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Criterion
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Definition
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| Clinical criteria
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Acute cutaneous lupus
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- Lupus malar rash
- Fixed erythema, fl at or raised, over the malar eminences, tending to spare the nasolabial folds
- Bullous lupus
- Toxic epidermal necrolysis variant of SLE
- Maculopapular lupus rash
- Photosensitive lupus rash (in the absence of dermatomyositis);
- Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation OR
- Subacute cutaneous lupus
- Nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias
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| Chronic cutaneous lupus
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- 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
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| Nonscarring alopecia
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- 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)
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| Oral or nasal ulcers
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- 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)
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| Joint disease
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- 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
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| Serositis
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- Typical pleurisy for more than one day, pleural effusions, or pleural rub, OR
- 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
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| Renal
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- Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours, OR
- Red blood cell casts
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| Neurologic
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- 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)
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| Hematologic criteria
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Hemolytic anemia
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| Leukopenia or lymphopenia
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- 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)
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| Thrombocytopenia
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- Thrombocytopenia (<100,000/mm3) at least once in the absence of other known causes, such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura
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| ANA
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- ANA level above laboratory reference range
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| Anti-dsDNA
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- Anti-dsDNA antibody level above laboratory reference range (or >twofold the reference range if tested by ELISA)
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| Anit-SM
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- Presence of antibody to Sm nuclear antigen
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| Immunologic criteria
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Antiphospholipid
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- 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)
- Positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM)
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| Low complement
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- Low C3 OR
- Low C4 OR
- Low CH50
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| Direct Coombs' test
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- Direct Coombs' test in the absence of hemolytic anemia
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