Objectives: The authors aimed to evaluate the role of N-terminal proANP (NT-proANP) and of NT-proBNP circulating levels as predictive markers of death due to systemic sclerosis (SSc).Methods: The authors retrospectively enrolled 51 SSc patients. At baseline, NT-proBNP and NT-proANP circulating levels and clinical features were collected. Date and causes of death were extracted during a 6-year follow-up.Results: 13 SSc patients (23.2%) died for SSc complications (9 for interstitial lung disease and 4 for pulmonary arterial hypertension). The median NT-proBNP plasma level did not significantly differ (p > 0.05) in SSc patients died or alive [645 (448-1026) fmol/ml vs 592 (409-789) fmol/ml]. The median NT-proANP plasma level was significantly (p < 0.01) higher in SSc died than in SSc patients alive [4000 (2100-6722) fmol/ml vs 1640 (1381-2721) fmol/ml]. The Kaplan-Meier analysis revealed that SSc patients with increased NT-proANP level had increased mortality (p < 0.05). In the multivariate analysis, DLco [HR 0.966 (0.934-0.999), p < 0.05] and NT-proANP level [HR 1 (1-1), p < 0.05] were predictive markers of death due to SSc.Conclusions: NT-proANP plasma level is a predictive marker of death due to SSc.