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Review
. 2019 Sep;78(9):1151-1159.
doi: 10.1136/annrheumdis-2018-214819. Epub 2019 Aug 5.

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   10 Diane L Kamen  11 David Jayne  12 Ricard Cervera  13 Nathalie Costedoat-Chalumeau  14 Betty Diamond  15 Dafna D Gladman  16 Bevra Hahn  17 Falk Hiepe  18 Søren Jacobsen  19 Dinesh Khanna  20 Kirsten Lerstrøm  21 Elena Massarotti  22   23 Joseph McCune  20 Guillermo Ruiz-Irastorza  24 Jorge Sanchez-Guerrero  25   26 Matthias Schneider  27 Murray Urowitz  28 George Bertsias  29 Bimba F Hoyer  18   30 Nicolai Leuchten  31 Chiara Tani  32 Sara K Tedeschi  23   33 Zahi Touma  34 Gabriela Schmajuk  3 Branimir Anic  35 Florence Assan  36 Tak Mao Chan  37 Ann Elaine Clarke  38 Mary K Crow  39 László Czirják  40 Andrea Doria  41 Winfried Graninger  42 Bernadett Halda-Kiss  40 Sarfaraz Hasni  43 Peter M Izmirly  44 Michelle Jung  38 Gábor Kumánovics  40 Xavier Mariette  45   46 Ivan Padjen  35 José M Pego-Reigosa  47 Juanita Romero-Diaz  26 Íñigo Rúa-Figueroa Fernández  48 Raphaèle Seror  36 Georg H Stummvoll  49 Yoshiya Tanaka  50 Maria G Tektonidou  51 Carlos Vasconcelos  52 Edward M Vital  53   54 Daniel J Wallace  55 Sule Yavuz  56 Pier Luigi Meroni  57 Marvin J Fritzler  58 Ray Naden  59 Thomas Dörner  18 Sindhu R Johnson  60   61
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Free article
Review

2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus

Martin Aringer et al. Ann Rheum Dis. 2019 Sep.
Free article

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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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 reflect current thinking about SLE and provide an improved foundation for SLE research.

Keywords: classification criteria; consensus methods; lupus; multi-criteria decision analysis; systemic lupus erythematosus; validation.

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Conflict of interest statement

Competing interests: None declared.

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