SupplementaryTable 1.The ACR and SLICC classification criteria for SLE

(case definitions of the criteria may differ between the two classifications)

Criteria / ACR criteria (1997 update) 1,2 / SLICC criteria (2012) 3
Skin /
  1. Malar rash. Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
/
  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 (noninduratedpsoriaform and/or annular polycyclic lesions that resolve without scarring)

  1. Discoid rash. Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring occur in older lesions
/
  1. Chronic cutaneous lupus (classic discoid rash: localized or generalized, hypertrophic [verrucous] lupus, lupus panniculitis [profundus], mucosal lupus, lupus erythematosustumidus, chillblains lupus, discoid lupus/lichen planus overlap

  1. Photosensitivity. Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation
/
  1. Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs)

Ulcers /
  1. Oral or nasopharyngeal ulceration
/
  1. Oral or nasal ulcers

Synovitis /
  1. Non-erosive arthritis involving ≥2 peripheral joints, characterized by tenderness, swelling or effusion
/
  1. Inflammatory synovitis in ≥2 joints:
  2. characterized by swelling or effusion, or
  3. tenderness and ≥30 minutes of morning stiffness

Serositis /
  1. Any of:
  1. Pleuritis: convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion
  2. Pericarditis: documented by ECG or rub or evidence of pericardial effusion
/
  1. Serositis: any of
  2. Typical pleurisy lasting>1 day, or pleural effusions, or pleural rub
  3. Typical pericardial pain (pain with recumbency improved by sitting forward) for >1 day, or pericardial effusion, or pericardial rub, or pericarditis by electrocardiography

Renal disorder /
  1. Any of:
  1. Persistent proteinuria >0.5 g per day or >3+ if quantitation is not performed
  2. Cellular casts: red cell, haemoglobin, granular tubular, or mixed
/
  1. Any of:
  2. Urine protein/creatinine (or 24 hr urine protein) representing ≥500 mg of protein/24 hr, or
  3. red blood cell casts

Neurological disorder /
  1. Any of:
  1. Seizures: in the absence of offending drugs or known metabolic derangements
  2. Psychosis: in the absence of offending drugs or known metabolic derangements
/
  1. Any of:
  2. seizures,
  3. psychosis,
  4. mononeuritis multiplex,
  5. myelitis,
  6. peripheral or cranial neuropathy,
  7. cerebritis (acute confusional state)

Haematologic disorder /
  1. Any of:
  1. Haemolytic anemia with reticulocytosis
  2. Lymphopenia: <1500/mm3
  3. Thrombocytopenia: <100,000/mm3
/
  1. Haemolyticanaemia
  2. Leukopenia (<4000/mm3 at least once), or lymphopenia (<1000/mm3 at least once)
  3. Thrombocytopenia (<100,000/mm3) at least once

Immunologic disorder /
  1. Any of:
  1. Anti-DNA: antibody to native DNA in abnormal titer
  2. Anti-Sm: presence of antibody to Sm nuclear antigen
  3. Positive finding of antiphospholipid antibodies based on: (1) an abnormal serum concentration of IgG or IgManticardiolipin 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 byTreponemapallidum immobilization or fluorescent treponemal antibody absorption test
/
  1. Anti-dsDNA above laboratory reference range (except ELISA: twice above laboratory reference range)
  2. Anti-Sm
  3. Antiphospholipid antibody, lupus anticoagulant, false-positive test for syphilis, anticardiolipin (at least twice normal or medium-high titer), or anti-b2 glycoprotein 1
  4. Low complement: low C3, or low C4, or low CH50
  5. Direct Coombs test in the absence of haemolyticanaemia

Antinuclear antibody /
  1. Abnormal titer of ANA by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with “drug-induced SLE” syndrome
/
  1. ANA above laboratory reference range

Diagnosis of SLE / At least 4 out of 11 criteria / 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

Abbreviations: ANA, antinuclear antibody; SLE systemic lupus erythematosus.

1.Tan, E.M. et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum25, 1271-7 (1982).

2.Hochberg, M.C. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum40, 1725 (1997).

3.Petri, M. et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum64, 2677-86 (2012).