Antiphospholipid antibodies occur in various clinical states, including the primary antiphospholipid syndrome. Clinical features in these conditions appear to be caused by vasculopathy associated with the presence of these antibodies.. We report the case of a patient with primary antiphospholipid syndrome who experienced cardiac necrosis secondary to myocardial microvasculopathy in the absence of vasculitis. This case demonstrates unequivocally that noninflammatory myocardial microvasculopathy occurs in the primary antiphospholipid syndrome per se without any clinical or immunologic signs of systemic lupus erythematosus or other disease process. The histopathologic findings in the skin and myocardial biopsies showed a noninflammatory vasculopathy characterized by bland thrombi and lack of infiltration of the vessel wall by inflammatory cells. Ultrastructural examination of the myocardial biopsy confirmed the vascular thrombosis and endothelial activation and showed no deposits in basement ...

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OBJECTIVE To determine the prevalence of cardiac valvular involvement in patients with the primary antiphospholipid syndrome. DESIGN Cross-sectional study with evaluation of case patients and control patients by Doppler echocardiography. The mean follow-up for case patients was 22 months. SETTING University-based tertiary medical center. PATIENTS Twenty-eight consecutive patients who were diagnosed with the primary antiphospholipid syndrome during a 10-year period; 28 age- and sex-matched healthy controls. MEASUREMENTS AND MAIN RESULTS Ten patients (36%; 95% Cl, 19% to 56%) with the primary antiphospholipid syndrome had cardiac valvular involvement: Four patients had mitral valve involvement; four patients, aortic valve involvement; and two patients, both mitral and aortic valvular involvement; no patients had tricuspid or pulmonary valve disease. Eight of 10 patients had a regurgitant murmur. None of the control patients had valvular disease. The mean mitral valve thickness in patients with

Antiphospholipid syndrome is an autoimmune disease, in which antiphospholipid antibodies (anticardiolipin antibodies and lupus anticoagulant) react against proteins that bind to anionic phospholipids on plasma membranes. Like many autoimmune diseases, it is more common in women than in men. The exact cause is not known, but activation of the system of coagulation is evident. Clinically important antiphospholipid antibodies (those that arise as a result of the autoimmune process) are associated with thrombosis and vascular disease. The syndrome can be divided into primary (no underlying disease state) and secondary (in association with an underlying disease state) forms. Anti-ApoH and a subset of anti-cardiolipin antibodies bind to ApoH, which in turn inhibits Protein C, a glycoprotein with regulatory function upon the common pathway of coagulation (by degradating activated factor V). Lupus anticoagulant (LAC) antibodies bind to prothrombin, thus increasing its cleavage to thrombin, its active ...

Systemic Antiphospholipid Syndrome, Antiphospholipid Syndrome information and help pages for APS patients, doctors and medical professionals Antiphospholipid Syndrome - Systemic Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory abnormalities. These include persistently elevated levels of antibodies directed against membrane anionic phospholipids (ie, anticardiolipin [aCL] antibody, antiphosphatidylserine) or their associated plasma proteins, predominantly beta-2 glycoprotein I (apolipoprotein H), or evidence of a circulating anticoagulant.Multiple terms exist for APS. Unfortunately, some synonyms can be confusing. Lupus anticoagulant (LA) syndrome, for example, is misleading because patients may not ... Antiphospholipid Syndrome - Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory

Systemic Antiphospholipid Syndrome, Antiphospholipid Syndrome information and help pages for APS patients, doctors and medical professionals Antiphospholipid Syndrome - Systemic Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory abnormalities. These include persistently elevated levels of antibodies directed against membrane anionic phospholipids (ie, anticardiolipin [aCL] antibody, antiphosphatidylserine) or their associated plasma proteins, predominantly beta-2 glycoprotein I (apolipoprotein H), or evidence of a circulating anticoagulant.Multiple terms exist for APS. Unfortunately, some synonyms can be confusing. Lupus anticoagulant (LA) syndrome, for example, is misleading because patients may not ... Antiphospholipid Syndrome - Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal losses associated with typical laboratory

Donadini, MP, Crowther, M. Antiphospholipid syndrome: a challenging hypercoagulable state with systemic manifestations. Hematol-Oncol. vol. 24. 2010. pp. 669-76. (Recent concise review and update of this syndrome. Many of the clinical statistics were cited from this review.). Ruiz-Irastorza, G, Crowther, M, Branch, W, Khamashta, MA. Antiphospholipid syndrome. Lancet. vol. 376. 2010. pp. 1498-1509. (Along with the first listed reference, a key source for this chapter, especially with respect to treatment recommendations.). Frances, C. Dermatological manifestations of Hughes antiphospholipid antibody syndrome. Lupus. vol. 19. 2010. pp. 1071-77. (Current review of cutaneous manifestations.). Weinstein, S, Piette, W. Cutaneous manifestations of antiphospholipid antibody syndrome. Hematol Oncol Clinics N Am. vol. 22. 2008. pp. 67-77. (Relatively recent review of cutaneous findings.). Hughes, GRV. Antiphospholipid syndrome (Hughes syndrome): 10 clinical topics. Lupus. vol. 19. 2010. pp. ...

How is Seronegative Antiphospholipid Antibody Syndrome abbreviated? SNAPS stands for Seronegative Antiphospholipid Antibody Syndrome. SNAPS is defined as Seronegative Antiphospholipid Antibody Syndrome rarely.

TY - JOUR. T1 - Bioprosthetic mitral valve thrombosis complicating antiphospholipid antibody syndrome, successfully treated with thrombolysis. AU - Chamsi-Pasha, Mohammed A.. AU - Alyousef, Tareq. AU - Sayyed, Samer H. PY - 2014/1/1. Y1 - 2014/1/1. N2 - The incidence of bioprosthetic valve thrombosis and related embolic complications is extremely rare, obviating the need for long-term anticoagulation. As a result, experience in the diagnosis and treatment of bioprosthetic valve thrombosis is fairly limited. We report the first case of antiphospholipid antibody syndrome presenting as bioprosthetic mitral valve thrombosis, 15 months after valve replacement, and successfully treated with thrombolytic therapy. (Echocardiography 2014;31:E278-E281).. AB - The incidence of bioprosthetic valve thrombosis and related embolic complications is extremely rare, obviating the need for long-term anticoagulation. As a result, experience in the diagnosis and treatment of bioprosthetic valve thrombosis is fairly ...

Antiphospholipid antibody syndrome is a fascinatingly complex disorder. It is an autoimmune disease, whereby the immune system attacks our own tissues. This article summarises the basic mechanisms of the condition and main diseases it is involved in. Learn about the diverse effects antiphospholipid antibody syndrome (APS) can have, from heart attack to miscarriage and how APS can be treated.

TY - JOUR. T1 - De novo infantile primary antiphospholipid antibody syndrome. AU - Alshekaili, J.. AU - Reynolds, G.. AU - Cook, M. C.. PY - 2010/11. Y1 - 2010/11. N2 - Most autoimmune diseases are rare in infants. Early onset can represent an extreme phenotype arising from strong genetic predisposition relatively independent of environmental influence. Alternatively, neonatal autoimmunity can arise from transplacental passage of maternal pathogenic IgG autoantibodies. Distinguishing between these possible explanations is crucial for determining the prognosis in the specific patient, and has important implications for understanding pathogenesis. We report a case of neonatal thrombotic stroke associated with both cardiolipin and β2-glycoprotein I antibodies in neonatal serum but absent from cord blood and maternal serum. While the child also carried one prothrombotic allele of factor V (Leiden allele), which may have contributed to the risk of thromboembolic disease, the serological analysis ...

