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.
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.
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%) (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.
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.
J scleroderma relat disord 2017; 2(3): 169 - 182
Article Type: ORIGINAL RESEARCH ARTICLE
AuthorsSusanna 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)
- • Accepted on 15/08/2017
- • Available online on 28/09/2017
- • Published in print on 05/10/2017
This article is available as full text PDF.
- Proudman, Susanna M. [PubMed] [Google Scholar] 1, 2, * Corresponding Author (firstname.lastname@example.org)
- Huq, Molla [PubMed] [Google Scholar] 3, 4
- Stevens, Wendy [PubMed] [Google Scholar] 3
- Wilson, Michelle E. [PubMed] [Google Scholar] 3
- Sahhar, Joanne [PubMed] [Google Scholar] 5, 6
- Baron, Murray [PubMed] [Google Scholar] 7
- Hudson, Marie [PubMed] [Google Scholar] 7
- Pope, Janet [PubMed] [Google Scholar] 8
- Allanore, Yannick [PubMed] [Google Scholar] 9
- Distler, Oliver [PubMed] [Google Scholar] 10
- Kowal-Bielecka, Otylia [PubMed] [Google Scholar] 11
- Matucci-Cerinic, Marco [PubMed] [Google Scholar] 12
- Low, Andrea H.L. [PubMed] [Google Scholar] 13
- Teng, Gim Gee [PubMed] [Google Scholar] 14
- Law, Weng Giap [PubMed] [Google Scholar] 15
- Santosa, Amelia [PubMed] [Google Scholar] 13
- Nikpour, Mandana [PubMed] [Google Scholar] 3, 4
- Australian Scleroderma Interest Group (ASIG) *
- Canadian Scleroderma Research Group (CSRG) *
- EULAR Scleroderma Trials and Research group (EUSTAR) *
- Singapore Scleroderma Workgroup (SCORE) *
Rheumatology Unit, Royal Adelaide Hospital, North Terrace, Adelaide - Australia
Discipline of Medicine, University of Adelaide, Adelaide - Australia
Department of Rheumatology, St. Vincent’s Hospital Melbourne, Melbourne - Australia
Department of Medicine, The University of Melbourne at St. Vincent’s Hospital, Melbourne - Australia
Department of Rheumatology, Monash Health, Clayton - Australia
Department of Medicine, Monash University, Clayton - Australia
Department of Rheumatology, Lady Davis Institute for Medical Research and Jewish General Hospital, Montreal - Canada
Schulich School of Medicine, Western University, London and St. Joseph’s Health Care, London, Ontario - Canada
Paris Descartes University, Cochin Hospital, Paris - France
Department of Rheumatology, University Hospital, Zurich - Switzerland
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok - Poland
Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Florence - Italy
Department of Rheumatology and Immunology, Singapore General Hospital and Duke-National University of Singapore - Singapore
Division of Rheumatology, University Medicine Cluster, National University Health System, and Yong Loo Lin School of Medicine, National University of Singapore - Singapore
Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital - Singapore
The complete list of investigators in ASIG, CSRG, EUSTAR and SCORE is available in the Acknowledgement section.