Please use this identifier to cite or link to this item:
https://hdl.handle.net/2440/139960
Citations | ||
Scopus | Web of Science® | Altmetric |
---|---|---|
?
|
?
|
Type: | Journal article |
Title: | What have multicentre registries across the world taught us about the disease features of systemic sclerosis? |
Author: | Proudman, S.M. Huq, M. Stevens, W. Wilson, M.E. Sahhar, J. Baron, M. Hudson, M. Pope, J. Allanore, Y. Distler, O. Kowal-Bielecka, O. Matucci-Cerinic, M. H L Low, A. Teng, G.G. Law, W.G. Santosa, A. Nikpour, M. Hill, C. Lester, S. Nash, P. et al. |
Citation: | Journal of Scleroderma and Related Disorders, 2017; 2(3):169-182 |
Publisher: | Sage |
Issue Date: | 2017 |
ISSN: | 2397-1983 2397-1991 |
Statement of Responsibility: | Susanna M. Proudman, Molla Huq, Wendy Stevens, Michelle E. Wilson, Joanne Sahhar, Murray Baron, Marie Hudson, Janet Pope, Yannick Allanore, Oliver Distler, Otylia Kowal-Bielecka, Marco Matucci-Cerinic, Andrea H.L. Low, Gim Gee Teng, Weng Giap Law, Amelia Santosa, Mandana Nikpour, Australian Scleroderma Interest Group (ASIG), Canadian Scleroderma Research Group (CSRG), EULAR Scleroderma Trials and Research group (EUSTAR), Singapore Scleroderma Workgroup (SCORE) |
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 wasthe most prevalent(37.6%). More patientsin SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (p<0.001). Patients with dcSSc were more likely to be younger and male (p<0.001) and have shorter disease duration, more calcinosis, tendon friction rubs and synovitis(all p<0.001). Interstitial lung disease (ILD) occurred more frequently in dcSSc but prevalence of pulmonary arterial hypertension (PAH) was similar in both subtypes. More deaths occurred among SCORE patients who had the shortest median survival (p<0.001). The survival of patients with early disease, males and those with dcSSc was shorter than that of patients with prevalent disease, female gender and lcSSc, respectively. SSc-related complications accounted for more than 50% of deaths, with PAH and ILD being the most common. Conclusions: 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. |
Keywords: | Clinical features; Cohort study; Multicentre registries; Survival; Systemic sclerosis |
Rights: | © 2017 Wichtig International |
DOI: | 10.5301/jsrd.5000256 |
Grant ID: | http://purl.org/au-research/grants/nhmrc/1126370 |
Published version: | http://dx.doi.org/10.5301/jsrd.5000256 |
Appears in Collections: | Medicine publications |
Files in This Item:
There are no files associated with this item.
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.