This guideline reviews the features of the Antiphospholipid syndrome [APS]- definition, clinical association, pathophysiology and the laboratory detection of Antiphospholipid antibodies. It includes a section on who should be tested for aPL antibodies and how this should influence their management. It also includes sections on the management of APS and the Catastrophic Antiphospholipid syndrome [CAPS].. ...

Antiphospholipid Antibody Syndrome (APS) is a highly prevalent cause of antibody-mediated thrombosis manifesting in venous thrombosis (DVT and PE), arterial thrombosis (most commonly stroke), and pregnancy complications. The diagnosis of definite APS requires both clinical and laboratory criterion as established by the working group of the International Congress on Antiphospholipid Antibodies (based on expert opinion). Since thrombosis and pregnancy loss are common in the general population, and antiphospholipid antibodies (aPL) occurs in a small percentage of the healthy public, it is important to demonstrate antibody persistence in patients who have the proper clinical indications in order to avoid misdiagnosis. Unfortunately, laboratory testing in this area lacks standardization, resulting in wide inter-laboratory variance. However, due to the commercialization of tests and automation, inter-laboratory variance has improved. Data on several new non-criterion tests suggest that they may ...

Antiphospholipid Antibody Carriers - Antiphospholipid Antibody Syndrome. Rare Diseases of the Immune System - by Pier Luigi Meroni

New life-saving treatments for Antiphospholipid syndrome in clinical trial on Genetic Risk Factors Associated With Antiphospholipid Antibody Syndrome

Pulmonary embolism is the most common pulmonary manifestation of primary antiphospholipid syndrome (PAPS). However, PAPS may manifest in the respiratory system also due to non-thrombotic processes. In the following paper we present a case of PAPS-related diffuse alveolar hemorrhage (DAH). Because of sparse literature and a lack of randomized controlled trials, there are currently no recommendations regarding the optimal choice of steroid-sparing agent in treating PAPS-related DAH. In our patient, treatment with cyclophosphamide or mycophenolate mofetil along with low dose prednisone was ineffective, partially because of infectious complications, whereas addition of monthly intravenous immunoglobulin to mycophenolate mofetil and prednisone, appears to control the disease. ...

Antiphospholipid antibody syndrome (APS) is a recently-diagnosed syndrome presenting with arterial and venous thrombosis, recurrent miscarriages and thrombocytopenia in the presence of antiphospholipid antibodies. A 16- year-old man referred due to right sided chest pain, dyspnea and cyanosis of two fingers presented for 2 months. After a complete workup, diagnosis of pulmonary thromboembolism was confirmed through clinical examination, spiral chest CTscan and lower limb Doppler sonography. He had positive anticardiolipin antibody, lupus anticoagulant, ANA and anti dsDNA. Based on these findings, diagnosis of APS (probably secondary to SLE) was made. Symptoms were improved by anticoagulant, prednisolone and chloroquine therapy. In a conclusion, pulmonary embolism may be the first presentation of APS, especially in young adults. (Tanaffos 2008; 7(1): 71-74)

TY - JOUR. T1 - Evaluation of phosphatidylserine-dependent antiprothrombin antibody testing for the diagnosis of antiphospholipid syndrome. T2 - Results of an international multicentre study. AU - Amengual, O.. AU - Forastiero, R.. AU - Sugiura-Ogasawara, M.. AU - Otomo, K.. AU - Oku, K.. AU - Favas, C.. AU - Delgado Alves, J.. AU - Žigon, P.. AU - Ambrožič, A.. AU - Tomšič, M.. AU - Ruiz-Arruza, I.. AU - Ruiz-Irastorza, G.. AU - Bertolaccini, M. L.. AU - Norman, G. L.. AU - Shums, Z.. AU - Arai, J.. AU - Murashima, A.. AU - Tebo, A. E.. AU - Gerosa, M.. AU - Meroni, P. L.. AU - Rodriguez-Pintó, I.. AU - Cervera, R.. AU - Swadzba, J.. AU - Musial, J.. AU - Atsumi, T.. PY - 2017/1/1. Y1 - 2017/1/1. N2 - Objective A task force of scientists at the International Congress on Antiphospholipid Antibodies recognized that phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT) might contribute to a better identification of antiphospholipid syndrome (APS). Accordingly, initial and ...

Rationale: Antiphospholipid syndrome (APS) in pregnancy may trigger the life-threatening catastrophic antiphospholipid syndrome (CAPS). Complement activation is implicated in the pathogenesis, and inhibition of complement factor C5 is suggested as an additional treatment option. Patient concerns, diagnosis and interventions: We present a pregnant patient treated with the C5-inhibitor eculizumab due to high risk of developing devastating APS-related complications. The complement inhibitory effects of the treatment were examined both in the patient and the premature infant. Outcomes: Complement activity in the mother recovered considerably faster than anticipated; however, no new thrombosis or CAPS developed during the last week of pregnancy or postpartum. Blood sampling from the umbilical vein and artery, and from the infant after delivery showed low complement activity; however, only 0.3% of the eculizumab concentration detected in the mother, consistent with low placental passage of eculizumab. ...

Sources Used in Current Review. Berg, T. (Updated 2015 April 15). Antiphospholipid syndrome and pregnancy. Medscape. Available online at http://emedicine.medscape.com/article/261691-overview. Accessed 2/15/2017.. (Reviewed 2016 March 21). Antiphospholipid syndrome. Lupus Foundation of America. Available online at http://www.lupus.org/answers/entry/antiphospholipid-syndrome. Accessed 2/15/2017.. Movva, S. (Updated 2016 July 1). Antiphospholipid syndrome workup. Medscape. Available online at http://emedicine.medscape.com/article/333221-workup. Accessed 2/15/2017.. Moake, J. (Revised 2016 September) Antiphospholipid antibody syndrome. Merck Manual Professional Version. Available online at http://www.merckmanuals.com/professional/hematology-and-oncology/thrombotic-disorders/antiphospholipid-antibody-syndrome-aps. Accessed 2/15/2017.. (Updated 2016 December). Antiphospholipid syndrome - APS. ARUP. Available online at http://www.arupconsult.com/Topics/APS.html?client_ID=LTD. Accessed 2/15/2017.. (© ...

TY - JOUR. T1 - IgG/IgM antiphospholipid antibodies present in the classification criteria for the antiphospholipid syndrome: a critical review of their association with thrombosis. AU - Kelchtermans, H.. AU - Pelkmans, L.. AU - de Laat, B.. AU - Devreese, K. M.. PY - 2016/8. Y1 - 2016/8. KW - anti-cardiolipin. KW - anti-phospholipid antibody. KW - antiphospholipid syndrome. KW - (2)-glycoprotein I. KW - IgM. U2 - 10.1111/jth.13379. DO - 10.1111/jth.13379. M3 - Article. VL - 14. SP - 1530. EP - 1548. JO - Journal of Thrombosis and Haemostasis. JF - Journal of Thrombosis and Haemostasis. SN - 1538-7933. IS - 8. ER - ...

