Background. Systemic sclerosis (SSc) is a complex chronic autoimmune disease, with an unpredictable evolution and high morbidity and mortality rates. Objective. Evaluation of long-term survival and identification of prognostic factors in patients with systemic sclerosis. Methods. All patients with SSc of the EUSTAR100 center, having at least one visit between 2004 and 2016, were included. Data were analyzed for survival, cause of death, as well as for the following events defining disease worsening: increase in modified Rodnan score (mRSS) with at least 25% and 5 points (compared to baseline visit), decrease with at least 10% (compared to baseline) of predicted forced vital capacity (FVC) and predicted diffusing capacity of the lungs for carbon monoxide (DLCO), and presence of new digital ulcers (DUs). Logistic regression (LR), Cox proportional hazards regression and Kaplan-Meier survival curves were used in univariate and multivariate analysis to study survival and identify prognostic factors. Results. 137 patients were included in the study (89.1% females, mean age ± SD 56.7 ± 12.6 years, disease duration 9.7 ± 7.1 years), with a follow-up duration of up to 19 years. 96 patients
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