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Meta-Analysis
. 2019 Apr 3;21(1):86.
doi: 10.1186/s13075-019-1867-1.

Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature

M R Pokeerbux  1   2   3   4 , J Giovannelli  1   2   3   4 , L Dauchet  5 , L Mouthon  6 , C Agard  7 , J C Lega  8   9 , Y Allanore  10 , P Jego  11 , B Bienvenu  12 , S Berthier  13 , A Mekinian  14 , E Hachulla  1   2   3   4 , D Launay  15   16   17   18
Affiliations
Meta-Analysis

Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature

M R Pokeerbux et al. Arthritis Res Ther. .

Abstract

Background: 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.

Methods: A 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.

Results: A 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.

Conclusions: Overall, 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.

Keywords: Meta-analysis; Prognosis factors; Survival; Systemic sclerosis.

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Conflict of interest statement

Ethics approval and consent to participate

This study was authorized by the French Ministry of Research. The authorization number is 13145. To issue such authorization, the Ministry of Research has sought the advice of an independent ethics committee, namely the “Comité consultatif sur le traitement de l’information en matière de recherche (CCTIRS),” which voted positively. French legislation on non-interventional studies requires collecting the non-opposition of patients but does not require written consent. As such, non-opposition was obtained from each patient included in the study for the use of their de-identified medical record data.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Kaplan-Meier survival curves from diagnosis for lcSSc and dcSSc patients in the French cohort
Fig. 2
Fig. 2
Flow chart showing search strategy to identify studies in the meta-analysis
See this image and copyright information in PMC

References

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