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 |