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EULAR/ACR Criteria for the Classification of Systemic Lupus Erythematosus[a]

Domain[b]

Weight[c]

Clinical domains

Constitutional:

  • Fever (> 38° C)

  1. 2

Hematologic:

  • Leukopenia (< 4000/mcL)

  • Thrombocytopenia (platelet count < 100,000/mcL)

  • Autoimmune hemolysis[d]

  1. 3

  2. 4

  3. 4

Neuropsychiatric:

  • Delirium

  • Psychosis

  • Seizure (generalized or partial/focal)

  1. 2

  2. 3

  3. 5

Mucocutaneous:

  1. 2

  2. 2

  3. 4

  4. 6

Serosal:

  • Pleural effusion or pericardial effusion

  • Acute pericarditis

  1. 5

  2. 6

Musculoskeletal:

  • Joint involvement[f]

  1. 6

Renal:

  • Proteinuria (> 0.5 g/24 hours)

  • Renal biopsy class II or V lupus nephritis

  • Renal biopsy class III or IV lupus nephritis

  1. 4

  2. 8

  3. 10

Immunologic domains

Antiphospholipid antibodies:

  • Anticardiolipin antibodies or

  • Anti-beta2 glycoprotein 1 antibodies or

  • Lupus anticoagulant

  3. 2

Complement proteins:

  • Low C3 or low C4

  • Low C3 and low C4

  1. 3

  2. 4

SLE-specific antibodies:

  • Anti-dsDNA antibody or

  • Anti-Smith antibody

  2. 6

[a] Patients are eligible for these criteria only if they have a positive ANA ≥ 1:80.

[b] Criteria do not need to occur simultaneously. Only the highest-weighted criterion score within a single domain should be used. SLE must be the most likely explanation for each criterion.

[c] Each criterion is assigned a weight of 2 to 10. If the patient's score is 10 or more, and at least one clinical criterion is fulfilled, disease is classified as SLE.

[d] Evidence of autoimmune hemolysis (such as the presence of reticulocytosis, low haptoglobin, elevated indirect bilirubin, elevated lactate dehydrogenase) and a positive direct antiglobulin (direct Coombs) test.

[e] This criterion may be noted during physical examination or review of a photo.

[f] Joint involvement is defined as either synovitis involving ≥ 2 joints characterized by swelling or effusion or tenderness in ≥ 2 joints and at least 30 minutes of morning stiffness.

ANA = antinuclear antibodies; anti-dsDNA = anti–double-stranded (ds) DNA; EULAR/ACR = European League Against Rheumatism/American College of Rheumatology; SLE = systemic lupus erythematosus.

Adapted 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 71(9):1400–1412, 2019. doi: 10.1002/art.40930