Pediatric APS: State of the Art

The purpose of this report is to review recent research findings on APS in children and neonates.

European evidence-based recommendations for diagnosis and treatment of pediatric APS has recently been published by the SHARE Initiative. Recent studies have shown a high prevalence of non-thrombotic manifestations in children with aPL, domains 4/5 specificity of 'innocent' anti-β2GPI antibodies in infants, and a higher risk for developmental delays and learning disabilities, hence, the need for neurodevelopmental monitoring in children born to mothers with APS. An International effort on creating a new diagnostic criteria for APS is underway. Pediatric APS is a rare disease with significant differences from the APS in adults. Majority of the children with persistently positive aPL do not develop thrombotic events; however, relatively higher proportion of thrombosis in children is related to aPL positivity compared to adults; this may partly be due to the absence of common pro-thrombotic "second-hit" risk factors of adults such as atherosclerosis and cigarette smoking. Diagnosis of APS in children may be delayed or missed when adult APS criteria are used, because in pediatric APS, non-thrombotic clinical manifestations such as thrombocytopenia, hemolytic anemia, and neurological disorders such as migraine, epilepsy, and chorea may precede thrombotic manifestations. Around 20% of the children initially diagnosed with primary APS eventually develop SLE. Neonatal APS is rare; however, the offspring of mothers with APS are at a higher risk for developmental delays and learning disabilities; prematurity and IUGR may increase this risk. Regular assessment of neurodevelopmental status of these children should be performed. Thrombosis is a rare event in the offspring of mothers with APS; anticoagulation of such infants is not recommended. An international effort to create a new diagnostic criteria for APS is underway.