Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database

Abstract

Objective: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years’ duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets.

Method: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud’s SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud’s phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31–59, and ≥ 60 years. We performed descriptive and bivariate analyses.

Results: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud’s.

Conclusion: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice.

Acknowledgements

The authors and all EUSTAR centres are grateful for the support of the European League Against Rheumatism until 2013. We also would like to thank all EUSTAR collaborators who are listed in the online additional file 1, and all the EUSTAR collaborating patients.

This work was partially supported by FIS grant 11/01506 from the Spanish Ministry of Economy and Competitiveness (Instituto de Salud Carlos III, Spain), and by an unrestricted grant from Merck Sharp and Dome to the Research Institute of the Hospital 12 de Octubre, Madrid (2014 0062) (Instituto de Investigación del Hospital 12 de Octubre, Madrid) (2014 0062).

Disclosure statement

EUSTAR was supported by the European League Against Rheumatism until 2013. O. Distler has consulted for, or has received research funding from, 4D Science, Actelion, Active Biotec, Bayer-Schering, Biogen, Biovitrium, BMS, Boehringer Ingelheim Pharma, EpiPharm, Ergonex, GSK, Inventiva, Medac, Novartis, Pfizer, Pharmacyclics, Roche/Genentech, Sanofi/Genzyme, Serodapharm, Sinoxa, and United BioSource Corporation. S. Vettori has received speaking fees from Pfizer, Abbvie, and Bristol-Myers Squibb, consultant fees from Thermo Fisher, and educational support from Pfizer and Roche. E. Loza and L. Carmona have received research funding from Abbvie, MSD, Pfizer, Roche, Novartis, BMS, and UCB. JA has received funding to attend courses and congresses from Pfizer, Abbvie, Gebro, and Menarini, speaking fees from Abbvie, GSK, Roche, and UCB, and consultant fees from UCB and GSK. All other authors declare no conflicts of interest.

Supporting Information

Additional Supporting Information may be found in the online version of this article.

Supplementary file: EUSTAR co-authors

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Additional information

Funding

This work was supported by the Merck Sharp and Dome;Instituto de Salud Carlos III, Spain;