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Table 1-6ck: 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for SLE
Criteria | Definition |
ANA | ANA at a titer of ≥ 1:80 at least once. Testing by immunofluorescence on HEp-2 cells or a solid-phase ANA screening immunoassay with at least equivalent performance is highly recommended |
Fever | Temperature > 38.3°C (100.9°F) |
Leukopenia | WBC count < 4000/mcL (4.0 x 109/L) |
Thrombocytopenia | Platelet count < 100,000/mcL (100 x 109/L) |
Autoimmune Hemolysis | Evidence of hemolysis, such as reticulocytosis, low haptoglobin, elevated indirect bilirubin, elevated LD, and positive direct antiglobulin (Coombs) test |
Delirium | Characterized by: (1) change in consciousness or level of arousal with reduced ability to focus; (2) symptoms development over hours to < 2 days; (3) symptom fluctuation throughout the day; (4) either acute/subacute change in cognition (eg, memory deficit or disorientation) or change in behavior, mood, or affect (eg, restlessness, reversal of sleep/wake cycle) |
Psychosis | Characterized by delusions or hallucinations or both without insight and absence of delirium |
Seizure | Primary generalized seizure or partial/focal seizure |
Nonscarring Alopecia | Nonscarring alopecia observed by a clinician1 |
Oral Ulcers | Oral ulcers observed by a clinician1 |
Subacute Cutaneous or Discoid Lupus | Subacute cutaneous lupus erythematosus observed by a clinician1: – Annular or papulosquamous (psoriasiform) cutaneous eruption, usually photodistributed. – If skin biopsy is performed, typical changes must be present (interface vacuolar dermatitis consisting of a perivascular lymphohistiocytic infiltrate, often with dermal mucin noted). OR Discoid lupus erythematosus observed by a clinician1: – Erythematous-violaceous cutaneous lesions with secondary changes of atrophic scarring, dyspigmentation, often follicular hyperkeratosis/plugging (scalp), leading to scarring alopecia on the scalp. – If skin biopsy is performed, typical changes must be present (interface vacuolar dermatitis consisting of a perivascular and/or periappendageal lymphohistiocytic infiltrate. In the scalp, follicular keratin plugs may be seen. In longstanding lesions, mucin deposition may be noted). |
Acute Cutaneous Lupus | – Malar rash or generalized maculopapular rash observed by a clinician1 – If skin biopsy is performed, typical changes must be present (interface vacuolar dermatitis consisting of a perivascular lymphohistiocytic infiltrate, often with dermal mucin noted. Perivascular neutrophilic infiltrate may be present early in the course). |
Pleural or Pericardial Effusion | Imaging evidence (such as ultrasonography, radiography, CT scan, MRI) of pleural or pericardial effusion, or both. |
Acute Pericarditis | Presence of two or more of the following: 1. Pericardial chest pain (typically sharp, worse with inspiration, improved by leaning forward) 2. Pericardial rub 3. ECG with new widespread ST elevation or PR depression 4. New or worsened pericardial effusion on imaging (such as ultrasonography, radiography, CT scan, MRI) |
Joint Involvement | Presence of either synovitis involving > 2 joints characterized by swelling or effusion or tenderness in > 2 joints and at least 30 minutes of morning stiffness |
Proteinuria | > 0.5 g/24 hours by 24-hour urine or equivalent spot urine protein-to-creatinine ratio |
Class II or V Lupus Nephritis on Renal Biopsy (According to ISN/RPS 2003 Classification) | Class II: Mesangial proliferative lupus nephritis: purely mesangial hypercellularity of any degree or mesangial matrix expansion by light microscopy, with mesangial immune deposit. A few isolated subepithelial or subendothelial deposits may be visible by immunofluorescence or electron microscopy but not by light microscopy. Class V: Membranous lupus nephritis: global or segmental subepithelial immune deposits or their morphologic sequelae by light microscopy and by immunofluorescence or electron microscopy, with or without mesangial alterations. |
Class III or IV Lupus Nephritis on Renal Biopsy (According to ISN/RPS 2003 Classification) | Class III: Focal lupus nephritis: active or inactive focal, segmental, or global endocapillary, or extracapillary glomerulonephritis involving < 50% of all glomeruli, typically with focal subendothelial immune deposits, with or without mesangial alterations. Class IV: Diffuse lupus nephritis: active or inactive diffuse, segmental, or global endocapillary or extracapillary glomerulonephritis involving ≥ 50% of all glomeruli, typically with diffuse subendothelial immune deposits, with or without mesangial alterations. This class includes cases with diffuse wire loop deposits but with little or no glomerular proliferation. |
Positive Antiphospholipid Antibodies | Anti-cardiolipin antibodies (IgA, IgG, or IgM) at medium or higher titer (> 40 APL, GPL, or MPL, or > 99th percentile) or positive anti-beta-2GPI antibodies (IgA, IgG, or IgM) or positive lupus anticoagulant. |
Low C3 OR Low C4 | C3 OR C4 below the lower limit of normal |
Low C3 AND Low C4 | Both C3 AND C4 below the lower limit of normal |
Anti-dsDNA Antibodies OR Anti-Sm Antibodies | Anti-dsDNA antibodies in an immunoassay with demonstrated ≥ 90% specificity for SLE against relevant disease controls or anti-Sm antibodies |