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Introduction Systemic Sclerosis (SSc) is a low prevalence rheumatic disease with a higher mortality rate compared to other autoimmune diseases. The main cause of mortality in SSc patients remains pulmonary involvement.The aim of this work is to analyze the mortality rate and its main cause factors in an SSc cohort from a tertiary hospital.Material and Methods An observational retrospective study of pre-Scleroderma and SSc patients (ACR/EULAR 2013 criteria) followed since 1994 was performed. Clinical and therapeutic data was collected focusing in skin (calcinosis, sclerodactyly and mRSS), vascular (Raynaud’s phenomenon, digital ulcers and lower limbs vasculopathy), respiratory (pulmonary hypertension and ILD), cardiac and gastrointestinal domains (GERD and GAVE). Death cases were classified attending to the main cause of death (SSc or non-SSc related).Results 204 patients (88.24% female) were included, with a mean (SD) age at diagnosis of 51.34 (15.68) years old and a mean disease duration (SD) of 7.28 (6.14) years. The 60.29% of patients were classified as Limited-SSc, 19.12% as Diffuse-SSc and 20.59% as pre-Scleroderma. The overall mRSS at diagnosis (SD) was 4.16 (8.19) points. Alterations in vascular and skin domains were the most prevalent.Eighteen deaths (8.82%) were observed: 2 patients (11.11%) with pre-Scleroderma, 12 (66.67%) with Limited-SSc and 4 (22.22%) within the Diffuse-SSc category. 52.9% with a SSc non-related cause of death (mainly infections), the 47.06% with a SSc related cause of death (cardiac and respiratory) and one patient with an unknown death cause.Performing the multivariable Cox regression analysis several variables associated with mortality risk in SSc patients were identified. The presence of digital ulcers was significantly associated with increased mortality risk (HR = 6.58; p=0.011). Cardiac involvement was also a strong and statistically significant predictor (HR = 13.69; p=0.003). Age at diagnosis remained a significant independent risk factor, with each additional year increasing the risk of death by 15.7% (HR = 1.16; p < 0.001). Female gender showed a robust protective effect (HR = 0.036; p < 0.001), indicating a markedly lower mortality risk compared to males.Other variables including dermatologic, pulmonary and gastrointestinal involvement, SSc subtypes, anti-Scl-70 antibodies, DMARD or vasodilator treatment did not reach statistical significance.Conclusions Global mortality was 8.82%, mainly related to cardiac involvement. Digital ulcers, a higher age at diagnosis and male gender also were associated with a higher mortality rate.