2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus
- PMID: 31383717
- DOI: 10.1136/annrheumdis-2018-214819
2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus
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.
© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.
Conflict of interest statement
Competing interests: None declared.
Comment in
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New 2019 SLE EULAR/ACR classification criteria are valuable for distinguishing patients with SLE from patients with pSS.Ann Rheum Dis. 2021 Aug;80(8):e122. doi: 10.1136/annrheumdis-2019-216222. Epub 2019 Sep 9. Ann Rheum Dis. 2021. PMID: 31501139 No abstract available.
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Do the 2019 EULAR/ACR SLE classification criteria close the door on certain groups of SLE patients?Ann Rheum Dis. 2021 Aug;80(8):e126. doi: 10.1136/annrheumdis-2019-216253. Epub 2019 Sep 16. Ann Rheum Dis. 2021. PMID: 31527019 No abstract available.
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Response to: 'New 2019 SLE EULAR/ACR classification criteria are valuable for distinguishing patients with SLE from patients with pSS' by Assan et al.Ann Rheum Dis. 2021 Aug;80(8):e123. doi: 10.1136/annrheumdis-2019-216250. Epub 2019 Sep 18. Ann Rheum Dis. 2021. PMID: 31533932 No abstract available.
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Role of ANA testing in the classification of patients with systemic lupus erythematosus.Ann Rheum Dis. 2021 Aug;80(8):e124. doi: 10.1136/annrheumdis-2019-216259. Epub 2019 Sep 20. Ann Rheum Dis. 2021. PMID: 31540932 No abstract available.
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Response to: 'Do the 2019 EULAR/ACR SLE classification criteria close the door on certain groups of SLE patients?' by Chi et al.Ann Rheum Dis. 2021 Aug;80(8):e127. doi: 10.1136/annrheumdis-2019-216338. Epub 2019 Oct 4. Ann Rheum Dis. 2021. PMID: 31585929 No abstract available.
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Response to: 'Role of ANA testing in the classification of patients with systemic lupus erythematosus' by Pisetsky et al.Ann Rheum Dis. 2021 Aug;80(8):e125. doi: 10.1136/annrheumdis-2019-216337. Epub 2019 Oct 11. Ann Rheum Dis. 2021. PMID: 31604707 No abstract available.
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Development and initial validation of diagnostic gene signatures for systemic lupus erythematosus.Ann Rheum Dis. 2021 Dec;80(12):e192. doi: 10.1136/annrheumdis-2019-216695. Epub 2019 Dec 6. Ann Rheum Dis. 2021. PMID: 31811057 No abstract available.
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New 2019 SLE EULAR/ACR classification criteria are valid for identifying patients with SLE among patients admitted for pericardial effusion.Ann Rheum Dis. 2021 Dec;80(12):e190. doi: 10.1136/annrheumdis-2019-216712. Epub 2019 Dec 6. Ann Rheum Dis. 2021. PMID: 31811058 No abstract available.
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Response to: 'Development and initial validation of diagnostic gene signatures for systemic lupus erythematosus' by Wang et al.Ann Rheum Dis. 2021 Dec;80(12):e193. doi: 10.1136/annrheumdis-2019-216807. Epub 2019 Dec 20. Ann Rheum Dis. 2021. PMID: 31862701 No abstract available.
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Response to: 'New 2019 SLE EULAR/ACR classification criteria are valid for identifying patients with SLE among patients admitted for pericardial effusion' by Sacre et al.Ann Rheum Dis. 2021 Dec;80(12):e191. doi: 10.1136/annrheumdis-2019-216803. Epub 2019 Dec 20. Ann Rheum Dis. 2021. PMID: 31862703 No abstract available.
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Response to: 'Do 2019 European League against rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus also indicate the disease activity?' by Teng et al.Ann Rheum Dis. 2022 Feb;81(2):e23. doi: 10.1136/annrheumdis-2020-217105. Epub 2020 Mar 2. Ann Rheum Dis. 2022. PMID: 32122907 No abstract available.
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Do 2019 European League against rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus also indicate the disease activity?Ann Rheum Dis. 2022 Feb;81(2):e22. doi: 10.1136/annrheumdis-2020-217017. Epub 2020 Mar 2. Ann Rheum Dis. 2022. PMID: 32122908 No abstract available.
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