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The adjusted global antiphospholipid syndrome score (aGAPSS) is a recently developed thrombotic risk assessment score that considers the antiphospholipid antibody (aPL) profile and conventional cardiovascular risk factors. In this retrospective study, we aimed to evaluate the validity of the aGAPSS in predicting clinical manifestations (criteria and extra-criteria) of antiphospholipid syndrome (APS) in a single centre cohort of patients. Ninety-eight patients with APS +/- systemic lupus erythematosus (SLE) were classified according to clinical manifestations as vascular thrombosis (VT), pregnancy morbidity (PM) or both (VT + PM). The aGAPSS was calculated for each patient as previously defined. Mean aGAPSS of the cohort was calculated as 10.2 +/- 3.8. Significantly higher aGAPSS values were seen in VT (n = 58) and VT + PM (n = 29) groups when compared to PM (n = 11) group (10.6 +/- 3.7 vs 7.4 +/- 2.9,P = 0.005; 10.7 +/- 4 vs 7.4 +/- 2.9,P = 0.008, respectively), mainly due to lower frequencies ...

The objective of this study was to highlight the need for investigation of antiphospholipid (aPL) antibodies in patients presenting with myocardial infarction (MI) and normal coronary arteries at angiography. We present five patients who were found to have had an MI without evidence of atherosclerosis. All had aPL antibodies and thus fulfilled the diagnosis of antiphospholipid syndrome (APS). Who did not have recurrent events on long-term anticoagulation maintaining an international normalised ratio of 3-4. This study suggests that APS is probably a major cause of MI in those with normal coronary arteries at angiography. It is an important diagnosis to make as they do not require anti-atherosclerotic treatment but appear, from this case series, to do well on high-dose warfarin. Further clinical studies are necessary to look at prevalence and best management in these patients.

TY - JOUR. T1 - Use of fondaparinux in a patient with antiphospholipid antibody syndrome and heparin-associated thrombocytopenia [9]. AU - Holtan, Shernan G.. AU - Knox, S. K.. AU - Tefferi, Ayalew. PY - 2006/7. Y1 - 2006/7. UR - http://www.scopus.com/inward/record.url?scp=33745225893&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=33745225893&partnerID=8YFLogxK. U2 - 10.1111/j.1538-7836.2006.01961.x. DO - 10.1111/j.1538-7836.2006.01961.x. M3 - Article. C2 - 16839370. AN - SCOPUS:33745225893. VL - 4. SP - 1632. EP - 1634. JO - Journal of Thrombosis and Haemostasis. JF - Journal of Thrombosis and Haemostasis. SN - 1538-7933. IS - 7. ER - ...

The Antiphospholipid Antibody Syndrome Market is expected to grow at a CAGR of 8.31% and is poised to reach US$XX Billion by 2027 as compared to US$XX

NorthShore encourages patients to utilize our medical library. Read our Antiphospholipid antibody syndrome encyclopedia resources online.

TY - JOUR. T1 - Thrombosis and thrombocytopenia in antiphospholipid syndrome (idiopathic and secondary to SLE). T2 - First report from the Italian Registry. AU - Barbui, T.. AU - Finazzi, G.. AU - Galli, M.. AU - Ciavarella, N.. AU - Schiavoni, M.. AU - Palareti, G.. AU - Marongiu, F.. AU - Muleo, G.. AU - Ferrari, L.. AU - Testa, S.. AU - Ballerini, G.. AU - Rossi Ferrini, P. L.. AU - Damasio, E.. AU - Trotta, A.. AU - Baudo, F.. AU - Pogliani, E.. AU - Rossi, E.. AU - DAngelo, A.. PY - 1993. Y1 - 1993. KW - antiphospholipid antibodies. KW - antiphospholipid syndrome. KW - Registry. KW - thrombocytopenia. KW - thrombosis. UR - http://www.scopus.com/inward/record.url?scp=0027515801&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=0027515801&partnerID=8YFLogxK. M3 - Article. C2 - 8314161. AN - SCOPUS:0027515801. VL - 78. SP - 313. EP - 318. JO - Haematologica. JF - Haematologica. SN - 0390-6078. IS - 5. ER - ...

BACKGROUND Antiphospholipid syndrome (APS) is an autoimmune systemic disease characterized by vascular thrombosis (arterial or venous) and/or pregnancy complications associated with the occurrence of autoantibodies, specifically lupus anticoagulant, anticardiolipin antibodies, and/or anti-β2 glycoprotein-I antibodies confirmed at least twice over a 12 week period according to the 2006 Sydney criteria. Antiphospholipid antibodies are encountered in the general population with a reported prevalence of 1% to 5% However, APS is far more infrequent with a prevalence of 40-50/100,000 persons and an incidence of about 5 new patients/100,000 persons. APS can be diagnosed in patients with no apparent clinical or laboratory pathology (primary APS) or it may be related to numerous other conditions, autoimmune diseases (usually systemic lupus erythematosus), malignancies, infections and drugs (secondary APS). Women are at risk for APS since the disease is encountered in both the primary and the secondary state

Pregnancy morbidity is one of the clinical manifestations used for classification criteria of antiphospholipid syndrome (APS). During the 14th International Congress on Antiphospholipid Antibodies (aPL), a Task Force with internationally-known experts was created to carry out a critical appraisal of …

Pathogenic and Therapeutic Role of Vitamin D in Antiphospholipid Syndrome Patients By Svetlana Jelic, Dejan Nikolic, Dragomir Marisavljević and Ljudmila Stojanovich Submitted: November 5th 2015Reviewed: August 2nd 2016 Published: April 26th 2017 DOI: 10.5772/65071 Abstract In this chapter, the novel findings on interrelationship between vitamin D status and two well‐known prothrombotic states, antiphospholipid syndrome, particularly its thrombotic phenotype, and metabolic syndrome will be reviewed. We shall present the results obtained from patients included in Serbian National Antiphospholipid Syndrome Registry, 68 patients with primary antiphospholipid syndrome (PAPS) and 69 patients with antiphospholipid syndrome associated with certain autoimmune rheumatic disease (sAPS), as well as 50 patients with pure metabolic syndrome (MetS). These results will be analysed and compared with the novel literature data. Prevalence of MetS in APS is high, with the atherogenic dyslipidaemia as its most ...

Adams, MJ, Donohoe, S, Mackie, IJ and Machin, SJ 2001 , Anti-tissue factor pathway inhibitor activity in patients with primary antiphospholipid syndrome , British Journal of Haematology, vol. 114 , pp. 375-379 , doi: 10.1046/j.1365-2141.2001.02923.x. ...

Antiphospholipid syndrome (APS) is characterized by thromboses in the larger arteries and veins and thrombotic microangiopathy. Indications: Suspicion of antiphospholipid syndrome. Thromboembolism in ...

HCQ may improve pregnancy outcomes for women with antiphospholipid syndrome July 2, 2015Treatment with hydroxychloroquine may reduce pregnancy risks and increase gestational duration in women with antiphospholipid syndrome, according to data presented at the European League Against Rheumatism Ann...

Schmeding and Schneider [57] reviewed the literature from 2000-2010 on the QOL of patients with SLE. It has been shown to lower QOL in patients with SLE vs. patients with other chronic diseases. SLE significantly reduces patients ability to cope with everyday activities. It is conditioned by age, fatigue and coexistence of neurological and psychiatric disorders, depression and irritability. There was no direct correlation between the degree of disease activity and organ damage in SLE and QOL.. Choi et al. [58] evaluated that the QOL of patients with SLE was lower than the control group, consisting healthy people. Reduced QOL resulted in depression, taking GCS and fatigue, which was not the result of the degree of disease activity and organ damage. Understanding psychological problems and appropriate treatment can help to improve the QOL of patients with SLE, especially those taking high doses of glucocorticosteroids, even if the disease activity is low. The study included 108 patients with SLE, ...

