Systemic Lupus International Collaborating Clinics criteria classification for SLE

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)

CriterionDefinition aFrequency (%) b
Clinical criteria
1. Acute cutaneous lupus erythematosusMalar rash, bullous lupus, toxic epidermal necrolysis variant, maculopapular, photosensitive, subacute cutaneous lupus60–70
2. Chronic cutaneous lupus erythematosusClassic discoid, hypertrophic, lupus panniculitis/profundus, mucosal, lupus erythematosus tumidus, chilblains lupus, discoid/lichen planus overlap15–30
3. AlopeciaNonscarring, diffuse hair thinning, or visible broken hairs30–50
4. Oral ulcersOral (palate, buccal, tongue) or nasal ulceration15–45
5. SynovitisArthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion and morning stiffness > 30 minutes90
6. SerositisPleuritis: 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 effusion30–60
10–40
7. Renal disorderPersistent proteinuria ≥ 0.5 g/day (24 hour urine or urine protein/creatinine ratio) or red blood cell casts40–60
8. Neurologic disorderSeizures, psychosis, myelitis, mononeuritis multiplex, peripheral or cranial neuropathy, acute confusional state15–20
9. Hemolytic anemiaDirect Coombs positive5–10
10. LeukopeniaLeukopenia < 4000/mm at least once, or Lymphopenia < 1000/mm at least once15–20
15–20
11. ThrombocytopeniaPlatelets < 100,000/mm 315–20
Immunologic Critieria
1. ANALevel above laboratory reference range98
2. Anti-dsDNALevel above laboratory reference range (or >2-fold enzyme-linked immunosorbent assay reference range)60–70
3. Anti-SmPresence of antibody to Sm nuclear antigen20–30
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 complementLow C3, low C4, or low CH5055–60
6. Direct Coombs testIn the absence of hemolytic anemia10–30

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

DiseaseManifestationsLabs AssociatedPrognosis
SLEMultiple organs can be involvedANA/sub-serologies (dsDNA, Sm, SSA, SSB, RNP, APLab)Variable
Subacute cutaneous lupus erythematosusSkin onlyCan 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 diseaseAntibodies to histoneUsually improves when drug is stopped
Discoid lupus erythematosusSkin onlyTypically Negative ANARarely progresses to SLE unless ANA +

ANA, antinuclear antibody; RNP, ribonucleoprotein; SSA, Sjögren’s syndrome A; SSB, Sjögren’s syndrome B; SLE, systemic lupus erythematosus.

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