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Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes and ethnicities
  1. Sindhu R Johnson1,
  2. Ralph Brinks2,
  3. Karen H Costenbader3,4,
  4. David Daikh5,
  5. Marta Mosca6,
  6. Rosalind Ramsey-Goldman7,
  7. Josef S Smolen8,
  8. David Wofsy9,
  9. Dimitrios T Boumpas10,11,
  10. Diane L Kamen12,
  11. David Jayne13,
  12. R Cervera14,
  13. Nathalie Costedoat-Chalumeau15,16,
  14. Betty Diamond17,
  15. Dafna D Gladman18,19,
  16. Bevra Hahn20,
  17. Falk Hiepe21,
  18. Søren Jacobsen22,
  19. Dinesh Khanna23,
  20. Kirsten Lerstrøm24,
  21. Elena Massarotti4,25,
  22. Joseph McCune23,
  23. Guillermo Ruiz-Irastorza26,
  24. Jorge Sanchez-Guerrero27,28,
  25. Matthias Schneider29,
  26. Murray Urowitz30,
  27. George Bertsias31,32,
  28. Bimba F Hoyer33,
  29. Nicolai Leuchten34,
  30. Chiara Tani35,36,
  31. Sara K Tedeschi4,37,
  32. Zahi Touma38,
  33. Gabriela Schmajuk39,
  34. Branimir Anic40,
  35. Florence Assan41,
  36. Tak Mao Chan42,
  37. Ann Elaine Clarke43,
  38. Mary K Crow44,
  39. László Czirják45,
  40. Andrea Doria46,
  41. Winfried B Graninger47,
  42. Bernadett Halda-Kiss45,
  43. Sarfaraz Hasni48,
  44. Peter M Izmirly49,
  45. Michelle Jung50,
  46. Gábor Kumánovics45,
  47. Xavier Mariette51,
  48. Ivan Padjen52,
  49. José M Pego-Reigosa53,
  50. Juanita Romero-Diaz54,
  51. Íñigo Rúa-Figueroa55,
  52. Raphaèle Seror41,
  53. Georg H Stummvoll8,
  54. Yoshiya Tanaka56,
  55. Maria G Tektonidou57,
  56. Carlos Vasconcelos58,
  57. Edward M Vital59,60,
  58. D J Wallace61,
  59. Sule Yavuz62,
  60. Pier Luigi Meroni63,
  61. Marvin J Fritzler64,
  62. Ray Naden65,
  63. Thomas Dörner21,
  64. Martin Aringer66
  1. 1 Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Mount Sinai Hospital; Institute of Health Policy, Management and Evaulation, University of Toronto, Toronto, Ontario, Canada
  2. 2 Policlinic and Hiller Research Unit of Rheumatology, Heinrich-Heine-University Duesseldorf, Düsseldorf, Germany
  3. 3 Department of Medicine, Division of Rheumatology, Inflammation, and Immunity, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA
  4. 4 Rheumatology, Harvard Medical School, Boston, Massachusetts, USA
  5. 5 Medicine/Rheumatology, UCSF Medical Center, San Francisco, California, USA
  6. 6 Rheumatology Unit, University of Pisa, Pisa, Italy
  7. 7 Medicine/Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
  8. 8 Rheumatology, Medical University of Vienna, Vienna, Austria
  9. 9 Division of Rheumatology, , Russell/Engleman Rheumatology Research Center, University of California, San Francisco, San Francisco, California, USA
  10. 10 Medicine, National and Kapodestrian University of Athens, and Biomedical Research Foundation of the Athens Academy, Athens, Greece
  11. 11 Rheumatology, University of Cyprus Medical School, Nicosia, Nicosia, Cyprus
  12. 12 Rheumatology, Medical University of South Carolina, Charleston, South Carolina, USA
  13. 13 Medicine, University of Cambridge, Cambridge, UK
  14. 14 Autoimmune Diseases, Hospital Clinic, University of Barcelona, Barcelona, Catalonia, Spain
  15. 15 Internal Medicine, Hospital Cochin, Paris, Île-de-France, France
  16. 16 INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cité, Université Paris Descartes-Sorbonne Paris Cité, Paris, France
  17. 17 Autoimmune and Musculoskeletal, Feinstein Institutes for Medical Research, Manhasset, New York, USA
  18. 18 Medicine, University of Toronto, Toronto, Ontario, Canada
  19. 19 Medicine, Toronto Western Hospital, Toronto, Ontario, Canada
  20. 20 Rheumatology, UCLA School of Medicine, Los Angeles, California, USA
  21. 21 Rheumatology and Clinical Immunology, Charité–Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
  22. 22 Department of Rheumatology, Copenhagen Lupus and Vasculitis Clinic, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark
  23. 23 Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA
  24. 24 Lupus Europe, Romford, UK
  25. 25 Medicine, Brigham and Women’s Hospital, Boston, Massachusetts, USA
  26. 26 Autoimmune Diseases Unit, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, Barakaldo, País Vasco, Spain
  27. 27 Medicine, Mount Sinai Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada
  28. 28 Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion, Mexico City, Mexico
  29. 29 Policlinic and Hiller Research Unit for Rheumatology, Medical Faculty,Heinrich-Heine-University, Düsseldorf, Germany
  30. 30 Medicine, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada
  31. 31 Rheumatology, University of Crete School of Medicine, Iraklio, Crete, Greece
  32. 32 Laboratory of Autoimmunity-Inflammation, Institute of Molecular Biology and Biotechnology, Heraklion, Crete, Greece
  33. 33 Rheumatology and Clinical Immunology, University of Schleswig-Holstein at Kiel, Kiel, Germany
  34. 34 Medicine III, University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany
  35. 35 Rheumatology, Azienda Ospedaliero Universitaria Pisana, Pisa, Toscana, Italy
  36. 36 Rheumatology, University of Pisa, Pisa, Toscana, Italy
  37. 37 Medicine, Division of Rheumatology, Brigham and Women’s Hospital, Boston, Massachusetts, USA
  38. 38 Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada
  39. 39 Department of Medicine, Division of Rheumatology, University of California, San Francisco, San Francisco, California, USA
  40. 40 Division of Clinical Immunology and Rheumatology, Department of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia
  41. 41 INSERM UMR 1163, Université Paris Sud, Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR 1184, Paris, France
  42. 42 Department of Medicine, University of Hong Kong, Pokfulam, Hong Kong
  43. 43 Rheumatology, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada
  44. 44 Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, New York, New York, USA
  45. 45 Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary
  46. 46 Division of Rheumatology, University of Padova, Padova, Italy
  47. 47 Rheumatology and Immunology, Medical University Graz, Graz, Austria
  48. 48 Rheumatology, NIAMS, National Institutes of Health, Bethesda, Maryland, USA
  49. 49 Rheumatology, New York University School of Medicine, New York, New York, USA
  50. 50 Rheumatology, University of Calgary, Calgary, Alberta, Canada
  51. 51 Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR, Université Paris Sud, Le Kremlin-Bicêtre, France
  52. 52 Division of Clinical Immunology and Rheumatology, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia
  53. 53 Rheumatology, University Hospital of Vigo, IRIDIS Group, Instituto de Investigación Sanitaria Galicia Sur, Vigo, Spain
  54. 54 Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Tlalpan, Mexico
  55. 55 Rheumatology, Dr Negrin University Hospital of Gran Canaria, Las Palmas de Gran Canaria, Spain
  56. 56 First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan
  57. 57 Medical School, National and Kapodistrian University of Athens, Athinon, Greece
  58. 58 Centro Hospitalar do Porto, ICBAS, University of Porto, Porto, Portugal
  59. 59 Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK
  60. 60 NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK
  61. 61 Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA
  62. 62 Rheumatology, Istanbul Bilim Universitesi, Istanbul, Turkey
  63. 63 Clinical Immunology and Rheumatology, IRCCS Istituto Auxologico Italiano, Milan, Italy
  64. 64 Medicine, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada
  65. 65 Medicine, McMaster University Faculty of Health Sciences, Hamilton, Ontario, Canada
  66. 66 Rheumatology, University Medical Center and Faculty of Medicine Carl Gustav Carus, Dresden, Germany
  1. Correspondence to Dr Sindhu R Johnson, Division of Rheumatology, Ground Floor, East Wing, Toronto Western Hospital, 399 Bathurst Street, Toronto, ON M5T2S8, Canada; Sindhu.Johnson{at}uhn.ca; Professor Martin Aringer, Division of Rheumatology, Department of Medicine III, University Medical Center TU Dresden, Fetscherstrasse 74, 01307 Dresden, Germany; martin.aringer{at}uniklinikum-dresden.de

