What are EULAR/ACR diagnostic criteria for systemic lupus erythematosus (SLE)?

Updated: Aug 04, 2021
  • Author: Christie M Bartels, MD, MS; Chief Editor: Herbert S Diamond, MD  more...
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Answer

In September 2019, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) published new criteria for the classification of SLE. [5, 6]  The EULAR/ACR criteria have sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the 1997 ACR criteria, and 96.7% sensitivity and 83.7% specificity of the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. [7, 101]  

The EULAR/ACR classification requires an antinuclear antibody (ANA) titer of at least 1:80 on HEp-2 cells or an equivalent positive test at least once; otherwise, the patient is considered not to have SLE. If it is present, 22 "additive weighted" classification criteria are considered, comprising seven clinical domains and three immunologic domains (see Tables 1 and 2, below). Each criterion is assigned points, ranging from 2 to 10. Patients with at least one clinical criterion and 10 or more points are classified as having SLE.

Table 1. EULAR/ACR Clinical Domains and Criteria for SLE (Open Table in a new window)

 Domain

Criteria

Points

Constitutional

Fever

2

Hematologic

Leukopenia

Thrombocytopenia

Autoimmune hemolysis

3

4

4

Neuropsychiatric

Delirium

Psychosis

Seizure

2

3

5

Mucocutaneous

Non-scarring alopecia

Oral ulcers

Subacute cutaneous or discoid lupus

Acute cutaneous lupus

2

2

4

6

Serosal

Pleural or pericardial effusion

Acute pericarditis

5

6

Musculoskeletal

Joint involvement

6

Renal

Proteinuria > 0.5 g/24 h

Renal biopsy class II or V lupus nephritis

Renal biopsy class III or IV lupus nephritis

4

8

10

Table 2. EULAR/ACR Immunologic Domains and Criteria for SLE (Open Table in a new window)

Domain

Criteria

Points

Antiphospholipid antibodies

Anti-cardiolipin antibodies or

Anti-β2GP1 antibodies or

Lupus anticoagulant

2

Complement proteins

Low C3 or low C4

Low C3 and low C4

3

4

SLE-specific antibodies

Anti-dsDNA antibody or

Anti-Smith antibody

6

Note the following [5, 6] :

  • A criterion should not be counted if there is a more likely explanation for it than SLE
  • Occurrence of a criterion on at least one occasion is sufficient
  • Criteria need not occur simultaneously
  • Within each domain, only the highest-weighted criterion is counted toward the total score

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