Cited by 1,612Systemic Lupus Erythematosus ResearchSystemic Sclerosis and Related DiseasesVasculitis and related conditions

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

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Aringer, Martin, Costenbader, Karen, Daikh, David, Brinks, Ralph, Mosca, Marta, Ramsey-Goldman, Rosalind, Smolen, Josef S, Wofsy, David +56 more·2019·Annals of the Rheumatic Diseases

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.

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  • A positive anti-dsDNA result gives 6 out of 10 points required for classification.

    “Given the high impact of a positive result, i.e., 6 out of 10 points required for classification”

    From Anti-dsDNA antibodies in the classification criteria of systemic lupus erythematosus (2022) · cites this paper as [20]

    The Derivation cohort passage identifies anti-dsDNA as a weighted SLE-specific antibody criterion and gives a classification threshold of at least 10 points. The Criteria section requires an anti-dsDNA assay with demonstrated specificity of at least 90%, but the passages available from our copy do not state that this criterion receives six points. Thus the specific 6-out-of-10 claim is not verified by the available text. (LLM, conf=0.78)

    Experimental — accuracy not yet measured
  • The more specific antibodies for SLE as anti-dsDNA and anti-Sm are grouped in one domain and weighted equally by 6 points, while aPL antibodies are grouped in a second domain accounting for only 2 points.

    “The more specific antibodies for SLE as anti-dsDNA and anti-Sm are grouped in one domain and weighted equally by 6 points, while aPL antibodies (IgG, IgA and IgM anti-cardiolipin and anti-β2GPI antibodies) are grouped in a second domain accounting for only 2 points.”

    From Anti-dsDNA antibodies in the classification criteria of systemic lupus erythematosus (2022) · cites this paper as [20]

    The Derivation cohort passage groups anti-dsDNA and anti-Sm in the SLE-specific antibody domain and antiphospholipid antibodies in a separate domain. The Criteria passage includes the listed anticardiolipin and anti-β2-glycoprotein I antibodies, but also includes lupus anticoagulant. The passages available from our copy do not state the claimed six-point and two-point weights. (LLM, conf=0.76)

    Experimental — accuracy not yet measured
  • The 2019 EULAR/ACR criteria assigned the highest score (6 points) to anti-dsDNA antibodies in the fully weighted scoring of the disease.

    “These 2019 criteria reinforced the importance of autoantibodies in SLE diagnosis, assigning the highest score (6 points) to anti-dsDNA antibodies in the fully weighted scoring of the disease.”

    From Anti-dsDNA antibodies in the classification criteria of systemic lupus erythematosus (2022) · cites this paper as [20]

    The Derivation cohort section identifies anti-dsDNA as an SLE-specific antibody within the weighted criteria. But the Discussion says renal biopsy showing class III or IV lupus nephritis carries the most weight, and class II or V nephritis carries eight points. Thus, anti-dsDNA does not receive the highest score in the full classification system; the retrieved passages do not establish its six-point value. (LLM, conf=0.88)

    Experimental — accuracy not yet measured
  • To enable alternative solid phase assays for detection of anti-dsDNA antibodies, the criteria defined the specificity to be ≥90%.

    “To enable alternative solid phase assays for detection of anti-dsDNA antibodies, the criteria defined the specificity to be ≥ 90%.”

    From Anti-dsDNA antibodies in the classification criteria of systemic lupus erythematosus (2022) · cites this paper as [20]

    The Criteria section defines anti-dsDNA positivity as detection by an immunoassay with demonstrated ≥90% specificity for SLE against relevant disease controls. The paper does not state that this threshold was chosen to enable alternative solid-phase anti-dsDNA assays; its mention of a solid-phase assay in Phase I concerns ANA screening instead. (LLM, conf=0.84)

    Experimental — accuracy not yet measured
  • In the 2019 ACR/EULAR criteria, ANA at a titer of ≥1:80 on HEp-2 cells is positioned as an entry criterion (mandatory requirement).

    “In the 2019 ACR/EULAR criteria, ANA at a titer of ≥1:80 on HEp-2 cells is positioned as an entry criterion (mandatory requirement).”

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Research works printed but not linked: 2

  1. [1]1. Internal Medicine Department, Centre de référence maladies auto-immunes et systémiques rares d'île deFrance, Cochin Hospital, Université Paris Descartes- Sorbonne Paris Cité, INSERM U1153, Center for Epidemiology and Statistics Sorbonne Paris Cité (CRESS), Paris, France on August 12, 2023 by guest. Protected by copyright. http://ard.bmj.com/ Ann Rheum Dis: first published as 10.1136/annrheumdis-2018-214819 on 5 August 2019. Downloaded from RefeRences
  2. [32]32. Ganczarczyk L, Urowitz MB, Gladman DD, et al. "Latent lupus".. J Rheumatol 1989;16:475-8.

