Abstract Purpose The aim of the study was to assess the predictors of major adverse cardiovascular events (MACE) in patients with systemic sclerosis (SSc) without pulmonary arterial hypertension. Methods The study comprised 68 patients with SSc who were followed up for the median time of 97 (74.5; 105) months. The main exclusion criteria involved tricuspid regurgitation maximal velocity >2.8 m/s and structural heart disease. At baseline the patients underwent clinical assessment of cardiovascular risk factors, 6-minute walk test, transthoracic echocardiography and biomarker testing for fetuin-A, growth differentiation factor 15 (GDF-15) and N-terminal pro-B-type natriuretic peptide. The primary composite endpoint was onset of MACE defined as death, myocardial infarction, myocardial revascularization and hospitalization for heart failure. The follow-up consisted of outpatient visits at 1 year intervals and telephone interview every 6 months. Results The baseline analysis revealed that chronic kidney disease (HR 28.13, 95% CI: 4.84–163.38), lung fibrosis on high resolution computed tomography (HR 4.36, 95% CI: 1.04–18.26) and GDF-15 concentration (unit HR 1.0006, 95% CI: 1.0002–1.0010) were independent predictors of MACE occurrence. CHLD score was formulated which assigned 1 point for the presence of arterial hypertension, hyperlipidaemia, diabetes mellitus and chronic kidney disease. After inclusion of CHLD score in Cox proportional model, it remained the only independent predictor of MACE onset (unit HR per 1 point 3.46; 95% CI: 2.06–5.82, p<0.0001). Receiver operating curve (ROC) analysis (Figure 1) showed that CHLD score accurately predicted MACE (AUC 0.839; 95% CI: 0.745–0.932, p<0.0001) and death (AUC 0.914, 95% CI: 0.849–0.978, p<0.0001) on follow-up. Conclusion Joint assessment of traditional risk factors in the form of CHLD score may serve as a reliable predictor of long-term outcome in patients with SSc without pulmonary arterial hypertension. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland Figure 1