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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 |
|---|---|---|
| Clinical criteria | ||
| 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 10–40 |
| 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 15–20 |
| 11. Thrombocytopenia | Platelets < 100,000/mm 3 | 15–20 |
| Immunologic Critieria | ||
| 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
| Disease | Manifestations | Labs Associated | Prognosis |
|---|---|---|---|
| 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.
