SLEEP-DISORDERED BREATHING WITH SCLERODERMA

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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.