ACR criteria[1,2] | SLICC criteria[3] | ||
(4 of 11 criteria)* | (4 of 17 criteria, including at least 1 clinical criterion and 1 immunologic criterion;¶ OR biopsy-proven lupus nephritisΔ) | ||
Criterion | Definition | Criterion | Definition |
Clinical criteria | |||
Malar rash | Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds | 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 (in the absence of dermatomyositis); OR subacute cutaneous lupus (nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias) |
Photosensitivity | Skin rash as a result of unusual reaction to sunlight, by patient history or clinician observation | ||
Discoid rash | Erythematosus raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions | Chronic cutaneous lupus | Classic discoid rash; localized (above the neck); generalized (above and below the neck); hypertrophic (verrucous) lupus; lupus panniculitis (profundus); mucosal lupus; lupus erythematosus tumidus; chilblains lupus; OR discoid lupus/lichen planus overlap |
Nonscarring alopecia | Diffuse thinning or hair fragility with visible broken hairs (in the absence of other causes, such as alopecia areata, drugs, iron deficiency, and androgenic alopecia) | ||
Oral ulcers | Oral or nasopharyngeal ulceration, usually painless, observed by a clinician | Oral or nasal ulcers | Palate, buccal, tongue, OR nasal ulcers (in the absence of other causes, such as vasculitis, Behçet syndrome, infection [herpesvirus], inflammatory bowel disease, reactive arthritis, and acidic foods) |
Arthritis | Nonerosive arthritis involving 2 or more peripheral joints, characterized by tenderness, swelling, or effusion | Joint disease | Synovitis involving 2 or more joints, characterized by swelling or effusion OR |
Tenderness in 2 or more joints and at least 30 minutes of morning stiffness | |||
Serositis | Pleuritis – Convincing history of pleuritic pain or rubbing heard by a clinician or evidence of pleural effusion OR | Serositis | Typical pleurisy for more than 1 day, pleural effusions, or pleural rub, OR |
Pericarditis – Documented by ECG, rub, or evidence of pericardial effusion | Typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day, pericardial effusion, pericardial rub, or pericarditis by electrocardiography in the absence of other causes, such as infection, uremia, and Dressler syndrome | ||
Renal disorder | Persistent proteinuria greater than 500 mg/24 hours or greater than 3+ if quantitation not performed OR | Renal | Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours, OR |
Cellular casts – May be red cell, hemoglobin, granular, tubular, or mixed | Red blood cell casts | ||
Neurologic disorder | Seizures OR psychosis – In the absence of offending drugs or known metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance) | Neurologic | Seizures; psychosis; mononeuritis multiplex (in the absence of other known causes, such as primary vasculitis); myelitis; peripheral or cranial neuropathy (in the absence of other known causes, such as primary vasculitis, infection, and diabetes mellitus); OR acute confusional state (in the absence of other causes, including toxic/metabolic, uremia, drugs) |
Hematologic disorder | Hemolytic anemia – With reticulocytosis OR Leukopenia – Less than 4000/mm3 total on 2 or more occasions OR Lymphopenia – Less than 1500/mm3 on 2 or more occasions OR Thrombocytopenia – Less than 100,000/mm3 (in the absence of offending drugs) | Hemolytic anemia | Hemolytic anemia |
Leukopenia or lymphopenia | Leukopenia (<4000/mm3 at least once) (in the absence of other known causes, such as Felty syndrome, drugs, and portal hypertension), OR | ||
Lymphopenia (<1000/mm3 at least once) (in the absence of other known causes, such as glucocorticoids, drugs, and infection) | |||
Thrombocytopenia | Thrombocytopenia (<100,000/mm3) at least once in the absence of other known causes, such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura | ||
Immunologic criteria | |||
ANA | An 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 lupus" syndrome | ANA | ANA level above laboratory reference range |
Immunologic disorders | Anti-DNA – Antibody to native DNA in abnormal titer OR Anti-Sm – Presence of antibody to Sm nuclear antigen OR Positive antiphospholipid antibody on:
| Anti-dsDNA | Anti-dsDNA antibody level above laboratory reference range (or >2-fold the reference range if tested by ELISA) |
Anti-Sm | Presence of antibody to Sm nuclear antigen | ||
Antiphospholipid | Antiphospholipid antibody positivity as determined by any of the following: Positive test result for lupus anticoagulant; false-positive test result for rapid plasma reagin; medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM); or positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM) | ||
Low complement | Low C3; low C4; OR low CH50 | ||
Direct Coombs test | Direct Coombs test in the absence of hemolytic anemia |