Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality

• DOI: 10.1186/s13075-021-02672-y
• Publication Date: 2021-12-07T12:03:26Z
• Authors: Sébastien De Alme...; Tiphaine Porel; Mickael Mounié; Laurent Alric; Léonardo Astudillo; Antoine Huart; Olivier Lairez; Martin Michaud; Grégoire Prévot; David Ribes; Laurent Sailler; Francis Gaches; Daniel Adoue; Gregory Pugnet
• Keywords: DLCO; Scleroderma (fungus)

Abstract Background Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature. Objective To determine and its predictors long-term follow-up bi-centric cohort SSc patients. Methods A retrospective observational study by systematically analyzing medical records patients diagnosed Toulouse University Hospital Ducuing Hospital. Standardized Mortality Ratio (SMR), at 1, 3, 5, 10, 15 years disease causes death were described. Predictors using Cox regression assessed. Results Three hundred seventy-five included: 63 diffuse cutaneous SSc, 279 limited 33 sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46–1.97). overall survival 97.6% 1 year, 93.4% 3 years, 87.1% 5 77.9% 10 61.3% years. Sixty-nine deaths recorded. 46.4% related secondary to interstitial lung (ILD) (34.4%), pulmonary hypertension (31.2%), digestive tract involvement (18.8%). 53.6% non-related SSc: cardiovascular disorders (37.8%) various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p =0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; =0.0174), cardiac (HR=2.86; =0.0012), fact being male (HR=3.25; =0.0004). Conclusion Long-term data confirmed high SSc. Male sex, DLCO <70%, involvement, CRP> 5mg/l identified as mortality.