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Longterm survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol
Journal of Rheumatology, 09/14/09
Badesch DB et al. – This study reports longterm survival rates for patients with scleroderma-associated PAH treated with IV epoprostenol. Although comparisons to historical data should be made with caution, this study reports a better survival outcome than natural history data on patients with scleroderma-associated PAH.
Methods- One hundred two patients diagnosed with PAH in association with scleroderma who received epoprostenol
- 51 PAH patients from a subject population of 56 who received epoprostenol
- 46 patients from an initial population of 55 subjects on conventional therapy who received epoprostenol in the extension study
- Open-label epoprostenol
- Adverse events, survival, and dosing information collected
- Probabilities of survival during the first and second years for all subjects who received epoprostenol during the initial randomized controlled study or during the extension study were 0.71 and 0.52
- Measure remained constant at 0.48 during the third and fourth years
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