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O8 Performance of the EULAR/ACR 2019 classification criteria for systemic lupus erythematosus in men, ethnicities, and early disease
  1. Martin Aringer1,
  2. Ralph Brinks2,
  3. Karen Costenbader3,
  4. Dimitrios Boumpas4,
  5. David Daikh5,
  6. David Jayne6,
  7. Diane L Kamen7,
  8. Marta Mosca8,
  9. Rosalind Ramsey-Goldman9,
  10. Josef S Smolen10,
  11. David Wofsy11,
  12. Betty Diamond12,
  13. Søren Jacobsen13,
  14. W Joseph McCune14,
  15. Guillermo Ruiz-Irastorza15,
  16. Matthias Schneider16,
  17. Murray Urowitz17,
  18. George Bertsias18,
  19. Bimba Hoyer19,
  20. Nicolai Leuchten20,
  21. Chiara Tani21,
  22. Sara K Tedeschi22,
  23. Zahi Touma23,
  24. Branimir Anic24,
  25. Florence Assan25,
  26. Tak Mao Chan26,
  27. Ann E Clarke27,
  28. Peggy Crow28,
  29. Lazlo Czirjak29,
  30. Andrea Doria30,
  31. Winfried Graninger31,
  32. Bernadette Halda-Kiss32,
  33. Sarfaraz A Hasni33,
  34. Peter M Izmirly34,
  35. Michelle Jung35,
  36. Gabor Kumanovics32,
  37. Xavier Mariette25,
  38. Ivan Padjen24,
  39. JM Pego-Reigosa36,
  40. Juanita Romero-Díaz37,
  41. Iñigo Rúa-Figueroa38,
  42. Raphaele Seror39,
  43. Georg Stummvoll40,
  44. Yoshiya Tanaka41,
  45. Maria Tektonidou4,
  46. Carlos Vasconcelos42,
  47. Edward M Vital43,
  48. Daniel J Wallace44,
  49. Sule Yavuz45,
  50. Raymond P Naden46,
  51. Thomas Dorner47 and
  52. Sindhu Johnson48
  1. 1Medicine III, University Medical Center and Faculty of Medicine at the TU Dresden, Dresden
  2. 2Dept. of Rheumatology and Hiller Research Unit, Heinrich-Heine University, Düsseldorf, Germany
  3. 3Rheumatology, Immunology and All, Brigham and Women’s Hospital, Boston, USA
  4. 4University of Athens, Athens, Greece
  5. 5Rheumatology, UCSF/VA Medical Center, San Francisco, USA
  6. 6Dept. of Medicine, University of Cambridge, Cambridge, UK
  7. 7Medicine/Rheumatology and Immunology, Medical University of South Carolina, Charleston, USA
  8. 8Rheumatology Unit, University of Pisa, Pisa, Italy
  9. 9FSM, Northwestern University, Chicago, USA
  10. 10Devision of Rheumatology, Dept. of Medicine 3, Medical University of Vienna, Vienna, Austria
  11. 11University of California San Francisco, San Francisco
  12. 12Autoimmune Musculoskeletal and Hematopoietic Diseases, The Feinstein Institute, Manhasset, USA
  13. 13University of Copenhagen, Copenhagen, Denmark
  14. 14Int Med/Rheum, University of Michigan, Ann Arbor, USA
  15. 15Autoimmune Diseases Research Unit, BioCruces, Hospital Universitario Cruces, Baracaldo, Spain
  16. 16Dept. and Hiller Research Center for Rheumatology, University Hospital Duesseldorf, Duesseldorf, Germany
  17. 17Centre for Prognosis Studies in the Rheumatic Diseases, University of Toronto, Toronto Western Hospital, Toronto, Canada
  18. 18University of Crete, Heraklion, Greece
  19. 19University of Schleswig-Holstein, Kiel
  20. 20University Medical Center and Faculty of Medicine TU Dresden, Dresden, Germany
  21. 21University of Pisa, Pisa, Italy
  22. 22Rheumatology Immunology and Allergy, Brigham and Women’s Hospital, Boston, USA
  23. 23Rheumatology, University of Toronto, Division of Rheumatology, Institute of Health Policy, Management and Evaluation, Toronto, Canada
  24. 24University Hospital Zagreb, Zagreb, Croatia
  25. 25Université Paris Sud, Paris, France
  26. 26University of Hong Kong, Hong Kong
  27. 27Division of Rheumatology, University of Calgary, Calgary, Canada
  28. 28Dept. of Medicine, Hospital for Special Surgery, New York, USA
  29. 29Dept. of Rheumatology and Immunology, University of Pécs, Pécs, Hungary
  30. 30University and Azienda Ospedaliera of Padova, Padova, Italy
  31. 31Rheumatology and Immunology, Medical University of Graz, Graz, Austria
  32. 32University of Pécs, Pécs, Hungary
  33. 33Lupus Clinical Research Program, Office of the Clinical Director, NIH/NIAMS, Bethesda
  34. 34New York University School of Medicine, New York, USA
  35. 35University of Calgary, Calgary, Canada
  36. 36Rheumatology Section, Hospital de Meixoeiro, Pontevedra, Vigo, Spain
  37. 37Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion, Mexico City, Mexico
  38. 38Rheumatology Division, Hospital Doctor Negrin, Las Palmas GC, Spain
  39. 39Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France
  40. 40Rheumatology, Medical University Vienna, Vienna, Austria
  41. 41First Dept. of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan
  42. 42University of Porto, Porto, Portugal
  43. 43University of Leeds, Leeds, UK
  44. 44Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, USA
  45. 45Dept. of Rheumatology, Marmara University, Istanbul, Turkey
  46. 46New Zealand Ministry of Health, New Zealand Ministry of Health, Auckland, New Zealand
  47. 47Charité Universitätsmedizin Berlin, Berlin, Germany
  48. 48Rheumatology, Mount Sinai Hospital and University Health Network, Toronto, Canada


