It is associated with obstetrical complications mainly spontaneous abortion as well as venous and arterial thrombotic risks. Antiphospholipid antefosfolipid syndrome occurs when your immune system mistakenly creates antibodies that make your blood much more likely to clot. Antiphospholipid syndrome aps represents a serious risk factor in pregnancy. The pathogenesis of the antiphospholipid syndrome nejm. Antiphospholipid antibodies apl are associated with the recurrent pregnancy loss and thrombosis that characterizes the antiphospholipid. Nantiphospholipid antibodies are directed against phos pholipidprotein. Immunotherapies for neurological manifestations in the context of. This can cause dangerous blood clots in the legs, kidneys, lungs and brain. Antiphospholipid syndrome symptoms and causes mayo clinic. Pathogenesis and management of antiphospholipid syndrome. Neurologic manifestations of antiphospholipid syndrome i f ricarte. The antiphospholipid syndrome is a prothrombotic disorder associated with autoantibodies. In sle, a variety of pathogenetic mechanisms has been implicated, including ischemic vasculopathy, frank.
Antiphospholipid antibody syndrome archives of pathology. Pdf the pathophysiology of antiphospholipid syndrome. Article information, pdf download for neurologic manifestations of. A diagnosis of the anti phos pho lip id syndrome is made if at least one. Its serological marker is the presence of antiphospholipid antibodies in the blood of these. Pregnancy outcome in women with antiphospholipid antibodies. Pdf endothelial dysfunction as a consequence of endothelial.
Proposed pathogenetic mechanisms in the antiphospholipid syndrome aps, supporting evidence from studies in humans and animals, and implications for targeted therapy. Pathophysiology of the antiphospholipid antibody syndrome ncbi. Serological testing for antinuclear antibody, auto. Pathophysiology of the antiphospholipid antibody syndrome.
Alzheimers disease, heart defects, leukemia, hyperten sion and gastrointestinal problems figure 1. Advances in our knowledge of the pathogenic mechanisms of antiphospholipid syndrome have been achieved in the past few years. Aps provokes blood clots thrombosis in both arteries and veins as well as pregnancyrelated complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia. Antiphospholipid syndrome, or antiphospholipid antibody syndrome aps or apls, is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. The role of apls in thrombosis is widely recognized, but the pathogenic mechanisms triggering thrombosis in aps are only partially understood. Arachchillage and mike laffan department of haematology, imperial college healthcare nhs trust and imperial college london, hammersmith hospital, london, uk summary antiphospholipid antibodies are a heterogeneous group of autoantibodies that have clear associations with thrombosis. Pdf the pathogenesis of the antiphospholipid syndrome. Thrombosis must be confirmed by objective validated criteria ie, unequivocal findings of appropriate imaging studies or histopathology. Pathogenesis and management of antiphospholipid syndrome deepa r. Insights into disease mechanisms have led to new therapies. Neurological involvement in antiphospholipid antibody syndrome aps is common. It is now widely accepted that apl are a heterogenous group of antibodies abs that react with a myriad of phospholipids pls, plprotein.
Mononeuritis multiplex associated with antiphospholipid antibodies. The new engl and journal of medicine 1034 n engl j med 368. The pathogenic mechanisms behind the syndrome are complex and not. Antiphospholipid syndrome aps is an autoimmune disease in which thrombosis andor pregnancy morbidity occur in patients with persistently positive antiphospholipid antibodies apls. Pdf the endothelial glycocalyx is an intravascular compartment which consists of carbohydrate. Antiphospholipid antibody syndrome is present if at least one of the clinical criteria and one of the laboratory criteria that follow are met clinical criteria 1. In pregnant women, antiphospholipid syndrome also can result in miscarriage and stillbirth.
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