Background The European Scleroderma Trials and Research Group (EUSTAR) recently developed a preliminarily revised activity index (AI) that performed better than the European Scleroderma Study Group Activity Index (EScSG-AI) in systemic sclerosis (SSc).
Objective To assess the predictive value for short-term disease severity accrual of the EUSTAR-AI, as compared with those of the EScSG-AI and of known adverse prognostic factors.
Methods Patients with SSc from the EUSTAR database with a disease duration from the onset of the first non-Raynaud sign/symptom ≤5 years and a baseline visit between 2003 and 2014 were first extracted. To capture the disease activity variations over time, EUSTAR-AI and EScSG-AI adjusted means were calculated. The primary outcome was disease progression defined as a Δ≥1 in the Medsger’s severity score and in distinct items at the 2-year follow-up visit. Logistic regression analysis was carried out to identify predictive factors.
Results 549 patients were enrolled. At multivariate analysis, the EUSTAR-AI adjusted mean was the only predictor of any severity accrual and of that of lung and heart, skin and peripheral vascular disease over 2 years.
Conclusion The adjusted mean EUSTAR-AI has the best predictive value for disease progression and development of severe organ involvement over time in SSc.
- systemic sclerosis
- autoimmune diseases
- outcomes research
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Handling editor Josef S Smolen
Contributors GV contributed to the study conception and design, acquisition of data, analysis and design of the study. SF and GV contributed to the data interpretation and analysis. All authors contributed to the acquisition of data; drafting and revising the manuscript and have critically reviewed and approved the final submitted version to be published.
Funding This study was funded by European Scleroderma Trials and Research group (EUSTAR).
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval All contributing EUSTAR centres have obtained approval from their respective local ethics committee for including patients’ data in the EUSTAR database and written informed consent was obtained in those centres, where required by the ethics committee.
Provenance and peer review Not commissioned; externally peer reviewed.
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