PT  - JOURNAL ARTICLE
AU  - DAVID B. BADESCH
AU  - MICHAEL D. McGOON
AU  - ROBIN J. BARST
AU  - VICTOR F. TAPSON
AU  - LEWIS J. RUBIN
AU  - FREDRICK M. WIGLEY
AU  - KENNETH M. KRAL
AU  - IBRAHIM H. RAPHIOU
AU  - GLENN D. CRATER
TI  - Longterm Survival Among Patients with Scleroderma-associated Pulmonary Arterial Hypertension Treated with Intravenous Epoprostenol
AID  - 10.3899/jrheum.081277
DP  - 2009 Oct 01
TA  - The Journal of Rheumatology
PG  - 2244--2249
VI  - 36
IP  - 10
4099  - http://www.jrheum.org/content/36/10/2244.short
4100  - http://www.jrheum.org/content/36/10/2244.full
SO  - J Rheumatol2009 Oct 01; 36
AB  - Objective. Pulmonary arterial hypertension (PAH) remains challenging to treat, especially in association with scleroderma. We examined survival rates among patients with PAH in association with scleroderma who received epoprostenol (Flolan®) through continuous intravenous (IV) infusion in an uncontrolled open-label 3-year extension study following an initial randomized, controlled 12-week study. Methods. One hundred two patients diagnosed with PAH in association with scleroderma who received epoprostenol were included in the analyses. This included 51 PAH patients from a subject population of 56 who received epoprostenol in the randomized controlled study, and 46 patients from an initial population of 55 subjects on conventional therapy in the randomized controlled study, who received epoprostenol in the extension study. All patients in this extension study received open-label epoprostenol. Adverse events, survival, and dosing information were collected throughout the study. Results. The 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, respectively. This measure remained constant at 0.48 during the third and fourth years. Conclusion. 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.