Abstract
BackgroundData on survival and prognosis factors in incident cohorts are scarce in systemic sclerosis (SStc). To describe survival, standardized mortality ratio (SMR), and prognosis factors in systemic sclerosis (SSc), we analyzed a multicenter French cohort of incident patients and performed a systematic review of the literature and meta-analysis.MethodsA multicenter, French cohort study was conducted between January 1, 2000, and December 31, 2013. Patients were followed-up until July 1, 2016.A systematic review of the literature was carried out in MEDLINE and EMBASE up to July 2017. Meta-analysis was performed using all available data on SMR and hazard ratios of prognosis factors.ResultsA total of 625 patients (493 females, 446 lcSSc) were included. During the study period, 104 deaths (16.6%) were recorded and 133 patients were lost to follow-up. Overall survival rates at 1, 3, 5, and 10 years from diagnosis were 98.0%, 92.5%, 85.9%, and 71.7% respectively in the French cohort. Overall SMR was 5.73 (95% CI 4.68–6.94). Age at diagnosis > 60 years, diffuse cutaneous SSc, scleroderma renal crisis, dyspnea, 6-min walking distance (6MWD), forced vital capacity < 70%, diffusing capacity of the lungs for carbon monoxide < 70%, pulmonary hypertension (PH), telangiectasia, valvular disease, malignancy, anemia, and CRP > 8 mg/l were associated with a poorer survival after adjustment.Eighteen studies (11,719 patients) were included in the SMR meta-analysis and 36 studies (26,187 patients) in the prognosis factor analysis. Pooled SMR was 3.45 (95%CI 3.03–3.94). Age at disease onset, male sex, African origin, diffuse cutaneous SSc, anti-Scl70 antibodies, cardiac and renal involvement, interstitial lung disease, PH, and malignancy were significantly associated with a worse prognosis. Anti-centromere antibodies were associated with a better survival.ConclusionsOverall, our study highlights a high mortality rate in SSc patients and confirms previously described prognosis factors related to skin extension and organ involvement while identifying additional prognosis factors such as autoantibody status, telangiectasia, 6MWD, and valvular disease.
Highlights
Data on survival and prognosis factors in incident cohorts are scarce in systemic sclerosis (SStc)
Survival and standardized mortality ratio During the study period, 104 deaths (16.6%) were recorded and 133 patients were lost to follow-up
The main results of our study are (i) a high risk of mortality in our cohort of incident patients, as shown by a high standardized mortality ratio (SMR) of 5.73; (ii) the identification of age > 60 years, diffuse cutaneous systemic sclerosis (SSc) (dcSSc), dyspnea, pulmonary hypertension (PH), low forced vital capacity (FVC), low DLCO, kidney involvement, valvular disease, cancer, telangiectasia, shorter 6-min walking distance (6MWD), anemia, and inflammation as prognosis factors in our cohort; (iii) a high pooled SMR of 3.45 in the meta-analysis of the literature, including our new cohort; and (iv) the additional identification of male sex, African origin, Interstitial lung disease (ILD), cardiac involvement, and anti-Scl-70 antibodies as associated with worse prognosis in our meta-analysis, while Anti-centromere antibodies (ACA) were associated with better prognosis
Summary
Data on survival and prognosis factors in incident cohorts are scarce in systemic sclerosis (SStc). To describe survival, standardized mortality ratio (SMR), and prognosis factors in systemic sclerosis (SSc), we analyzed a multicenter French cohort of incident patients and performed a systematic review of the literature and meta-analysis. Systemic sclerosis (SSc) is an autoimmune disease, characterized by microvascular damage, dysregulation of both innate and adaptative immunity, and fibrosis of multiple organs. Two recent meta-analyses have reported that standardized mortality ratio (SMR) was stable over time [5, 6]. Data on survival in incident cohorts are scarce in SSc [7,8,9,10,11,12]
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