NATURAL HISTORY

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。这是一项硬皮病患者的登记研究，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，再到临床疾病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进行随访，通过计划随访和事件驱动随访记录疾病的临床病程。纳入标准包括运动超声心动图，静息sPAP >35mmHg或肺功能检查，FVC >70%, DLCO <预测的55%或FVC/ DLCO比值>1.6。对于确诊肺动脉高压，有症状的肺动脉高压患者右心导管穿刺显示平均肺动脉压> 25mmHg。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.