2012 SLICC Classification Criteria for Systemic Lupus Erythematosus (SLE)

SLICC classification criteria improved the clinical relevance of the ACR criteria, incorporated recent findings on the immunology of SLE, and resolved several problems attributed to the ACR criteria.

 

Clinical Criteria
1. Acute or Subacute Cutaneous Lupus

  • Acute cutaneous lupus: including lupus malar rash (do not count if malar discoid), bullous lupus, toxic epidermal necrolysis variant of SLE, maculopapular lupus rash, photosensitive lupus rash (in the absence of dermatomyositis)
  • Subacute cutaneous lupus (nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias)

2. Chronic cutaneous lupus: including classical discoid rash, localized (above the neck), generalized (above and below the neck), hypertrophic (verrucous) lupus, lupus panniculitis (profundus), mucosal lupus, lupus erythematosus tumidus, chillblains lupus, discoid lupus/lichen planus overlap
3. Oral ulcers: palate, buccal, tongue, or nasal ulcers, in the absence of other causes, such as vasculitis, Behcet´s, infection (herpes), inflammatory bowel disease, reactive arthritis, and acidic foods
4. Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs)
in the absence of other causes such as alopecia areata, drugs, iron deficiency and androgenic alopecia
5. Synovitis involving two or more joints, characterized by swelling or effusion OR tenderness in 2 or more joints and thirty minutes or more of morning stiffness.
6. Serositis

  • typical pleurisy for more than 1 day, or pleural effusions, or pleural rub
  • typical pericardial pain (pain with recumbency improved by sitting forward) for more than 1 day, or pericardial effusion, or pericardial rub, or pericarditis by EKG

in the absence of other causes, such as infection, uremia, and Dressler’s pericarditis

7. Renal: Urine protein/creatinine (or 24 hr urine protein) representing 500 mg of protein/24 hr or Red blood cell casts
8. Neurologic: seizures, psychosis, mononeuritis multiplex (in the absence of other known causes such as primary vasculitis), myelitis, peripheral or cranial neuropathy
(in the absence of other known causes such as primary vasculitis, infection, and diabetes mellitus), acute confusional state (in the absence of other causes, including toxic-metabolic, uremia, drugs)
9. Hemolytic anemia
10. Leukopenia (< 4000/mm3 at least once), in the absence of other known causes such as Felty’s, drugs, and portal hypertension
OR
Lymphopenia (< 1000/mm3 at least once), in the absence of other known causes such as corticosteroids, drugs and infection
11. Thrombocytopenia (<100,000/mm3) at least once, in the absence of other known causes such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura (TTP)

Immunological Criteria

  1. ANA above laboratory reference range
  2. Anti-dsDNA above laboratory reference range, except ELISA: twice above laboratory reference range
  3. Anti-Sm
  4. Antiphospholipid antibody: any of the following
    • lupus anticoagulant
    • false-positive RPR
    • medium or high titer anticardiolipin (IgA, IgG or IgM)
    • anti-β2 glycoprotein I (IgA, IgG or IgM)
  5. Low complement
    • low C3
    • low C4
    • low CH50
  6. Direct Coombs test in the absence of hemolytic anemia

Requirements: ≥4 criteria (at least 1 clinical and 1 laboratory criteria) or biopsy-proven lupus nephritis with positive ANA or anti-DNA antibodies. Criteria are cumulative and need not be present concurrently.

Abbreviations: ANA, antinuclear antibodies; ECG, electrocardiography; ELISA, enzyme-linked immunosorbent assay; RBC, red blood cell; SLE, systemic lupus erythematosus.

 

 

References:

  1. Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG Jr, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G Jr, Magder LS. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012 Aug;64(8):2677-86. [Medline]
  2. Hartman EAR, van Royen-Kerkhof A, Jacobs JWG, Welsing PMJ, Fritsch-Stork RDE. Performance of the 2012 Systemic Lupus International Collaborating Clinics classification criteria versus the 1997 American College of Rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. A systematic review and meta-analysis. Autoimmun Rev. 2018 Mar;17(3):316-322. [Medline]
  3. Flynn A, Gilhooley E, O’Shea F, Wynne B. The use of SLICC and ACR criteria to correctly label patients with cutaneous lupus and systemic lupus erythematosus. Clin Rheumatol. 2018 Mar;37(3):817-818. [Medline]

 

Created Aug 20, 2018.

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