Nailfold capillaroscopy for prediction of novel future severe organ involvement in systemic sclerosis.甲褶毛细血管镜检查对系统性硬化症新的未来严重器官受累的预测。
J Rheumatol. 2013 Dec;40(12):2023-8. doi: 10.3899/jrheum.130528. Epub 2013 Oct 15.
2
Relationship between body composition and both cardiovascular risk factors and lung function in systemic sclerosis.系统性硬化症患者身体成分与心血管危险因素及肺功能之间的关系
Clin Rheumatol. 2014 Jan;33(1):77-82. doi: 10.1007/s10067-013-2388-y. Epub 2013 Sep 20.
3
Ischemic arterial events and atherosclerosis in patients with systemic sclerosis: a population-based case-control study.系统性硬化症患者的缺血性动脉事件和动脉粥样硬化:一项基于人群的病例对照研究。
Arthritis Res Ther. 2013 Aug 14;15(4):R87. doi: 10.1186/ar4267.
4
Scleroderma renal crises: case report and review of literature.
J Pak Med Assoc. 2013 Jul;63(7):916-8.
5
Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.基于证据的系统性硬化症肺动脉高压检测:DETECT 研究。
Ann Rheum Dis. 2014 Jul;73(7):1340-9. doi: 10.1136/annrheumdis-2013-203301. Epub 2013 May 18.
6
Macrovascular involvement in systemic sclerosis: evidence of correlation with disease activity.系统性硬化症的大血管受累:与疾病活动度相关的证据。
Clin Exp Rheumatol. 2012 Mar-Apr;30(2 Suppl 71):S76-80. Epub 2012 May 30.
7
Vascular involvement in systemic sclerosis (scleroderma).系统性硬皮病(硬皮病)中的血管病变。
J Inflamm Res. 2011;4:105-25. doi: 10.2147/JIR.S18145. Epub 2011 Jul 26.
8
Systemic sclerosis and the heart: current diagnosis and management.系统性硬化症与心脏:当前的诊断与治疗。
Curr Opin Rheumatol. 2011 Nov;23(6):545-54. doi: 10.1097/BOR.0b013e32834b8975.
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Early intervention in pulmonary arterial hypertension associated with systemic sclerosis: an essential component of disease management.早期干预系统性硬皮病相关肺动脉高压:疾病管理的重要组成部分。
Eur Respir Rev. 2010 Dec;19(118):314-20. doi: 10.1183/09059180.00007810.
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Primary myocardial involvement in systemic sclerosis: evidence for a microvascular origin.系统性硬化症的原发性心肌受累:微血管起源的证据。
Clin Exp Rheumatol. 2010 Sep-Oct;28(5 Suppl 62):S48-53. Epub 2010 Nov 3.