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin antibodies and anti-β2-glycoprotein 1 antibodies. APS can present with a variety of clinical phenotypes, including thrombosis in the veins, arteries and microvasculature as well as obstetrical complications. The pathophysiological hallmark is thrombosis, but other factors such as complement activation might be important. Prevention of thrombotic manifestations associated with APS includes lifestyle changes and, in individuals at high risk, low-dose aspirin. Prevention and treatment of thrombotic events are dependent mainly on the use of vitamin K antagonists. Immunosuppression and anticomplement therapy have been used anecdotally but have not been adequately tested. Pregnancy morbidity includes unexplained recurrent early miscarriage, fetal death and late obstetrical manifestation such as pre-eclampsia, premature birth or fetal ...

A 48-year-old woman with antiphospholipid syndrome (APS) had multiple skin necrosis caused by massive bleeding and hematoma collection at the right lower leg, left thigh, and abdomen. During the first month, we did surgical debridement every 2 to 3 days with meticulous coagulation and applied negative pressure wound therapy (NPWT). See the original post ...

During the past 12 years both clinicians and researchers involved in rheumatology, haematology, clinical immunology, and gynaecology have been inundated with hundreds of manuscripts regarding the so called antiphospholipid syndrome (APS).. As a result of the spread of information about the pathogenesis and clinical aspect of antiphospholipid antibodies (APA) in humans, non-professional APA readers have been often puzzled by APA characterisation, criteria for APS definition, reliability of assays for APA detection, and APS management.. None the less, thanks to the growing number of clinical findings concerning APS, it has became apparent that understanding of this syndrome may be of interest to other medical specialties.. Asherson and colleagues faced the difficult task of going through the great amount of information on APS and to present it in a systematic and didactic way.. The book is homogeneously structured in three sections dealing with aetiopathogenic mechanisms of APS (section I), its ...

Antiphospholipid syndrome is a systemic autoimmune disorder. It is usually defined as the clinical complex of vascular occlusion and ischemic events occurring in patients who have circulating antiphospholipid antibodies. Clinical presentation A...

Antiphospholipid syndrome is a systemic autoimmune disorder. It is usually defined as the clinical complex of vascular occlusion and ischaemic events occurring in patients who have circulating antiphospholipid antibodies. Pathology Patients hav...

Outcome of primary antiphospholipid syndrome in childhood. Gattorno, M.; Falcini, F.; Ravelli, A.; Zulian, F.; Buoncompagni, A.; Martini, G.; Resti, M.; Picco, P.; Martini, A. // Lupus;2003, Vol. 12 Issue 6, p449 The objective of this paper is to investigate the long-term outcome of primary antiphospholipid syndrome (APS) in the paediatric age. The features of unselected patients with primary APS who had disease onset before the age of 16 years were retrospectively analysed in three Italian referral... ...

The classical clinical picture of the antiphospholipid syndrome (APS) is characterized by venous and arterial thromboses, fetal losses and thrombocytopenia, in the presence of antiphospholipid...

A 32-year-old woman was admitted for mild gait difficulty and behavioural changes of 3 years duration. Familial and medical histories were unremarkable. Neurological examination revealed: mildly slowed gait, decreased synkinesias of the left arm (held abducted and flexed), increased deep tendon reflexes, Babinski signs, mild lower limb spasticity and dysmetria of left limbs. Depression and mild executive dysfunctions were also found.. Brain CT showed cerebral white matter (WM) calcifications (fig 1). MRI revealed T2 WM hyperintensities in the centra semiovalia, around the frontal horns and, to a lesser extent, in the posterior regions. Post-contrast MRI demonstrated points and stripes of enhancement in the periventricular WM, which are consistent with engorged medullary vessels (fig 2). Proton ...

Fingerprint Dive into the research topics of Antiphospholipid antibody syndrome mimicking multiple sclerosis clinically and by magnetic resonance imaging. Together they form a unique fingerprint. ...

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Blood. 117(25), 6939-6947. Agar C, de Groot PG, Marquart JA & Meijers JCM. (2011b) Evolutionary conservation of the LPS binding site of beta2-glycoprotein I. Thromb Haemost. In press. Arad A, Proulle V, Furie RA, Furie BC & Furie B. (2011) β₂-Glycoprotein-1 autoantibodies from patients with antiphospholipid syndrome are sufficient to potentiate arterial thrombus formation in a mouse model. Blood. 117(12), 3453-3459. Bevers EM & Galli M. (1990) Beta 2-glycoprotein I for binding of anticardiolipin antibodies to cardiolipin. Electron microscopy analysis of β2GPI. , 2010). Analysis of EM pictures showed that more than 99% of plasma β2GPI was in a closed conformation. These observations suggested that plasma β2GPI circulates in a circular (closed) conformation, whereas after interaction with antibodies β2GPI undergoes a major conformational change into a fishhook-like (open) structure. , 1996). When antibodies toward β2GPI were added to plasma, clotting times prolonged in a β2GPI-dependent ...

The antiphospholipid syndrome (APS) is characterised by the presence of antiphospholipid antibodies (aPA) associated with thrombosis (arterial and venous) and pregnancy morbidity. This thesis has aimed to investigate the frequency of protein C pathway defects in patients with aPA and to study clinical correlates examine the mechanisms of antiphospholipid interference in the protein C pathway and to assess activated protein C (APC) resistance in patients with aPA in terms of thrombin generation. Although have I have discovered a high degree of heterogeneity in the phenotype of patients with APS, I have demonstrated APC resistance and increased thrombin generation in the majority of patients with APS. While in some cases, APC resistance is clearly immunoglobulin mediated, it is a multifactorial phenomenon with many confounding variables. My data suggest that immunoglobulin dependent APC resistance may occur through P2 glycoprotein-I dependent and independent mechanisms. In a detailed study of ...

Erkan: Antiphospholipid Syndrome patients with vascular events generally receive life-long anticoagulation, despite the lack of high-quality data on the risk of recurrence and optimal duration of anticoagulation. Given that at least 50% of thrombotic events in aPL-positive patients have recognizable triggers, the possibility of discontinuing anticoagulation in highly selected patients exists, especially when the triggers are eliminated. However, discontinuation of anticoagulation is a critical decision that requires careful assessment of comorbidities, triggers, and residual thrombosis as well as patient education. The 13th International Congress on aPL Treatment Task Force recommended that in cases of first venous event, low-risk aPL profile, and a known transient precipitating factor, anticoagulation could be limited to 3-6 months. Given the importance of this question, i.e., anticoagulation withdrawal in APS, the development of a multicenter clinical trial protocol to determine whether ...

TY - JOUR. T1 - Efficacy of eculizumab in a patient with immunoadsorption- dependent catastrophic antiphospholipid syndrome. T2 - A case report. AU - Kronbichler, Andreas. AU - Frank, Renate. AU - Kirschfink, Michael. AU - Szilágyi, Ágnes. AU - Csuka, Dorottya. AU - Prohászka, Z.. AU - Schratzberger, Peter. AU - Lhotta, Karl. AU - Mayer, Gert. PY - 2014/11/14. Y1 - 2014/11/14. N2 - Catastrophic antiphospholipid syndrome (CAPS) is a rare but devastating complication in patients with antiphospholipid syndrome (APS) with a high morbidity and mortality. We describe a case of a 30-year old female patient with immunoglobulin A (IgA) deficiency who underwent splenectomy because of idiopathic thrombocytopenic thrombocytopenia. Subsequently, an APS and finally systemic lupus erythematosus was diagnosed. After an uncomplicated pregnancy that was terminated by cesarean section, the patient developed severe CAPS with cerebral, myocardial, renal, and pulmonary involvement. Because of IgA deficiency, ...

