Antiphospholipid syndrome in children

Antiphospholipid syndrome is considered as the most common acquired hypercoagulation state of autoimmune disorder in children. Besides vascular occlusion, antiphospholipid antibodies have been associated with various nonthrombotic clinical manifestations. This review highlights recent clinical advances in the field of neonatal and pediatric antiphospholipid syndrome and emphasizes differences in relation to the antiphospholipid syndrome in adult population.

Neonatal antiphospholipid syndrome is a rare clinical entity characterized by neonatal thrombotic disease due to the transplacental passage of maternal antiphospholipid antibodies. There is growing evidence that transplacentally transferred antiphospholipid antibodies act as a risk factor, but are not usually a sufficient condition for thrombosis and other thrombophilic risk factors should be systematically evaluated. Long-term studies of children born to antiphospholipid-antibody-positive mothers provided the evidence of possible neurodevelopmental changes in these children and regular neuropsychological assessments are recommended. Data from the pediatric antiphospholipid syndrome studies have confirmed that antiphospholipid-antibody-related thromboses in children are frequently associated with multiple antiphospholipid antibodies positivity and concomitant presence of inherited prothrombotic disorders. Children with antiphospholipid syndrome have frequently demonstrated associated nonthrombotic manifestations, particularly hematological, skin and neurological manifestations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have recognized certain differences that suggest two distinct subgroups with specific clinical characteristics.

Clinical and laboratory characterization of pediatric patients with antiphospholipid syndrome continues to improve and implies some important differences between antiphospholipid syndrome in pediatric and adult populations.