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
PURPOSE: Pulmonary 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.
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 <65% predicted).
RESULTS: There 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.
CONCLUSIONS: All 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.