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Summary
Patients with scleroderma are at increased risk for
the development of pulmonary hypertension, and the development
of unexplained dyspnea or an isolated decrease in diffusing
capacity should prompt evaluation. Echocardiography
is often helpful in this situation. Because the prognosis
of untreated pulmonary hypertension occurring in the
setting of scleroderma is generally quite poor, vigilance
is required on the part of physicians following this
“at risk” group of patients. The past decade has seen
important advances in the treatment of pulmonary arterial
hypertension, including intravenous epoprostenol, oral
bosentan, and subcutaneously infused treprostinil. As
new therapies are developed for the treatment of pulmonary
arterial hypertension, it is essential that patients
with scleroderma-related disease are included in clinical
trials. PH
Acknowledgement
Portions of this report are similar to upcoming articles
written by the same authors in the 2nd Edition of Pulmonary
Circulation edited by Drs Andrew Peacock and Lewis J.
Rubin and in Progress in Cardiovascular Diseases.
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