Antiphospholipid syndrome

Service of Internal Medicine, Hospital de Cruces-University of the Basque Country, Bizkaia, Spain.
The Lancet (Impact Factor: 45.22). 10/2010; 376(9751):1498-509. DOI: 10.1016/S0140-6736(10)60709-X
Source: PubMed


The antiphospholipid syndrome causes venous, arterial, and small-vessel thrombosis; pregnancy loss; and preterm delivery for patients with severe pre-eclampsia or placental insufficiency. Other clinical manifestations are cardiac valvular disease, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, and cognitive impairment. Antiphospholipid antibodies promote activation of endothelial cells, monocytes, and platelets; and overproduction of tissue factor and thromboxane A2. Complement activation might have a central pathogenetic role. Of the different antiphospholipid antibodies, lupus anticoagulant is the strongest predictor of features related to antiphospholipid syndrome. Therapy of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thromboprophylaxis is recommended in patients with systemic lupus erythematosus and probably in purely obstetric antiphospholipid syndrome. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin. Hydroxychloroquine is a potential additional treatment for this syndrome. Possible future therapies for non-pregnant patients with antiphospholipid syndrome are statins, rituximab, and new anticoagulant drugs.

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Available from: Guillermo Ruiz-Irastorza, Feb 13, 2014
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    • "Antiphospholipid antibodies (aPLAs) are members of a heterogeneous family of immunoglobulins that recognize a variety of phospholipids or proteins that bind to phospholipids. The persistent presence of aPLAs can lead to the development of Antiphospholipid Syndrome (APS), a complex autoimmune disease characterized by venous and/or arterial thrombosis and/or pregnancy morbidity[1,2,3]. As a complex disease, APS is caused by a combination of genetic and environmental factors like some drugs or infections[4,5]. "
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    ABSTRACT: The identification of the genetic risk factors that could discriminate non- thrombotic from thrombotic antiphospholipid antibodies (aPLA) carriers will improve prognosis of these patients. Several human studies have shown the presence of aPLAs associated with atherosclerotic plaque, which is a known risk factor for thrombosis. Hence, in order to determine the implication of atherosclerosis in the risk of developing thrombosis in aPLA positive patients, we performed a genetic association study with 3 candidate genes, APOH, LDLR and PCSK9.
    Full-text · Article · Jan 2016 · PLoS ONE
    • "A recent case–control study has also demonstrated that monocyte count may be a risk factor for venous thromboembolism [29]. For the pathogenesis of thrombotic APS, the immune-complex formed by aCL or aβ2GP1 and their antigens, in particular LDL oxidized, would activate monocytes to express tissue factor , to secrete inflammatory cytokines [11] [27] and possibly contribute to the formation of atherosclerotic plaques on the vascular wall [30]. "
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    ABSTRACT: Introduction: Antiphospholipid syndrome (APS) is a pro-thrombotic autoimmune disease that affects different vascular beds, with potential risk for recurrence. Systemic lupus erythematosus (SLE), specific autoantibodies profile and atherogenic disorders have been described as risk factors for the occurrence of first thrombosis in patients with antiphospholipid antibodies (aPL). However, factors associated with recurrent thrombosis have not yet been completely elucidated in APS. The aimof this study was to evaluate the association of recurrent thrombosis with markers of inflammation, autoimmunity and the presence of atherogenic disorders in APS patients. Materials and methods:Weperformed a retrospective evaluation of a cohort of APS patients in order to determine if markers of inflammation, autoimmunity and cardiovascular risk were associated with recurrence of thrombosis. Results: One hundred fifteen patients with APS were included, 60% had primary APS. History of recurrent thrombosis was positive in 38.3% of patients, and 40% of them were on oral anticoagulants at the time of recurrence. Independent risk factors associated with recurrent thrombosis were arterial hypertension (OR = 3.7, 95% CI = 1.68.5, P = 0.002) and monocytosis above 500 u/mm3 (OR = 2.4, 95% CI = 1.25.3, P = 0.02). These factors were particularly relevant in cases of venous index event. Conclusion: The results suggest that arterial hypertension and monocyte counts may be independent factors for thrombosis recurrence in APS. Given the morbidity of recurrent cases, the results may support the evaluation of therapeutic measures to a rigid control of blood pressures and modulation of inflammatory response in APS, as additional prophylaxis against the recurrence of vascular events.
    No preview · Article · Oct 2015 · Thrombosis Research
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    • "There are two different prophylactic settings: primary thromboprophylaxis for patients who have not yet experienced a thrombotic event, and secondary thromboprophylaxis for patients who have already had a previous thrombotic event. For primary prophylaxis, patients are prescrived low dose aspirin, and for secondary prophylaxis, patients take warfarin with target PT/INR 2.0 to 3.0 [6]. "
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    ABSTRACT: 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) is particularly challenging in these patients because of the unique features of APS. Patients with APS are constantly at risk of arterial and venous thrombotic events. Therefore it is very important to maintain proper anticoagulation perioperatively, especially during CPB. In this paper, we present three successful cases of APS patients who underwent cardiac surgery with CPB.
    Full-text · Article · Feb 2014 · Korean journal of anesthesiology
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