Table 1: American College of Rheumatology (ACR) revised classification criteria for systemic lupus erythematosus.

CriteriaDefinition

Malar rashFixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds

Discoid rashErythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring occurs in older lesions

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

Oral ulcersOral or nasopharyngeal ulceration, usually painless, observed by a physician

ArthritisNonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion

Serositis(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(a) Persistent proteinuria > 0.5 g/day > 3+ if quantitation is not performed
(b) Cellular casts—may be red blood cell, hemoglobin, granular tubular, mixed

Neurologic disorder(a) Seizures—in the absence of offending drugs or known metabolic derangements (e.g., uremia, acidosis, or  electrolyte imbalance)
(b) Psychosis—in the absence of offending drugs or known metabolic derangements (e.g., uremia, acidosis,  or electrolyte imbalance)

Hematologic disorder(a) Hemolytic anemia with reticulocytosis
(b) Leukopenia—<4000/mm
(c) Lymphopenia—<1500/mm
(d) Thrombocytopenia—<100,000/mm in the absence of offending drugs

Immunologic disorder(a) Anti-DNA—antibody to native DNA in abnormal titer
(b) Anti-Sm—presence of antibody to Sm nuclear antigen
(c) Positive finding of antiphospholipid antibodies based on (1) abnormal serum concentration of IgG or  IgM anticardiolipin antibodies, (2) positive test result for lupus anticoagulant using a standard method,  or (3) false-positive serologic test for syphilis known to be positive for at least 6 mo and confirmed by  Treponema pallidum immobilization or fluorescent treponemal antibody absorption test

ANAAbnormal titer of ANA by immunofluorescence or equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome