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.Scleroderma patients have a high risk for developing pulmonary hypertension (PAH). We know that some of the abnormalities found in PAH occur many years prior to the definite diagnosis of PAH. Exercise induced PAH occurs in idiopathic PAH, and we have found that 47% of scleroderma patients without definite PAH have increases in the pulmonary pressures. This study is a double blind controlled trial of bosentan, one of the approved treatments for PAH, vs placebo in a 4 month study. It will determine whether any of the more subtle findings of early PAH respond to bosentan.
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