2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus
Open accessAbstract
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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The EULAR/ACR criteria do not recommend a method.
“The EULAR/ACR criteria do not recommend a method.”
From Laboratory evaluation of anti-dsDNA antibodies. (2022) · cites this paper as [28]
The claim does not specify what kind of method it means. For ANA testing, the paper's Phase Ia section explicitly recommends immunofluorescence on HEp-2 cells or a solid-phase screening immunoassay with equivalent performance. The paper also describes its criteria as a method for classifying SLE. (LLM, conf=0.86)
Checked by a reasoning model — may be wrong.
Experimental — accuracy not yet measuredPositivity for polyreactive aCL antibodies is a classification criterion for APS and SLE.
“Positivity for polyreactive aCL antibodies is a criterion in the classification of two autoimmune conditions: APS and SLE251,252.”
From The role of mitochondria in rheumatic diseases (2022) · cites this paper as [251]
The SLE classification-criteria figure lists anti-cardiolipin antibodies as a weighted antiphospholipid-antibody criterion, alongside other requirements for classifying SLE (page not supplied in our copy). It does not specify polyreactive aCL antibodies. This paper sets out SLE criteria, not APS criteria; the sentence also cites another source for APS. (LLM, conf=0.86)
Experimental — accuracy not yet measuredAnti-dsDNA antibodies are an important laboratory parameter for diagnosis, monitoring and classification of systemic lupus erythematosus (SLE).
“Antibodies to dsDNA are an important laboratory parameter for diagnosis, monitoring and classification of systemic lupus erythematosus (SLE).”
From Laboratory evaluation of anti-dsDNA antibodies. (2022) · cites this paper as [28]
The Derivation cohort section (page not provided) identifies anti-dsDNA antibodies as SLE-specific antibodies in the classification criteria, supporting the classification part of the claim. The passages do not establish their use for diagnosis or monitoring, so those parts are unsupported by this paper. (LLM, conf=0.88)
Experimental — accuracy not yet measuredAnti-dsDNA testing is an essential part of the diagnosis, classification, and management of SLE.
“Consequently, anti-dsDNA testing is an essential part of the diagnosis, classification, and management of SLE (2,3,(11)(12)(13)”
From Anti-dsDNA Testing Specificity for Systemic Lupus Erythematosus: A Systematic Review. (2022) · cites this paper as [2]
The classification criteria list anti-dsDNA antibodies among the SLE-specific antibodies that contribute to a classification score (Derivation cohort/validation passage; criteria table). That supports their role in classification, but not the claim that testing is essential to diagnosis or management. The paper explicitly says its classification criteria are not designed for diagnosis or treatment decisions (unlabelled concluding passage; page not given). (LLM, conf=0.89)
Experimental — accuracy not yet measuredThe latest EULAR/ACR SLE classification criteria (2019) stated that the test to measure anti-dsDNA autoantibodies should have a specificity of at least 90%.
“The latest EULAR/ACR SLE classification criteria (2019) stated that the test to measure anti-dsDNA autoantibodies should have a specificity of at least 90% [28].”
From Laboratory evaluation of anti-dsDNA antibodies. (2022) · cites this paper as [28]
The EULAR/ACR criteria do not specify which immunoglobulin isotype should be tested.
“Neither do they specify which immunoglobulin isotype should be tested.”
From Laboratory evaluation of anti-dsDNA antibodies. (2022) · cites this paper as [28]
Assessment of whether studies measured specificity in a relevant disease control group was guided by derivation and validation cohorts used to develop the 2019 EULAR/ACR criteria.
“the assessment of whether studies measured specificity in a relevant disease control group was guided by the derivation and validation cohorts used to develop the 2019 EULAR/ACR classification criteria”
From Anti-dsDNA Testing Specificity for Systemic Lupus Erythematosus: A Systematic Review. (2022) · cites this paper as [2]
Some anti-dsDNA tests used in practice have relatively low specificity.
“the EULAR/ACR noted that some anti-dsDNA tests used in practice have relatively low specificity (2).”
