SynthesisArthritis research & therapy2019
Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature.
Synthesis in Arthritis research & therapy, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 93 papers, 1 of them a synthesis that pooled it.
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Who cites it
93 citing papers in PubMed, 1 synthesis or guideline pooled it, 210 citations in OpenAlex.
- ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease.The European respiratory journal · 2026Guideline
- Systemic Autoimmune Rheumatic Disease-Associated Interstitial Lung Disease: A Pulmonary Perspective on Recent Guidelines.Advances in therapy · 2026Review
- Cardiac involvement in systemic sclerosis: mechanisms, manifestations and management.Rheumatology (Oxford, England) · 2026Review
- Assessment of Skin in Patients With Systemic Sclerosis Using High-Frequency Ultrasound and Shear Wave Elastography: A Comparative Study With Histology, Molecular, and Clinical Parameters.Arthritis care & research · 2026Article
- Transcatheter Aortic Valve Replacement in Systemic Sclerosis: A Case Series and Review of Short- and Long-Term Outcomes.Mediterranean journal of rheumatology · 2026Article
- Interstitial lung disease in systemic sclerosis-a retrospective cross-sectional study in Taiwan.Clinical rheumatology · 2026Article
- Serum CCL18 May Reflect Multiorgan Involvement with Poor Outcome in Systemic Sclerosis.Biomolecules · 2026Article
- Biomarker-based clustering identifies distinct pulmonary function trajectories in early systemic sclerosis.Frontiers in immunology · 2026Observational
- Clinical characteristics and treatment patterns of systemic sclerosis patients in China: a single-center retrospective study.Clinical and experimental medicine · 2025Observational
- Systemic sclerosis: bridging clinical and molecular insights: results from the PRECISESADS study.Journal of translational medicine · 2025Article
- Sclerosing diseases of the skin.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2025Review
- [Journal of nuclear medicine : official publication, Society of Nuclear Medicine · 2025Article
- A comparative analysis of risk stratification tools in SSc-associated pulmonary arterial hypertension: a EUSTAR analysis.Rheumatology (Oxford, England) · 2025Article
- Cardiac Involvement and Heart Failure Staging in Patients with Systemic Sclerosis Without Pulmonary Arterial Hypertension.Journal of clinical medicine · 2025Article
- Characterization of Circulating Vesicles of Complicated and Uncomplicated Systemic Sclerosis Patients and Their Role in Vascular Dysfunction.International journal of molecular sciences · 2025Article
- A scoping review of the epidemiology of systemic sclerosis and its organ manifestations: 2018-2024.Current opinion in rheumatology · 2025Article
- Natural killer cells are decreased in systemic sclerosis and have diagnostic value for pulmonary arterial hypertension incorporation.Scientific reports · 2025Article
- The pathogenesis, diagnostic utility and clinical relevance of cutaneous telangiectasia in systemic sclerosis.Seminars in arthritis and rheumatism · 2025Review
- Increased risk of acute myocardial infarction in Swedish patients with systemic sclerosis: a population-based study.Rheumatology advances in practice · 2025Article
- Esomeprazole inhibits proliferation of scleroderma fibroblasts via cell cycle regulation.Frontiers in pharmacology · 2025Article
33 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
13 authors at 9 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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.
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