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Authors
Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., Smolen, J. S., Wofsy, D., Boumpas, D. T., Kamen, D. L., Jayne, D., Cervera, R., Costedoat-Chalumeau, N., Diamond, B., Gladman, D. D., Hahn, B., Hiepe, F., Jacobsen, S., Khanna, D., Lerstrøm, K., Massarotti, E., McCune, J., Ruiz-Irastorza, G., Sanchez-Guerrero, J., Schneider, M., Urowitz, M., Bertsias, G., Hoyer, B. F., Leuchten, N., Tani, C., Tedeschi, S. K., Touma, Z., Schmajuk, G., Anic, B., Assan, F., Chan, T. M., Clarke, A. E., Crow, M. K., Czirják, L., Doria, A., Graninger, W., Halda-Kiss, B., Hasni, S., Izmirly, P. M., Jung, M., Kumánovics, G., Mariette, X., Padjen, I., Pego-Reigosa, J. M., Romero-Diaz, J., Rúa-Figueroa Fernández, Í., Seror, R., Stummvoll, G. H., Tanaka, Y., Tektonidou, M. G., Vasconcelos, C., Vital, E. M., Wallace, D. J., Yavuz, S., Meroni, P. L., Fritzler, M. J., Naden, R., Dörner, T., & Johnson, S. R.
Affiliations
  • University Hospital Carl Gustav Carus (DE)
  • Brigham and Women's Hospital (US)
  • San Francisco VA Medical Center (US)
  • Heinrich Heine University Düsseldorf (DE)
  • University of Pisa (IT)
  • Medical University of Vienna (AT)
  • University of California, San Francisco (US)
  • National and Kapodistrian University of Athens (GR)
  • Medical University of South Carolina (US)
  • University of Cambridge (GB)
  • Hospital Clínic de Barcelona (ES)
  • Mount Sinai Hospital (CA)
  • Feinstein Institute for Medical Research (US)
  • University of Toronto (CA)
  • University of California, Los Angeles (US)
  • Humboldt-Universität zu Berlin (DE)
  • Copenhagen University Hospital (DK)
  • University of Michigan (US)
  • University of Crete (GR)
  • Christian-Albrechts-Universität zu Kiel (DE)
  • Université Paris-Sud (FR)
  • University of Hong Kong (HK)
  • University of Calgary (CA)
  • Hospital for Special Surgery (US)
  • University of Pecs (HU)
  • University of Padua (IT)
  • Medical University of Graz (AT)
  • National Institutes of Health (US)
  • New York University (US)
  • University Hospital Centre Zagreb (HR)
  • University of Occupational and Environmental Health Japan (JP)
  • Universidade do Porto (PT)
  • University of Leeds (GB)
  • Cedars-Sinai Medical Center (US)
  • IRCCS Istituto Auxologico Italiano (IT)
  • McMaster University (CA)
  • Inserm (FR)
Corresponding author
Aringer, Martin
Venue
Annals of the Rheumatic Diseases
Publisher
Elsevier BV
Published
2019-09
Volume
78
Issue
9
Pages
1151-1159
ISSN
0003-4967 (ISSN-L)
OpenAlex
W2966041078
Cited by
1,612
References
35
Citations by year
2026: 280 · 2025: 267 · 2024: 293 · 2023: 222 · 2022: 186 · 2021: 192 · 2020: 141 · 2019: 29 · 2018: 1 · 2014: 1
FWCI
103.56
Type
journal article
Language
en
Open access
Locations
  • Annals of the Rheumatic Diseases (publishedVersion)
  • PubMed (publishedVersion)
  • Research at the University of Copenhagen (University of Copenhagen) (publishedVersion)
  • Istanbul Science University (Istanbul Bilim University) (submittedVersion, other-oa)
  • CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion)
  • HighWire Press Open Archive (submittedVersion)
  • Padua Research Archive (University of Padova) (submittedVersion)
Funders
  • American College of Rheumatology
  • European League Against Rheumatism
Topics
  • Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
Keywords
systemic lupus erythematosus, classification criteria, antinuclear antibodies, weighted criteria, multi-criteria decision making, antiphospholipid antibodies, complement proteins, sensitivity and specificity, SLICC 2012 criteria
MeSH terms
Systematic Reviews as Topic, Humans, Lupus Erythematosus, Systemic, Rheumatic Diseases, Rheumatology, Societies, Medical

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