| 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 |
Pediatric lupus nephritis
