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Validation of EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient indexes (ESSPRI)
  1. Raphaèle Seror1,2,
  2. Elke Theander3,
  3. Johan G Brun4,
  4. Manel Ramos-Casals5,
  5. Valeria Valim6,
  6. Thomas Dörner7,
  7. Hendrika Bootsma8,
  8. Athanasios Tzioufas9,
  9. Roser Solans-Laqué10,
  10. Thomas Mandl3,
  11. Jacques-Eric Gottenberg11,
  12. Eric Hachulla12,
  13. Kathy L Sivils13,
  14. Wan-Fai Ng14,
  15. Anne-Laure Fauchais15,
  16. Stefano Bombardieri16,
  17. Guido Valesini17,
  18. Elena Bartoloni18,
  19. Alain Saraux19,
  20. Matija Tomsic20,
  21. Takayuki Sumida21,
  22. Sumusu Nishiyama22,
  23. Roberto Caporali23,
  24. Aike A Kruize24,
  25. Cristina Vollenweider25,
  26. Philippe Ravaud2,
  27. Claudio Vitali26,
  28. Xavier Mariette1,
  29. Simon J Bowman27,
  30. on behalf of the EULAR Sjögren's Task Force
  1. 1Center of Clinical Epidemiology, Hôpital Hotel Dieu; INSERM U738, Université Paris-René Descartes, Paris, France
  2. 2Department of Rheumatology, INSERM U802, Assistance Publique–Hopitaux de Paris, Hôpital Bicêtre; Université Paris-Sud 11; INSERM U1012, Le Kremlin Bicêtre, France
  3. 3Department of Rheumatology, Skane University Hospital Malmö, Lund University, Malmö, Sweden
  4. 4Department of Rheumatology, Haukeland University Hospital, Bergen, Norway
  5. 5Laboratory of Autoimmune Diseases “Josep Font”, IDIBAPS, ICMiD, Hospital Clinic, Barcelona, Spain
  6. 6Division of Rheumatology, Department of Medicine, Federal University of Espírito Santo, Brazil
  7. 7Rheumatology Department, Charité, University Hospital, Berlin, Germany
  8. 8Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
  9. 9Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece
  10. 10Departement of Autoimmune systemic Diseases, Vall d'Hebron University Hospital, Barcelona, Spain
  11. 11Departement of Rheumatology, Centre National de Référence des Maladies Auto-Immunes Rares, INSERM UMRS_1109, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Strasbourg university Hospital, Université de Strasbourg, Strasbourg, France
  12. 12Department of Internal Medicine, Claude Huriez Hospital, Université Nord de France, Lille, France
  13. 13Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, USA
  14. 14Musculoskeletal Research Group, University of Newcastle, Newcastle, UK
  15. 15Department of Rheumatology, University Hospital, Limoges, France
  16. 16Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy
  17. 17Department of Internal Medicine, Rheumatology, La Sapienza Università di Roma, Roma, Italy
  18. 18Department of Rheumatology, Perugia University Hospital, Perugia, Italy
  19. 19Department of Rheumatology, Hopital Cavale Blanche, and EA 2216, Université Bretagne occidentale, Brest, France
  20. 20Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia
  21. 21Department of Internal Medicine, University of Tsukuba, Japan
  22. 22Rheumatic Disease Center, Kurashiki Medical Center, Kurashiki, Japan
  23. 23Department of Rheumatology, University of Pavia, IRCCS S, Matteo Foundation, Pavia, Italy
  24. 24Department of Rheumatology & Clinical Immunology, Department of Rheumatology, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands
  25. 25Department of Rheumatology, German Hospital, Buenos-Aires, Argentina
  26. 26Sections of Rheumatology, Instituto San Giuseppe, Como and Casa di Cura di Lecco, Lecco, Italy
  27. 27Rheumatology Department, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
  1. Correspondence to Dr Raphaèle Seror, Department of Rheumatology, Hôpital Bicêtre, 78 rue du Général Leclerc, Le Kremlin Bicêtre 94275, France; raphaele.se{at}gmail.com

Abstract

Objectives To validate the two recently developed disease activity indexes for assessment of primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) SS Patient Reported Index (ESSPRI) and the EULAR SS Disease Activity Index (ESSDAI).

Methods A prospective international 6-month duration validation study was conducted in 15 countries. At each visit, physicians completed ESSDAI, SS disease activity index (SSDAI), Sjögren's Systemic Clinical Activity Index (SCAI) and physician global assessment (PhGA); and patients completed ESSPRI, Sicca Symptoms Inventory (SSI), Profile of Fatigue and Discomfort (PROFAD) and patient global assessment (PGA). Psychometric properties (construct validity, responsiveness and reliability) were evaluated and compared between scores.

Results Of the 395 patients included, 145 (37%) and 251 (64%) had currently active or current or past systemic manifestations, respectively. EULAR scores had higher correlation with the gold standard than other scores (ESSDAI with PhGA: r=0.59; ESSRPI with PGA: r=0.70). Correlations between patient and systemic scores were very low (ranging from 0.07 to 0.29). All systemic scores had similar large responsiveness in improved patients. Responsiveness of patient scores was low but was significantly higher for ESSPRI compared with SSI and PROFAD. Reliability was very good for all scores.

Conclusions ESSDAI and ESSPRI had good construct validity. All scores were reliable. Systemic scores had a large sensitivity to change in patients whose disease activity improves. Patient scores had a small sensitivity to change, however, significantly better for ESSPRI. Systemic and patient scores poorly correlated, suggesting that they are 2 complementary components that should be both evaluated, but separately.

  • Sjögren's Syndrome
  • Outcomes research
  • Disease Activity

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