Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis.
Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis.
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DOI：
10.1093/rheumatology/kes068
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发表时间：
2012-09
期刊：
影响因子：
--
通讯作者：
QUINs Trial Study Group
中科院分区：
文献类型：
--
作者：
Barnes T;Gliddon A;Doré CJ;Maddison P;Moots RJ;QUINs Trial Study Group
目标。本研究旨在探讨血管性血友病因子(von Willebrand factor，vWF)作为一种生物标志物在LcSSc中的应用，特别是vWF预测该病未来疾病表现的能力。方法：研究方法。在Quins试验[用血管紧张素转换酶(ACE)抑制预防硬皮病的血管损伤]期间，在基线和3年期间，测量了lcSSc患者血清中的vWFAg浓度。分别于治疗前和治疗3年时测定%DLCO、%KCO、%FVC、超声心动图估测的肺动脉压(PAP)、雷诺发作次数、雷诺严重程度、指端溃疡发生率、尿蛋白排泄量、估计肾小球滤过率(EGFR)、改良Rodnan皮肤评分和Medsger疾病活动度评分。结果。基线血清vWF浓度与并发Medsger疾病活动评分、%DLCO、%FVC、尿蛋白排泄量、表皮生长因子受体和PAP>；30 毫米汞柱相关。在Logistic回归模型中，基础血清vWF浓度能够通过超声心动图预测PAP升高的未来发展(PAP>；40 = ，P<0.001)。结论。肺动脉高压是LcSSc的一个危及生命的并发症。VWF是血管内皮细胞活化的标志。LcSSc患者血清vWF浓度升高增加了PAP继发升高的风险。因此，筛查lcSSc患者的vWF可以确定有发生PAH的风险的人群。这些患者可能会被稳定内皮的药物作为靶点，例如他汀类药物。
Objectives. This study aims to examine the utility of von Willebrand factor (vWF) as a biomarker in lcSSc, in particular the ability of vWF to predict the future development of disease manifestations in this disease. Methods. vWFAg concentrations were measured in the serum of patients with lcSSc at baseline and at 3 years, during the QUINs trial [Prevention of Vascular Damage in Scleroderma with Angiotensin-Converting Enzyme (ACE) Inhibition]. %DLCO, %KCO, %FVC, pulmonary artery pressure (PAP) estimation by echocardiography, Raynaud’s attack frequency, Raynaud’s severity, digital ulcer frequency, urinary protein excretion, estimated glomerular filtration rate (eGFR), modified Rodnan skin score and Medsger disease activity score were also measured at baseline and 3 years. Results. Baseline serum vWF concentrations were related to concurrent Medsger disease activity score, %DLCO, %FVC, urinary protein excretion, eGFR and PAP >30 mmHg. In logistic regression models, baseline serum vWF concentrations were able to predict the future development of elevated PAP by echocardiography (PAP >40 mmHg, P = 0.001). Conclusions. Pulmonary artery hypertension is a life-threatening complication of lcSSc. vWF is a marker of endothelial cell activation. Raised serum concentrations of vWF in lcSSc increase the risk of developing subsequent elevation in PAP. Therefore screening patients with lcSSc for vWF may identify a group at risk of developing PAH. These patients could potentially be targeted with agents that stabilize the endothelium, e.g. statins.
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DOI：
10.1111/j.1365-2362.2006.01688.x
发表时间：
2006-09-01
影响因子：
5.5
作者：
McLaughlin, V. V.
通讯作者：
McLaughlin, V. V.
影响因子：
3.2
作者：
Rossi, Rosario;Nuzzo, Annachiara;Modena, Maria Grazia
通讯作者：
Modena, Maria Grazia
DOI：
10.1093/rheumatology/ken488
发表时间：
2009-03
期刊：
Rheumatology (Oxford, England)
影响因子：
--
作者：
Hachulla E;Carpentier P;Gressin V;Diot E;Allanore Y;Sibilia J;Launay D;Mouthon L;Jego P;Cabane J;de Groote P;Chabrol A;Lazareth I;Guillevin L;Clerson P;Humbert M;ItinérAIR-Sclérodermie Study Investigators
通讯作者：
ItinérAIR-Sclérodermie Study Investigators
影响因子：
5
作者：
Sweiss, Nadera J;Hushaw, Linda;Archer, Stephen L
通讯作者：
Archer, Stephen L
影响因子：
27.4
作者：
Herrick, AL;Illingworth, K;Jayson, MIV
通讯作者：
Jayson, MIV