2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus

The entry criterion is necessary to classify SLE.
Entry criterion: ANA at a titer of ≥1:80 on HEp-2 cells or an equivalent positive test (ever).*
At least 1 clinical criterion required to classify SLE. Additional additive (clinical or immunology) criteria are counted toward the total score.
Additive criteria: Do not count a criterion if there is a more likely explanation than SLE. Occurrence of a criterion on ≥1 occasion is sufficient. Criteria need not occur simultaneously. Within each domain (eg, mucocutaneous, complement proteins), only the highest-weighted criterion is counted toward the total score if more than 1 is present.^¶
Clinical domains and criteria	Weight
Constitutional
Fever	2
Hematologic
Leukopenia	3
Thrombocytopenia	4
Autoimmune hemolysis	4
Neuropsychiatric
Delirium	2
Psychosis	3
Seizure	5
Mucocutaneous
Nonscarring alopecia	2
Oral ulcers	2
Subacute cutaneous or discoid lupus	4
Acute cutaneous lupus	6
Serosal
Pleural or pericardial effusion	5
Acute pericarditis	6
Musculoskeletal
Joint involvement	6
Renal
Proteinuria >0.5 g per 24 hours	4
Renal biopsy Class II or V lupus nephritis	8
Renal biopsy Class III or IV lupus nephritis	10
Immunology domains and criteria	Weight
Antiphospholipid antibodies
Anti-cardiolipin antibodies or anti-beta-2GP1 antibodies or lupus anticoagulant	2
Complement proteins
Low C3 or low C4	3
Low C3 and low C4	4
SLE-specific antibodies
Anti-dsDNA antibody^Δ or anti-Smith antibody	6
A total score of ≥10 and ≥1 clinical criterion are required to classify SLE.
Total score

SLE: systemic lupus erythematosus; ANA: antinuclear antibody; HEp-2: human epithelial type 2; anti-beta-2GP1: anti-beta-2 glycoprotein 1; anti-dsDNA: anti-double-stranded DNA.

* If ANA is absent, do not classify as SLE.

¶ Additional criteria within the same domain will not be counted.

Δ In an assay with 90% specificity against relevant disease controls.

From: Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol 2019; 71(9):1400-1412. https://onlinelibrary.wiley.com/doi/full/10.1002/art.40930. Copyright © 2019 American College of Rheumatology. Adapted with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).

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2019 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus