Classification of Systemic Lupus Erythematosus

1982 Criteria including 1997 revisions

The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have systemic lupus erythematosus if any 4 or more of the 11 criteria are 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 or more peripheral joints, characterized by tenderness, swelling, or effusion

6. Serositis a) Pleuritis--convincing history of pleuritic pain or rubbing heard by a physician or evidence of pleural effusion

OR

b) Pericarditis--documented by ECG or rub or evidence of pericardial effusion

7. Renal disorder a) Persistent proteinuria greater than 0.5 grams per day or grater than 3+ if quantitation not performed

OR

b) Cellular casts--may be red cell, hemoglobin, granular, tubular, or mixed

8. Neurologic disorder a) Seizures--in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance

OR

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

9. Hematologic disorder a) Hemolytic anemia--with reticulocytosis

OR

b) Leukopenia--less than 4,000/mm<>3<> total on 2 or more occasions

OR

c) Lyphopenia--less than 1,500/mm<>3<> on 2 or more occasions

OR

d) Thrombocytopenia--less than 100,000/mm<>3<> in the absence of offending drugs

10. Immunologic disorder

a) Anti-DNA: antibody to native DNA in abnormal titer

OR

b) Anti-Sm: presence of antibody to Sm nuclear antigen

OR

c) Antiphospholipid antibodies based on 1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, 2) a positive test result for lupus anticoagulant using a standard method, or 3) 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 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