SLEEP DISORDERED BREATHING AND SCLERODERMA-ASSOCIATED PULMONARY HYPERTENSION

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。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，周期性呼吸（Cheyne-Stokes）伴中心性呼吸暂停和夜间通气不足，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，但尚未对SScPH患者进行充分研究。我们的总体假设是SDB可能在SScPH的发病和进展中发挥作用，就像它在原发性肺动脉高压中明显发挥的作用一样。我们的具体目的是：1)确定SDB与硬皮病患者肺动脉高压的关系；2)确定SScPH患者睡眠呼吸障碍的患病率；3)确定SScPH患者存在的SDB类型。提出的研究的源人群是约翰霍普金斯大学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，该研究招募伴有和不伴有肺动脉高压的硬皮病患者进行夜间多导睡眠检查，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第一步干预措施。

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.