Antoine Huart, Leonardo Astudillo, Sébastien De Almeida Chaves, Grégoire Prévot, Daniel Adoue, David Ribes, Laurent Alric, T. Porel, Laurent Sailler, Mickael Mounié, Grégory Pugnet, Francis Gaches, Martin Michaud, and Olivier Lairez
Background Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature. Objective To determine the mortality and its predictors in a long-term follow-up of a bi-centric cohort of SSc patients. Methods A retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital. Standardized Mortality Ratio (SMR), mortality at 1, 3, 5, 10, and 15 years of disease and causes of death were described. Predictors of mortality using Cox regression were assessed. Results Three hundred seventy-five patients were included: 63 with diffuse cutaneous SSc, 279 with limited cutaneous SSc, and 33 with sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46–1.97). The overall survival rates were 97.6% at 1 year, 93.4% at 3 years, 87.1% at 5 years, 77.9% at 10 years, and 61.3% at 15 years. Sixty-nine deaths were recorded. 46.4% were SSc related deaths secondary to interstitial lung disease (ILD) (34.4%), pulmonary hypertension (31.2%), and digestive tract involvement (18.8%). 53.6% were non-related to SSc: cardiovascular disorders (37.8%) and various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors were identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p=0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; p=0.0174), cardiac involvement (HR=2.86; p=0.0012), and the fact of being male (HR=3.25; p=0.0004). Conclusion Long-term data confirmed high mortality of SSc. Male sex, DLCO 5mg/l were identified as independent predictors of mortality. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-021-02672-y., • Male sex, cardiac involvement, DLCO 5 mg/l are strong predictors of mortality in systemic sclerosis. • This study shows the survival of subtypes and in particular sine scleroderma. • Sine scleroderma subtype has better survival than diffuse or limited cutaneous subtypes. • Non-systemic sclerosis-related deaths are more frequent than systemic sclerosis-related deaths. • Cardiovascular events non-systemic sclerosis-related are the main deaths. Supplementary Information The online version contains supplementary material available at 10.1186/s13075-021-02672-y.