WorldWideScience

Sample records for antiphospholipid antibodies paradigm

  1. Antiphospholipid Antibody and Antiphospholipid Syndrome

    Institute of Scientific and Technical Information of China (English)

    吴竞生

    2008-01-01

    @@ Antiphospholipid antibodies (APA) APA is a big category for all kinds of negative charge phospholipid or lecithin - a protein complex autoantibodies or the same antibody, through its recognition of antigen (target protein) different, and phospholipids or lecithin - protein complex combination of various rely on the interference Phospholipid clotting and anti-coagulation factor, and promote endothelial cells, platelets, complement activation and play a role. APA including lupus anticoagulant(LA) and anticardiolipin antibody (ACA), In addition, there are anti-β2 glycoprotein-I (β2-GPI) antibody, anti-prothrombin (a- PT) antibody, anti-lysophosphatidic acid antibody and anti-phosphatidylserine antibody, and so on. APA as the main target of phospholipid-binding protein, including β2-GPI, prothrombin, annexin, protein C (PC) and protein S (PS), plasminogen, and so on.

  2. What Is Antiphospholipid Antibody Syndrome?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Is Antiphospholipid Antibody Syndrome? Antiphospholipid (AN-te-fos-fo-LIP-id) antibody ... weeks or months. This condition is called catastrophic antiphospholipid syndrome (CAPS). People who have APS also are at ...

  3. Antiphospholipid antibody syndrome.

    Science.gov (United States)

    Kutteh, William H; Hinote, Candace D

    2014-03-01

    Antiphospholipid antibodies (aPLs) are acquired antibodies directed against negatively charged phospholipids. Obstetric antiphospholipid antibody syndrome (APS) is diagnosed in the presence of certain clinical features in conjunction with positive laboratory findings. Obstetric APS is one of the most commonly identified causes of recurrent pregnancy loss. Thus, obstetric APS is distinguished from APS in other organ systems where the most common manifestation is thrombosis. Several pathophysiologic mechanisms of action of aPLs have been described. This article discusses the diagnostic and obstetric challenges of obstetric APS, proposed pathophysiologic mechanisms of APS during pregnancy, and the management of women during and after pregnancy.

  4. Evolution of antiphospholipid antibody syndrome.

    Science.gov (United States)

    Baviskar, Rutuja R; Amonkar, Gayathri P; Chaudhary, Vinod A; Balasubramanian, Meenakshi; Mohite, Shailesh C; Puranik, Gururaj V

    2012-12-01

    Antiphospholipid antibody syndrome is a very important cause of cerebral infarction, myocardial infarction, and repeated pregnancy losses in women. We present an extremely rare case of a 44-year-old man with antiphospholipid syndrome who collapsed and died suddenly. At autopsy, he was found to have both cerebral and myocardial infarction. In all young patients with cerebral infarction, myocardial infarction, pulmonary embolism, recurrent miscarriages, and unexplained low platelet count, one must consider the strong possibility of antiphospholipid antibody syndrome.

  5. Pathogenic role of antiphospholipid antibodies

    NARCIS (Netherlands)

    Salmon, J. E.; de Groot, P. G.

    2008-01-01

    The antiphospholipid antibody syndrome (APS) is characterized by recurrent arterial and venous thrombosis and/or pregnancy in association with antiphospholipid (aPL) antibodies. The pathogenic mechanisms in APS that lead to in vivo injury are incompletely understood. Recent evidence suggests that AP

  6. Lupus anticoagulants and antiphospholipid antibodies

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000547.htm Lupus anticoagulants and antiphospholipid antibodies To use the sharing features on this page, please enable JavaScript. Lupus anticoagulants are antibodies against substances in the lining ...

  7. Antiphospholipid antibody syndrome and autoimmune diseases.

    Science.gov (United States)

    Ostrowski, Rochella A; Robinson, John A

    2008-02-01

    The arbitrary division between antiphospholipid antibody syndrome and secondary antiphospholipid antibody syndrome has not proven useful. Antiphospholipid antibodies in the absence of antiphospholipid antibody syndrome often occur as epiphenomena in many autoimmune diseases. They are very common in systemic lupus erythematosus. Antiphospholipid antibody syndrome is a significant comorbidity in lupus but is uncommon in Sjögren's syndrome, rheumatoid arthritis, scleroderma, and systemic vasculitis. Evidence is growing that antiphospholipid antibodies may have a pathogenic role in pulmonary hypertension and accelerated atherosclerosis of autoimmune diseases.

