Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.


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

Petri1,  Michelle, Orbai1,  Ana-Maria, Alarcon2,  Graciela S., Gordon3,  Caroline, Merrill4,  Joan T., Fortin5,  Paul R., Bruce6,  Ian N.

Johns Hopkins University School of Medicine, Baltimore, MD
University Hospital Lund, Lund, Sweden
Northwestern University, Chicago, IL
Hanyang University Hospital for Rheumatic Diseases, Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, South Korea
Nova Scotia Rehabilitation Center, Halifax, NS
University Health Network/Mount Sinai Hospital, Toronto, ON
Research Institute of the McGill Univ. Health, Montreal, QC
Feinstein Institute for Medical Research, Manhasset, NY
Allegheny Singer Research Institute, Pittsburgh, PA
Toronto Western Hospital and University of Toronto, Toronto, ON
Toronto Western Research Institute, University of Toronto, University Health Network, Toronto, ON
University of Alabama at Birmingham, Birmingham, AL
UCSD School of Medicine, La Jolla, CA
North Dallas Dermatology Assoc, Dallas, TX
University of Maryland, Baltimore, MD
University of Birmingham, Birmingham, United Kingdom
Oklahoma Medical Research Foundation, Oklahoma City, OK
Toronto Western Hospital, Toronto, ON
The University of Manchester, Manchester, United Kingdom
University College London, London WC1E 6JF, United Kingdom
Cedars-Sinai/UCLA, Los Angeles, CA
University Hospital, Lund, Sweden

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.

Methods:

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.

Results:

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

To cite this abstract, please use the following information:
Petri, Michelle, Orbai, Ana-Maria, Alarcon, Graciela S., Gordon, Caroline, Merrill, Joan T., Fortin, Paul R., et al; Derivation and Validation of Systemic Lupus International Collaborating Clinics Classification Criteria for Systemic Lupus Erythematosus. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1708
DOI:

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