systemic%20lupus%20erythematosus%20(pediatric)
SYSTEMIC LUPUS ERYTHEMATOSUS (PEDIATRIC)
Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation.
Clinical presentation varies in different patients and the disease activity varies over time in a single patient. Childhood-onset SLE has a greater disease severity and earlier disease damage than in adults with SLE.

Systemic%20lupus%20erythematosus%20(pediatric) Diagnosis

Diagnosis

Diagnosis of SLE is based on clinical symptoms and lab findings

  • Antinuclear antibodies (ANAs) are present in almost all patients with SLE  
    • A negative test indicates a low clinical probability of SLE; a positive test alone has a poor diagnostic value without the clinical features of autoimmune rheumatic disease  
  • Entry criterion: An ANA titer of ≥1:80 on HEp-2 cells or an equivalent positive test is measured at least once; it is recommended to test by immunofluorescence on HEp-2 cells or a solid-phase ANA screening immunoassay with at least equivalent performance
2019 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) Classification Criteria for SLE
  • A criterion is not included if there is an explanation other than SLE
  • It is sufficient for a criterion to occur on at least 1 occasion
  • Criteria need not be present all at the same time
  • Only the criterion with the highest weight within each domain will be counted
  • Classify as SLE if total score is ≥10 with at least 1 clinical criterion and entry criterion is met
  • Clinical Domains and Criteria Weight
  • Constitutional
  • - Fever (temperature >38.3°C) 2
  • Hematologic
  • - Leukopenia (white blood cell count <4000/mm3) 3
    - Autoimmune hemolysis [presence of elevated indirect bilirubin, elevated lactate dehydrogenase (LDH), low haptoglobin, reticulocytosis, AND positive Coombs’ (direct antiglobulin) test] 4
    - Thrombocytopenia (platelet count <100,000/mm3) 4
  • Mucocutaneous
  • - Non-scarring alopecia (observed by a clinician) 2
    - Oral ulcers (observed by a clinician) 2
    - Subacute cutaneous OR discoid lupus (observed by a clinician) 4
    - Acute cutaneous lupus (malar or generalized maculopapular rash observed by a clinician) 6
  • Musculoskeletal
  • - Joint involvement (either synovitis involving ≥2 joints OR tenderness in ≥2 joints and at least 30 minutes of morning stiffness) 6
  • Neuropsychiatric
  • - Delirium (change in consciousness with decreased ability to focus; symptoms developing over hours to <2 days or fluctuating throughout the day; either acute/subacute change in cognition or change in behavior, mood, or affect) 2
    - Psychosis (hallucinations and/or delusions without insight and no delirium) 3
    - Seizure (primary generalized seizure or partial/focal seizure) 5
  • Renal
  • - Proteinuria (>0.5 g/24 hr by 24-hour urine or equivalent spot urine protein-to-creatinine ratio) 4
    - Class II or V lupus nephritis on renal biopsy 8
    - Class III or IV lupus nephritis on renal biopsy 10
  • Serosal
  • - Pleural or pericardial effusion (with imaging evidence) 5
    - Acute pericarditis (≥2 of the following: Pericardial chest pain, pericardial rub, ECG with new widespread ST elevation or PR depression, or new or worsened pericardial effusion on imaging) 6
    Immunologic Domains and Criteria Weight
  • Antiphospholipid antibodies
  • - Anti-cardiolipin antibodies (IgA, IgG, or IgM) at medium or high titer OR
    - Anti-beta-2 glycoprotein 1 antibodies (IgA, IgG, or IgM) OR
    - Lupus anticoagulant positive 2
  • Complement proteins
  • - Low C3 OR low C4 3
    - Low C3 AND low C4 4
  • SLE-specific antibodies
  • - Anti-dsDNA antibody in an immunoassay with demonstrated ≥90% specificity for SLE versus relevant disease controls OR
    - Anti-Smith antibody 6

    Other Classification Criteria   

    1997 American College of Rheumatology (ACR) Criteria

    • A patient can be classified as having SLE if at least 4 of the 11 criteria are present  

    2012 Systemic Lupus International Collaborating Clinics (SLICC) Criteria

    • Include 11 clinical and 6 immunologic criteria  
    • Classification requires ≥4 criteria with at least 1 clinical and 1 immunologic criterion, or biopsy-proven lupus nephritis in the presence of ANAs or anti-dsDNA  
    • More sensitive criteria especially in early cSLE

    Disease Activity Indices

    • Several reliable and validated measuring tools are available for assessing disease activity in SLE  
      • BILAG and SLEDAI are the indices more commonly used and most completely validated  

    British Isles Lupus Assessment Group (BILAG)

    • Assesses 9 organ systems; also evaluates relapse occurrence  
    • BILAG 2004 consists of several questions assessed on a 0-4 scale: 0 = not present, 1 = improving, 2 = same, 3 = worse, 4 = new event  
    • Responses are then combined into 5 states of disease activity:  
      • A: Very active disease needing immunosuppressants, medium- or high-dose corticosteroids or high-dose anticoagulation
      • B: Moderate disease activity needing a lower dose of corticosteroid, antimalarials, or NSAIDs
      • C: Little disease activity needing only symptomatic treatment
      • D: No disease activity at the time but previously present
      • E: No current or previous disease activity

    Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)

    • Global index for assessing disease activity in the preceding 10 days  
    • Used in both clinical and research purposes  
    • Consists of 24 items which include specific manifestations from 9 organ systems with a total score of 105  
    • Other versions of this index include SELENA-SLEDAI, SLEDAI 2000 and MEX-SLEDAI  

    European Consensus Lupus Activity Measurement (ECLAM)

    • Measures disease activity in the previous month  
    • Evaluates 10 organ systems and 2 lab values, eg erythrocyte sedimentation rate (ESR) and complement levels  
    • Consists of 33 items evaluated from 0.5 to 2 based on the type of involvement, and a combined global score that ranges from 0 to 17.5  

    Physician Global Assessment (PGA)

    • A visual analogue scale (VAS) reflecting the physician's judgment of the overall disease activity of SLE  
    • Most common VAS tool ranges from 0-3 where 0 = none, 1 = mild, 2 = moderate, 3 = severe  
      • A flare is indicated by an increase in the score of ≥1 since the last patient visit  
    • Reliability is affected by the scale used thus the need for standardization of scoring

    Classification

    Categories of Disease Activity in SLE

    Mild

    • Constitutional symptoms, mild arthritis, rash ≤9% body surface area (BSA)
    • Platelet count 50-100 x 103/mm3
    • SLEDAI ≤6
    • BILAG C or ≤1 BILAG B manifestation

    Moderate

    • Rheumatoid arthritis-like arthritis, serositis, rash 9-18% BSA, cutaneous vasculitis ≤18% BSA
    • Platelet count 20-50 x 103/mm3
    • SLEDAI 7-12
    • ≥2 BILAG B manifestations

    Severe

    • Major organ- or life-threatening disease (eg cerebritis, mesenteric vasculitis, myelitis, nephritis, pneumonitis, thrombotic thrombocytopenic purpura-like disease, acute hemophagocytic syndrome)  
    • Platelet count <20 x 103/mm3
    • SLEDAI >12
    • ≥1 BILAG A manifestations
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