  8. Antiphospholipid syndrome, antiphospholipid antibodies and solid organ transplantation.

    Science.gov (United States)

    González-Moreno, J; Callejas-Rubio, J L; Ríos-Fernández, R; Ortego-Centeno, N

    2015-11-01

    Antiphospholipid syndrome is considered a high risk factor for any kind of surgery. Considering that all solid organ transplants are critically dependent on the patency of vascular anastomosis, there is much concern about the consequences this pro-thrombotic condition may have on transplantation. Relatively little information is available in the literature assessing the real risk that antiphospholipid syndrome or the presence of antiphospholipid antibodies represent in solid organ transplantation. The aim of this article is to review the literature related to transplantation of solid organs in patients diagnosed with antiphospholipid syndrome or patients with positive antiphospholipid antibodies.

  9. Antiphospholipid Antibodies and Systemic Scleroderma

    Directory of Open Access Journals (Sweden)

    Awa Oumar Touré

    2013-03-01

    Full Text Available Objective: Antiphospholipid antibodies (APLs could be associated with an increased risk of vascular pathologies in systemic scleroderma. The aim of our study was to search for APLs in patients affected by systemic scleroderma and to evaluate their involvement in the clinical manifestations of this disease. Materials and Methods: We conducted a cross-sectional descriptive study, from January 2009 until August 2010, with patients received at the Department of Dermatology (Dakar, Senegal. Blood samples were taken at the hematology laboratory and were analyzed for the presence of APLs. Results: Forty patients were recruited. Various types of either isolated or associated APLs were found in 23 patients, i.e. 57.5% of the study population. The most frequently encountered antibody was IgG anti-β2 GPI (37.5% of the patients, followed by anticardiolipins (17.5% and lupus anticoagulants (5%. No statistically significant association of positive antiphospholipid-related tests to any of the scleroderma complications could be demonstrated. Conclusion: A high proportion of patients showing association of systemic scleroderma and APLs suggests the presence of a morbid correlation between these 2 pathologies. It would be useful to follow a cohort of patients affected by systemic scleroderma in order to monitor vascular complications following confirmation of the presence of antiphospholipid syndrome.

  10. Antiphospholipid Antibodies and Antiphospholipid Syndrome during Pregnancy: Diagnostic Concepts

    OpenAIRE

    Roger A. Levy; Flavia eCunha; de Jesús, Guilherme R.; Jesús,Nilson R. de

    2015-01-01

    Antiphospholipid syndrome comprises of a wide spectrum of clinical and obstetric manifestations linked to the presence of antiphospholipid antibodies. APS was described in the context of lupus and later as an isolated syndrome or primary APS. The Classification Criteria was designed for the definition of APS in epidemiologic and clinical studies is generally misused for clinical diagnostic decisions on an individual basis. The presence of aPL, especially the lupus anticoagulant test, is known...

  11. Primary Antiphospholipid Antibody Syndrome: A Case Report.

    Science.gov (United States)

    Kadeli, Deepak K; Hanjagi, Siddaraya Y

    2015-10-01

    Primary Antiphospholipid antibody syndrome is a rare disease associated with thromboembolic events which may affect either the arterial or the venous vasculature. It presents with an increased risk of thrombosis in pregnant woman leading to repeated fetal losses. We present here a case of primary antiphospholipid antibody syndrome in young women who had previous event of gangrene of toes leading to their amputation and repeated fetal losses.

  12. Antiphospholipid Antibody Syndrome Presenting with Unilateral Adrenal Hemmorhage.

    Science.gov (United States)

    Ullah, Kifayat; Butt, Ghias; Neopane, Sippy; Arshi, Shahana

    2016-06-01

    The antiphospholipid antibody syndrome presents with vascular thrombosis which involve both arterial and venous systems. The clinical presentation of antiphospholipid antibody syndrome includes obstetric complications leading to recurrent abortions, presence of circulating antibodies against phospholipids, and multi-organ thromboembolisms. We report a case of a patient who presented with unilateral adrenal hemorrhage and subsequently found to have antiphospholipid antibody syndrome and lupus nephritis.

  13. Graves' Disease Associated with Cerebrovascular Disease and Antiphospholipid Antibody Syndrome

    Directory of Open Access Journals (Sweden)

    Ines Khochtali

    2010-01-01

    have increased risk for developing thromboembolic accidents, which are favoured by a simultaneous presence of antiphospholipid antibodies syndrome. in this paper, we describe the case of a patient with Graves' disease, who developed strokes with antiphospholipid antibodies syndrome.

  14. Calciphylaxis in catastrophic antiphospholipid antibody syndrome.

    Science.gov (United States)

    Shah, Surbhi; Larson, Andrew; Datta, Yvonne

    2015-06-01

    Antiphospholipid antibody syndrome (APS) is a multisystem disorder characterized by vascular thrombosis and presence of circulating autoantibodies. The presence of APS can predispose to macrovascular as well as microvascular thrombotic events. Renal involvement is a common occurrence especially in the background of systemic lupus erythematosus. Skin appears to be another frequent target organ and a significant proportion of patients may present with skin lesions at the time of diagnosis. We present the case of a patient who presented with skin necrosis secondary to antiphospholipid antibody syndrome despite being on therapeutic anticoagulation and then developed dystrophic calcification secondary to her renal insufficiency. This complex skin condition eventually leads to her demise, as she was not a candidate for surgical management of these lesions. Why is this important? This case brings to our attention the need to consider calciphylaxis as a cause of ecchymotic-appearing skin lesions in dialysis patients on warfarin in patients with antiphospholipid antibody syndrome.

