Several studies have assessed prognosis factors in systemic sclerosis (SSc), but only few have investigated extensive clinical and laboratory factors in a large, incident and well phenotyped population. The aim of the present study was to describe survival, standardized mortality ratio (SMR) and prognostic factors in a well-phenotyped and multicenter French cohort of incident SSc patients. A systematic review and meta-analysis of cohort studies to assess SMR and prognostic factors in SSc was then performed.

Survival, SMR and prognosis factors were assessed in a cohort of SSc patients followed in five referral centers in France. A systematic review of the literature was carried out in Pubmed up to July 2017. Cohort studies of unselected SSc patients reporting SMR and prognostic factors were included in the analysis. We calculated the pooled summary estimates of SMR and hazard ratios (HR) of prognosis factors.

Out of the 42 referral centers included in the French SSc registry, the present analysis was restricted to 5 centers to ensure the lowest rate of missing data. 625 SSc patients [28.6% of diffuse SSc (dcSSc)] with recent diagnosis were followed from January 2000 to December 2013. Mean age at disease onset was 50.6±14.5 years and mean follow-up was 5.2±3.6years. In total, 104 deaths were recorded. Overall survival rates at 1, 3, 5 and 10 years from diagnosis were 98.0%, 92.5%, 85.9% and 71.7% respectively. Overall SMR was 5.73 [95% confidence interval (CI) 4.68–6.94]. Age at diagnosis >60years, dcSSc, scleroderma renal crisis, severe dyspnoea, FVC <70%, DLCO <70%, pulmonary hypertension (PH), telangiectases, valvular disease, malignancy, anemia, CRP >8mg/L were associated with a poorer survival. Eighteen articles were included in the SMR meta-analysis, representing a total population of 11,329 patients and 33 studies were included in the prognosis factors meta-analysis, representing a total of 23,520 patients. Pooled SMR was 3.45 [IC95% 3.03–3.94]. Age at disease onset, age at diagnosis, male sex, black race, dcSSc subtype, anti-Scl70 autoantibodies, cardiac and renal involvements, interstitial lung disease, PH and malignancy were significantly associated with a worse prognosis. Presence of ACA autoantibodies was associated with a better survival.

Our study confirms a high mortality rate in SSc and previously described prognosis factors related to skin extension, autoantibody status and organ involvement while reporting new association such as telangiectases and valvular disease.