| ACR Criteria for Classification of Systemic Lupus Erythematosus [48, 49] | SLICC Criteria for the Classification of Systemic Lupus Erythematous [50] | ||
|---|---|---|---|
| (4 of 11 criteria)* | (4 of 17 criteria, including at least one clinical criterion and one immunologic criterion; OR biopsy proven lupus nephritis) Δ | ||
| 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 | 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 |
| Discoid Rash | Erythematosus raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions | 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's disease, infection [herpesvirus], inflammatory bowel disease, reactive arthritis, and acidic foods) |
| Arthritis | Nonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion | Joint Disease | Synovitis involving two or more joints, characterized by swelling or effusion OR Tenderness in two 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 one 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 one day, pericardial effusion, pericardial rub, or pericarditis by electrocardiography in the absence of other causes, such as infection, uremia, and Dressler's 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 two or more occasions OR Lymphopenia – Less than 1500/mm3 on two 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's 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) | |||
| Thrombocyto-penia | 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 finding of antiphospholipid antibody based on an abnormal serum level of IgG or IgM anticardiolipin antibodies, on a positive test result for lupus anticoagulant using a standard method, or on a false-positive serologic test for syphilis known to be positive for at least six months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test |
Anti-dsDNA | Anti-dsDNA antibody level above laboratory reference range (or >twofold 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 | ||