  15. Origin and pathogenesis of antiphospholipid antibodies

    Directory of Open Access Journals (Sweden)

    C.M. Celli

    1998-06-01

    Full Text Available Antiphospholipid antibodies (aPL are a heterogeneous group of antibodies that are detected in the serum of patients with a variety of conditions, including autoimmune (systemic lupus erythematosus, infectious (syphilis, AIDS and lymphoproliferative disorders (paraproteinemia, myeloma, lymphocytic leukemias. Thrombosis, thrombocytopenia, recurrent fetal loss and other clinical complications are currently associated with a subgroup of aPL designating the antiphospholipid syndrome. In contrast, aPL from patients with infectious disorders are not associated with any clinical manifestation. These findings led to increased interest in the origin and pathogenesis of aPL. Here we present the clinical features of the antiphospholipid syndrome and review the origin of aPL, the characteristics of experimentally induced aPL and their historical background. Within this context, we discuss the most probable pathogenic mechanisms induced by these antibodies.

  16. Imaging spectrum of primary antiphospholipid antibody syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Kwon Ha; Won, Jong Jin [Wonkwang University Hospital, Iksan (Korea, Republic of); Ha, Hyun Kwon; Kim, Jung Hoon; Kim, Jeong Gon; Ki, Won Woo; Kim, Pyo Nyun; Lee, Moon Gyu; Auh, Yong Ho [Asan Medical Center, Seoul (Korea, Republic of)

    1998-04-01

    Antiphospholipid antibody syndrome is recognized as one of the most important causes of hypercoagulability. It can be clinically diagnosed if patients have experienced unexplained recurrent venous or arterial thrombosis, recurrent fetal loss, or thrombocytopenia in the presence of circulating autoantibodies to phospholipids, such as anticardiolipin antibody or lupus anticoagulant. Approximately half of all patients with this syndrome do not have associated systemic disease, and their condition is described as primary antiphospholipid antibody syndrome (PAPS). In the remainder, the syndrome is accompanied by systemic lupus erythematosus or other connective tissue diseases, and is known as secondary antiphospholipid syndrome (1). The purpose of this paper is to illustrate the systemic manifestation of PAPS, focusing on the radiological findings of CT, MR and angiography in clinically proven patients. (author). 8 refs., 10 figs.

  17. Antiphospholipid Antibodies and Antiphospholipid Syndrome during Pregnancy: Diagnostic Concepts.

    Science.gov (United States)

    Levy, Roger A; Dos Santos, Flavia Cunha; de Jesús, Guilherme R; de Jesús, Nilson R

    2015-01-01

    Antiphospholipid syndrome (APS) comprises of a wide spectrum of clinical and obstetric manifestations linked to the presence of antiphospholipid antibodies (aPL). APS was described in the context of lupus, and later as an isolated syndrome or primary APS. The presence of aPL, especially the lupus anticoagulant test, is associated with adverse pregnancy outcomes, such as fetal death, recurrent early miscarriages, pre-eclampsia, and placental insufficiency, but does not seem to influence infertility. High quality scientific data to support these associations, however, are lacking, and controversies arise about the definition of positive aPL (low vs medium-high titers) or even the definition of the adverse events. This review discusses APS classification criteria and the current debate about it.

  18. Antiphospholipid Antibodies in Lupus Nephritis.

    Directory of Open Access Journals (Sweden)

    Ioannis Parodis

    Full Text Available Lupus nephritis (LN is a major manifestation of systemic lupus erythematosus (SLE. It remains unclear whether antiphospholipid antibodies (aPL alter the course of LN. We thus investigated the impact of aPL on short-term and long-term renal outcomes in patients with LN. We assessed levels of aPL cross-sectionally in SLE patients diagnosed with (n = 204 or without (n = 294 LN, and prospectively in 64 patients with active biopsy-proven LN (52 proliferative, 12 membranous, before and after induction treatment (short-term outcomes. Long-term renal outcome in the prospective LN cohort was determined by the estimated glomerular filtration rate (eGFR and the Chronic Kidney Disease (CKD stage, after a median follow-up of 11.3 years (range: 3.3-18.8. Cross-sectional analysis revealed no association between LN and IgG/IgM anticardiolipin or anti-β2-glycoprotein I antibodies, or lupus anticoagulant. Both aPL positivity and levels were similar in patients with active LN and non-renal SLE. Following induction treatment for LN, serum IgG/IgM aPL levels decreased in responders (p<0.005 for all, but not in non-responders. Both at active LN and post-treatment, patients with IgG, but not IgM, aPL had higher creatinine levels compared with patients without IgG aPL. Neither aPL positivity nor levels were associated with changes in eGFR from either baseline or post-treatment through long-term follow-up. Moreover, aPL positivity and levels both at baseline and post-treatment were similar in patients with a CKD stage ≥3 versus 1-2 at the last follow-up. In conclusion, neither aPL positivity nor levels were found to be associated with the occurrence of LN in SLE patients. However, IgG aPL positivity in LN patients was associated with a short-term impairment of the renal function while no effect on long-term renal outcome was observed. Furthermore, IgG and IgM aPL levels decreased following induction treatment only in responders, indicating that aPL levels are

