SAT0273 PREDICTIVE VALUE OF THE REVISED EUROPEAN SCLERODERMA TRIALS AND RESEARCH GROUP ACTIVITY INDEX (EUSTAR-AI)
Authors: Serena Fasano, Veronica Giacco, Antonella Riccardi, Valentina Messiniti, Alessandra Vacca, Oliver Distler, Otylia Kowalka Bielecka, Yannick Allanore, Gabriele Valentini
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Disease activity as measured by the European Scleroderma Study Group Activity Index (EScSG-AI) (1) has been recently found to predict the development of damage over time in an early systemic sclerosis (SSc) cohort (2). The European Scleroderma Trials and Research Group Task Force for the Development of Revised Activity Criteria for SSc recently succeeded in identifying a preliminarily revised activity index (EUSTAR-AI) that had a greater construct validity than the EScSG-AI i.e. performing better in identifying patients with active disease (3). <h3>Objectives</h3> To assess in patients with SSc the predictive value of the EUSTAR-AI for disease severity accrual. <h3>Methods</h3> SSc patients from the EUSTAR database with a disease duration from the onset of the first non-Raynaud sign/symptom≤5 years were first extracted. Patients were considered for the study if they presented the following features: a)availability of items included in the EUSTAR-AI, in the EScSG-AI and in the Medsger severity scale at baseline and yearly for 2 consecutive years; b)availability of vital status at the last observation; c) availability of other disease features known to predict disease progression (male sex, clinical and serological subset). To capture the disease activity variations over time, we calculated the EUSTAR-AI and EScSG-AI adjusted mean (area under the curve over time divided by time interval). Disease progression was based on the Medsger severity scale and included accrual of a Δ≥1 summed severity score and of a Δ≥1 severity in each organ systems at the 2 year follow-up visit compared with the initial visit. To explore specific determinants of disease progression, logistic regression analysis was carried out. <h3>Results</h3> A total of 549 patients satisfied the entry criteria. At univariate logistic regression analysis (among sex, age, clinical and serological subset, EScSG-AI and EUSTAR-AI adjusted mean and baseline severity score), EScSG-AI adjusted mean (OR 1.41 95%CI 1.20-1.67), antiScl70 antibody positivity (OR 1.72 95%CI 1.20-2.47), diffuse subset (OR:1.46 95%CI 1.01-2.10) and EUSTAR-AI adjusted mean (OR 1.41 95%CI 1.23-1.61) predicted disease severity accrual. Multivariate analysis revealed that the EUSTAR-AI adjusted mean was the best predictor of disease progression (Table1). Moreover, at multivariate analysis the EUSTAR-AI adjusted mean also predicted severity accrual of lung (OR 1.32), heart (OR 1.40), skin (OR 1.48) and peripheral vascular disease (OR 1.45). <h3>Conclusion</h3> The adjusted mean EUSTAR-AI has a distinct predictive value for disease progression and development of severe organ involvement in SSc and works better than EScSG-AI. <h3>References</h3> [1] Valentini G, et al. Ann Rheum Dis 2001;60:592-8. [2] Nevskaya T, et al. Rheumatology (Oxford). 2017;56:1111-1122. [3] Valentini G, et al. Ann Rheum Dis. 2017;76:270-276. <h3>Disclosure of Interests</h3> Serena Fasano: None declared, Veronica Giacco: None declared, Antonella Riccardi: None declared, Valentina Messiniti: None declared, ALESSANDRA VACCA: None declared, Oliver Distler Grant/research support from: Prof. Distler received research funding from Actelion, Bayer, Boehringer Ingelheim and Mitsubishi Tanabe to investigate potential treatments of scleroderma and its complications, Consultant for: Prof. Distler has/had consultancy relationship within the last 3 years with Actelion, AnaMar, Bayer, Boehringer Ingelheim, ChemomAb, espeRare foundation, Genentech/Roche, GSK, Inventiva, Italfarmaco, iQvia, Lilly, medac, MedImmune, Mitsubishi Tanabe Pharma, Pharmacyclics, Novartis, Pfizer, Sanofi, Serodapharm and UCB in the area of potential treatments of scleroderma and its complications. In addition, he had/has consultancy relationship within the last 3 years with A. Menarini, Amgen, Abbvie, GSK, Mepha, MSD, Pfizer and UCB in the field of arthritides and related disorders, Otylia Kowalka Bielecka Consultant for: “OK-B received consulting fees or other remuneration from Bayer, Boehringer Ingelheim, Inventiva, Medac, Novartis and Roche”, Yannick Allanore Grant/research support from: Inventiva, F Hoffman La-Roche, Sanofi, BMS, Pfizer, Consultant for: Actelion, Bayer, BMS, Boehringer, Roche, Sanofi, Gabriele Valentini Grant/research support from: MSD, Phizer, Consultant for: MSD, Phizer, biogen, Speakers bureau: MSD, amgen, biogen, lilly, sanofi, phizer