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Revised European Scleroderma Trials and Research Group Activity Index is the best predictor of short-term severity accrual
  1. Serena Fasano1,
  2. Antonella Riccardi1,
  3. Valentina Messiniti1,
  4. Paola Caramaschi2,
  5. Edoardo Rosato3,
  6. Britta Maurer4,
  7. Vanessa Smith5,
  8. Elise Siegert6,
  9. Ellen De Langhe7,
  10. Valeria Riccieri8,
  11. Paolo Airó9,
  12. Carina Mihai10,
  13. Jerome Avouac11,
  14. Elisabetta Zanatta12,
  15. Ulrich A Walker13,
  16. Florenzo Iannone14,
  17. Paloma García De la Peña Lefebvre15,
  18. Jörg H W Distler16,
  19. Alessandra Vacca17,
  20. Oliver Distler18,
  21. Otylia Kowal-Bielecka19,
  22. Yannick Allanore11,
  23. Gabriele Valentini1
  1. 1 Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy
  2. 2 Department of Rheumatology, University of Verona, Verona, Italy
  3. 3 Dipartimento di Medicina Traslazionale e di Precisione, Sapienza University of Rome, Roma, Italy
  4. 4 Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  5. 5 Department of Rheumatology, University Hospital Ghent, Gent, Belgium
  6. 6 Department of Rheumatology, Charit University Hospital, Berlin, Germany
  7. 7 Department of Development and Regeneration, Laboratory of Tissue Homeostasis and Disease, Skeletal Biology and Engineering Research Center, KU Leuven, Leuven, Belgium
  8. 8 Clinical Medicine and Therapy, Sapienza University of Rome, Rome, Italy
  9. 9 Rheumatology and Clinical Immunology Department, Spedali Civili di Brescia, Brescia, Italy
  10. 10 Department of Rheumatology, Carol Davila University of Medicine and Pharmacy, Bucarest, Romania
  11. 11 Department of Rheumatology, Paris Descartes University, Rheumatology A and INSER U1016, Cochin Hospital, Paris, France
  12. 12 Dipartimento di Medicina, DIMED, Universita degli Studi di Padova, Padova, Italy
  13. 13 Department of Rheumatology, Basel University, Basel, Switzerland
  14. 14 Department of Rheumatology, University of Bari, Bari, Italy
  15. 15 Department of Rheumatology, Ramon y Cajal University Hospital, Madrid, Spain
  16. 16 Department of Internal Medicine III, University of Erlangen, Erlangen, Germany
  17. 17 Chair and Rheumatology Unit, University Clinic AOU Cagliari, Monserrato, Italy
  18. 18 Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  19. 19 Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
  1. Correspondence to Dr Serena Fasano, Università degli Studi della Campania Luigi Vanvitelli, Napoli 80131, Italy; serena.fasano{at}


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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