Clinical criteria
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:
· Classic discoid rash
· Localized (above the neck)
· Generalized (above and below the neck)
· Hypertrophic (verrucous) lupus
· Lupus panniculitis (profundus)
· Mucosal lupus
· Lupus erythematosus tumidus
· Chilblainsns lupus
· Discoid lupus/lichen planus overlap
3. Oral ulcers
· Palate
· Buccal
· Tongue
· OR nasal ulcers
· in the absence of other causes, such as vasculitis, Behc¸et’s disease, infection (herpesvirus), inflammatory bowel disease, reactive arthritis,
· and acidic foods
4. Nonscarring alopecia (diffuse thinning or hair fragility with visibly broken hairs)
in the absence of other causes such as alopecia areata, drugs, iron deficiency, and androgenic alopecia
5. Synovitis involving 2 or more joints, characterized by swelling or effusion
OR
Tenderness in 2 or more joints and at least 30 minutes of morning stiffness
6. Serositis
· Typical pleurisy for more than 1 day
OR pleural effusions
OR pleural rub
· Typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 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) representing 500 mg protein/24 hours
OR 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 (_4,000/mm3 at least once)
in the absence of other known causes such as Felty’s syndrome, drugs, and portal hypertension
OR
Lymphopenia (_1,000/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, and thrombotic thrombocytopenic purpura
Immunologic criteria
1. ANA level above the 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 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–_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
Note -* Criteria are cumulative and need not be present concurrently. SLICC _ Systemic Lupus International Collaborating Clinics; SLE _ systemic lupus erythematosus;
ANA _ antinuclear antibody; anti-dsDNA _ anti-double-stranded DNA; ELISA _ enzyme-linked immunosorbent assay. |