1. Acute cutaneous lupus, including: |
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 psoriasiform and/or annular polycyclic lesions that resolve without scarring, although occasionally with post-inflammatory dyspigmentation or telangiectasias) |
2. Chronic cutaneous lupus, including: |
Classical discoid rash |
Localized (above the neck) |
Generalized (above and below the neck) |
Hypertrophic (verrucous) lupus |
Lupus panniculitis (Profundis) |
Mucosal lupus |
Lupus erythematosus tumidus |
Chilblains lupus |
Discoid lupus/lichen planus overlap |
3. Oral 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
|
4. Non-scarring 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. |
5. 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 |
6. Serositis |
Typical pleurisy for more than one day |
OR pleural effusions |
OR pleural rub |
Typical pericardial pain (pain with recumbency improved by sitting forward) for more than one day |
OR pericardial effusion |
OR pericardial rub |
OR pericarditis by electrocardiography |
In the absence of other causes such as infection, uremia, and Dressler’s pericarditis
|
7. Renal |
Urine protein-to-creatinine ratio (or 24-hour urine protein) equal to or greater than 500 mg protein/24 hours OU red blood cell casts |
8. 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
|
Acute confusional state |
In the absence of other causes, including toxic/metabolic, uremia, drugs
|
9. Hemolytic anemia |
10. 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 corticosteroids, drugs, and infection |
11. Thrombocytopenia (< 100,000/mm3 at least once) |
In the absence of other known causes such as drugs, portal hypertension, thrombotic thrombocytopenic purpura
|
Immunologic criteria
|
1. ANA level above laboratory reference range |
2. Anti-dsDNA antibody level above laboratory reference range (or 2-fold the reference range if tested by ELISA) |
3. Anti-Sm: the presence of antibody to Sm nuclear antigen |
4. Antiphospholipid antibody positivity, as determined by: |
Positive test for lupus anticoagulant |
False-positive test result for rapid plasma reagin |
Moderate titer anticardiolipin level (IgA, IgG, or IgM) |
Positive test result for anti-2-glycoprotein I (IgA, IgG, or IgM) |
5. Low complement |
Low C3 |
Low C4 |
Low CH50 |
6. Direct Coombs’ test in the absence of hemolytic anemia |