TY - JOUR T1 - Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.01708-2016 VL - 50 IS - 1 SP - 1601708 AU - Gabor Kovacs AU - Alexander Avian AU - Nora Wutte AU - Franz Hafner AU - Florentine Moazedi-Fürst AU - Sonja Kielhauser AU - Elisabeth Aberer AU - Marianne Brodmann AU - Winfried Graninger AU - Vasile Foris AU - Andrea Olschewski AU - Horst Olschewski Y1 - 2017/07/01 UR - http://erj.ersjournals.com/content/50/1/1601708.abstract N2 - Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.Scleroderma patients without pulmonary hypertension develop mild deterioration in pulmonary exercise haemodynamics http://ow.ly/hva930aEW4E ER -