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

ABSTRACT 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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    • "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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    • "Antiphospholipid syndrome (APS) is a systemic autoimmune disease in which the excessive blood clotting and/or certain complications of pregnancy (fetal loss or premature birth) are associated with the presence of antibodies that recognize anionic phospholipid–protein complexes (Ruiz-Irastorza et al. 2010). Although antiphospholipid antibodies such as those against cardiolipin , ␤2 glycoprotein 1 or lupus anticoagulant are both diagnostic markers for, and pathogenic drivers of APS (Sherer et al. 2007), the exact pathophysiological mechanisms of this disorder have Abbreviations: ␤2GPI␤, 2 glycoprotein-I; CL, cardiolipin; LA, lupus anticoagulant ; PAPS, primary antiphospholipid syndrome; SNP, single nucleotide polymorphism. "
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    ABSTRACT: The aim of the study was to investigate serum concentrations of interleukin (IL)-17 and IL-17-inducing cytokines IL-23 and transforming growth factor (TGF)-β, as well as IL-17 single nucleotide polymorphism (SNP) rs2275913 in patients with primary antiphospholipid syndrome (PAPS). We studied fifty patients with PAPS and fifty age- and sex-matched healthy controls. The cytokine levels were measured by ELISA, while the rs2275913 SNP located in promoter region of IL-17 gene was genotyped using real-time PCR. The significantly higher levels of IL-17 (p=0.002), IL-23 (p<0.001) and TGF-β (p=0.042) were found in PAPS patients (median 13.1, 9.4, and 125.6pg/ml, respectively) compared to the control group (6.8, 4.9 and 44.4pg/ml). There was a significant positive correlation between concentrations of IL-17 and IL-23 (r=0.540, p<0.001), but not between those of IL-17 and TGF-β. No statistically significant differences were observed in the distribution of genotypes and alleles of the IL-17 rs2275913 variants in patients with PAPS compared to healthy subjects. The blood concentrations of IL-17 did not differ in subjects with different rs2275913 genotypes or patients with or without antiphospholipid antibodies. Finally, a trend toward higher IL-17 levels (p=0.063) and the significantly higher IL-17 concentrations (p=0.012) were observed in PAPS patients with deep vein thrombosis and thrombocytopenia, respectively. These data demonstrate that IL-23/IL-17 axis, stimulated independently of TGF-β increase IL-17A gene polymorphism and antiphospholipid antibody production, might contribute to vascular manifestations of PAPS.
    Immunobiology 03/2012; 218(2). DOI:10.1016/j.imbio.2012.03.002 · 3.18 Impact Factor
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    • "Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by the presence of arterial or venous thrombosis and/or recurrent fetal loss, in addition to serum antiphospholipid (aPL) antibodies, mainly lupus anticoagulant (LA) and anticardiolipin antibody (aCL) [1]. APS can occur in persons without an underlying systemic autoimmune disease (primary APS or PAPS) or in the presence of other systemic autoimmune diseases, particularly systemic lupus erythematosus (SLE) [1] [2] [3]. "
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    ABSTRACT: To evaluate the frequency of seizures in primary antiphospholipid syndrome (PAPS) and their possible clinical and laboratory associations. Eighty-eight PAPS patients (Sydney's criteria) were analyzed by a standard interview, physical examination and review of medical charts. Risk factors for seizures, clinical manifestations, associated comorbidities, and antiphospholipid antibodies were evaluated. Nine (10.2%) patients with seizures were identified, 77.8% had convulsions onset after PAPS diagnosis. Mean age, gender, and race were comparable in groups with or without seizures. Interestingly, a higher frequency of current smoking (44.4 versus 10.1%, P = 0.019) was observed in the first group. Stroke, Sneddon's syndrome, and livedo reticularis were more frequent in PAPS patients with seizures than those without seizures, although not statistically significant (P > 0.05). Comparison between patients with seizures onset after PAPS diagnosis (n = 7) and those without convulsions (n = 79) demonstrated a higher frequency of current smoking (42.9 versus 10%, P = 0.042) and stroke in the first group (71.4 versus 30.4%, P = 0.041). Regression analysis confirmed that smoking (P = 0.030) and stroke (P = 0.042) were independently associated to seizures. About 10.2% of PAPS patients had convulsions, predominantly after PAPS diagnosis, and seizures were associated to current smoking and stroke.
    Clinical and Developmental Immunology 03/2012; 2012:981519. DOI:10.1155/2012/981519 · 2.93 Impact Factor
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