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) Management

Follow-up and Monitoring

Lifelong monitoring is required for SLE patients. Frequency of visits depends on disease activity, severity and extent, response to treatment, type of treatment and monitoring of toxicity

  • The most important tool in the management of SLE is careful, frequent clinical and lab evaluation to detect disease flares, appearance of infections and to tailor management based on patient response
    • Do active surveillance of opportunistic infections
    • Adjust current therapy (eg corticosteroids and immunosuppressants) according to the severity of organ involvement during SLE flares
    • Patients with severe SLE, complications, comorbidities and drug toxicity will require more frequent follow-up
  • Monitoring during clinic visit should include history-taking, physical exam and lab tests (eg CBC, platelet count, creatinine measurement, urinalysis, renal and liver function tests, immunology, ECG, imaging)
    • For active SLE disease, monitoring can be done every 1-3 months after diagnosis or flare while for lowithno disease activity or with stable treatment course, monitoring can be every 6-12 months
  • Physicians may be aided by at least 1 of the following several global or body system-specific indices developed for long-term observation of SLE: BILAG, SLEDAI, ECLAM and PGA
    • Disease damage should be assessed yearly using the pediatric SLICC/ACR Damage Index
  • 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


  • Patients on long-term glucocorticoids should be monitored for:
    • Linear growth  
    • Physical body changes, eg Cushingoid features and striae  
    • Electrolyte, glucose and lipid levels to identify metabolic conditions
    • Development of visual field loss and avascular necrosis  
    • Bone densitometry to identify osteoporosis and monitor response to treatment  
  • Patients on glucocorticoids and Hydroxychloroquine should have ophthalmological screening (eg visual fields examination and/or spectral domain-optical coherence tomography) performed at baseline and then annually
  • Patients on immunosuppressants should be monitored for hematologic, liver and renal toxicity, and occurrence of infection

Hospital Admission  

  • Hospital admission is advised for those:  
    • with life-threatening or serious organ system involvement
    • Requiring high-dose IV corticosteroids or chemotherapeutic/immunosuppressant drugs (eg Cyclophosphamide, Mycophenolic acid)    
    • with severe hypertension
    • with unexplained fever to rule out sepsis

Expert Referral

  • Establish good working relationship amongst the primary care physician, pediatrician, rheumatologist, nurse, pharmacist, other healthcare providers, the patient and his/her family
    • Team approach will allow for earlier identification of disease flares and medication toxicity
  • Patient will initially present to a primary care physician/pediatrician who is responsible for:
    • Recognizing the signs and symptoms of SLE in their patients
    • Making an initial diagnosis
    • Referring patient to a rheumatologist and participating in further SLE diagnosis, management and monitoring
  • The rheumatologist is the integral part of the medical team managing an SLE patient
  • Referral to a rheumatologist is indicated for:
    • Confirmation of diagnosis
    • Management plan for patient
    • Periodic evaluation of disease activity and severity
    • Management of uncontrolled disease
    • Management of organ involvement or life-threatening disease
    • Prevention and/or management of treatment toxicities
    • Special situations (eg pregnancy, antiphospholipid syndrome, life-threatening disease, concomitant infection and surgery)
  • Multidisciplinary care involves referral of SLE patients to a psychologist, psychiatrist, neurologist, hematologist, ophthalmologist, dermatologist, nephrologist, cardiologist, pulmonologist, orthopedic surgeon, physical and/or occupational therapist, and other specialists when necessary
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