Table 15.21-1. 2019 EULAR/ACR classification criteria for systemic lupus erythematosus

Only one criterion, with the highest score, should count per each domain. It is sufficient to meet each criterion once and not necessarily simultaneously. Do not count a given criterion if it is likely explained by other conditions. Classification as SLE requires ≥10 points and ≥1 clinical criterion present.

Entry criterion: ANA titer ≥1/80a

Clinical domain

Criteria

Points

Comments

Fever

Fever >38.3 degrees Celsius

2

 

Mucous membrane and skin

Acute cutaneous lupus

6

Observed by a clinician (direct examination or review of photographs)

Subacute cutaneous or discoid lupus

4

Oral ulcers

2

Nonscarring alopecia

2

Musculoskeletal

Joint involvement

6

≥2 joints involved with synovitis (swelling/effusion)

or

Tenderness and ≥30 min of morning stiffness

Serous membranes

Acute pericarditis

6

 

Pleural or pericardial effusion

5

On any of ultrasonography, radiography, CT, or MRI

Renal involvement

Renal biopsy class III or IV

10

 

Renal biopsy class II or V

8

 

Proteinuria >0.5 g/24 h

4

 

Neurologic and psychiatric

Seizures

5

Generalized or partial/focal

Psychosis

3

Delusions or hallucinations without insight and with absence of delirium

Delirium

2

 

Hematologic

Autoimmune hemolysis

4

Positive Coombs (direct antiglobulin) test

and

Low haptoglobin, elevated indirect bilirubin, elevated LDH

Thrombocytopenia <100,000/microL

4

 

Leukopenia <4000/microL

3

 

SLE-specific antibodies

Anti-dsDNA or

anti-Sm

6

 

Antiphospholipid antibodies

Anti-cardiolipin,

anti-beta2GPI,

lupus anticoagulant

2

 

Complement protein

Low C3 and low C4

4

 

Low C3 or low C4

3

 

a Testing using HEp-2 cells or equivalent.

Based on Arthritis Rheumatol. 2019;71(9):1400-1412.

ACR, American College of Rheumatology; ANA, antinuclear antibody; CT, computed tomography; dsDNA, double-stranded DNA; EULAR, European League Against Rheumatism; GPI, glycoprotein I; LDH, lactate dehydrogenase; MRI, magnetic resonance imaging; SLE, systemic lupus erythematosus.