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What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

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%) (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.

J scleroderma relat disord 2017; 2(3): 169 - 182

Article Type: ORIGINAL RESEARCH ARTICLE

DOI:10.5301/jsrd.5000256

Authors

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)

Article History

Disclosures

Financial support: This work was supported by Scleroderma Australia, Arthritis Australia, Actelion Australia, Bayer, CSL Biotherapies, GlaxoSmithKline Australia and Pfizer. Dr. Nikpour holds a National Health and Medical Research Council of Australia Career Development Fellowship (APP1126370). The Canadian Scleroderma Research Group (CSRG) is funded by the Canadian Institutes of Health Research (CIHR) (grant #FRN 83518), the Scleroderma Society of Canada and its provincial Chapters, Scleroderma Society of Ontario, Scleroderma Society of Saskatchewan, Sclérodermie Québec, Cure Scleroderma Foundation, INOVA Diagnostics Inc. (San Diego, CA), Dr. Fooke Laboratorien GmbH (Neuss, Germany), Euroimmun (Lubeck, Germany), Mikrogen GmbH (Neuried, Germany), Fonds de la recherche en santé du Québec (FRSQ), the Canadian Arthritis Network (CAN), and the Lady Davis Institute of Medical Research of the Jewish General Hospital, Montreal, QC. The CSRG has also received educational grants from Pfizer and Actelion pharmaceuticals. EUSTAR: Oliver Distler has/had consultancy relationship and/or has received research funding from 4 D Science, Actelion, Active Biotec, Bayer, Biogen Idec, Boehringer Ingelheim Pharma, BMS, ChemomAb, EpiPharm, Ergonex, espeRare foundation, GSK, Roche-Genentech, Inventiva, Lilly, medac, MedImmune, Mitsubishi Tanabe, Pharmacyclics, Pfizer, Sanofi, Serodapharm, Sinoxa and UCB in the area of potential treatments of scleroderma and its complications. He has a patent on mir-29 for the treatment of systemic sclerosis licensed. The SCORE cohort was supported by the Singapore Clinical Research Institute multi-centre research electronic data capture, with funding support from the National Medical Research Council. Dr. Low holds a NMRC transition award (NMRC/TA/013/2012) and clinician scientist individual research grant (CIRG14nov021). The SCORE cohort has received patient support grants from Actelion pharmaceuticals.
Conflict of interest: One or more authors have financial interest related to this study to disclose. Dr. Nikpour holds a National Health and Medical Research Council of Australia Career Development Fellowship (APP1126370). The Canadian Scleroderma Research Group (CSRG) is funded by the Canadian Institutes of Health Research (CIHR) (grant #FRN 83518) Dr. Low holds a NMRC transition award (NMRC/TA/013/2012) and clinician scientist individual research grant (CIRG14nov021).

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Authors

Affiliations

  • 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.

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