What are the clinical features and treatment options for Systemic Lupus Erythematosus (SLE) in pediatric patients?

Clinical Features and Treatment of Systemic Lupus Erythematosus (SLE) in Pediatric Patients

Pediatric SLE presents with more severe disease manifestations than adult-onset SLE and requires immunosuppression regimens similar to adults with special considerations for growth, fertility, and psychosocial factors.

Epidemiology and Presentation

• Approximately 20% of SLE cases are diagnosed before 18 years of age, with genetic components being more common in childhood-onset SLE 1
• Pediatric SLE typically presents with more severe disease compared to adult-onset SLE 1, 2
• The most frequent presenting symptoms in pediatric SLE include fatigue, arthritis, fever, weight loss, and malar rash 3

Clinical Features in Pediatric SLE

• Mucocutaneous manifestations:

  • Higher prevalence of malar rash (61% vs 35.5% in adults) 2
  • Increased photosensitivity (20% vs 9% in adults) 2
  • More frequent mucosal ulcerations (11.4% vs 4% in adults) 2

• Renal involvement:

  • Lupus nephritis is more common in pediatric patients (43% vs 26.4% in adults) 2
  • In adolescents with isolated proteinuria, orthostatic/postural proteinuria should be excluded 1

• Hematological abnormalities:

  • More frequent in pediatric SLE compared to adult-onset disease (57% vs 36.4%) 2
  • Anemia is the most common hematologic abnormality (74.5%), with autoimmune hemolytic anemia being predominant 4
  • Associated with positive antiphospholipid antibodies and higher SLEDAI scores 4

• Other organ involvement:

  • Less frequent neurological symptoms compared to adults (6% vs 30%) 2
  • Lower incidence of polyarthritis than adults (68% vs 86%) 2

Diagnostic Approach

• Initial workup should include ANA, anti-dsDNA, complement levels, CBC, renal function, and urinalysis 5
• Positive ANA warrants further evaluation with additional immunological tests including anti-Sm antibodies, anti-Ro/SSA, anti-La/SSB, and antiphospholipid antibodies 5
• Regular monitoring is essential as kidney involvement can remain asymptomatic for significant periods 1
• Higher index of suspicion should be maintained for patients of Asian, African/Caribbean, and Hispanic descent 1

Treatment Approach for Pediatric SLE

• First-line therapy:

  • Hydroxychloroquine is the cornerstone of treatment for most pediatric SLE patients 5
  • All SLE patients should receive hydroxychloroquine unless contraindicated 5

• Immunosuppressive therapy:

  • Similar immunosuppression regimens to adults but with important pediatric considerations 1
  • Induction therapy typically includes glucocorticoids with either mycophenolic acid (MPAA) (36%) or cyclophosphamide (34%) 1
  • Maintenance therapy commonly involves MPAA (55%) 1

• Special pediatric considerations:

  • Dose adjustments based on age and weight 1
  • Growth concerns favor limiting glucocorticoid exposure 1
  • Fertility preservation, especially in adolescents, may favor limiting cyclophosphamide exposure 1
  • Adherence concerns may favor intravenous medications in some cases 1
  • Psychosocial concerns related to school and peer socialization 1

• Management of severe manifestations:

  • For hematologic abnormalities resistant to corticosteroids and IVIG, rituximab may be considered 4
  • For hyperlipidemia, statins may be given to children aged 8 years or above 1

Multidisciplinary Care

• Pediatric SLE patients should be co-managed by pediatric nephrologists and rheumatologists with expertise in lupus 1
• Additional support from clinical psychologists, psychiatrists, or social workers is often beneficial 1

Prognosis

• Long-term data shows survival rates without advanced CKD, kidney failure, or death of 94.2%, 92.7%, and 83.2% at 5,10, and 20 years, respectively 1
• Poor prognostic indicators include arthritis, anemia, and seizures at disease onset 3
• Mean SLICC/ACR damage index of 2.6 after 4.7 years of follow-up indicates substantial morbidity 3

Vaccination Considerations

• Due to high risk of pneumococcal infection, immunization is recommended for pediatric SLE patients 1
• Ideally, vaccinations should be administered prior to initiation of immunosuppressive therapy 1