Kampolis C, Plastiras Sc, Vlachoyiannopoulos Pg, Moyssakis I, Tzelepis Ge
Department of Pathophysiology, University of Athens School of Medicine, Athens, Greece.
Scand J Rheumatol. 2008 Jul-Aug;37(4):278-83. doi: 10.1080/03009740801978871.
To define the risk factors associated with a relatively rapid increase in estimated pulmonary arterial systolic pressure (PASP) in patients with systemic sclerosis (SSc).
SSc patients undergoing screening for pulmonary arterial hypertension (PAH) by echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded PASP, pulmonary function, and clinical and laboratory data. PAH was defined as an estimated PASP> or =40 mmHg. In each patient, the PASP values with their corresponding time intervals were fitted to a linear function and the slope of the line was calculated.
Seventy-one patients with at least two echocardiographic studies each were analysed. In 16 (23%) patients, the rate of PASP progression was > or =2.5 mmHg/year whereas in the remaining 55 (77%) patients the rate of progression was <2.5 mmHg/year. In multiple logistic regression analysis, anti-centromere antibodies (ACA) (OR 8.75, CI 1.12-68.38, p = 0.039) and age > or =50 years at diagnosis (OR 8.76, CI 1.28-60.14, p = 0.027) were independently associated with a rise of PASP by > or =2.5 mmHg/year. Baseline forced vital capacity (FVC) <70% (predicted), Raynaud's duration preceding skin manifestations by > or =5 years, and fibrosis on lung computed tomography (CT) were not associated with a rapid rise of PASP (p>0.05).
Old age at diagnosis and ACA are associated with a relatively rapid rise of PASP estimated by echocardiography in SSc. Screening for PAH in these patients may, if followed by right heart catheterization, detect PAH at an earlier stage and guide therapeutic decisions.
确定与系统性硬化症（SSc）患者肺动脉收缩压（PASP）估计值相对快速升高相关的危险因素。
识别出通过超声心动图筛查肺动脉高压（PAH）的SSc患者，并对其病历进行回顾性审查。在所有患者中，我们记录了PASP、肺功能以及临床和实验室数据。PAH定义为估计的PASP≥40 mmHg。在每位患者中，将PASP值及其相应的时间间隔拟合为线性函数，并计算直线的斜率。
分析了71例每位患者至少有两项超声心动图检查的患者。16例（23%）患者的PASP进展速率≥2.5 mmHg/年，而其余55例（77%）患者的进展速率<2.5 mmHg/年。在多因素logistic回归分析中，抗着丝点抗体（ACA）（比值比8.75，可信区间1.12 - 68.38，p = 0.039）和诊断时年龄≥50岁（比值比8.76，可信区间1.28 - 60.14，p = 0.027）与PASP升高≥2.5 mmHg/年独立相关。基线用力肺活量（FVC）<70%（预测值）、皮肤表现前雷诺现象持续时间≥5年以及肺部计算机断层扫描（CT）显示纤维化与PASP快速升高无关（p>0.05）。
诊断时年龄较大和ACA与SSc患者超声心动图估计的PASP相对快速升高相关。对这些患者进行PAH筛查，如果随后进行右心导管检查，可能会在更早阶段检测到PAH并指导治疗决策。
