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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. The study comprised 68 patients with SSc who were followed up for the median time of 99 (96; 107) 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-min walk test, transthoracic echocardiography and biomarker testing, including growth differentiation factor 15 (GDF-15). 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. 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 (Chronic kidney disease, Hypertension, hyperLipidaemia, Diabetes mellitus) 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). 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.

CHLD score, a new score based on traditional risk factor evaluation and long-term cardiovascular outcomes in patients with systemic sclerosis