Introduction: Catastrophic Antiphospholipid syndrome (CAPS) and heparin-induced thrombocytopenia (HIT) are immune- mediated thrombotic conditions caused by an...

Free Online Library: The Wolf Hidden behind the Clots: Catastrophic Antiphospholipid Antibody Syndrome.(Case Report) by Case Reports in Medicine; Health, general Antibodies Health aspects Anticardiolipin antibodies Cytomegalovirus infections Development and progression Diagnosis Fondaparinux Infection Lupus Systemic lupus erythematosus Valganciclovir Viral antibodies

Catastrophic Antiphospholipid Antibody Syndrome (CAPS) is a rare condition in which life-threatening blood clots form in multiple organs simultaneously and can lead to multi-organ system failure and death. The causes of CAPS are not entirely understood, but CAPS episodes are often triggered by stressful events such as infections, surgery, or trauma. For patients who survive an episode of CAPS, permanent kidney failure is not uncommon because the kidneys are the organ system most frequently affected in CAPS. Kidney transplantation is the treatment of choice for end-stage kidney disease, but patients with a history of CAPS are exceptionally high-risk kidney transplant recipients because the chance that surgery itself could trigger a life-threatening or transplant-threatening episode of CAPS is significant. As a result, patients with CAPS are not generally considered candidates for transplantation. Despite this, these patients have a severely decreased life-expectancy on dialysis and their ...

Catastrophic Antiphospholipid Antibody Syndrome (CAPS) is a rare condition in which life-threatening blood clots form in multiple organs simultaneously and can lead to multi-organ system failure and death. The causes of CAPS are not entirely understood, but CAPS episodes are often triggered by stressful events such as infections, surgery, or trauma. For patients who survive an episode of CAPS, permanent kidney failure is not uncommon because the kidneys are the organ system most frequently affected in CAPS. Kidney transplantation is the treatment of choice for end-stage kidney disease, but patients with a history of CAPS are exceptionally high-risk kidney transplant recipients because the chance that surgery itself could trigger a life-threatening or transplant-threatening episode of CAPS is significant. As a result, patients with CAPS are not generally considered candidates for transplantation. Despite this, these patients have a severely decreased life-expectancy on dialysis and their ...

TY - JOUR. T1 - Libman-Sacks Endocarditis in a Patient with Antiphospholipid Syndrome. AU - Kotkar, Kunal D.. AU - Said, Sameh M.. N1 - Publisher Copyright: © 2016 The Society of Thoracic Surgeons. Copyright: Copyright 2017 Elsevier B.V., All rights reserved.. PY - 2016/7/1. Y1 - 2016/7/1. N2 - Antiphospholipid syndrome is a systemic autoimmune syndrome with cardiac manifestations such as nonbacterial thrombotic endocarditis, also known as Libman-Sacks endocarditis. A 61-year-old female with history of antiphospholipid syndrome presented in acute pulmonary edema. Echocardiography demonstrated mobile vegetations on the free margins of both the anterior and the posterior mitral valve leaflets. Blood cultures and fungal serologies were negative. During mitral valve replacement both the anterior and posterior mitral leaflets were covered with multiple small vegetations with features of nonbacterial thrombotic endocarditis. Though mostly asymptomatic, Libman-Sacks endocarditis may be an indication ...

Looking for online definition of antiphospholipid-antibody syndrome in the Medical Dictionary? antiphospholipid-antibody syndrome explanation free. What is antiphospholipid-antibody syndrome? Meaning of antiphospholipid-antibody syndrome medical term. What does antiphospholipid-antibody syndrome mean?

Mycoplasma penetrans, a rare bacterium so far only found in HIV-infected persons, was isolated in the blood and throat of a non-HIV-infected patient with primary antiphospholipid syndrome (whose etiology and pathogenesis are unknown).

Sixty six per cent of patients who survive an initial catastrophic APS event remained symptom free with anticoagulation during an average follow up of 67.2 months. Twenty six per cent of the survivors developed further APS related events and the mortality rate of these patients was about 25%.. Doctors have been increasingly recognising catastrophic APS but still many unresolved questions exist. The optimal treatment combination at the time of a catastrophic APS event is controversial,6 but anticoagulation is the preferred treatment in the long term. Although short term outcomes of catastrophic APS are documented in two different series,3,4 the long term outcomes are unknown. This is the first study demonstrating that catastrophic APS recurrence is unusual and patients treated with anticoagulation generally have a stable course.. Long term anticoagulation with warfarin is the standard of care to prevent a recurrent vascular event in patients with APS. The recurrence rate in untreated patients is ...

TY - JOUR. T1 - Antiphospholipid antibodies and retinal vascular disease. AU - Dunn, James P.. AU - Noorily, S. W.. AU - Petri, M.. AU - Finkelstein, D.. AU - Rosenbaum, J. T.. AU - Jabs, D. A.. PY - 1996. Y1 - 1996. N2 - Background. Antiphospholipid antibody retinopathy is a rare and poorly defined clinical entity in which the proper diagnosis has important ocular and systemic implications. The clinical course of five patients with antiphospholipid antibody retinopathy is described and the role of such antibodies in idiopathic retinal venous occlusive disease is investigated. Methods. Five case reports are presented highlighting the presentation, course, therapy, associations and outcomes of patients with antiphospholipid antibody retinopathy. In addition, twenty additional patients presenting with idiopathic retinal venous occlusive disease were tested for the presence of antiphospholipid antibodies. Results. All five patients with antiphospholipid antibody retinopathy had diffuse retinal ...

A 27-year-old Hispanic female was admitted to hospital with fever, a sudden marked decrease in vision, and multi-organ failure shortly after preterm delivery by cesarean section for eclampsia. Her past history was significant for a spontaneous first

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This page includes the following topics and synonyms: Antiphospholipid Antibody Test, aPL Antibody, Antiphospholipid Antibodies, Antiphospholipid Antibody, Anticardiolipin Antibody Test, Anticardiolipin Antibody.

Hepatitis C, a worldwide viral infection, is an important health problem in Brazil. The virus causes chronic infection, provoking B lymphocyte dysfunction, as represented by cryoglobulinemia, non-organ-specific autoantibody production, and non-Hodgkins lymphoma. The aim of this research was to screen for the presence of antiphospholipid autoantibodies in 109 Brazilian hepatitis C virus carriers without clinical history of antiphospholipid syndrome. Forty healthy individuals were used as the control group. IgA, IgG, and IgM antibodies against cardiolipin and β2-glycoprotein I were measured with an enzyme-linked immunosorbent assay, using a cut-off point of either 20 UPL or 20 SBU. While 24 (22.0%) hepatitis C carriers had moderate titers of IgM anticardiolipin antibodies (median, 22.5 MPL; 95%CI: 21.5-25.4 MPL), only three carriers (,3%) had IgG anticardiolipin antibodies (median, 23 GPL; 95%CI: 20.5-25.5 GPL). Furthermore, IgA anticardiolipin antibodies were not detected in these individuals. ...

