Clinical criteria
Acute cutaneous – lupus malar rash, bullous lupus, toxic epidermal necrolysis variant of lupus, maculopapular lupus rash, photosensitive lupus rash
Chronic cutaneous – discoid lupus rash, hypertrophic (verrucous) lupus, lupus panniculitis, mucosal lupus, lupus erythematosus tumidus, chilblains lupus, discoid lupus
Oral or nasal ulcers – palate, buccal, tongue, or nasal in the absence of other causes
Non-scarring alopecia – in the absence of other causes
Arthritis – synovitis involving 2 or more joints, characterised by swelling or effusion or tenderness in 2 or more joints and at least 30 minutes of morning stiffness
Serositis – typical pleurisy for more than one day, pleural effusions, pleural rub or typical pericardial pain for more than one day, pericardial effusion, pericardial rub or pericarditis by ECG
Renal involvement – urine protein:creatinine ratio or 24-hour urine protein with >500 mg protein/24 hours or red cell casts
Neurological symptoms – seizures, psychosis, mononeuritis multiplex, myelitis, peripheral or cranial neuropathy, acute confusional state
Haemolytic anaemia – in the absence of other causes
Leucopenia – in the absence of other causes
Thrombocytopenia – in the absence of other causes
Immunological criteria
Antinuclear antibodies
Anti-dsDNA antibodies – above reference range (or >2-fold if tested by ELISA)
Anti-Smith antibodies
Antiphospholipid antibodies – positive lupus anticoagulant, false-positive rapid plasma reagin test, medium- or high-titre cardiolipin antibody, positive anti-b2-glycoprotein
Low complement C3, C4, CH50
Positive Direct Coombs test – in the absence of haemolytic anaemia
* For a positive diagnosis, patients must have 4 or more of the listed criteria, with at least 1 clinical and 1 laboratory criterion
dsDNA double-stranded DNA
ELISA enzyme-linked immunosorbent assay
Source: reference 1