  19. Pulmonary manifestations of the antiphospholipid antibody syndrome.

    Science.gov (United States)

    Ford, H James; Roubey, Robert A S

    2010-09-01

    A broad spectrum of pulmonary disease may occur in antiphospholipid antibody syndrome. The most common pulmonary manifestations are pulmonary thromboembolism and pulmonary hypertension. In this article the authors review these manifestations, as well as less common findings including acute respiratory distress syndrome, alveolar hemorrhage, and pulmonary capillaritis.

  20. The antiphospholipid antibody syndrome: a case report.

    Science.gov (United States)

    Luma, Henry Namme; Doualla, Marie-Solange; Temfack, Elvis; Bagnaka, Servais Albert Fiacre Eloumou; Mankaa, Emmanuella Wankie; Fofung, Dobgima

    2012-01-01

    Antiphospholipid antibody syndrome is defined by the presence of thromboembolic complications and/or pregnancy morbidity in the presence of persistently increased titers of antiphospholipid antibodies. Its clinical presentation can be diverse and any organ can be involved, with a current impact in most surgical and medical specialties. The authors present the case of a 43-year-old man who, over a 13-year period of follow-up, presented with thrombosis of the mesenteric vein, inferior vena cava, and axillary and subclavian veins in a setting where diagnostic and therapeutic options are limited and costly. Through this case report, the authors aim to describe the evolution of this complex pathology, which to date has not been described in the authors' milieu - probably because of its challenging diagnosis and the limited treatment options available. The authors conclude that clinicians need to have a high index of suspicion of APS in patients who present with a thrombotic episode - clinicians should investigate for the presence of antiphospholipid antibodies, as early diagnosis may influence the course of the disease. Furthermore, resources for the detection of antiphospholipid antibodies should be made readily available in resource-limited settings. Finally, patient education on the importance of drug compliance, periodic monitoring, and prevention of thrombosis is indispensable, especially as mortality could be associated with the effects of vascular thrombosis and/or the effects of bleeding due to anticoagulants.

  1. An atypical presentation of antiphospholipid antibody syndrome.

    Science.gov (United States)

    D'souza, Deepti; Dandakeri, Sukumar; Bhat, M Ramesh; Srinath, M K

    2015-01-01

    Cutaneous manifestations in antiphospholipid antibody syndrome (APS) though common, are extremely diverse and it is important to know which dermatological finding should prompt consideration of antiphospholipid syndrome. The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis, and systemic involvement is invariably an accomplice in APS. Cutaneous ulcers with sharp margins can be seen in APS and they are usually seen on the legs. This case had an atypical presentation, as the initial presentation was painful necrotic ulcers over the legs, which resembled pyoderma gangrenosum and she had no systemic manifestations. There was no history of any arterial or venous thrombosis or any abortions. Antiphospholipid syndrome can be tricky to diagnose when cutaneous lesions are atypical. Nonetheless, it is very important to pin down this syndrome early due to its systemic complications.

  2. An atypical presentation of antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Deepti D′Souza

    2015-01-01

    Full Text Available Cutaneous manifestations in antiphospholipid antibody syndrome (APS though common, are extremely diverse and it is important to know which dermatological finding should prompt consideration of antiphospholipid syndrome. The cutaneous manifestations of APS vary from livedo reticularis to cutaneous necrosis, and systemic involvement is invariably an accomplice in APS. Cutaneous ulcers with sharp margins can be seen in APS and they are usually seen on the legs. This case had an atypical presentation, as the initial presentation was painful necrotic ulcers over the legs, which resembled pyoderma gangrenosum and she had no systemic manifestations. There was no history of any arterial or venous thrombosis or any abortions. Antiphospholipid syndrome can be tricky to diagnose when cutaneous lesions are atypical. Nonetheless, it is very important to pin down this syndrome early due to its systemic complications.