Background Supported by both the ACR and EULAR, the EULAR/ACR 2019 Classification Criteria for SLE employ positive ANA (ever) as an entry criterion and use a weighted scheme with values ranging from 2 to 10, for a classification cut-off of 10. Criteria items are attributed to SLE only if there is no more likely alternative diagnosis in the individual patients. Items are organized in domains, and only the highest ranking item within a domain is counted. These criteria have been validated in a cohort of 696 SLE patients and 574 non-SLE patients from a total of 21 centers, reaching an overall sensitivity of 96.1% and a specificity of 93.4%. To at least estimate the performance in groups underrepresented in the validation cohort of this transatlantic project, we analyzed this cohort for patient subsets with regard to sex, ethnicity, and disease duration.

Methods The full EULAR/ACR 2019 classification criteria validation cohort was analyzed for female (n=1,098) and male (n=172) patients, Asian (n=118), Black (n=68), Hispanic (n=124) and White (n=941) patients, and patients with an SLE duration of less than 1 year (n=34), one to less than 3 years (n=196), 3 to less than 5 years (n=157), and 5 or more years (n=879). Sensitivity and specificity were calculated for the EULAR/ACR 2019 criteria, the SLICC 2012 criteria and the ACR 1997 criteria each.

Results As shown in table 1, most of the point estimates for sensitivity and specificity in subsets lay within the 95% confidence intervals of the sensitivity and specificity of the EULAR/ACR 2019 criteria validation. In particular, sensitivity and specificity for all ethnic groups were within the confidence intervals or even higher. Formally, the sensitivity was slightly lower for male patients, corresponding to a higher specificity, but the male 95% confidence intervals (0.86–0.98 for sensitivity, 0.90–0.99 for specificity) overlapped. While sensitivity appeared independent of disease duration from year 1 on, sensitivity was only 89% in the first year of disease, identical to the SLICC criteria (89%) and numerically higher than the ACR criteria (56%), but all confidence intervals overlapped.

Abstract O8 Table 1

Conclusion While not all subgroups of SLE patients in the validation cohort are of adequate size to fully explore the sensitivity and specificity of the EULAR/ACR 2019 SLE classification criteria in the respective subsets, the point estimates of sensitivity and specificity suggest that the new criteria perform at least reasonably well in all ethnic groups, in men and in early disease. Nevertheless, sensitivity and specificity should be independently validated in larger groups of Asian, Black and Hispanic patients, male patients and in early disease.

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