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
    Digital Edition
    Asia's trusted medical magazine for healthcare professionals. Get your MIMS JPOG - Malaysia digital copy today!
    Sign In To Download
    Editor's Recommendations
    Most Read Articles
    Pearl Toh, 21 Nov 2020
    Antiviral treatment with tenofovir alafenamide fumarate (TAF) during pregnancy in highly viraemic mothers effectively prevents mother-to-child transmission (MTCT) of hepatitis B virus (HBV) with no safety concerns, according to two studies presented during the AASLD 2020 Liver Meeting.