Background Patients with normal (mean pulmonary arterial pressure (mPAP) ≤20 mm Hg) and borderline mean pulmonary pressures (21–24 mm Hg) are “at risk” of developing pulmonary hypertension (PH). The objectives of this analysis were to examine the baseline characteristics in systemic sclerosis (SSc) with normal and borderline mPAP and to explore long-term outcomes in SSc patients with borderline mPAP versus normal haemodynamics.
Methods PHAROS is a multicentre prospective longitudinal cohort of patients with SSc “at risk” or recently diagnosed with resting PH on right heart catheterisation (RHC). Baseline clinical characteristics, pulmonary function tests, high-resolution CT, 2-dimensional echocardiogram and RHC results were analysed in normal and borderline mPAP groups.
Results 206 patients underwent RHC (results showed 35 normal, 28 borderline mPAP, 143 resting PH). There were no differences in the baseline demographics. Patients in the borderline mPAP group were more likely to have restrictive lung disease (67% vs 30%), fibrosis on high-resolution CT and a higher estimated right ventricular systolic pressure on echocardiogram (46.3 vs 36.2 mm Hg; p<0.05) than patients with normal haemodynamics. RHC revealed higher pulmonary vascular resistance and more elevated mPAP on exercise (≥30; 88% vs 56%) in the borderline mPAP group (p<0.05 for both). Patients were followed for a mean of 25.7 months and 24 patients had a repeat RHC during this period. During follow-up, 55% of the borderline mPAP group and 32% of the normal group developed resting PH (p=NS).
Conclusions Patients with borderline mPAP have a greater prevalence of abnormal lung physiology, pulmonary fibrosis and the presence of exercise mPAP ≥30 mm Hg.
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Funding The PHAROS registry is funded by unrestricted grant from Actelion Inc and Gilead Inc and the Scleroderma Foundation. DK was supported by a National Institutes of Health award (NIAMS K23 AR053858-05).
Ethics approval The study received ethics approval from the review board at each institution.
Patient consent Obtained.
Competing interests SB, RS, MBB, RD, AG, MH, VH, JM, NN and PM have no conflicts of interest to declare; LC reports being involved in clinical trials with Gilead, United Therapeutics and Pfizer; MEC reports serving on the advisory board meeting on PAH and rheumatology by Gilead; CD has received research grants from Aspreva and Gilead; AF reports being a speaker at Actelion and Gilead, serving as a consultant, advisory board member at Actelion; LH reports research support from Actelion and Medimmune; MDM reports consulting, speakers bureaux and/or received grant/research support from Actelion, Gilead, United Therapeutics and Novartis; VMcL reports receiving research grants from Actelion, Novartis, United Therapeutics, acting as a consultant for Actelion, BMS, Mondo Biotech, Gilead, United Therapeutics and as a speaker's bureau for Actelion, Gilead and United Therapeutics; DEF reports honoraria, speaking, receiving research grants, and serving on the advisory board of Actelion and Gilead, grants and advisory board of Pfizer; VS reports research grants from United Therapeutics, Pfizer, Gilead, Actelion, Bristol Myers Squibb and Sibley foundation, acting as a consultant to United Therapeutics and Gilead, and as a speaker at Gilead and Actelion; DK reports serving on the advisory board of Actelion and Pfizer and received grants from Actelion and Gilead.
Provenance and peer review Not commissioned; externally peer reviewed.
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