Systemic Lupus International Collaborating Clinics (SLICC) criteria used in the classification for SLE
The 2012 SLICC criteria replaced the 1997 ACR criteria because of possessing better sensitivity (97%) although specificity was less (84%). Any person who satisfies four or more of the following clinical and immunologic criteria including at least one clinical criterion and one immunologic criterion, or any person who has biopsy-proven lupus nephritis in the presence of a positive ANA or anti-dsDNA antibodies is considered to have SLE for the purposes of clinical studies.
Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus (2012)
|Criterion||Definition a||Frequency (%) b|
|1. Acute cutaneous lupus erythematosus||Malar rash, bullous lupus, toxic epidermal necrolysis variant, maculopapular, photosensitive, subacute cutaneous lupus||60–70|
|2. Chronic cutaneous lupus erythematosus||Classic discoid, hypertrophic, lupus panniculitis/profundus, mucosal, lupus erythematosus tumidus, chilblains lupus, discoid/lichen planus overlap||15–30|
|3. Alopecia||Nonscarring, diffuse hair thinning, or visible broken hairs||30–50|
|4. Oral ulcers||Oral (palate, buccal, tongue) or nasal ulceration||15–45|
|5. Synovitis||Arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion and morning stiffness > 30 minutes||90|
|6. Serositis||Pleuritis: convincing history of pleuritic pain for > 1 day or pleural rub or evidence of pleural effusion, or pericarditis documented by electrocardiogram or rub or evidence of pericardial effusion||30–60|
|7. Renal disorder||Persistent proteinuria ≥ 0.5 g/day (24 hour urine or urine protein/creatinine ratio) or red blood cell casts||40–60|
|8. Neurologic disorder||Seizures, psychosis, myelitis, mononeuritis multiplex, peripheral or cranial neuropathy, acute confusional state||15–20|
|9. Hemolytic anemia||Direct Coombs positive||5–10|
|10. Leukopenia||Leukopenia < 4000/mm 3 at least once, or Lymphopenia < 1000/mm 3 at least once||15–20|
|11. Thrombocytopenia||Platelets < 100,000/mm 3||15–20|
|1. ANA||Level above laboratory reference range||98|
|2. Anti-dsDNA||Level above laboratory reference range (or >2-fold enzyme-linked immunosorbent assay reference range)||60–70|
|3. Anti-Sm||Presence of antibody to Sm nuclear antigen||20–30|
|4. Antiphospholipid antibody positivity as determined by any of the following:• Positive test for lupus anticoagulant• False-positive test result for rapid plasma reagin• Medium- or high-titer anticardiolipin antibody level (IgG, IgM, or IgA)• Positive test result for anti-β2-glycoprotein I (IgG, IgM, or IgA)||30–50|
|5. Low complement||Low C3, low C4, or low CH50||55–60|
|6. Direct Coombs test||In the absence of hemolytic anemia||10–30|
a Other causes for the clinical and immunologic manifestations must be ruled out prior to attributing them to systemic lupus erythematosus.
b Frequency = chance of occurrence at any time during the patient’s illness.
Different Types of Lupus
|SLE||Multiple organs can be involved||ANA/sub-serologies (dsDNA, Sm, SSA, SSB, RNP, APLab)||Variable|
|Subacute cutaneous lupus erythematosus||Skin only||Can be ANA-negative SSA or SSB+||5%–10% can progress to SLE|
|Drug-induced lupus erythematosus (see Chapter 17 )||Systemic but typically not central nervous system or renal disease||Antibodies to histone||Usually improves when drug is stopped|
|Discoid lupus erythematosus||Skin only||Typically Negative ANA||Rarely progresses to SLE unless ANA +|
ANA, antinuclear antibody; RNP, ribonucleoprotein; SSA, Sjögren’s syndrome A; SSB, Sjögren’s syndrome B; SLE, systemic lupus erythematosus.