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

Fever

Fever >38.3°C

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 <100×109/L

4

Leukopenia <4×109/L

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.