Abstract

Objectives The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance of the new criteria with regard to disease duration, sex and race/ethnicity, and compared its performance against the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and ACR 1982/1997 criteria.

Methods Twenty-one SLE centres from 16 countries submitted SLE cases and mimicking controls to form the validation cohort. The sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated.

Results The cohort consisted of female (n=1098), male (n=172), Asian (n=118), black (n=68), Hispanic (n=124) and white (n=941) patients; with an SLE duration of 1 to <3 years (n=196) and ≥5 years (n=879). Among patients with 1 to <3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 81%). The EULAR/ACR criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). Among women, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 83%) and better specificity than the SLICC criteria (94% vs 82%). Among white patients, the EULAR/ACR criteria had better sensitivity than the ACR criteria (95% vs 83%) and better specificity than the SLICC criteria (94% vs 83%). The EULAR/ACR criteria performed well among black patients (sensitivity of 98%, specificity 100%), and had better sensitivity than the ACR criteria among Hispanic patients (100% vs 86%) and Asian patients (97% vs 77%).

Conclusions The EULAR/ACR 2019 criteria perform well among patients with early disease, men, women, white, black, Hispanic and Asian patients. These criteria have superior sensitivity than the ACR criteria and/or superior specificity than the SLICC criteria across many subgroups.

  • systemic lupus erythematosus
  • epidemiology
  • lupus nephritis
  • outcomes research
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Footnotes

  • Handling editor David S Pisetsky

  • Twitter @none, @edvital

  • Contributors All authors contributed data and critical review of the manuscript.

  • Funding This body of work was jointly supported by the European League Against Rheumatism and the American College of Rheumatology. This research was supported (in part) by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health.

  • Competing interests None declared.

  • Patient and public involvement Patients and/or the public were involved in the design, or conduct, or reporting, or dissemination plans of this research. Refer to the Methods section for further details.

  • Patient consent for publication Not required.

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

  • Data availability statement Data are available upon reasonable request.

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