  3. Antiphospholipid Antibodies and Antiphospholipid Syndrome During Pregnancy: Diagnostic Concepts

    Directory of Open Access Journals (Sweden)

    Roger A Levy

    2015-05-01

    Full Text Available Antiphospholipid syndrome comprises of a wide spectrum of clinical and obstetric manifestations linked to the presence of antiphospholipid antibodies. APS was described in the context of lupus and later as an isolated syndrome or primary APS. The Classification Criteria was designed for the definition of APS in epidemiologic and clinical studies is generally misused for clinical diagnostic decisions on an individual basis. The presence of aPL, especially the lupus anticoagulant test, is known to be a recurrence predictor for arterial events and fetal death. When the serologic assays are used it is important that the validated tests for anticardiolipin and anti-beta 2 glycoprotein I and cut-off are employed. Pathogenic mechanisms are various, beyond the inhibition of anticoagulant action of beta 2 glycoprotein I and other natural anticoagulants, including cell-mediated events involving endothelial and dendritic cells, monocytes and platelets. The mechanisms are not self-exclusive and may in fact be related, offering different targets for potential future therapies. During pregnancy a high-risk care setting, following a protocol of tests and prediction of events, is imperative. The relationship of aPL and infertility is not clear, w

  4. The antiphospholipid antibody syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Luma HN

    2012-10-01

    Full Text Available Henry Namme Luma,1,2 Marie-Solange Doualla,1,2 Elvis Temfack,1 Servais Albert Fiacre Eloumou Bagnaka,1 Emmanuella Wankie Mankaa,3 Dobgima Fofung41Department of Internal Medicine, Douala General Hospital, Douala, Cameroon; 2Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon; 3Department of Radiology, Douala General Hospital Douala, Cameroon; 4Department of Abdominal Surgery, Daniel Muna Memorial Clinic, Douala, CameroonAbstract: Antiphospholipid antibody syndrome is defined by the presence of thromboembolic complications and/or pregnancy morbidity in the presence of persistently increased titers of antiphospholipid antibodies. Its clinical presentation can be diverse and any organ can be involved, with a current impact in most surgical and medical specialties. The authors present the case of a 43-year-old man who, over a 13-year period of follow-up, presented with thrombosis of the mesenteric vein, inferior vena cava, and axillary and subclavian veins in a setting where diagnostic and therapeutic options are limited and costly. Through this case report, the authors aim to describe the evolution of this complex pathology, which to date has not been described in the authors' milieu – probably because of its challenging diagnosis and the limited treatment options available. The authors conclude that clinicians need to have a high index of suspicion of APS in patients who present with a thrombotic episode – clinicians should investigate for the presence of antiphospholipid antibodies, as early diagnosis may influence the course of the disease. Furthermore, resources for the detection of antiphospholipid antibodies should be made readily available in resource-limited settings. Finally, patient education on the importance of drug compliance, periodic monitoring, and prevention of thrombosis is indispensable, especially as mortality could be associated with the effects of vascular thrombosis and/or the effects

  5. Antiphospholipid antibody syndrome presenting as transverse myelitis

    Directory of Open Access Journals (Sweden)

    Javvid M Dandroo

    2015-01-01

    Full Text Available The antiphospholipid syndrome (APS is characterized by arterial and/or venous thrombosis and pregnancy morbidity in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disorder or secondary to a connective tissue disease, most frequently systemic lupus erythematosus. Central nervous system involvement is one of the most prominent clinical manifestations of APS, and includes arterial and venous thrombotic events, psychiatric features, and a variety of other nonthrombotic neurological syndromes. Although the mechanism of neurological involvement in patients with APS is thought to be thrombotic in origin and endothelial dysfunction associated with antiphospholipid antibodies. APS presenting as acute transverse myelitis is very rarely seen with a prevalence rate of 1%. We are describing a foreigner female presenting as acute transverse myelitis which on evaluation proved to be APS induced. So far, very few cases have been reported in literature with APS as etiology.

  6. Antiphospholipid antibody: laboratory, pathogenesis and clinical manifestations

    Directory of Open Access Journals (Sweden)

    T. Ziglioli

    2011-06-01

    Full Text Available Antiphospholipid antibodies (aPL represent a heterogeneous group of antibodies that recognize various antigenic targets including beta2 glycoprotein I (β2GPI, prothrombin (PT, activated protein C, tissue plasminogen activator, plasmin and annexin A2. The most commonly used tests to detect aPL are: lupus anticoagulant (LAC, a functional coagulation assay, anticardiolipin antibody (aCL and anti-β2GPI antibody (anti-β2GPI, which are enzyme-linked immunoassay (ELISA. Clinically aPL are associated with thrombosis and/or with pregnancy morbidity. Apparently aPL alone are unable to induce thrombotic manifestations, but they increase the risk of vascular events that can occur in the presence of another thrombophilic condition; on the other hand obstetrical manifestations were shown to be associated not only to thrombosis but mainly to a direct antibody effect on the trophoblast.

  7. Graves' Disease Associated with Cerebrovascular Disease and Antiphospholipid Antibody Syndrome

    OpenAIRE

    2010-01-01

    Thyroid disorders are commonly associated with coagulopathy. Patients with hyperthyroidism have increased risk for developing thromboembolic accidents, which are favoured by a simultaneous presence of antiphospholipid antibodies syndrome. in this paper, we describe the case of a patient with Graves' disease, who developed strokes with antiphospholipid antibodies syndrome.

