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Purpose: Pulmonary arterial hypertension (PAH) is a common complication and established prognostic determinant in systemic sclerosis (SSc). Aim of the study was to verify whether a screening strategy with exercise-Doppler echocardiography (EDE) unmasks the presence of stress PAH.Methods: 73 consecutive female patients (61±12 years) affected by SSc (66% limited) underwent a bicycle exercise test in the semi-supine position on a tilting exercise table with an increase in workload every 2 min by 25 W. Echocardiography examinations were performed simultaneously throughout the exercise with the evaluation of pulmonary artery systolic pressure (PASP) and right ventricular function. Clinical data, N-terminal pro-brain natriuretic peptide (NT-proBNP), and carbon dioxide diffusing capacity (DLCO) were collected.Results: All patients had normal left ventricle ejection fraction and normal left ventricle filling pressures, estimated by E/E' ratio. Mean PASP at resting condition was 31±8 mmHg and the mean increase in PASP during exercise was 20±10 mmHg. Fifty-nine (81%) patients had normal rest PASP (≤36 mmHg); among these patients, 21 (36%) showed an increase during stress over 50 mmHg. The peak systolic velocity of tricuspidal lateral annulus on Doppler tissue imaging was normal at rest in the majority of patients (>0.12 m/sec in 92%), as well as the tricuspid annular plane systolic excursion (>18 mm in 96%), and right ventricle outflow tract acceleration time (>100 msec in 82%); no significant worsening of the above parameters during stress occurred with the exception of acceleration time (128±31 msec at rest vs 95±20 msec at stress, p<0.0001). Multivariate analysis showed significant independent correlations (p<0.05 for all) between peak PASP and the number of years from diagnosis, the presence of Raynaud's phenomenon, rest PASP, and right ventricle diastolic function (E'/A').Conclusions: Rest and stress echocardiography provides useful information in SSc patients; an abnormal increase in PASP at EDE is frequent even in patients with rest PASP within normal limits.