OBJECTIVES: Systemic sclerosis-related pulmonary arterial hypertension (SSc PAH) is a major complication of both limited and diffuse systemic sclerosis and leads to substantial morbidity and mortality. Natriuretic peptides (NP) are clinically useful markers of right ventricular dysfunction and pulmonary hypertension. The aim of our review was to examine the evidence for the physiologic, diagnostic, and prognostic role of NP in the context of SSc PAH.METHODS: A Medline search for articles published between January 1999 and December 2008 was conducted using the following keywords: natriuretic peptides, systemic sclerosis or scleroderma, and pulmonary arterial hypertension.RESULTS: In patients with SSc PAH, NP levels increase in proportion to the extent of right ventricular dysfunction and correlate significantly with functional capacity and echocardiographic and hemodynamic parameters. NP may also provide prognostic information beyond conventional risk markers but their use has to be considered against the background of the parameters that may influence their concentration.CONCLUSION: There is growing evidence that NP, along with the traditional assessment modalities such as echocardiography and the 6-minute walking test, may be a suitable marker for SSc PAH in terms of screening, diagnostic evaluation, risk stratification, and response to therapy; this merits prospective evaluation.