SLEEP DISORDERED BREATHING AND SCLERODERMA-ASSOCIATED PULMONARY HYPERTENSION

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。所列机构为中心机构，不一定是研究者机构。硬皮病相关性肺动脉高压（SScPH）与高死亡率相关，目前尚无有效的治疗选择。睡眠呼吸障碍（SDB）是一系列疾病，包括阻塞性睡眠呼吸暂停和呼吸不足、伴有中枢性呼吸暂停的周期性呼吸（Cheyne-Stokes）和夜间通气不足，已知在其他慢性肺部疾病（包括原发性肺动脉高压）患者中常见，但尚未在SScPH患者中进行充分研究。我们的总体假设是，SDB可能在SScPH的发病机制和进展中发挥作用，因为它显然在原发性肺动脉高压中发挥作用。 我们的具体目的是：1）确定硬皮病患者中SDB与肺动脉高压的相关性; 2）确定SScPH患者中睡眠呼吸障碍的患病率; 3）表征SScPH患者中存在的SDB类型。拟定研究的源人群是约翰霍普金斯肺动脉高压诊所和硬皮病诊所的硬皮病患者组。具体目的将通过实施一项前瞻性病例对照研究来解决，招募硬皮病伴和不伴肺动脉高压的患者进行夜间多导睡眠描记术，以检测和诊断SDB。SScPH患者SDB的诊断和治疗可能是改善SScPH患者发病率和死亡率的首选干预措施。

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-associated pulmonary hypertension (SScPH) is associated with high mortality and currently has no effective therapeutic options. Sleep disordered breathing (SDB) is a constellation of disorders, including obstructive sleep apneas and hypopneas, periodic breathing (Cheyne-Stokes) with central apneas, and nocturnal hypoventilation, known to be common in patients with other chronic pulmonary diseases, including primary pulmonary hypertension, but has not been adequately studied in patients with SScPH. Our overall hypothesis is that SDB may play a role in the pathogenesis and progression of SScPH, as it apparently does in primary pulmonary hypertension. Our Specific Aims are 1) to determine the association of SDB to pulmonary hypertension in patients with scleroderma; 2) to determine the prevalence of sleep-disordered breathing in patients with SScPH; and 3) to characterize the types of SDB present in patients with SScPH. The source population for the study proposed is the group of patients with scleroderma at The Johns Hopkins Pulmonary Hypertension clinic and Scleroderma clinic. The Specific Aims will be addressed by implementation of a prospective case-control study, recruiting patients with scleroderma with and without pulmonary hypertension for nocturnal polysomnography for detection and diagnosis of SDB. Diagnosis and treatment of SDB in patients with SScPH may prove to be the first intervention to improve morbidity and mortality patients with SScPH.