Antiphospholipid syndrome (APS) is a thrombophilic disorder that classically presents with vascular thrombosis and/or obstetric complications. APS is associated with antiphospholipid antibodies: a heterogeneous group of autoantibodies that are directed against membrane phospholipids in complex with phospholipid-binding proteins. Beta-2-glycoprotein I (B2GPI) binds anionic phospholipids and is c...

The antiphospholipid syndrome is characterized by recurrent fetal loss, venous and/or arterial thrombosis, and thrombocytopenia associated with elevated titers of lupus anticoagulant and anticardiolipin antibodies. Although thrombosis is the characteristic vascular involvement in APS, the development of vascular aneurysms in patients with APS has been reported. We describe four patients with es...

Antiphospholipid syndrome (APS) is a rare disease in which patients display prolonged coagulation test results in vitro, but usually develop thrombotic symptoms in vivo. Patients with APS are at increased risk of valvular heart disease or coronary vascular disease, conditions that often necessitate cardiac surgery via bypass. The management of anticoagulation during cardiopulmonary bypass (CPB)...

Antiphospholipid antibodies may signal the formation of vascular thrombi in the Antiphospholipid syndrome (APS). A rare complication of APS is adrenal insufficiency resulting from venous thrombus followed by hemorrhagic infarction. We describe the case of a 42-year-old male with APS presenting with vomiting and abdominal pain. Through laboratory and imaging diagnostic exams, we confirmed the di...

The family of antiphospholipid antibodies (aPL) includes a heterogeneous population of autoantibodies whose specificity is directed against not only phospholipids, but their complex with plasma proteins. Anticardiolipin antibodies (aCL) and lupus anticoagulant (LA) tests are widely performed to screen the aPL family which is associated with thrombotic complications in patients with systemic lup...

OBJECTIVE Antiphospholipid antibodies (aPL), especially those targeting β2 -glycoprotein I (β2 GPI), are well known to activate endothelial cells, monocytes, and platelets, with prothrombotic implications. In contrast, the interaction of aPL with neutrophils has not been extensively studied. Neutrophil extracellular traps (NETs) have recently been recognized as an important activator of the coa...

OBJECTIVE To evaluate the prevalence of diastolic dysfunction in patients with anticardiolipin antibodies (aCL) and to examine whether the antiphospholipid syndrome (APS) is associated with diastolic dysfunction independently of valvular abnormalities and systolic dysfunction. METHODS Pulsed, continuous, colour Doppler echocardiography was performed in 179 subjects, of whom 15 were excluded f...

OBJECTIVE To develop diagnostic criteria for a familial form of antiphospholipid antibody syndrome (APS), identify families with >1 affected member, examine possible modes of inheritance, and determine linkage to potential candidate genes. METHODS Family members of probands with primary APS were analyzed for clinical and laboratory abnormalities associated with APS. Families with > or =2 affe...

Antiphospholipid syndrome (APS) is rare in children, and evidence-based guidelines are sparse. Consequently, management is mostly based on observational studies and physician's experience, and treatment regimens differ widely. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative was launched to develop diagnostic and management regimens for children and young...