From Anti-dsDNA Testing Specificity for Systemic Lupus Erythematosus: A Systematic Review. (2022) · cites this paper as [2]
The classification-criteria figure lists anti-dsDNA antibodies, but the retrieved passage does not explain its asterisk or discuss the specificity of anti-dsDNA tests. Searches for anti-dsDNA assay specificity and low-specificity tests returned passages about ANA or the specificity of the overall classification criteria, not the claim. (LLM, conf=0.68)
Experimental — accuracy not yet measured
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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)
- Northwestern University (US)
- 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)
- Cochin Hospital Center de référence maladies auto‐immunes et systémiques rares d'île de France Paris France
- 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)
- Lupus Europe Essex UK
- University of the Basque Country (ES)
- Mount Sinai Hospital/University Health Network University of Toronto, Toronto, Ontario, Canada and Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán Mexico City Mexico
- University of Crete (GR)
- University of Zagreb School of Medicine and University Hospital Center Zagreb Zagreb Croatia
- 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)
- University of Pécs Pécs Hungary
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (US)
- New York University (US)
- AP‐HP, Hôpitaux universitaires Paris‐Sud – Hôpital Bicêtre and Université Paris‐Sud INSERM UMR 1184 Université Paris‐Saclay Le Kremlin Bicêtre France
- University Hospital Centre Zagreb (HR)
- University Hospital of Vigo IRIDIS Group Instituto de Investigación Sanitaria Galicia Sur (IISGS) Vigo Spain
- Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán Mexico City Mexico
- Doctor Negrín University Hospital Las Palmas de Gran Canaria Las Palmas Spain
- Université Paris Sud Hôpitaux Universitaires Paris‐Sud, AP‐HP, INSERM 1184 Le Kremlin‐Bicêtre France
- University of Occupational and Environmental Health Japan (JP)
- Universidade do Porto (PT)
- University of Leeds (GB)
- Cedars-Sinai Medical Center (US)
- Istanbul Bilim University Istanbul Turkey
- IRCCS Istituto Auxologico Italiano (IT)
- McMaster University (CA)
- Mount Sinai Hospital (CA)
- Corresponding author
- Aringer, Martin
- Johnson, Sindhu R.
- ORCID
- Aringer, Martin
- Costenbader, Karen
- Brinks, Ralph
- Mosca, Marta
- Ramsey‐Goldman, Rosalind
- Smolen, Josef S.
- Wofsy, David
- Boumpas, Dimitrios T.
- Kamen, Diane L.
- Jayne, David
- Cervera, Ricard
- Diamond, Betty
- Gladman, Dafna D.
- Hiepe, Falk
- Jacobsen, Søren
- Khanna, Dinesh
- Massarotti, Elena
- Ruiz‐Irastorza, Guillermo
- Schneider, Matthias
- Urowitz, Murray
- Bertsias, George
- Hoyer, Bimba F.
- Leuchten, Nicolai
- Tani, Chiara
- Tedeschi, Sara K.
- Touma, Zahi
- Schmajuk, Gabriela
- Assan, Florence
- Chan, Tak Mao
- Clarke, Ann Elaine
- Crow, Mary K.
- Doria, Andrea
- Graninger, Winfried
- Hasni, Sarfaraz
- Izmirly, Peter M.
- Jung, Michelle
- Padjen, Ivan
- Pego‐Reigosa, José M.
- Tanaka, Yoshiya
- Tektonidou, Maria G.
- Vasconcelos, Carlos
- Vital, Edward M.
- Wallace, Daniel J.
- Meroni, Pier Luigi
- Fritzler, Marvin J.
- Dörner, Thomas
- Johnson, Sindhu R.
- Venue
- Arthritis & Rheumatology
- Publisher
- Wiley
- Published
- 2019-08-06
- Volume
- 71
- Issue
- 9
- Pages
- 1400-1412
- ISSN
- 2326-5191 (ISSN-L)
- 2326-5205
- PMC
- PMC6827566
- OpenAlex
- W2965565685
- Cited by
- 2,677
- References
- 36
- Citations by year
- 2026: 428 · 2025: 497 · 2024: 447 · 2023: 420 · 2022: 367 · 2021: 301 · 2020: 184 · 2019: 27 · 2017: 1 · 2013: 2
- FWCI
- 166.73
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Arthritis & Rheumatology (publishedVersion)
- PubMed (publishedVersion)
- White Rose Research Online (University of Leeds, The University of Sheffield, University of York) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Deep Blue (University of Michigan) (submittedVersion)
- eScholarship (California Digital Library) (submittedVersion)
- CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion, other-oa)
- Funders
- European League Against Rheumatism
- National Institute of Arthritis and Musculoskeletal and Skin Diseases
- National Institutes of Health
- 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
- Adult, Antibodies, Antinuclear, Autoantibodies, Complement System Proteins, Decision Support Techniques, Delphi Technique, Europe, Female, Humans, International Cooperation, Lupus Erythematosus, Systemic, Male, Middle Aged, Rheumatology, Sensitivity and Specificity, Societies, Medical, United States, Cohort Studies, Antibodies, Antiphospholipid
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Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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