Hickey Peter M, Lawrie Allan, Condliffe Robin
Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom.
Pulmonary Vascular Diseases Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
Front Med (Lausanne). 2018 Jun 6;5:175. doi: 10.3389/fmed.2018.00175. eCollection 2018.
Pulmonary arterial hypertension (PAH) develops in 7-12% of patients with systemic sclerosis (SSc) and is associated with a 3 year survival of 52%. Early detection by screening is therefore recommended for all patients with SSc. Historically, screening has been performed using echocardiography and measurement of gas transfer. More recently the DETECT protocol, using a combination of biomarkers (including N-terminal pro-brain natriuretic peptide) and clinical parameters, has been developed. The optimal method of screening for PAH with high sensitivity and specificity is, however, not clear. Protein expression differences between different SSc disease phenotypes have been reported, and include alterations in concentration of NT-proBNP, endoglin, soluble vascular endothelial growth factor receptor 1, placenta growth factor, growth differentiation factor-15, vascular endothelial growth factor alpha, resistin-like molecule beta, and soluble thrombomodulin. This review summarizes the current knowledge of these protein changes in patients with SSc and PAH.
7%至12%的系统性硬化症（SSc）患者会发生肺动脉高压（PAH），且其3年生存率为52%。因此，建议对所有SSc患者进行筛查以早期发现。过去，筛查通过超声心动图和气体交换测量来进行。最近，已开发出使用生物标志物（包括N末端脑钠肽前体）和临床参数组合的DETECT方案。然而，尚不清楚具有高灵敏度和特异性的PAH最佳筛查方法。已有报道不同SSc疾病表型之间存在蛋白质表达差异，包括NT-脑钠肽前体、内皮糖蛋白、可溶性血管内皮生长因子受体1、胎盘生长因子、生长分化因子-15、血管内皮生长因子α、抵抗素样分子β和可溶性血栓调节蛋白浓度的改变。本综述总结了目前关于SSc和PAH患者这些蛋白质变化的知识。
