| 1) Acute cutaneous lupus |
| a) including lupus malar rash (do not count if malar discoid) |
| i) bullous lupus |
| ii) toxic epidermal necrolysis variant of SLE |
| iii) maculopapular lupus rash |
| iv) photosensitive lupus rash |
| (1) in the absence of dermatomyositis |
| b) or subacute cutaneous lupus |
| i) nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias) |
| 2) Chronic cutaneous lupus |
| a) including classical discoid rash |
| i) localized (above the neck) |
| ii) generalized (above and below the neck) |
| b) hypertrophic (verrucous) lupus |
| c) lupus panniculitis (profundus) |
| d) mucosal lupus |
| e) lupus erythematosus tumidus |
| f) chillblains lupus |
| g) discoid lupus/lichen planus overlap |
| 3) Oral ulcers: palate |
| a) buccal |
| b) tongue |
| c) or nasal ulcers |
| d) in the absence of other causes, such as vasculitis, Behcets, infection (herpes), inflammatory bowel disease, reactive arthritis, and acidic foods |
| 4) Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs) |
| a) 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 2 or more joints and thirty minutes or more of morning stiffness. |
| 6) Serositis |
| a) typical pleurisy for more than 1 day |
| i) or pleural effusions |
| ii) or pleural rub |
| b) typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day |
| i) or pericardial effusion |
| ii) or pericardial rub |
| iii) or pericarditis by EKG |
| (1) in the absence of other causes, such as infection, uremia, and Dressler's pericarditis |
| 7) Renal |
| a) Urine protein/creatinine (or 24 hr urine protein) representing 500 mg of protein/24 hr or |
| b) Red blood cell casts |
| 8) Neurologic |
| a) seizures |
| b) psychosis |
| c) mononeuritis multiplex |
| i)in the absence of other known causes such as primary vasculitis |
| d) myelitis |
| e) peripheral or cranial neuropathy |
| i) in the absence of other known causes such as primary vasculitis, infection, and diabetes mellitus |
| f) acute confusional state |
| i) in the absence of other causes, including toxic-metabolic, uremia, drugs |
| 9) Hemolytic anemia |
| 10) Leukopenia (< 4,000/mm3 at least once) |
| i)in the absence of other known causes such as Felty's, drugs, and portal hypertension |
| b) Lymphopenia (< 1,000/mm3 at least once) |
| ii)in the absence of other known causes such as corticosteroids, drugs and infection |
| 11)Thrombocytopenia (<100,000/mm3) at least once |
| a)in the absence of other known causes such as drugs, portal hypertension, and TTP |
| Immunological Criteria |
| 1) ANA above laboratory reference range |
| 2) Anti-dsDNA above laboratory reference range, except ELISA: twice above laboratory reference range |
| 3) Anti-Sm |
| 4) Antiphospholipid antibody: any of the following |
| a) lupus anticoagulant |
| b) false-positive RPR |
| c) medium or high titer anticardiolipin (IgA, IgG or IgM |
| d) anti- _2 glycoprotein I (IgA, IgG or IgM) |
| 5) Low complement |
| a) low C3 |
| b) low C4 |
| c) low CH50 |
| 6) Direct Coombs test in the absence of hemolytic anemia |