You may also need to take blood thinners for 3 to 4 weeks after surgery to lower your risk of blood clots.. ANTIPHOSPHOLIPID ANTIBODY SYNDROME (APS) In general, you will need long-term treatment with a blood thinner for a long time if you have the APS. Initial treatment may be heparin, either unfractionated or low-molecular heparin. These medicines are given by injection.. In most cases, warfarin (Coumadin), which is given by mouth, is then started. It is necessary to monitor the level of anticoagulation frequently. This is most often done using the INR test.. If you have APS and become pregnant, you will need to be followed closely by a provider expert in this condition. You will not take warfarin during pregnancy, but will be given low-molecular weight heparin instead.. If you have SLE and APS your provider will also recommend that you take hydroxychloroquine. ...

Antiphospholipid syndrome is a thrombotic disorder characterized by the association of arterial and venous thrombosis with the antibodies directed toward phospholipids. The presence of these antibodies in systemic lupus erythematosus(SLE) has been shown to be related to several clinical and analytical alterations. We experienced one case of lupus nephritis with positive antiphospholipid antibodies in a 10-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24) ...

TY - JOUR. T1 - Antiphospholipid antibodies and fetal loss. T2 - Clinical association and possible pathogenetic role in experimental models. AU - Meroni, P. L.. AU - Piona, A.. AU - La Rosa, L.. AU - Tincani, A.. AU - Balestrieri, G.. AU - Casali, P.. PY - 1995/1/1. Y1 - 1995/1/1. N2 - It is widely accepted that the persistent presence of antiphospholipid antibodies (aPL) represents a high risk factor for recurrent spontaneous abortion, fetal growth retardation, and complicated pregnancies (early and severe preeclampsia). However, whether aPL can be regarded as direct pathogenetic antibodies and which pathogenetic mechanism sustains the clinical manifestations are still open questions. We approached the problem by (a) setting up an experimental model of fatal loss in naive mice; and (b) by investigating the deposition of β2 glycoprotein I (β2 GPI)-the plasma cofactor for aPL-on placentae from women suffering from aPL-associated recurrent abortion. In the experimental model we found that ...

TY - JOUR. T1 - Restricted T-cell receptor β-chain usage by T cells autoreactive to β2-glycoprotein I in patients with antiphospholipid syndrome. AU - Yoshida, Kazue. AU - Arai, Takahide. AU - Kaburaki, Junichi. AU - Ikeda, Yasuo. AU - Kawakami, Yutaka. AU - Kuwana, Masataka. PY - 2002/4/1. Y1 - 2002/4/1. N2 - We recently identified CD4+ T cells that are autoreactive to β2-glycoprotein I (β2GPI) and that promote antiphospholipid antibody production in patients with antiphospholipid syndrome (APS). In this study, T-cell receptor (TCR) β chains of β2GPI-reactive T cells were examined in 8 β2GPI-responders, including 5 patients with APS and 3 healthy subjects, using polymerase chain reaction and single-strand conformation polymorphism (PCRSSCP) analysis combined with in vitro stimulation of peripheral blood T cells with recombinant β2GPI. The TCR Vβ segments that expanded oligoclonally after stimulation with β2GPI varied among responders, but the Vβ7 and Vβ8 segments were commonly ...

TY - JOUR. T1 - Comparative analysis of different enzyme immunoassays for assessment of phosphatidylserine-dependent antiprothrombin antibodies. AU - Amengual, Olga. AU - Horita, Tetsuya. AU - Binder, Walter. AU - Norman, Gary L.. AU - Shums, Zakera. AU - Kato, Masaru. AU - Otomo, Kotaro. AU - Fujieda, Yuichiro. AU - Oku, Kenji. AU - Bohgaki, Toshiyuki. AU - Yasuda, Shinsuke. AU - Atsumi, Tatsuya. PY - 2014/9. Y1 - 2014/9. N2 - Phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT) were strongly correlated with the presence of lupus anticoagulant showing a high specificity for the diagnosis of antiphospholipid syndrome. However, the main criticism for the clinical applicability of aPS/PT testing is the lack of reproducibility of the results among laboratories. In this study, we measured IgG and IgM aPS/PT using our original in-house enzyme-linked immunosorbent assays (ELISA) and commercial ELISA kits to assess the assay performance and to evaluate the accuracy of aPS/PT results. The ...

Of the several types of antiphospholipid antibodies (aPL) described, lupus anticoagulant (LA) antibodies and anticardiolipin antibodies (aCL) are the types most clearly associated with antiphospholipid antibody syndrome (APS), the latter being the focus of this chapter. The Venereal Disease Research Laboratory (VDRL) and rapid plasma reagin tests were then developed, both employing purified cardiolipin combined with cholesterol and lecithin as the antigen. Classification of patients with APS considers both clinical and laboratory criteria. The syndrome is classified as primary APS when not associated with another disease and accounts for over 50% of cases. The first category of aPL, LA antibodies, are detected with certain phospholipid-dependent coagulation assays. Definite APS is present when at least one clinical criterion and one laboratory criterion are present. LAs are antibodies that are prothrombotic in vivo but display anticoagulant properties in vitro (i.e., they prolong certain coagulation

The objective of the study is to analyze the efficacy and safety of splenectomy in the management of refractory autoimmune thrombocytopenia (AT)/autoimmune hemolytic anemia (AIHA) associated or not with systemic lupus erythematosus. Thirty-four patients after splenectomy due to severe AT and/or AIHA were divided into group 1 (G1) 18 SLE/APS patients: 9 AT/SLE patients, 6 SLE/antiphospholipid syndrome (APS), and 3 primary APS. Group 2 (G2): 16 patients without SLE/APS: 2 Fisher-Evans syndrome and 14 AIHA. Surgery approach when (1) platelets ≤ 50,000/ml despite 2 weeks on medical therapy, (2) medically dependent, and (3) medically intolerant or after two hemolytic crises in AIHA patients ...

Myasthenia is a rare neurological condition with risk of death in case of inappropriate management. The outcome of this pathology is very bad in developing countries with inadequate technical Platform. We underlined the main difficulties of diagnostic and the management of Myasthenie and antisynthetase antibody syndrome in a 44 years-old Togolese man.

Summary Antiphospholipid antibodies (APLA) have been shown to activate endothelial cells (EC) in vitro, as documented by an increased expression of tissue factor as well as leukocyte adhesion molecules such as intercellular adhesion molecule-1, vascular cell adhesion molecule (VCAM)-1 and E-selecti...

Antiphospholipid antibodies (aPL) have been reported in the cerebrospinal fluids (CSF) of neurology patients but no CSF studies with psychiatric patients exist. We tested serum from 100 hospitalized psychotic patients having hallucinations and/or delusions for aPL. Patients with positive serum aPL f …

Read Antiphospholipid antibodies increase the levels of mitochondrial DNA in placental extracellular vesicles: Alarmin-g for preeclampsia, Scientific Reports on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips.

Our data suggest that subsequent thrombo-occlusive events and death after focal cerebral ischemia may occur sooner and more frequently in patients found to have IgG aCL immunoreactivity ,40 GPL units at the time of their index focal cerebral ischemic event when compared with patients who harbor 10 to 40 GPL at the time of their index event. Therefore GPL ,40 may be an important prognostic marker for subsequent thrombo-occlusive events in patients with index focal cerebral ischemia. Patients with ,40 GPL also appear to have more features of the antiphospholipid syndrome: younger age at onset and more recurrent strokes at the time of diagnosis.21 Furthermore, being in the GPL ,40 group appears to confer a sixfold-increased risk of developing subsequent TIAs only compared with being in the GPL ≤40 group. TIAs do not generally have as equally bad an outcome as ischemic stroke, although many hemispheric TIAs are minor infarcts by persistent subtle signs or neuroimaging abnormalities, and cognitive ...

