摘要 目的：临界肺动脉高压有一个显著的特征，就是安静状态下肺动脉压正常，而运动可以显著诱发肺动脉高压，这可能是系统性硬化患者肺动脉高压的早期表现，我们假定对这类病人进行降低肺动脉高压的治疗是安全的，而且可以减慢血液动力学的发展。

方法：对合并临界肺动脉高压的系统性硬化症患者进行一次基线右心导管，12个月后观察作为第一个实验组，波生坦治疗6个月后作为第二个实验组。主要研究重点为比较治疗组和对照组在50瓦的平均肺动脉压改变。

结果：10个病人完成了该实验，观察期间安静状态，50瓦和最大运动量的平均肺动脉压显著升高(增加2.5±3.0mmHg; p=0.03, 增加4.0±2.9mmHg; p=0.002,和增加6.8±4.1mmHg; p<0 .005)，治疗期间肺动脉压则趋于降低(降低.5±3.9mmhg; p="0.07," 降低1.5±4.5mmhg; p="0.32," 和降1.8±7.0mmhg; p="0.43)，治疗期和观察期肺动脉压的变化还是很显著的(p=0.03," p="0.01" (主要终点), 和p="0.02)。在安静状态下肺动脉血管阻力是显著不同的(+8±25" vs. -45±22dyn*s*cm(-5) ; p<0.001)，肺动脉楔压则没有明显差别(+2.6±2.5mmhg (p<0.01) vs. -0.6±3.8mmhg (ns) ,p="0.12)，但是观察期间则有显著的增长。试验中没有出现相关不良反应。

结论：伴有临界肺血流动力学改变的系统性硬化症患者，一年期观察期间内安静状态和运动诱发的肺动脉压可能均会有显著升高。波生坦治疗可能是一种安全有效的治疗方法。这种用来产生假说的探索性研究的结果需要随机对照研究来证实。

附原文：OBJECTIVE: Borderline pulmonary arterial hypertension (PAH), characterised by a marked exercise-induced increase in pulmonary arterial pressure with normal resting values may precede manifest PAH in systemic sclerosis. We hypothesised that PAH treatment is safe in these patients and might attenuate hemodynamic progression.METHODS: Systemic sclerosis patients with borderline PAH underwent a baseline right heart catheterisation, a control after a 12-month observation and a second control after 6 months bosentan therapy. As primary end point the changes of mean pulmonary arterial pressure at 50 Watts during the observation vs. therapy were compared.RESULTS: Ten patients completed the study. Mean pulmonary arterial pressure at rest, 50 Watt, and maximal exercise increased significantly during the observation period (+2.5±3.0mmHg; p=0.03, +4.0±2.9mmHg; p=0.002, and +6.8±4.1mmHg; p<0 .005) and tended to decrease during the treatment period (-2.5±3.9mmhg; p="0.07," -1.5±4.5mmhg; p="0.32," and -1.8±7.0mmhg; p="0.43)." the changes during observation vs. therapy period were significantly different (p="0.03," p="0.01" (primary end point), and p="0.02," respectively). the changes in resting pulmonary vascular resistance were also significantly different (+8±25 vs. -45±22dyn*s*cm(-5) ; p<0.001). the changes in resting pulmonary arterial wedge pressure (+2.6±2.5mmhg (p<0.01) vs. -0.6±3.8mmhg (ns)) were not significant (p="0.12)," despite the significant increase during observation period. no relevant adverse effects were reported.conclusion: in systemic sclerosis patients with borderline pulmonary hemodynamics, resting and exercise pulmonary arterial pressure may significantly increase within one year of observation. bosentan might be safe and effective to attenuate these changes. randomised controlled trials are warranted to confirm these explorative findings of a hypothesis generating study.

引自：Kovacs G, Maier R, Aberer E, Brodmann M, Graninger W, Kqiku X, Scheidl S, Tr?ster N, Hesse C, Rubin L, Olschewski H.Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary pressures. Arthritis Rheum， 2011 Nov 29. doi: 10.1002/art.33460. [Epub ahead of print]