BOSENTAN VS PLACEBO IN NYHA CLASS I/II SCLERODERMA PATIENTS WITH EXERCISE INDUCE

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 研究中心，而研究中心不一定是研究者所在的机构。 硬皮病患者发生肺动脉高压（PAH）的风险较高。 我们知道，PAH中发现的一些异常发生在PAH确诊前多年。 运动诱发的PAH发生在特发性PAH中，我们发现47%没有明确PAH的硬皮病患者肺压升高。 本研究是一项在4个月研究中比较波生坦（PAH的获批治疗之一）与安慰剂的双盲对照试验。 它将确定早期PAH的任何更微妙的发现是否对波生坦有反应。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Scleroderma patients have a high risk for developing pulmonary hypertension (PAH). We know that some of the abnormalities found in PAH occur many years prior to the definite diagnosis of PAH. Exercise induced PAH occurs in idiopathic PAH, and we have found that 47% of scleroderma patients without definite PAH have increases in the pulmonary pressures. This study is a double blind controlled trial of bosentan, one of the approved treatments for PAH, vs placebo in a 4 month study. It will determine whether any of the more subtle findings of early PAH respond to bosentan.