  8. Antiphospholipid antibody syndrome presenting with hemichorea.

    Science.gov (United States)

    Ayalew, Yezenash; Khattak, Fazlihakim

    2012-01-01

    A 25-year-old Bangladeshi lady presented to neurology with a three-month history of involuntary movements of her right arm, associated with loss of power. There was progression to the right leg, and she subsequently developed episodes of slurred speech and blurred vision. At the time of presentation, she was 12 weeks pregnant and the symptoms were reported to have started at conception. Past medical history was unremarkable apart from one first trimester miscarriage and there was no significant family history suggestive of a hereditary neurological condition. MRI of the head revealed no abnormalities but serology showed positive antinuclear antibodies (ANAs) at a titre of 1/400. Further investigations revealed strongly positive anticardiolipin antibodies (>120) and positive lupus anticoagulant antibodies. The patient had a second miscarriage at 19 weeks gestation strengthening the possibility that the chorea was related to antiphospholipid antibody syndrome and she was started on a reducing dose of Prednisolone 40 mg daily and aspirin 300 mg daily. Six months later, she had complete resolution of neurological symptoms. There are several reports of chorea as a feature of antiphospholipid syndrome, but no clear consensus on underlying pathophysiology.

  9. Pathophysiology of the antiphospholipid antibody syndrome.

    Science.gov (United States)

    Willis, Rohan; Pierangeli, Silvia S

    2011-11-01

    Antiphospholipid antibodies (aPL) are associated with the recurrent pregnancy loss and thrombosis that characterizes the antiphospholipid antibody syndrome (APS). Although the ontogeny of these pathogenic antibodies has not been fully elucidated, there is evidence that indicates the involvement of both genetic and environmental factors. The ability of aPL to induce a procoagulant phenotype in APS patients plays a central role in the development of arterial and venous thrombotic manifestations typical of the disease. Inflammation serves as a necessary link between this procoagulant phenotype and actual thrombus development and is an important mediator of the placental injury seen in APS patients with obstetric complications. Recent evidence has indicated a role for abnormal cellular proliferation and differentiation in the pathophysiology of APS, especially in those patients with pregnancy morbidity and other more atypical manifestations that have no identifiable thrombotic cause. The interplay of genetic and environmental factors responsible for aPL development and the mechanisms by which these antibodies produce disease in APS patients is the focus of this review.

  10. Antiphospholipid Antibody Syndrome Presenting with Hemichorea

    Directory of Open Access Journals (Sweden)

    Yezenash Ayalew

    2012-01-01

    Full Text Available A 25-year-old Bangladeshi lady presented to neurology with a three-month history of involuntary movements of her right arm, associated with loss of power. There was progression to the right leg, and she subsequently developed episodes of slurred speech and blurred vision. At the time of presentation, she was 12 weeks pregnant and the symptoms were reported to have started at conception. Past medical history was unremarkable apart from one first trimester miscarriage and there was no significant family history suggestive of a hereditary neurological condition. MRI of the head revealed no abnormalities but serology showed positive antinuclear antibodies (ANAs at a titre of 1/400. Further investigations revealed strongly positive anticardiolipin antibodies (>120 and positive lupus anticoagulant antibodies. The patient had a second miscarriage at 19 weeks gestation strengthening the possibility that the chorea was related to antiphospholipid antibody syndrome and she was started on a reducing dose of Prednisolone 40 mg daily and aspirin 300 mg daily. Six months later, she had complete resolution of neurological symptoms. There are several reports of chorea as a feature of antiphospholipid syndrome, but no clear consensus on underlying pathophysiology.

  11. The investigation of relationship between preeclampsia and antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Mehmet Tayyar

    2013-03-01

    Full Text Available Aim. The aim of this study was evaluate the relationship between preeclampsia and antiphospholipid antibodies. Methods. A total of 116 pregnant women between 20th and 40th weeks of gestation admitted to our department were investigated. 63 of them were allocated our preeclampsia group and 53 of them were allocated our control group. Lupus anticoagulant, anti-cardiolipin antibodies (IG G ve M and antiphosphatidylserine antibodies (IG G ve M were measured. Results. There was no statistical significance between preeclampsia and control group for antiphospholipid antibodies but these were two times higher in preeclamptic group compared to control group. (22.2% in preeclampsia, 11.3% in control group p=0.193. Conclusions. In an unselected population we were not able to demonstrate an association between preeclampsia and antiphospholipid antibody syndrome but antiphospholipid antibody ratio elevated in women with preeclampsia. These findings show that, there is a need for large scale studies.

  12. Mechanisms of anti-phospholipid antibody formation and action

    NARCIS (Netherlands)

    de Groot, Philip G.

