Criteria

2019 European League Against Rheumatism/American College of Rheumatology classification system[1]

Entry criterion

  • Antinuclear antibodies (ANA) at a titer of ≥1:80 on HEp-2 cells or an equivalent positive test (ever)

  • If absent, do not classify as SLE; if present, apply additive criteria

Additive criteria

  • Additive criterion should not be counted if there is a more likely explanation than SLE

  • Occurrence of a criterion on at least one occasion is sufficient

  • SLE classification requires at least one clinical criterion and ≥10 points

  • Criteria need not occur simultaneously

  • Within each domain, only the highest criterion is counted toward the score*

Clinical domains and criteria

Constitutional

  • Fever, 2 points

Hematologic

  • Leukopenia, 3 points

  • Thrombocytopenia, 4 points

  • Autoimmune hemolysis, 4 points

Neuropsychiatric

  • Delirium, 2 points

  • Psychosis, 3 points

  • Seizure, 5 points

Mucocutaneous

  • Nonscarring alopecia, 2 points

  • Oral ulcers, 2 points

  • Subacute cutaneous OR discoid lupus, 4 points

  • Acute cutaneous lupus, 6 points

Serosal

  • Pleural or pericardial effusion, 5 points

  • Acute pericarditis, 6 points

Musculoskeletal

  • Joint involvement, 6 points

Renal

  • Proteinuria >0.5 g/24 hours, 4 points

  • Renal biopsy class II or V lupus nephritis, 8 points

  • Renal biopsy class III or IV lupus nephritis, 10 points

Immunology domains and criteria

Antiphospholipid antibodies

  • Anticardiolipin antibodies OR anti-beta2-glycoprotein 1 antibodies OR lupus anticoagulant, 2 points

Complement proteins

  • Low C3 OR low C4, 3 points

  • Low C3 AND low C4, 4 points

SLE-specific antibodies

  • Anti-double-stranded (ds)DNA antibody** OR anti-Smith antibody, 6 points

Total score

Scores of 10 or more are classified as systemic lupus erythematosus if the entry criterion has been fulfilled.

* Additional criteria items within the same domain will not be counted.

** In an assay with ≥90% specificity against relevant disease controls.

1997 update of the 1982 American College of Rheumatology revised criteria for the classification of SLE[2][3]

These criteria were initially developed to identify patients for clinical studies and were based on a white population. Any ≥4 of the 11 criteria are required to classify a patient as having SLE. These criteria can be present serially or simultaneously during any interval of observation.

  1. Malar rash

    • Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds.

  2. Discoid rash

    • Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions.

  3. Photosensitivity

    • Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation.

  4. Oral ulcers

    • Oral or nasopharyngeal ulceration, usually painless, observed by physician.

  5. Arthritis

    • Nonerosive arthritis involving ≥2 peripheral joints, characterized by tenderness, swelling, or effusion.

  6. Serositis (one of the following):

    • Pleuritis: convincing history of pleuritic pain, pleural rubs on auscultation, or evidence of pleural effusion

    • Pericarditis: documented by ECG, pericardial rub, or evidence of pericardial effusion.

  7. Renal disorder (one of the following):

    • Persistent proteinuria >0.5 g/day or >3+ if quantification not performed

    • Cellular casts: may be red cell, hemoglobin, granular, tubular, or mixed.

  8. Neurologic disorder (one of the following):

    • Seizures: in the absence of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance)

    • Psychosis: in the absence of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance).

  9. Hematologic disorder (one of the following):

    • Hemolytic anemia: with reticulocytes

    • Leukopenia: <4000/mm³ on ≥2 occasions

    • Lymphopenia: <1500/mm³ on ≥2 occasions

    • Thrombocytopenia: <100,000/mm³ in the absence of offending drugs.

  10. Immunologic disorder (one of the following):

    • Anti-DNA: presence of antibody to native DNA in abnormal titer

    • Anti-Smith: presence of antibody to Smith nuclear antigen

    • Positive findings of antiphospholipid antibodies based on:

      • An abnormal serum level of IgG or IgM anticardiolipin antibodies

      • Positive test result for lupus anticoagulant using a standard method

      • A false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test. 

  11. Antinuclear antibody (ANA)

    • An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome.

2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for systemic lupus erythematosus[4]

The SLICC criteria for SLE classification requires:

  1. Fulfillment of at least 4 criteria, with at least one clinical criterion AND one immunologic criterion

    OR

  2. Lupus nephritis as the sole clinical criterion in the presence of ANA or anti-dsDNA antibodies.

Clinical criteria:

  • Acute cutaneous lupus

  • Chronic cutaneous lupus

  • Oral ulcers: palate

  • Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs)

  • Synovitis involving two or more joints, characterized by swelling or effusion OR tenderness in two or more joints and 30 minutes or more of morning stiffness

  • Serositis

  • Renal

  • Neurologic

  • Hemolytic anemia

  • Leukopenia (<4000/mm³ at least once)

  • Thrombocytopenia (<100,000/mm³) at least once.

Immunologic criteria:

  • ANA above laboratory reference range

  • Anti-dsDNA above laboratory reference range, except enzyme-linked immunosorbent assay: twice above laboratory reference range

  • Anti-Smith

  • Antiphospholipid antibody: any of the following

  • Low complement

  • Direct Coombs test in the absence of hemolytic anemia.

Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices[5]

  • Class I: minimal mesangial lupus nephritis

  • Class II: mesangial proliferative lupus nephritis

  • Class III: focal lupus nephritis

  • Class III (A): active lesions - focal proliferative lupus nephritis

  • Class III (A/C):

    • Active and chronic lesions: focal proliferative and sclerosing lupus nephritis class III (C)

    • Chronic inactive lesions: focal sclerosing lupus nephritis

  • Class IV: diffuse lupus nephritis

  • Class V: membranous lupus nephritis

  • Class VI: advanced sclerosis lupus nephritis.

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