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Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): Two-Year Outcomes for Patients with Pulmonary Hypertension

Virginia Steen


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Conference: 2010 International PHA Conference and Scientific Sessions

Release Date: 06.24.2010

Presentation Type: Abstracts

Steen VD, The PHAROS Investigators.
Georgetown University, Washington, DC, USA

PURPOSEPulmonary hypertension (PH) in scleroderma is very complex. PHAROS is a multicenter, prospective, observational study to determine the outcomes of high risk patients for getting PH and incident cases of PH.

METHODSPH 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 <65% predicted).

RESULTSThere were 133 PH patients (19 developed PH after first visit). The age at PH diagnosis was a mean (m) 57y, 87% were women, 22% African–American, scleroderma disease duration to PH was m10.8 y, follow up duration from PH was m1.7y.

Group 1: 92 had PAH: mPAP–38mmHg, m DLCO–40%, m FVC%/DLCO% (ratio)–2.34. Only 6 patients had anti–Scl 70 (p<0.03 compared to other groups).

Group 2 = 24 (wedge m 21 mmHg): mPAP–33mmHg, DLCO–38%. ratio–2.1.

Group 3 = 17 patients (m FVC 55%), mPAP–31mmHg, DLCO–32%, ratio–1.9. In the PAH group, 37% were on oxygen, the m6 minute walk distance was 342m. Most patients received a single oral therapy for PAH: 22 endothelin receptor antagonist, 20 sildenafil, but 20 received a combination of these agents. 17 were on a prostacylcin: (only 3 on intravenous).

There was a 94% cumulative survival at 1 y and 91% at 2 y. and 84% 1 yr and 68% 2 yr time to clinical worsening which was defined as death, hospitalization for PH, or the addition of another PH medication.

CONCLUSIONSAll PH groups had very low DLCOs, 31% of SSc patients with mPAP>25mmHg did not have PAH. PAH patients had higher mPAP and more anticentromere patients.

CLINICAL IMPLICATIONS: Careful evaluation of all SSc-PH patients is necessary to determine who has PAH versus other types of PH. Early diagnosis and treatment should improve outcome.