PT - JOURNAL ARTICLE AU - Sangmee Bae AU - Rajeev Saggar AU - Marcy B Bolster AU - Lorinda Chung AU - Mary Ellen Csuka AU - Chris Derk AU - Robyn Domsic AU - Aryeh Fischer AU - Tracy Frech AU - Avram Goldberg AU - Monique Hinchcliff AU - Vivien Hsu AU - Laura Hummers AU - Elena Schiopu AU - Maureen D Mayes AU - Vallerie McLaughlin AU - Jerry Molitor AU - Nausheen Naz AU - Daniel E Furst AU - Paul Maranian AU - Virginia Steen AU - Dinesh Khanna TI - Baseline characteristics and follow-up in patients with normal haemodynamics versus borderline mean pulmonary arterial pressure in systemic sclerosis: results from the PHAROS registry AID - 10.1136/annrheumdis-2011-200546 DP - 2012 Aug 01 TA - Annals of the Rheumatic Diseases PG - 1335--1342 VI - 71 IP - 8 4099 - http://ard.bmj.com/content/71/8/1335.short 4100 - http://ard.bmj.com/content/71/8/1335.full SO - Ann Rheum Dis2012 Aug 01; 71 AB - 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.