    2011-01-01

    The antiphospholipid syndrome is an autoimmune disease characterised by the clinical features of recurrent thrombosis in the venous or arterial circulation and foetal losses in combination with circulating anti-phospholipid antibodies in the blood of the afflicted patients. Over the last 25 years nu

  13. Progression of antiphospholipid antibody syndrome to catastrophic antiphospholipid antibody syndrome acutely with cessation of antithrombotic therapy.

    Science.gov (United States)

    Katikireddi, V S; Kandiah, D A

    2012-05-01

    Catastrophic antiphospholipid antibody syndrome (CAPS) is a serious condition that is often unrecognised with a high mortality. Cessation of anticoagulation in antiphospholipid antibody syndrome (APS) can have devastating consequences with progression to CAPS. Making a diagnosis of APS can however be challenging because of the evolving diagnostic criteria and difficulty in confirming thromboses. Management of these patients can also be complex, especially in those with coexistent thrombocytopenia. New potential treatments are emerging targeted on the immunomodulation of APS rather than just prevention of thrombosis. This article aims to highlight these diagnostic and management difficulties by reporting and discussing three cases of APS with progression to CAPS following cessation of anticoagulation, one with fatal consequences, with confirmation of CAPS on autopsy, and two with successful treatment and outcomes.

  14. Anticoagulation in management of antiphospholipid antibody syndrome in pregnancy.

    Science.gov (United States)

    Lockshin, Michael D

    2013-06-01

    Knowledge of antiphospholipid antibodies and their impact on pregnancy continues to evolve. A variety of antiphospholipid antibodies have been identified, but not all of them seem to be pathologic for pregnancy outcome. Understanding of which patients are at high risk for adverse pregnancy outcome and the most effective treatment will require clinical trials based on risk stratification and long-term follow-up of infants.

  15. Catastrophic antiphospholipid antibody syndrome in a child with thrombotic microangiopathy.

    Science.gov (United States)

    Prasad, N; Bhadauria, D; Agarwal, N; Gupta, A; Gupta, P; Jain, M; Lal, H

    2012-07-01

    Thrombotic microangiopathic hemolytic anemia (TMHA) is not uncommon in clinical nephrology practice while antiphospholipid syndrome (APS) is uncommon. Although less than 1% of patients with APS develop catastrophic APS (CAPS), its potential lethal outcome because of thrombosis in multiple organs and subsequent multiorgan failure emphasizes its importance in nephrology practice. Here is a case of catastrophic APS in a 7-year-old girl, who presented to us with TMHA associated with antiphospholipid antibodies and subsequently died because of CAPS.

  16. Aortitis with antiphospholipid antibodies: CT and MR findings

    Energy Technology Data Exchange (ETDEWEB)

    Seror, O.; Dordea, M.; Ghenassia, C.; Coderc, E.; Sellier, N. [Department of Radiology, Centre Hospitalo-Universitaire Paris XIII, Bondy (France); Fain, O. [Department of Medicine, Centre Hospitalo-Universitaire Paris XIII, Bondy (France)

    1998-10-01

    Two cases of aortitis associated with the presence of antiphospholipid antibodies (APAs) are reported. Only CT and MR imaging were able to show these unusual form of aortitis preferentially affecting the outer aortic tunics. We conclude that aortitis could be a new manifestation of primary antiphospholipid syndrome (APS) and the initial pathological process before the development of aortic thrombosis, reported as a classical complication of APS. (orig.) (orig.) With 2 figs., 6 refs.

  17. Transverse myelitis and polymyositis associated with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Mori, Atsuko; Nodera, Hiroyuki; Nakane, Syunya; Kaji, Ryuji

    2010-10-01

    Antiphospholipid antibody syndrome (APS) has been widely recognized to be associated with various neurological complications. In addition to the classical notion of APS as a thrombotic disorder, APS has been suggested to be an autoinflammatory disease as well. We present a previously healthy 46-year-old man who concurrently developed transverse myelitis and polymyositis whose laboratory studies were significant for the elevated antiphospholipid antibodies such as anti-cardiolipin (CL)/beta2-glycoprotein I (beta 2GPI) antibody. This report further enhances the recognized clinical phenotypes of the neurological complications of APS and the understanding of its pathomechanism.

  18. Antiphospholipid antibodies in Brazilian hepatitis C virus carriers

    Directory of Open Access Journals (Sweden)

    A.M. Atta

    2008-06-01

    Full Text Available 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-Hodgkin's 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. Male gender and IgM anticardiolipin seropositivity were associated in the hepatitis C group (P = 0.0004. IgA anti-β2-glycoprotein-I antibodies were detected in 29 of 109 (27.0% hepatitis C carriers (median, 41 SAU; 95%CI: 52.7-103.9 SAU. Twenty patients (18.0% had IgM anti-β2-glycoprotein I antibodies (median, 27.6 SMU; 95%CI: 23.3-70.3 SMU, while two patients had IgG antibodies against this protein (titers, 33 and 78 SGU. Antiphospholipid antibodies were detected in only one healthy individual, who was seropositive for IgM anticardiolipin. We concluded that Brazilian individuals chronically infected with hepatitis C virus present a significant production of antiphospholipid antibodies, mainly IgA anti-β2-glycoprotein I antibodies, which are not associated with clinical manifestations of antiphospholipid syndrome.