Renal vein thrombosis (RVT) is the formation of a clot in the vein that drains blood from the kidneys, ultimately leading to a reduction in the drainage of one or both kidneys and the possible migration of the clot to other parts of the body. First described by German pathologist Friedrich Daniel von Recklinghausen in 1861, RVT most commonly affects two subpopulations: newly born infants with blood clotting abnormalities or dehydration and adults with nephrotic syndrome. Nephrotic syndrome, a kidney disorder, causes excessive loss of protein in the urine, hypoalbuminemia, hypercholesterolemia and edema, triggering a hypercoagulable state and increasing chances of clot formation. Other less common causes include hypercoagulable state, cancer, renal transplantation, behcet syndrome, antiphospholipid antibody syndrome or blunt trauma to the back or abdomen. Treatment of RVT mainly focuses on preventing further blood clots in the kidneys and maintaining stable renal function. The use of ...

Antiphospholipid antibodies (APAs) are detected in various autoimmune diseases, such as antiphospholipid syndrome (APS) and systemic lupus erythematosus. In addition to their binding to negatively charged phospholipids, APAs often cross-react with other molecules. Their potential biological effects are not fully understood. Apoptotic cells are a potential source of auto-antigens during systemic autoimmunity. Inefficient clearance of apoptotic cells results in the development of autoimmune manifestations and intracellular antigens such as nucleosomes become accessible during apoptosis. We examined a panel of monoclonal APAs generated from NZW/BXSB F1, a strain which spontaneously develops autoimmune symptoms reminiscent of APS. These APAs did not bind to live cells, but reacted strongly with different structures within apoptotic cells. Further analysis with various inhibitors indicated that the binding of APAs to apoptotic cells depends on specific caspase activities and on the modification of ...

Systemic lupus erythematosus (SLE) is an autoimmune disorder that occurs mainly in young women and can affect multiple organ systems. The clinical course of SLE is often one of spontaneous remissions and relapses. One of the most feared complications of SLE is acute neuropsychiatric disorders because of the profound effect on the patient and the association with high mortality (1). These disorders can present as seizures, psychosis, organic brain syndrome, strokes, and transient ischemic attacks (TIA) and may result in cumulative neurocognitive dysfunction. Recognized potential causes of these syndromes include cerebritis and hypercoagulable states such as antiphospholipid antibody syndrome and vasculitis (2). SLE is also known to promote the premature development of atherosclerosis, which is a common cause of stroke/TIA and dementia (3). Recently, attention has focused on the cardiac valvular abnormalities seen in SLE and their potential role in acute neuropsychiatric syndromes (4-7). The paper ...

Burgundy Awareness Ribbon Meaning What causes are associated with the burgundy awareness ribbon? * Amyloidosis * Antiphospholipid Antibody Syndrome * Aortic Aneurysm * AV Malformation * Brain Aneurysm * Congenital Vascular Cavernous Malformation * Cystic Hygroma * Disabled Adults * Factor V Leiden * Headache * Hemangioma * Hemiplegic Migraine * Hemochromatosis * Hirschsprungs Disease * Hughes Syndrome * Meningitis * Multiple Myeloma * Parkes Weber Syndrome * Port-Wine Stain Birthmark

An interdisciplinary faculty of neurologists and rheumatologists will provide an update on neuro-rheumatology-the neurological manifestations of systemic inflammatory and autoimmune syndromes. Part I of this course will review rheumatological diagnosis, physical examination techniques, and interpretation of laboratory studies; discuss neurological manifestations and treatment of Systemic Lupus Erythematosus, Sjogrens Syndrome, Antiphospholipid Antibody Syndrome, Mixed Connective Tissue Disease, and Rheumatoid Arthritis; and review CNS Vasculitis and other vasculitides. Part II will review diagnosis and treatment of Neurosarcoidosis, Pachymeningitis and Behcets Disease; discuss Peripheral Nervous System Manifestations of Rheumatological Diseases; and conclude with an interdisciplinary case discussion with faculty highlighting how rheumatologists and neurologists approach differential diagnosis, treatment, and management. This program complements C78: Neuro-rheumatology: Neurological ...

The antiphospholipid syndrome (APS) is a prothrombotic state characterized by recurrent arterial and venous thrombosis, recurrent pregnancy loss, and the presence of circulating antiphospholipid antibodies (aPL). Thrombophilia may be associated with premature atherosclerosis, and accelerated atherosclerosis was suggested as an additional clinical feature of APS. This pathological process may be mediated by the direct proinflammatory and procoagulant activity that aPLs exert on endothelial cells or indirectly, via the inflammatory/immune mechanisms that have been implicated in autoantibody-mediated thrombosis.40,41 In 1993, Vaarala et al42 provided the first evidence that aPLs may be involved in atherosclerosis. This study also suggested that some aPLs were capable of cross-reacting with oxLDL in SLE. Hypercholesterolemia, diabetes mellitus, smoking, obesity, arterial hypertension, and sedentary lifestyle in APS appear to be similar to those in the general population.41 Therefore, the ...

Antibodies directed toward phosphatidylethanolamine (anti-PE) appear to occur particularly frequently in women with unexplained early fetal loss (UFL). Two studies have shown that the presence of anti-PE antibodies is a higher independent risk factor for early UFL than either aCL or anti-β2GPI antibodies.22,23 Moreover, anti-PE antibodies have been reported as the only aPL antibodies found in cases of UFL (73%). Regarding thrombosis, which is the other main clinical feature of APS, a multicenter study conducted within the framework of the European Forum on aPL antibodies found the prevalence of anti-PE was 15% in patients with unexplained venous thrombosis; this specificity was found mainly as the sole aPL antibody.24 At present, there is no accepted standardized method for the measurement of anti-PE, and the heterogeneity of these antibodies increases the difficulties in attaining such a goal. This problem significantly limits the utility of this assay. Hence, following current evidence, the ...

The objective of the study is to analyze the efficacy and safety of splenectomy in the management of refractory autoimmune thrombocytopenia (AT)/autoimmune hemolytic anemia (AIHA) associated or not with systemic lupus erythematosus. Thirty-four patients after splenectomy due to severe AT and/or AIHA were divided into group 1 (G1) 18 SLE/APS patients: 9 AT/SLE patients, 6 SLE/antiphospholipid syndrome (APS), and 3 primary APS. Group 2 (G2): 16 patients without SLE/APS: 2 Fisher-Evans syndrome and 14 AIHA. Surgery approach when (1) platelets ≤ 50,000/ml despite 2 weeks on medical therapy, (2) medically dependent, and (3) medically intolerant or after two hemolytic crises in AIHA patients ...

If a woman suffers from autoimmune disease (AD), several factors can affect pregnancy or neonatal outcome: repeated spontaneous pregnancy losses (frequently related to antiphospholipid antibodies (aPL)), neonatal lupus with complete congenital heart block (CHB) (linked to transplacental passage of IgG anti Ro/SS-A antibodies) and the disease activity itself that can affect the mother, the pregnancy and fetal outcome. If appropriately managed, the antiphospholipid syndrome (APS) is bone of the few tractable causes of pregnancy losses.Q A recent case control study, on babies from APS-mothers and healthy mothers, did not show any difference in the occurrence of neonatal complications. There are few data about the longterm outcome of babies born to patients with AD. We recently reported increased occurrence of learning disabilities in children born to aPL positive mothers with systemic lupus erythematosus (SLE). The modern management of pregnancy in patients with AD includes the treatment of disease ...

