This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.This is a prospective, randomized, placebo-controlled trial of Bosentan in systemic sclerosis patients with exercise-induced pulmonary hypertension. Subjects will be randomized to receive Bosentan 62.5 mg PO Bid x 1 month followed by 125 mg PO Bid thereafter versus placebo for 16 weeks. The primary outcome variable is total exercise time during the standard Bruce stress protocol with echocardiogram.The secondary outcome variables to be evaluated include: 6 minute walk distance, BMP levels, ET-1 levels, Short-Form 36 questionnaire, sHAQ, UCDS Dyspnea index.At the completion of 16 weeks, subjects will be eligible to receive long term Bosentan. Measurements of exercise endurance, non-invasive hemodynamics, and quality of life will be repeated every 6 months until the completion of the trial in 36 months as part of the PHRAOS long term (observational) study in which participants will be urged to enter, if they are not already a part of it.
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