Pooled SMR was 3

Pooled SMR was 3.45 (95%CI 3.03C3.94). 2016. A systematic review of the literature was carried out in Mouse monoclonal to Cytokeratin 17 MEDLINE and EMBASE up to July 2017. Meta-analysis was performed using all available data on SMR and risk ratios of prognosis factors. Results A total of 625 individuals PF-04620110 (493 females, 446 lcSSc) were included. During the study period, 104 deaths (16.6%) were recorded and 133 individuals were lost to follow-up. Overall survival rates at 1, 3, 5, and 10?years from analysis were 98.0%, 92.5%, 85.9%, and 71.7% respectively in the People from france cohort. Overall SMR was 5.73 (95% CI 4.68C6.94). Age at analysis ?60?years, diffuse cutaneous SSc, scleroderma renal problems, dyspnea, 6-min going for walks range (6MWD), forced vital capacity ?70%, diffusing capacity of the lungs for carbon monoxide ?70%, PF-04620110 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 individuals) were included in the SMR meta-analysis and 36 studies (26,187 individuals) in the prognosis element analysis. Pooled SMR was 3.45 (95%CI 3.03C3.94). Age at disease onset, male sex, African source, 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. Conclusions Overall, our study highlights a high mortality rate in SSc individuals and confirms previously explained prognosis factors related to pores and skin extension and organ involvement while identifying additional prognosis factors such as autoantibody status, telangiectasia, 6MWD, and valvular disease. Electronic supplementary material The online version of this article (10.1186/s13075-019-1867-1) contains supplementary material, which is available to authorized users. test or Wilcoxon test in case of non-normality for quantitative variables and Fishers precise test for qualitative variables. Survival was estimated from analysis using the Kaplan-Meier method. Prognosis factors were assessed by Cox regression analysis in the non-adjusted analysis and subsequently modified for age, sex, and SSc subtype. The assumption that risk ratios were constant over time was verified. SMR was determined as the percentage of observed death in the cohort to the number of death of the French age/sex-matched human population in 2014. We determined weighted pooled summary estimations of SMR and HR of prognosis factors. For each meta-analysis, we used the DerSimonian and Laird method. Accordingly, studies were considered to be a random sample from a human population of studies. Heterogeneity was assessed using an ideals less than PF-04620110 0.05 were considered significant. Results French cohort study Baseline characteristicsA total of 625 individuals (493 females, 446 lcSSc) were included. Mean age at disease onset was 52.7??14.9?years. The median disease duration from disease onset was 0.8 (IQR 2.2) years. Median follow-up time was 4.4 (IQR 5.3) years. The baseline characteristics are demonstrated in Table?1. Table 1 Demographics and medical characteristics of 625 individuals with SSc at baseline (for dcSSc)(%) for qualitative variables and imply??SD or median [IQR] for quantitative variables quantity of individuals with available data, limited cutaneous systemic sclerosis, modified Rodnan score, glomerular filtration rate, atrioventricular block, package branch block, remaining ventricular ejection portion, pulmonary hypertension, pulmonary arterial hypertension, echocardiography, ideal heart catheterization, 6-min going for walks range, systolic pulmonary arterial pressure, total lung capacity, forced vital capacity, diffusing capacity of the lungs for carbon monoxide, C reactive protein, body mass index, anti-centromere antibodies, antiphospholipid antibodies Survival and standardized mortality percentage During the study period, PF-04620110 104 deaths (16.6%) were recorded and 133 individuals were lost to follow-up. Overall survival rates at 1, 3, 5, 10, and 15?years from analysis were 98.0% (95% CI 96.9C99.1%), 92.5% (90.4C94.7%), 85.9% (82.8C89.1%), 71.7% (66.3C77.5%), and 53% (33.8C83.4%) respectively. Survival rates for the diffuse and limited cutaneous subtypes are demonstrated in Fig.?1 and in Additional?file?1:.