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Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI)
  1. Raphaèle Seror1,2,3,
  2. Hendrika Bootsma4,
  3. Alain Saraux5,
  4. Simon J Bowman6,
  5. Elke Theander7,
  6. Johan G Brun8,
  7. Gabriel Baron2,3,
  8. Véronique Le Guern9,
  9. Valérie Devauchelle-Pensec5,
  10. Manel Ramos-Casals10,
  11. Valeria Valim11,
  12. Thomas Dörner12,
  13. Athanasios Tzioufas13,
  14. Jacques-Eric Gottenberg14,
  15. Roser Solans Laqué15,
  16. Thomas Mandl7,
  17. Eric Hachulla16,
  18. Kathy L Sivils17,
  19. Wan-Fai Ng18,
  20. Anne-Laure Fauchais19,
  21. Stefano Bombardieri20,
  22. Roberta Priori21,
  23. Elena Bartoloni22,
  24. Vincent Goeb23,
  25. Sonja Praprotnik24,
  26. Takayuki Sumida25,
  27. Sumusu Nishiyama26,
  28. Roberto Caporali27,
  29. Aike A Kruize28,
  30. Cristina Vollenweider29,
  31. Philippe Ravaud2,3,
  32. Petra Meiners30,
  33. Pilar Brito-Zerón10,
  34. Claudio Vitali31,
  35. Xavier Mariette1
  36. on behalf of the EULAR Sjögren's Task Force
    1. 1Department of Rheumatology, Hôpitaux Universitaires Paris-Sud, Assistance Publique–Hopitaux de Paris (AP-HP), Université Paris-Sud, INSERM U1012, Le Kremlin Bicêtre, France
    2. 2Center of Clinical Epidemiology, Hôpital Hotel Dieu, Paris, France
    3. 3INSERM U738, Université Paris-René Descartes, Paris, France
    4. 4Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
    5. 5Department of Rheumatology, Hôpital Cavale Blanche, CHU Brest, and EA 2216, Université Bretagne occidentale, Brest, France
    6. 6Rheumatology Department, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK
    7. 7Department of Rheumatology, Skane University Hospital Malmö, Lund University, Malmö, Sweden
    8. 8Department of Rheumatology, Haukeland University Hospital, Bergen, Norway
    9. 9Department of Internal Medicine and National Referral Centre for Rare Systemic Auto-immune Diseases, Assistance Publique–Hopitaux de Paris, Hôpital Cochin, Paris Descartes University, Paris, France
    10. 10Laboratory of Autoimmune Diseases “Josep Font”, IDIBAPS, ICMiD, Hospital Clinic, Barcelona, Spain
    11. 11Division of Rheumatology, Department of Medicine, Federal University of Espírito Santo, Espírito Santo, Brazil.
    12. 12Rheumatology Department, Charité, University Hospital, Berlin, Germany
    13. 13Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece
    14. 14Department 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
    15. 15Department of Autoimmune systemic Diseases, Vall d'Hebron University Hospital, Barcelona, Spain
    16. 16Department of Internal Medicine, Claude Huriez Hospital, Université Nord de France, Lille, France
    17. 17Department of Arthritis and Clinical Immunology, Oklahoma Medical research foundation, Oklahoma City, USA
    18. 18Musculoskeletal Research Group, Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
    19. 19Department of Rheumatology, University Hospital, Limoges, France
    20. 20Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy
    21. 21Department of Medicina Interna e Specialità Mediche, Rheumatology Clinic, La Sapienza University of Rome, Rome, Italy
    22. 22Department of Rheumatology, Perugia University Hospital, Perugia, Italy
    23. 23Department of Rheumatology, Amiens university hospital, Amiens, France
    24. 24Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia
    25. 25Department of Internal Medicine, University of Tsukuba, Tsukuba, Japan
    26. 26Rheumatic Disease Center, Kurashiki Medical Center, Kurashiki, Japan
    27. 27Department of Rheumatology, University of Pavia, IRCCS S. Matteo Foundation, Pavia, Italy
    28. 28Department of Rheumatology & Clinical Immunology, University Medical Center Utrecht (UMCU), Utrecht, The Netherlands
    29. 29Department of Rheumatology, German Hospital, Buenos-Aires, Argentina
    30. 30Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
    31. 31Sections of Rheumatology, Instituto San Giuseppe, Como and Casa di Cura di Lecco, Lecco, Italy
    1. Correspondence to Dr Raphaèle SEROR, Department of Rheumatology, Hôpital Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France; raphaele.se{at}gmail.com.

    Abstract

    Objectives To define disease activity levels, minimal clinically important improvement (MCII) and patient-acceptable symptom state (PASS) with the primary Sjögren's syndrome (SS) disease activity indexes: European League Against Rheumatism (EULAR) SS disease activity index (ESSDAI) and EULAR SS patient-reported index (ESSPRI).

    Methods For 790 patients from two large prospective cohorts, ESSDAI, physician evaluation of disease activity, ESSPRI and patients’ satisfaction with their current health status were recorded. Receiver operating characteristic curve analyses and anchoring methods were used to estimate disease activity levels of ESSDAI and the PASS of ESSPRI. At follow-up visit, patients and physicians assessed, respectively, whether symptoms and disease activity have improved or not. An anchoring method based on this evaluation was used to estimate MCII of ESSDAI and ESSPRI.

    Results Low-activity (ESSDAI<5), moderate-activity (5≤ESSDAI≤13) and high-activity (ESSDAI≥14) levels were defined. MCII of ESSDAI was defined as an improvement of at least three points. The PASS estimate was defined as an ESSPRI<5 points and MCII as a decrease of at least one point or 15%.

    Conclusions This study determined disease activity levels, PASS and MCII of ESSDAI and ESSPRI. These results will help designing future clinical trials in SS. For evaluating systemic complications, the proposal is to include patients with moderate activity (ESSDAI≥5) and define response to treatment as an improvement of ESSDAI at least three points. For addressing patient-reported outcomes, inclusion of patients with unsatisfactory symptom state (ESSPRI≥5) and defining response as an improvement of ESSPRI at least one point or 15% seems reasonable.

    • Sjøgren's Syndrome
    • Outcomes research
    • Patient perspective

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