@article {Kovacs1601708, author = {Gabor Kovacs and Alexander Avian and Nora Wutte and Franz Hafner and Florentine Moazedi-F{\"u}rst and Sonja Kielhauser and Elisabeth Aberer and Marianne Brodmann and Winfried Graninger and Vasile Foris and Andrea Olschewski and Horst Olschewski}, title = {Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study}, volume = {50}, number = {1}, elocation-id = {1601708}, year = {2017}, doi = {10.1183/13993003.01708-2016}, publisher = {European Respiratory Society}, abstract = {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}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/50/1/1601708}, eprint = {https://erj.ersjournals.com/content/50/1/1601708.full.pdf}, journal = {European Respiratory Journal} }