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)

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.