1708 - Derivation and Validation of Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus

Monday, November 7, 2011: 4:45 PM
W183a (McCormick Place West)
Michelle Petri1, Ana-Maria Orbai1, Graciela S. Alarcón2, Caroline Gordon3, Joan T. Merrill4, Paul R. Fortin5, Ian N. Bruce6, David A. Isenberg7, Daniel J. Wallace8, Ola Nived9, Gunnar K. Sturfelt10, Rosalind Ramsey-Goldman11, Sang-Cheol Bae12, John G. Hanly13, Jorge Sanchez-Guerrero14, Ann E. Clarke15, Cynthia Aranow16, Susan Manzi17, Murray B. Urowitz18, Dafna D. Gladman19, Kenneth C. Kalunian20, Melissa I. Costner21, Hong Fang1, Systemic Lupus International Collaborating Clinics (SLICC)22 and Laurence S. Magder23, 1Johns Hopkins University School of Medicine, Baltimore, MD, 2University of Alabama at Birmingham, Birmingham, AL, 3University of Birmingham, Birmingham, United Kingdom, 4Oklahoma Medical Research Foundation, Oklahoma City, OK, 5Toronto Western Hospital, Toronto, ON, 6The University of Manchester, Manchester, United Kingdom, 7University College London, London, United Kingdom, 8Cedars-Sinai/UCLA, Los Angeles, CA, 9University Hospital, Lund, Sweden, 10University Hospital Lund, Lund, Sweden, 11Northwestern University, Chicago, IL, 12Hanyang University Hospital for Rheumatic Diseases, Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, South Korea, 13Dalhousie University and Capital Health, Halifax, NS, 14Mount Sinai Hospital/University Health Network, Toronto, ON, 15Research Institute of the McGill Univ. Health, Montreal, QC, 16Feinstein Institute for Medical Research, Manhasset, NY, 17Allegheny Singer Research Institute, Pittsburgh, PA, 18Toronto Western Hospital and University of Toronto, Toronto, ON, 19Toronto Western Research Institute, University of Toronto, University Health Network, Toronto, ON, 20UCSD School of Medicine, La Jolla, CA, 21North Dallas Dermatology Assoc, Dallas, TX, 22Chicago, 23University of Maryland, Baltimore, MD
Presentation Number: 1708

Background/Purpose: The Systemic Lupus International Collaborating Clinics (SLICC) revised the American College of Rheumatology (ACR) SLE classification criteria and then validated the new criteria in order to improve clinical relevance, meet more stringent methodology requirements and incorporate new knowledge in SLE immunology since 1982. 

Method: The new classification criteria were derived from a set of 702 expert-rated patient scenarios.  Recursive partitioning and logistic regression were used to derive an initial rule that was simplified and refined based on SLICC physician consensus.  The SLICC classification rule was validated in a new sample of 690 SLE patients and controls. 

Result: The SLICC criteria rule for SLE classification requires: 1) Four criteria, with at least one clinical criterion AND one immunologic criterion OR 2)  Lupus nephritis alone in the presence of ANA or anti-dsDNA antibodies. In the derivation set, the SLICC classification rule resulted in fewer misclassifications than the current ACR classification rule (49 compared to 70, p=0.0082), had greater sensitivity (94% versus 86%, p<0.0001) and equal specificity (92% versus 93%, p=0.39).  In the validation set, the SLICC classification rule resulted in fewer misclassifications (66 compared to 74, p=0.43), had greater sensitivity (97% versus 83%, p<0.0001) but less specificity (84% versus 96%, p<0.0001).

Conclusion: The new SLICC classification criteria performed well on a large set of patient scenarios rated by experts.  In particular, the new criteria provide updated and more inclusive definitions for each criterion.  Importantly, they require that at least one clinical criterion and one immunologic criterion be present to have a classification of SLE.  Under the new SLICC classification lupus nephritis by biopsy (in the presence of SLE autoantibodies) is sufficient for classification. 

Table:  Clinical and Immunologic Criteria Used in the Classification Rule

 

Clinical Criteria

1. 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
or 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

chilblains lupus

discoid lupus/lichen planus overlap

3. Oral ulcers: palate

buccal

tongue

or nasal ulcers

in the absence of other causes, such as vasculitis, Behcets, infection (herpes), inflammatory bowel disease, reactive arthritis, 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, 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, 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
anti-b2 glycoprotein I

5. Low complement
low C3
low C4
low CH50

6. Direct Coombs test in the absence of hemolytic anemia

 


Keywords: classification criteria, lupus nephritis and systemic lupus erythematosus (SLE)

Disclosure: M. Petri, None; A. M. Orbai, None; G. S. Alarcón, None; C. Gordon, None; J. T. Merrill, None; P. R. Fortin, None; I. N. Bruce, None; D. A. Isenberg, None; D. J. Wallace, None; O. Nived, None; G. K. Sturfelt, None; R. Ramsey-Goldman, None; S. C. Bae, None; J. G. Hanly, None; J. Sanchez-Guerrero, None; A. E. Clarke, None; C. Aranow, None; S. Manzi, None; M. B. Urowitz, None; D. D. Gladman, None; K. C. Kalunian, None; M. I. Costner, None; H. Fang, None; L. S. Magder, None.