Antiphospholipid syndrome

The description of the antiphospholipid syndrome (APS) has been one of the most striking developments in the field of clinical immunology in the last 2 decades. APS is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss, accompanied by elevated titers of antiphospholipid antibodies (aPL), namely the lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) [1,2]. The syndrome may occur in isolation (primary APS) or in association with an underlying systemic disease, particularly systemic lupus erythematosus (SLE) (secondary APS). Because aPL-related thrombosis can occur anywhere in the body, the recognition of APS has had a major impact on several medical specialties, including pediatrics. The earliest descriptions of the association between a circulating anticoagulant and vascular thrombosis in the pediatric population are those of Olive et at in 1979 [3] and St Clair et at in 1981 [4]. In recent years, the features of A-PS have been increasingly recognized in children [5,6]. Most information on APS in the pediatric population, however, comes from individual case reports or small patient series; large-scale multicenter studies are lacking. The following case highlights some of the important clinical and laboratory features of the APS.