Antiphospholipid syndrome in pediatrics

Abstract

APS is recognized increasingly as a leading cause of vascular thrombosis in the pediatric population. With the obvious exception of pregnancy morbidity, most of the clinical features that may occur in adults with APS have been described also in children. Because the coincident prothrombotic factors that are common in adults have little or no impact in children, pediatric patients with APS constitute a suitable sample to investigate the relationship of aPL with the associated clinical manifestations, such as thrombocytopenia, hemolytic anemia, chorea, and livedo reticularis, and the specificities of aPL that are more linked to thrombosis. On the other hand, because of the high frequency of infectious processes in early life, children may have a greater prevalence of nonpathogenic and transient aPL. For these reasons, the diagnostic and therapeutic approach to APS in childhood may be different from that for adults. Because of the rarity of aPL-related thrombosis in children, the natural history and optimal management can be defined only through large, multicenter, controlled studies. A internet-based registry for pediatric patients with APS (Ped-APS Register) has been recently established as part of the activities of the Euro-aPL Forum and the Lupus Working Group of the Pediatric Rheumatology European Society. This registry is aimed to obtain information on APS in childhood, particularly regarding association of aPL with clinical manifestations, specificity of aPL, impact of treatment and long-term outcome (http://www.med.ub.es/MIMMUN/FORUM/PEDIATRIC.HTM).