systemic%20lupus%20erythematosus%20(pediatric)
SYSTEMIC LUPUS ERYTHEMATOSUS (PEDIATRIC)
Systemic lupus erythematosus is a chronic, multisystem, inflammatory, autoimmune disorder characterized by formation of autoantibodies directed against self-antigens and immune-complex formation.
It can be suspected when ≥2 organ systems are involved.
It is predominantly diagnosed in females of childbearing age, rarely diagnosed before 8 years old.
Clinical presentation varies in different patients and the disease activity varies over time in a single patient. Majority of patients have arthralgia of the hand.

Monitoring

  • Monitoring during clinic visit should include:
    • History-taking
    • Physical exam
    • Lab tests
      • Complete blood count (CBC)
      • Creatinine measurement
      • Urinalysis
  • Results of lab tests that may precede a disease flare:
    • Decrease in serum complement levels
    • Increase in anti-double stranded deoxyribonucleic acid (dsDNA)
    • Increase in erythrocyte sedimentation rate (ESR)
    • Decrease in hemoglobin level, leukocyte or platelet counts
    • Increase in creatine phosphokinase (CPK) levels
    • Appearance of microscopic hematuria or proteinuria

Comorbidities & Complications of Systemic Lupus Erythematosus (SLE)

  • Comorbidities for which systemic lupus erythematosus (SLE) patients are at an increased risk include infections, hypertension, atherosclerosis, dyslipidemia, diabetes, osteoporosis, avascular necrosis & malignancies

Neuropsychiatric Systemic Lupus Erythematosus (NPSLE)

  • Monitor systemic lupus erythematosus (SLE) patients for neurological &/or psychiatric manifestations as in non-neuropsychiatric systemic lupus erythematosus (NPSLE) patients
  • Usually appears w/in 1 year from the time of diagnosis; may also appear before or at the time of diagnosis
  • Diagnostic work-up may include the following:
    • Lumbar puncture
    • Nerve conduction studies (NCS)
    • Neuropsychological assessment of cognitive function
    • Neuroimaging: T1/T2 magnetic resonance imaging (MRI), diffusion-weighted imaging, gadolinium-enhanced T1 sequences
    • Cerebrospinal fluid (CSF) analysis
    • Electroencephalography (EEG)
  • Patients found to have neuropsychiatric systemic lupus erythematosus (NPSLE) should be referred to a team of psychiatrists, psychologists, neurologist & rheumatologist

Toxicity

  • Patients on long-term glucocorticoids should be monitored for:
    • Electrolyte, glucose & lipid levels to identify metabolic conditions
    • Bone densitometry to identify osteoporosis & monitor response to treatment
  • Patients on Hydroxychloroquine should have ophthalmological exam every 6-12 months to detect retinal toxicity
  • Patients on immunosuppressants should be monitored for hematologic, liver & renal toxicity, & occurrence of infection

Lupus Nephritis

  • Patients suspected to have lupus nephritis should immediately undergo renal biopsy to confirm diagnosis, evaluate severity, determine prognosis & therapy
  • Indications for renal biopsy in systemic lupus erythematosus (SLE) patients
    • Unexplained increase in serum creatinine in the absence of alternative causes (eg sepsis, hypovolemia, medications)
    • Confirmed proteinuria (≥1.0 gm/24 hours)
    • Presence of the following (result of 2 tests done w/in a short period of time, w/o other causes):
      • Proteinuria of >5.0 gm/24 hours & hematuria [≥5 red blood cells/high-power field (RBCs/hpf)] or
      • Proteinuria of >5.0 gm/24 hours & cellular casts
  • Recommended tests used for monitoring of lupus nephritis:
  •  

      Active nephritis at onset of treatment Previous active nephritis, none currently No prior or current nephritis
    Blood pressure 1 3 3
    Urinalysis 1 3 6
    Protein/ Creatinine ratio 1 3 6
    PSerum creatinine 1 3 6
    C3/C4 levels 2 3 6
    Anti-DNA 1 3 3

    *Values are at monthly intervals indicating minimum time at which the test should be measured
    Modified from: 2012 American College of Rheumatology Guidelines for Screening, Treatment, and Management of Lupus Nephritis

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