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, 71 (9), 1400-1412

2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus

Martin Aringer  1 Karen Costenbader  2 David Daikh  3 Ralph Brinks  4 Marta Mosca  5 Rosalind Ramsey-Goldman  6 Josef S Smolen  7 David Wofsy  8 Dimitrios T Boumpas  9 Diane L Kamen  10 David Jayne  11 Ricard Cervera  12 Nathalie Costedoat-Chalumeau  13 Betty Diamond  14 Dafna D Gladman  15 Bevra Hahn  16 Falk Hiepe  17 Søren Jacobsen  18 Dinesh Khanna  19 Kirsten Lerstrøm  20 Elena Massarotti  2 Joseph McCune  19 Guillermo Ruiz-Irastorza  21 Jorge Sanchez-Guerrero  22 Matthias Schneider  23 Murray Urowitz  24 George Bertsias  25 Bimba F Hoyer  26 Nicolai Leuchten  1 Chiara Tani  27 Sara K Tedeschi  2 Zahi Touma  15 Gabriela Schmajuk  3 Branimir Anic  28 Florence Assan  29 Tak Mao Chan  30 Ann Elaine Clarke  31 Mary K Crow  32 László Czirják  33 Andrea Doria  34 Winfried Graninger  35 Bernadett Halda-Kiss  33 Sarfaraz Hasni  36 Peter M Izmirly  37 Michelle Jung  31 Gábor Kumánovics  33 Xavier Mariette  38 Ivan Padjen  28 José M Pego-Reigosa  39 Juanita Romero-Diaz  40 Íñigo Rúa-Figueroa Fernández  41 Raphaèle Seror  29 Georg H Stummvoll  7 Yoshiya Tanaka  42 Maria G Tektonidou  43 Carlos Vasconcelos  44 Edward M Vital  45 Daniel J Wallace  46 Sule Yavuz  47 Pier Luigi Meroni  48 Marvin J Fritzler  31 Ray Naden  49 Thomas Dörner  17 Sindhu R Johnson  50
Affiliations

2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus

Martin Aringer et al. Arthritis Rheumatol.

Abstract

Objective: To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR).

Methods: This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects.

Results: The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria.

Conclusion: These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.

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