Patients with renal diseases are prone to both thrombosis and bleeding, as they have profound changes in all three classic components of coagulation, defined approximately 150 years ago by Virchow: blood flow, vessel wall (endothelial injury), and coagulation properties of the blood (e.g., coagulation and fibrinolytic systems and platelets). The prothrombotic state in chronic kidney disease (CKD), glomerular diseases (including systemic lupus and vasculitis), and some less frequent conditions (idiopathic retroperitoneal fibrosis, antiphospholipid syndrome, hemolytic‐uremic syndrome, etc.) is associated with vascular endothelial damage, increase in certain coagulation and antifibrinolytic factors, decrease in anticoagulation proteins, dyslipidemia, hypoalbuminemia, changes in platelet membranes, hemo‐ and peritoneal dialysis and heparin treatment, increased microRNAs and circulating microparticles, antiphospholipid antibodies, nephrotic syndrome, anemia with high platelet count, and so on.

Results At least one EE was detected in 118 (63%) patients (52 cerebral, 95 other locations) after IE diagnosis in 80 (time interval between IE and EE diagnosis: 5.9±11.3 days). At least one aPL antibody was found in 31 patients (17%).. Detection of EE over time after IE diagnosis was more frequent among patients with anti-β2GPI IgM (log-rank P=0.0036) and that of cerebral embolisms, among patients with aCL IgM and anti-β2GPI IgM (log-rank P=0.002 and P,0.0001, respectively).. Factors predictive of EE were anti-β2GPI IgM (HR=3.45 (1.47-8.08), P=0.0045), creatinine (2.74 (1.55-4.84), P=0.0005) and vegetation size (2.41 (1.41-4.12), P=0.0014). Those of cerebral embolism were aCL IgM (2.84 (1.22-6.62), P=0.016) and anti-β2GPI IgM (4.77 (1.79-12.74), P=0.0018). ...

We report in Figure 2 the annual rate (∼ 0.4%) of first cardiovascular event (including VTE) in normal white population at 35 to 55 years of age,14 that (1.36%) of 125 single aPL positive carriers with a mean age of 41 years (data taken by a recently published study by our group),15 and that (5.3%) of triple-positive carriers of this study (mean age, 45 years). Annual rate in a cohort of SLE patients half of whom had aPL was 2.9 per 100 patient-years.16 Hence, because less than half of our cohort had autoimmune disorders, the higher incidence of TEs recorded in this study may be related to the selection of high-risk aPL-positive population. Likewise, in patients with APS and triple positivity,7 TEs were equally distributed among arterial and venous circulation. Some patients in this study were treated with aspirin for primary thromboprophylaxis, behavior that did not lead to a significant reduction of events. To our knowledge, only one prospective clinical trial addresses the question of ...

What are the effects of treatments for unexplained recurrent miscarriage? What are the effects of treatments for recurrent miscarriage caused by antiphospholipid syndrome?

An examination of the symptoms, rupture of blood cells (fibrin production), liver damage, clotting (low serum heparin), high blood pressure (capillary apoptosis), proteinuria (low heparan sulfate (HS) to prevent protein loss), pointed to some obvious treatments and the causes. Infertility is often treated by in vitro fertilization/insemination, supported with aspirin and heparin injections to maintain gestation. These treatments are consistent with high levels of chronic inflammation that block implantation and stimulate labor. Infertility is also associated with antiphospholipid antibodies. A closer look at the antiphospholipid antibodies showed that they were directed against β2-glycoprotein-I. So, I expected the β2-glycoprotein-I protein to be the original target for the antibodies, the initiating antigen, but when I looked up the sequence of that protein, it lacked the expected basic triplet I have found in all other autoantigens and allergens. This meant to me that there was a different ...

Accumulating evidence supports the concept of increased thrombin generation, placental vascular lesions, and inflammation as crucial points in the development of the great obstetrical syndromes [preeclampsia, intrauterine growth restriction (IUGR), preterm labor (PTL), preterm prelabor rupture of membranes (PROM), fetal demise and recurrent abortions]. In light of this, the role of heparins for primary or secondary prevention of these syndromes is becoming more and more apparent, mainly due to the antithrombotic and anti-inflammatory effects of heparins. There is agreement regarding the use of heparin in the prevention of gestational complications in patients with antiphospholipid syndrome, while its use for other obstetrical complications is under debate. In the present review we will describe the physiologic role of heparins on coagulation and inflammation and we will discuss current evidence regarding the use of heparins for the prevention/treatment of obstetrical syndromes ...

Stephan Moll, MD and Damon Houghton, MD write … In patients with antiphospholipid syndrome (APS) who require anticoagulation for the treatment of DVT or PE, warfarin or a low molecular weight heparin have traditionally been used. A question that comes up is whether one of the new oral anticoagulants (DOACs) can be effectively and safely used instead.. It is not known at this point whether DOACs are equally, more or less effective as/than warfarin in patients with APS. Data from clinical trials directly comparing DOACs with warfarin are not yet available. Given the absence of data, no formal recommendations or guidelines exist on this topic. It is an individualized decision between a physician and patient with APS whether to use warfarin or a DOAC for the treatment of DVT or PE.. Published Data. Several case reports and case series of patients with APS treated with a DOAC have been published. All data (from a total of 122 patients) have recently been summarized [1]: Sixteen percent of patients ...

97,. Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Lakos G, Tincani A, Kontopoulou-Griva I, Galeazzi M, Meroni PL, Derksen RH, de Groot PG, Gromnica-Ihile E, Baleva M, Mosca M, Bombardieri S, Houssiau F, Gris JC, Quere I, Hachulla E, Vasconcelos C, Roch B, Fernandez-Nebro A, Boffa MC, Hughes GR, Ingelmo M, Euro-Phospholipid Project Group. .Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 2002; 46: 1019-27 267). Cerca con Google ...

TY - JOUR. T1 - Autoimmune-mediated atherothrombosis. AU - Matsuura, Eiji. AU - Lopez, L. R.. PY - 2008. Y1 - 2008. N2 - Autoimmune vascular inflammation and oxidative stress (lipid peroxidation) are common in systemic autoimmune diseases and contribute to the oxidative modification of low-density lipoprotein (oxLDL) and oxLDL/β2GPI complex formation. Circulating oxLDL/β2GPI complexes have been detected in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). The presence of antibodies to oxLDL/β2GPI complexes indicates that these complexes are immunogenic, and the coexistence of complexes and antibodies has pointed to an active proatherogenic role in the development of autoimmune vascular complications. Immunohistochemical staining of atherosclerotic lesions suggest that these complexes are formed in the arterial wall and released into circulation. The in vitro macrophage uptake of oxLDL/β2GPI complexes was significantly increased in the presence of ...

Dr Deepa Jayakody Arachchillage trained in medicine at the North Colombo University in Sri Lanka and trained in haematology at the Newcastle Upon Tyne University Hospitals and University College London (UCL). She was awarded an MD (res) from UCL in July 2015 following successful completion of research related to antiphospholipid syndrome (APS), complement activation and direct acting oral anticoagulants. She has published in many peer reviewed journals and written several book chapters in the field. She has presented in a number of national and international conferences. She has been a co-investigator for several clinical trials and received funding for research projects. Dr Jayakody Arachchillage is a member of the British Society of Haematologists haemostasis and thrombosis task force. She has a specialised clinic for patients with APS at Hammersmith Hospital every Friday.. ...