Abstract: Poster Presentations |


Virginia Steen, MD*
Author and Funding Information

Georgetown University, Washington, DC


Chest. 2009;136(4_MeetingAbstracts):54S. doi:10.1378/chest.136.4_MeetingAbstracts.54S
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PURPOSE:  Pulmonary hypertension(PH) in scleroderma is a very complex disease. PHAROS is a multicenter, prospective, observational study to determine the outcomes of high risk patients for getting PH and incident cases of PH.

METHODS:  PH was defined as a mean(m)PAP > 25mmHg on right heart catheterization. Patients were then defined as Group 1 - PAH, Group 2 - PVH with wedge > 16mmHg and Group 3 –PH with fibrosis (moderate-severe fibrosis on high resolution CT scan with FVC < 60% predicted).

RESULTS:  There were 99 PH patients (14 got PH 1.2y(m)after 1st visit). The m age at PH diagnosis was 57y, 87% were women, 22% African-American, m scleroderma disease duration to PH was 10.8 y. m follow up duration from PH was 1.7y. This included patients in the following subgroups of PH. Group 1: 76 patients, mPAP - 36mmHg, m DLCO - 41%, m FVC%/DLCO% (ratio) - 2.24. Only 6 patients had anti-Scl 70 (p < 0.03 compared to other groups). Group 2: 12 patients (wedge m 21 mmHg): m PAP - 33mmHg, m DLCO - 38%. m ratio - 2.02, Group 3: 11 patients (m FVC 53.1%), m PAP - 27mmHg, DLCO –m 31%, ratio –1.78, In the PAH group, 48% were on oxygen, the m6 minute walk distance was 350m. Most patients received a single oral therapy for PAH: 17 endothelin receptor antagonist, 20 sildenafil, but 15 received a combination of these agent and 11 were on a prostacylcin: (only 2 on intravenous). There was a 94% cumulative survival at 1 y and 90% at 2 y and 90% at 3 y.

CONCLUSION:  20% of SSc patients with mPAP > 25mmHg did not have PAH. PAH patients had higher mPAP, higher ratio, and fewer Scl-70 patients. Newly diagnosed PAH patients managed with oral agents have an excellent 2 year survival.

CLINICAL IMPLICATIONS:  Careful evaluation of all SSc-PH patients is necessary to determine who has PAH versus other types of PH. PHAROS will help to define these patients and their different outcomes.

DISCLOSURE:  Virginia Steen, Grant monies (from sources other than industry) Sibley Foundation; Grant monies (from industry related sources) Gilead pharmaceutical; Consultant fee, speaker bureau, advisory committee, etc. Actelion, Gilead, United Therapeutics; No Product/Research Disclosure Information

Tuesday, November 3, 2009

12:45 PM - 2:00 PM




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