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EULAR Sjögren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren's syndrome
  1. Raphaèle Seror1,2,3,4,5,
  2. Philippe Ravaud1,2,3,
  3. Simon J Bowman6,
  4. Gabriel Baron1,2,3,
  5. Athanasios Tzioufas7,
  6. Elke Theander8,
  7. Jacques-Eric Gottenberg9,
  8. Hendrika Bootsma10,
  9. Xavier Mariette4,5,
  10. Claudio Vitali11
  11. on behalf of the EULAR Sjögren's Task Force
  1. 1Department of Epidemiology, Biostatistics and Clinical Research, Assistance Publique–Hopitaux de Paris, Hôpital Bichat, Paris, France
  2. 2INSERM U738, Paris, France
  3. 3Université Paris 7-Denis Diderot, UFR de Médecine, Paris, France
  4. 4Department of Rheumatology, Assistance Publique–Hopitaux de Paris, Hôpital Bicêtre, INSERM U802, Le Kremlin Bicêtre, France
  5. 5Université Paris-Sud 11, Le Kremlin Bicêtre, France
  6. 6Rheumatology Department, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
  7. 7Department of Pathophysiology, School of Medicine, University of Athens, Greece
  8. 8Department of Rheumatology, Malmö University Hospital, Lund University, Sweden
  9. 9Department of Rheumatology, Strasbourg University Hospital, Strasbourg, France
  10. 10Department of Rheumatology and Clinical Immunology, University Medical Center Groningen (UMCG), Groningen, The Netherlands
  11. 11Department of Internal Medicine and section of Rheumatology, ‘Villamarina’ Hospital, Piombino, Italy
  1. Correspondence to Dr Raphaèle Seror, Department of Epidemiology, Biostatistics and Clinical Research, Hôpital Bichat, INSERM U738, Hôpital Bichat, 46 rue Henri Huchard, Paris 75018, France; raphaele.se{at}gmail.com

Abstract

Objective To develop a disease activity index for patients with primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI).

Methods Thirty-nine SS experts participated in an international collaboration, promoted by EULAR, to develop the ESSDAI. Experts identified 12 organ-specific ‘domains’ contributing to disease activity. For each domain, features of disease activity were classified in three or four levels according to their severity. Data abstracted from 96 patients with systemic complications of primary SS were used to generate 702 realistic vignettes for which all possible systemic complications were represented. Using the 0–10 physician global assessment (PhGA) scale, each expert scored the disease activity of five patient profiles and 20 realistic vignettes. Multiple regression modelling, with PhGA used as the dependent variable, was used to estimate the weight of each domain.

Results All 12 domains were significantly associated with disease activity in the multivariate model, domain weights ranged from 1 to 6. The ESSDAI scores varied from 2 to 47 and were significantly correlated with PhGA for both real patient profiles and realistic vignettes (r=0.61 and r=0.58, respectively, p<0.001). Compared with 57 (59.4%) of the real patient profiles, 468 (66.7%) of the realistic vignettes were considered likely or very likely to be true.

Conclusions The ESSDAI is a clinical index designed to measure disease activity in patients with primary SS. Once validated, such a standardised evaluation of primary SS should facilitate clinical research and be helpful as an outcome measure in clinical trials.

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Footnotes

  • Funding This project was supported by a grant from the European League Against Rheumatism (EULAR).

  • Competing interests None.

  • Ethics approval This study was conducted with the approval of the institutional review board of GHU Paris Nord (no IRB0006477).

  • Provenance and peer review Not commissioned; externally peer reviewed.

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