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What have multicentre registries across the world taught us about the disease features of systemic sclerosis?
- Publication Year :
- 2017
-
Abstract
- Introduction The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (pConclusions This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.
- Subjects :
- 0301 basic medicine
medicine.medical_specialty
Pediatrics
Survival
Immunology
Disease
Scleroderma
03 medical and health sciences
0302 clinical medicine
Rheumatology
Internal medicine
Immunology and Allergy
Medicine
Multicentre registrie
030203 arthritis & rheumatology
Clinical features, Cohort study
Multicentre registries
Systemic sclerosis
business.industry
Interstitial lung disease
Autoantibody
Clinical features
medicine.disease
030104 developmental biology
Clinical feature
Cohort
business
Cohort study
Rheumatism
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....349091c6a90ca18ba70a2740348cc11a
- Full Text :
- https://doi.org/10.5301/jsrd.5000256