Susan Bernstein is a freelance journalist based in Atlanta.
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Proposed Draft Criteria
Entry Criterion: History of a positive ANA BY Hep-2 immunofluorescence >1:80.
Opening Statements: 1) For each criterion, do not score if a more likely cause for the symptom exists. 2) Occurrence of a criterion on at least one occasion is sufficient. 3) Criteria need not occur simultaneously. 4) At least one clinical criterion must be present. 5) Within each domain, only the highest weighted criterion is counted toward the total score.
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Constitutional Domain
- Unexplained fever >38.3º C (2)
Muco-Cutaneous Domain
- Non-scarring alopecia (2)
- Oral ulcers (2)
- Sub-acute cutaneous or discoid lupus (4)
- Acute cutaneous lupus (6)
Arthritis Domain
- Synovitis in two or more joints or tenderness in two or more joints or ≥30 minutes of morning stiffness (6)
Neurologic Domain
- Delirium (2)
- Psychosis (3)
- Seizure (5)
Serositis Domain
- Pleural or pericardial effusion (5)
- Acute pericarditis (6)
Hematological Domain
- Leukopenia (3)
- Thrombocytopenia (4)
- Autoimmune hemolysis (4)
Renal Domain
- 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)
Antiphospholipid Domain
- 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)
References
- Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012 Aug;64(8):2677–2686.
- Aringer M, Dorner T, Leuchten N, et al. Toward new criteria for systemic lupus erythematosus: A standpoint. Lupus. 2016 May;25(8):805–811.
- Singh JA, Solomon DH, Dougados M, et al. Development of classification and response criteria for rheumatic diseases. Arthritis Rheum. 2006 Jun;55(3):348–352.
- Touma Z, Costenbader KH, Johnson S, et al. Do patients with SLE at onset differ from mimickers? A comparison of clinical and serological manifestations in a multicenter cohort to inform the development of new classification criteria for SLE. Ann Rheum Dis. 2016;75(suppl 2):558.
- Leuchten M, Milke B, Winkler-Rohlfing B, et al. Early symptoms of systemic lupus erythematosus (SLE) recalled by 337 SLE patients. Ann Rheum Dis. 2016;75(suppl 2):313.
