Supplementary Table 1. The ACR and SLICC classification criteria for SLE
(case definitions of the criteria may differ between the two classifications)
ACR criteria (1997 update) 1,2
1. Malar rash. Fixed erythema, flat or raised, over the malar
eminences, tending to spare the nasolabial folds
SLICC criteria (2012) 3
1. Acute cutaneous lupus (lupus malar rash [do not count if malar
discoid], bullous lupus, toxic epidermal necrolysis variant of
SLE, maculopapular lupus rash, photosensitive lupus rash), or
subcute cutaneous lupus (nonindurated psoriaform and/or
annular polycyclic lesions that resolve without scarring)
2. Discoid rash. Erythematous raised patches with adherent
keratotic scaling and follicular plugging; atrophic scarring
occur in older lesions
2. Chronic cutaneous lupus (classic discoid rash: localized or
generalized, hypertrophic [verrucous] lupus, lupus panniculitis
[profundus], mucosal lupus, lupus erythematosus tumidus,
chillblains lupus, discoid lupus/lichen planus overlap
3. Photosensitivity. Skin rash as a result of unusual reaction to
sunlight, by patient history or physician observation
3. Nonscarring alopecia (diffuse thinning or hair fragility with
visible broken hairs)
Ulcers
Synovitis
4. Oral or nasopharyngeal ulceration
5. Non-erosive arthritis involving ≥2 peripheral joints,
characterized by tenderness, swelling or effusion
Serositis
6. Any of:
a. Pleuritis: convincing history of pleuritic pain or rub heard
by a physician or evidence of pleural effusion
b. Pericarditis: documented by ECG or rub or evidence of
pericardial effusion
Renal
disorder
7. Any of:
a. Persistent proteinuria >0.5 g per day or >3+ if quantitation
is not performed
b. Cellular casts: red cell, haemoglobin, granular tubular, or
mixed
4. Oral or nasal ulcers
5. Inflammatory synovitis in ≥2 joints:
a. characterized by swelling or effusion, or
b. tenderness and ≥30 minutes of morning stiffness
6. Serositis: any of
a. Typical pleurisy lasting >1 day, or pleural effusions, or
pleural rub
b. Typical pericardial pain (pain with recumbency improved
by sitting forward) for >1 day, or pericardial effusion, or
pericardial rub, or pericarditis by electrocardiography
7. Any of:
a. Urine protein/creatinine (or 24 hr urine protein)
representing ≥500 mg of protein/24 hr, or
b. red blood cell casts
Criteria
Skin
Neurological
disorder
8. Any of:
a. Seizures: in the absence of offending drugs or known
metabolic derangements
b. Psychosis: in the absence of offending drugs or known
metabolic derangements
Haematologic 9. Any of:
a. Haemolytic anemia with reticulocytosis
disorder
b. Lymphopenia: <1500/mm3
c. Thrombocytopenia: <100,000/mm3
Immunologic 10. Any of:
a. Anti-DNA: antibody to native DNA in abnormal titer
disorder
b. Anti-Sm: presence of antibody to Sm nuclear antigen
c. Positive finding of antiphospholipid antibodies based on:
(1) an abnormal serum concentration of IgG or IgM
anticardiolipin antibodies, (2) a positive test result for SLE
anticoagulant, or (3) a false positive serologic test for
syphilis known to be positive for ≥6 months and confirmed
by Treponema pallidum immobilization or fluorescent
treponemal antibody absorption test
11. Abnormal titer of ANA by immunofluorescence or an
Antinuclear
equivalent assay at any point in time and in the absence of
antibody
drugs known to be associated with “drug-induced SLE”
syndrome
Diagnosis of
SLE
At least 4 out of 11 criteria
Abbreviations: ANA, antinuclear antibody; SLE systemic lupus erythematosus.
8. Any of:
a. seizures,
b. psychosis,
c. mononeuritis multiplex,
d. myelitis,
e. peripheral or cranial neuropathy,
f. cerebritis (acute confusional state)
9. Haemolytic anaemia
10. Leukopenia (<4000/mm3 at least once), or lymphopenia
(<1000/mm3 at least once)
11. Thrombocytopenia (<100,000/mm3) at least once
12. Anti-dsDNA above laboratory reference range (except ELISA:
twice above laboratory reference range)
13. Anti-Sm
14. Antiphospholipid antibody, lupus anticoagulant, false-positive
test for syphilis, anticardiolipin (at least twice normal or
medium-high titer), or anti-b2 glycoprotein 1
15. Low complement: low C3, or low C4, or low CH50
16. Direct Coombs test in the absence of haemolytic anaemia
17. ANA above laboratory reference range
Either the biopsy-proven lupus nephritis in the presence of ANA or
anti-dsDNA as a “stand alone” criterion, OR
four criteria with at least one of the clinical and one of the
immunologic/ANA criteria
1.
Tan, E.M. et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25, 1271-7 (1982).
2.
Hochberg, M.C. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus
erythematosus. Arthritis Rheum 40, 1725 (1997).
3.
Petri, M. et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic
lupus erythematosus. Arthritis Rheum 64, 2677-86 (2012).