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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    • "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.
    Korean journal of anesthesiology 02/2014; 66(2):164-8. DOI:10.4097/kjae.2014.66.2.164
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    • "For several years, detection of autoantibodies has been recommended in clinical practice for women with infertility including the detection of ANA, APA and ATA. A link of APA with recurrent pregnancy loss has been established, and treatment based on anticoagulation such as subcutaneous heparin is effective [3]. As an organ-specific autoantibody, ATA affect pregnancy outcome negatively by damaging the thyroid function, and the thyroxin replacement therapy is efficacious in preventing foetal loss for patients with recurrent miscarriage [1,2]. "
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    ABSTRACT: Anti-nuclear antibodies (ANA) are suspected of having relevance to adverse reproductive events. This study aims to investigate the potential effect of ANA on IVF/ICSI outcome and the therapeutic role of prednisone plus low-dose aspirin (P + A) adjuvant treatment in ANA + patients. The first IVF/ICSI cycles without P + A of sixty-six ANA + women were enrolled as the ANA + group, and the 233 first IVF/ICSI cycles of matched ANA- women served as the ANA- group. The ANA + group was divided into the Titre < =1:320 subgroup and the Titre > 1:320 subgroup. Twenty-one ANA + women with adverse outcomes in their first cycles (ANA + cycles without P + A) received P + A adjuvant treatment for three months before the second IVF/ICSI cycle (ANA + cycles with P + A). The clinical characteristics and the IVF/ICSI outcomes were compared, respectively, between 1) the ANA + group and the ANA- group, 2) the Titre < =1:320 subgroup and the Titre > 1:320 subgroup, and 3) the ANA + cycles without P + A and the ANA + cycles with P + A. No significant differences were observed between each of the two-group pairs in the clinical characteristics. The ANA + group exhibited significantly lower MII oocytes rate, normal fertilisation, pregnancy and implantation rates, as well as remarkably higher abnormal fertilisation and early miscarriage rates. The Titre < =1:320 subgroup's IVF/ICSI outcomes were as poor as those of the Titre > 1:320 subgroup. After the P + A adjuvant treatment, the number of two pro-nuclei, perfect embryos and available embryos, and the implantation rate increased significantly. These observations suggest that ANA could exert a detrimental effect on IVF/ICSI outcome that might not be titre-dependent, and P + A adjuvant treatment could be useful for ANA + patients. This hypothesis should be verified in further prospective randomised studies.
    Reproductive Biology and Endocrinology 10/2013; 11(1):98. DOI:10.1186/1477-7827-11-98 · 2.23 Impact Factor
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    • "Primary thromboprophylaxis is recommended in patients with SLE and APS with hydroxychloroquine considered as a potential additional treatment for this syndrome. Possible future therapies for non-pregnant APS patients are statins, rituximab, and new anticoagulant drugs [14]. The most prevalent pathology in APS was thrombosis; venous (42.6%) and arterial (22%). "
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    ABSTRACT: Objective To detect the pulmonary involvement in asymptomatic secondary APS patients by pulmonary function tests (PFTs) and chest multislice HRCT angiography. Comparing the pulmonary findings to those of asymptomatic SLE patients without APS was considered.Patients and methodsThirty-four SLE patients with APS and another 34 SLE patients without APS and with a negative ACL test were included as control. All patients were asymptomatic for any pulmonary manifestations. Plain chest X-ray, HRCT angiography and PFTs were performed for all patients in an attempt to assess the pulmonary vasculature and lung parenchyma. Disease activity was assessed using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) while assessment of organ damage was made using the Systemic Lupus International Collaborating Clinics/ACR (SLICC/ACR) index.ResultsThere were abnormal pulmonary CT findings in 11 (32.35%) of the asymptomatic secondary APS patients with an obvious association to lupus anticoagulants. However, plain X-ray showed basal atelectasis and/or elevation of the copulae in four patients. Pulmonary abnormalities included a high frequency of pulmonary artery aneurysms (20.59%) thrombosis, basal atelectasis, embolism, bronchiectasis, pleural effusion and thickening. The SLEDAI and SLICC were significantly higher in APS patients. More SLE patients without APS were receiving hydroxychloroquine which suggests a protective role on the thromboembolic events occurring in APS.Conclusion Multislice HRCT pulmonary angiography, with its multiplanar capability, vascular reconstruction and high quality, is useful in demonstrating the entire thoracic spectrum in asymptomatic APS patients. Particular concern about the medications used in APS could prevent the risk of developing noticeable thromboembolic events.
    07/2012; 61(3):217–222. DOI:10.1016/j.ejcdt.2012.10.036
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