The European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) published criteria for the diagnosis of systemic lupus erythematosus (SLE) in 2019.
Initial criteria: ANA titer >= 1:80 on HEp-2 cells or equivalent
Additive criteria - findings should not be explained better by another diagnosis:
(1) clinical domains (16 scores)
(2) immunology domains (3 scores)
|
Clinical Domains |
Findings |
Points |
|
constitutional |
fever (temperature > 38.3°C) |
2 |
|
hematologic |
leukopenia (< 4,000 per µL) |
3 |
|
|
thrombocytopenia (<100,000 per µL) |
4 |
|
|
autoimmune hemolysis |
4 |
|
neuropsychiatric |
delirium |
2 |
|
|
psychosis |
3 |
|
|
seizures |
5 |
|
mucocutaneous |
non-scarring 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 |
|
|
biopsy: Class II nephritis |
8 |
|
|
biopsy: Class III nephritis |
10 |
|
|
biopsy: Class IV nephritis |
10 |
|
|
biopsy: Class V nephritis |
8 |
|
Immunology Domains |
Findings |
Points |
|
Antiphospholipid Antibodies |
lupus anticoagulant OR anticardiolipin antibody OR anti-beta2GP1 antibodies |
2 |
|
Complement Proteins |
low C3 OR low C4 |
3 |
|
|
low C3 AND low C4 |
4 |
|
SLE-specific Antibodies |
anti-double stranded DNA antibody OR anti-Smith antibody |
6 |
subscore for clinical domains =
= SUM(MAX(points for each criterion))
subscore for immunology domains =
= SUM(points for all clinical parameters)
total score =
= (points for clinical domains) + (points for immunology domains)
Interpretation:
• minimum subscores: 0
• maximum clinical subscore: 39
• maximum immunology subscore: 12
• maximum total score: 51
Criteria for a diagnosis of SLE:
(1) ANA titer
(2) total score >=10
(3) at least 1 clinical criterion
Specialty: Immunology/Rheumatology
