The challenges in diagnosing pediatric primary antiphospholipid syndrome

Area of Science:

• Pediatric Rheumatology
• Immunology
• Hematology

Background:

• Pediatric primary antiphospholipid syndrome (APS) is rare and distinct from adult APS.
• Diagnosis in children often requires adapting adult criteria, potentially leading to delays.

Purpose of the Study:

• To present experience in diagnosing and treating pediatric primary APS.
• To highlight distinctions between pediatric and adult APS.
• To emphasize the need for specific diagnostic considerations in children.

Main Methods:

• Retrospective review of six pediatric primary APS cases.
• Application of updated Sapporo criteria for APS diagnosis.
• Analysis of clinical manifestations, including vascular, hematologic, neurologic, and psychiatric involvement.

Main Results:

• Six pediatric patients diagnosed with primary APS, one with probable catastrophic APS (CAPS).
• Manifestations included vascular events, thrombocytopenia, psychiatric disorders, chorea, and valvular heart disease.
• All patients received immunosuppression and long-term anticoagulation.

Conclusions:

• Pediatric primary APS presents unique features not covered by adult classification criteria.
• Neurologic and hematologic manifestations are common in pediatric APS.
• Current adult criteria may lead to underdiagnosis in pediatric populations, necessitating tailored diagnostic approaches.