NATURAL HISTORY

该子项目是利用NIH/NCRR资助的中心赠款提供的资源的许多研究子项目之一。子项目和研究者（PI）可能从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。所列机构为中心，不一定是研究者所在机构。这是一项在硬皮病患者中进行的登记研究，旨在确定肺动脉高压从肺动脉高压前期到可诊断肺动脉高压，再到疾病临床恶化的病理进展时间。将以前瞻性方式对入组登记研究的患者进行随访，通过计划和事件驱动随访记录疾病的临床病程。入选标准包括静息sPAP> 35 mmHg的运动超声心动图或FVC >70%且DLCO <预测值的55%或FVC/DLco比值>1.6的肺功能测试。对于明确的肺动脉高压诊断，右心导管插入术显示平均PA压力> 25 mmHg的症状性肺动脉高压患者。在过去的六个月里确诊。

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. This is a registry study of scleroderma patients to determine the time of pathologic progression of pulmonary hypertension from pre-pulmonary hypertension, to diagnosable pulmonary hypertension, to clinical worsening of disease. Patients entered into the registry will be followed in prospective fashion noting the clinical course of disease by both scheduled and event driven follow up. Inclusion Criteria includes Exercise echocardiogram with a resting sPAP of >35mmHg or pulmonary function test with FVC >70% and a DLCO <55% of predicted or a FVC/DLco ratio >1.6. For diagnosis of definite pulmonary hypertension, patients with symptomatic pulmonary hypertension with a right heart catheterization showing a mean PA pressure > 25mmHg. diagnosed in the past 6 months.