Patients characteristics at baseline and after 36 months of follow-up. Results are presented as mean ± standard error of the mean. (a) reduction in N-terminal-pro-brain natriuretic peptide (NT-proBNP) levels, (b) an improvement of the skin involvement as assessed by the Modified Rodnan Skin Score (mRSS) was documented, (c) no statistical significant changes in pulmonary artery pressure when comparing baseline and 36 month levels, (d) no patient showed any change in the ejection fraction, (e) forced vital capacity (FVC), (f) forced expiratory volume in one second (FEV1), (g) mean values of diffusing capacity for carbon monoxide (DLCO).