Systemic sclerosis (SSc) is a connective tissue disease, which may lead to pulmonary artery hypertension (PAH). N-terminal pro-brain natriuretic peptide (NT-proBNP) is a biologic marker for the diagnosis and treatment of congestive heart failure. The aim of our study was to investigate the potential role of the plasma NT-proBNP assay in the assessment of functional status and right heart performance in systemic sclerosis-related pulmonary hypertension (SScPAH). Systolic pulmonary artery pressure (sPAP) assessed by echocardiography, six-minute walk test (SMWT) and plasma NT-proBNP levels were recorded from 45 SSc patients. Mean value of NT-proBNP for SSc patients with PAH (n=14) was 691.7+/-325.7 fmol/L compared to 417.4+/-167.1 fmol/L for patients without PAH (n=31) (p=0.0007). In SSc patients we found a statistically significant correlation between NT-proBNP values and sPAP (r=0.32, p=0.03). Amongst SScPAH patients, NT-proBNP values were significantly correlated with sPAP (r=0.73, p=0.003) and inversely correlated with the SMWT (r=-0.60, p=0.02). These results suggest NT-proBNP as a useful additional biological tool in the evaluation and management of SScPAH patients.