Antiphospholipid syndrome in children

Area of Science:

• Pediatric Rheumatology
• Hematology
• Immunology

Background:

• Antiphospholipid Syndrome (APS) in children is rare but severe, with higher risks of thrombosis and catastrophic APS than in adults.
• Non-thrombotic manifestations are common in pediatric APS and may precede thrombotic events.
• Recent classification criteria for APS require evaluation in pediatric populations.

Purpose of the Study:

• To provide a comprehensive overview of antiphospholipid antibody (aPL)-related clinical manifestations in pediatric patients.
• To analyze published cohorts and data from the international pediatric APS registry.
• To illustrate APS in infants due to maternal aPL transfer, focusing on perinatal thrombosis and neurodevelopmental outcomes.

Main Methods:

• Review of published cohorts on pediatric Antiphospholipid Syndrome.
• Analysis of data from the international pediatric APS registry.
• Examination of case studies on neonatal APS from transplacental maternal aPL transfer.

Main Results:

• Pediatric APS exhibits more severe presentations, frequent thrombotic recurrences, and a higher incidence of catastrophic APS compared to adult APS.
• Non-thrombotic manifestations are more prevalent in children and can occur before thrombotic events.
• Neonatal APS, resulting from maternal aPL, rarely causes acute perinatal thrombosis but is linked to long-term neurodevelopmental issues.

Conclusions:

• Pediatric APS requires specialized management due to its severity and distinct clinical course.
• Novel therapies, including B cell and complement inhibitors, show promise, particularly for catastrophic APS.
• Understanding transplacental aPL transfer is crucial for managing neonatal risks and long-term neurodevelopmental follow-up.