How to Use the Criteria
The development team’s goal is for the criteria to include a mobile app and online tool that defines each criterion and adds up the patient’s score, said Karen Costenbader, MD, director of the lupus program at Brigham and Women’s Hospital in Boston. She showed the audience how to score several patient cases.
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“Within each domain, there is a system of criteria in ascending order of importance. You only count the most heavily weighted criterion within that domain,” said Dr. Costenbader. “We are relying on your clinical acumen. If you feel there’s a more likely explanation for this patient to have a certain manifestation, you would not score it. If you think it’s as likely to be SLE as something else, then score it.”
Despite their many strengths, these criteria have some limitations, so discussion is welcome, she said.
“SLE is a complex, heterogeneous disease with many overlap syndromes. There is also incomplete lupus, pre-lupus, cutaneous-only lupus and early lupus,” said Dr. Costenbader. Patients with overlap syndromes may be classified as SLE if they meet the criteria, but excluded in specific studies. “Also, newer biomarkers and assays, such as complement deposition products and interferon signatures, may be very helpful in the classification of SLE, but they’re not widely available at this time.”
Susan Bernstein is a freelance journalist based in Atlanta.
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Proposed Draft Criteria
Here are the proposed ACR/EULAR Classification Criteria for SLE with scores in parentheses
Entry Criterion: History of a positive ANA BY Hep-2 immunofluorescence >1:80.
- For each criterion, do not score if a more likely cause for the symptom exists.
- Occurrence of a criterion on at least one occasion is sufficient.
- Criteria need not occur simultaneously.
- At least one clinical criterion must be present.
- Within each domain, only the highest weighted criterion is counted toward the total score.
- Unexplained fever >38.3 C (2)
- Non-scarring alopecia (2)
- Oral ulcers (2)
- Sub-acute cutaneous or discoid lupus (4)
- Acute cutaneous lupus (6)
- Synovitis in two or more joints or tenderness in two or more joints or ≥30 minutes of morning stiffness (6)
- Delirium (2)
- Psychosis (3)
- Seizure (5)
- Pleural or pericardial effusion (5)
- Acute pericarditis (6)
- Leukopenia (3)
- Thrombocytopenia (4)
- Autoimmune hemolysis (4)
- Proteinuria >0.5g 24 hours (4)
- Renal biopsy with Class II or V lupus nephritis (8)
- Renal biopsy with Class III or IV lupus nephritis (10)
- Present (anti-cardiolipin antibody positive with medium or high units, or anti-β2-GP1 positive or lupus anticoagulant positive) (2)
Complement Proteins Domain
- Low C3 or Low C4 (3)
- Low C3 and Low C4 (4)
Highly Specific Antibodies Domain
- Anti-dsDNA antibody (6)
- Anti-Smith antibody (6)