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.
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).
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.
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.
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.
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