NATURAL HISTORY

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 这是一项硬皮病患者的注册研究，以确定从肺动脉高压前期到可诊断的肺动脉高压，再到疾病的临床恶化的病理进展时间。进入登记处的患者将以前瞻性的方式接受跟踪，通过计划和事件驱动的后续行动记录疾病的临床过程。纳入标准包括 运动超声心动图，静息空间35毫米汞柱，或肺功能测试，FVC；70%，DLCO；55%，或FVC/DLCO；1.6。对于明确的肺动脉高压的诊断，有症状的肺动脉高压患者右心导管术显示平均动脉压为25毫米汞柱。在过去6个月内确诊。

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.