ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2576

Epoprostenol Rescue Therapy In Systemic Sclerosis-Associated Pulmonary Arterial Hypertension and Idiopathic Pulmonary Arterial Hypertension

Adrienne M. Roos1, Christopher Pasarikovski1, Amie T. Kron1, John T. Granton2, Peter Lee3, John Thenganatt4 and Sindhu R. Johnson5, 1Medicine, Toronto Scleroderma Research Program, Toronto Western Hospital, Mount Sinai Hospital, and University of Toronto, Toronto, ON, Canada, 2Medicine, Toronto Pulmonary Hypertension Programme, Toronto General Hospital and University of Toronto, Toronto, ON, Canada, 3Lebovic Bldg, Mt. Sinai Hospital, Toronto, ON, Canada, 4Medicine, University Health Network Pulmonary Hypertension Programme, Toronto General Hospital, Divisions of Respirology and Critical Care Medicine, Toronto, ON, Canada, 5Medicine, Division of Rheumatology, Toronto Western Hospital, University Health Network Pulmonary Hypertension Programme, Toronto General Hospital, Mount Sinai Hospital and University of Toronto, Toronto, ON, Canada

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords:

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Epoprostenol has been demonstrated to improve hemodynamics, functional class, and six-minute walk distance (6MWD) in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) and idiopathic PAH (IPAH) patients. In contemporary practice, it is usually reserved for patients who have failed treatment with endothelin receptor antagonists and/or phosphodiesterase-5 inhibitors. The effect of epoprostenol rescue therapy on survival has not been evaluated. The objective of this study was to evaluate the role of intravenous epoprostenol as rescue therapy in the SSc-PAH and IPAH patients.

Methods:

Patients attending the University Health Network Pulmonary Hypertension Program between 1998 and 2012 were included if they had a diagnosis of SSc-PAH and IPAH based on a mean pulmonary artery pressure (mPAP) of > 25 mmHg and a pulmonary capillary wedge pressure of <15 mmHg on cardiac catheterization, and had been treated with intravenous epoprostenol after treatment with endothelin receptor antagonists and/or phosphodiesterase-5 inhibitors for PAH. The primary outcome was survival. Survival was defined as the time from initiation of epoprostenol to death from any cause. Patients were censored as of May 1, 2012. Survival was evaluated using Kaplan Meier curves.

Results:

1140 patients were reviewed to identify 36 patients with SSc-PAH and 24 patients with IPAH treated with epoprostenol after failure with oral pulmonary hypertension specific therapies. 83% of SScPAH and 75% of IPAH patients were female. The mean (standard deviation) PAH duration prior to initiation of epoprostenol was 3.3 (5.7) years for SScPAH, and 2.1 (2.1) years for IPAH patients. Median 1-, 2-, 3-, 4-, 5-year survival for SSc patients was 85.7%, 60.7%, 53.6%, 46.1%, 42.3%; and for IPAH patients was 83.3%, 70.8%, 65.8%, 59.2%, 59.2%. There was no significant difference in survival between the SScPAH and IPAH patients treated with epoprostenol (p=0.13).

Conclusion: Our findings demonstrate desirable long-term survival and support the use of epoprostenol as rescue therapy for SSc-PAH and IPAH patients.

Disclosure:

A. M. Roos,
None;

C. Pasarikovski,
None;

A. T. Kron,
None;

J. T. Granton,

Support respirology program at the hospital foundation.,

9,

Pfizer support of research study via CIHR grant.,

9;

P. Lee,
None;

J. Thenganatt,
None;

S. R. Johnson,
None.

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