  19. Antiphospholipid Antibody Titers and Clinical Outcomes in Patients with Recurrent Miscarriage and Antiphospholipid Antibody Syndrome: A Prospective Study

    Directory of Open Access Journals (Sweden)

    Yu Song

    2017-01-01

    Conclusions: Anti-β2-GP1 IgM was the predominant form of antibody in patients with RM and APS. The decreases in antiphospholipid antibody titers correlated with better pregnancy outcomes. The shorter treatment regimen was effective and economical.

  20. [Antiphosphatidylethanolamine antibody as a marker of antiphospholipid syndrome?].

    Science.gov (United States)

    Yelnik, Cécile Marie; Dubucquoi, Sylvain; Houfflin-Debarge, Véronique; Lambert, Marc

    2015-03-01

    Antibody to phosphatidylethanolamine (aPE) are observed in thrombotic or obstetric manifestations suggestive of antiphospholipid syndrome (APS). aPE seem to be markers of thrombotic risk independent of conventional antiphospholipid antibodies (aPL). aPE assays are not standardized. There is no therapeutic recommendation for isolated aPE patients with thrombotic or obstetric events. Prospective studies have to be carried to better define the therapeutic management of these patients. Value of aPE in APS criteria is still not established.

  1. Antiphospholipid Antibody Titers and Clinical Outcomes in Patients with Recurrent Miscarriage and Antiphospholipid Antibody Syndrome: A Prospective Study

    Science.gov (United States)

    Song, Yu; Wang, Hai-Yan; Qiao, Jie; Liu, Ping; Chi, Hong-Bin

    2017-01-01

    Background: The management of patients with recurrent miscarriage (RM) and antiphospholipid antibody syndrome (APS) includes prolonged treatment with heparin and aspirin, starting from the confirmation of pregnancy and continuing until 6 weeks after birth. This study was conducted to determine the relationship between changes in antiphospholipid antibody titers and clinical outcomes. The effect of a shortened treatment regimen was also evaluated. Methods: A prospective study of 123 patients with RM and APS between March 2012 and May 2014 was conducted. Patients were pretreated with a low dose of prednisone plus aspirin before pregnancy, and heparin was added after conception. The levels of antiphospholipid antibodies and pregnancy outcomes were evaluated. Results: All patients were positive for anti-β2-glycoprotein 1 (anti-β2-GP1) IgM. After prepregnancy treatment with low-dose prednisone plus aspirin, 99 of 123 patients became pregnant, and 87 of those pregnancies resulted in successful live births, while 12 resulted in miscarriage, showing a success rate of 87.9%. In the live birth group, levels of anti-β2-GP1 were 56.8 ± 49.0 RU/ml before the pretreatment regimen, 32.1 ± 26.0 RU/ml after 2 months of pretreatment, and 24.1 ± 23.1 RU/ml during early pregnancy (P antiphospholipid antibody titers were 52.8 ± 30.7 RU/ml before pretreatment, 38.5 ± 34.2 RU/ml after pretreatment, and 33.9 ± 24.7 RU/ml during early pregnancy; the decrease in antiphospholipid antibodies was lower in the miscarriage group than in the live birth group (P antiphospholipid antibody titers correlated with better pregnancy outcomes. The shorter treatment regimen was effective and economical. PMID:28139508

  2. THE IX EUROPEAN FORUM ON ANTIPHOSPHOLIPID ANTIBODIES. A BRIEF REVIEW

    Directory of Open Access Journals (Sweden)

    Nataliya V Seredavkina

    2014-01-01

    Full Text Available The article presents a brief review of the proceedings of the IX European Forum on antiphospholipid antibodies held in May 2013 in Krakow (Poland. The aim of the Forum is to coordinate multicenter projects focused on antiphospholipid antibodies (aPL, both clinical and fundamental research, based on cooperation between the European countries. The main purpose is to stimulate research into all aspects of aPL, to facilitate the exchange of information between institutions, and to involve many centers in different countries into scientific research on this issue. The issues of standardization of the diagnostic criteria for antiphospholipid syndrome (APS, primarily serological markers (their specificity, sensitivity and correlation with clinical manifestations, as well as non-criterial manifestations of APS, were considered at the meeting. In addition, the therapy problems were discussed.

  3. The emerging role of multiple antiphospholipid antibodies positivity in patients with antiphospholipid syndrome.

    Science.gov (United States)

    Forastiero, Ricardo; Martinuzzo, Marta

    2015-01-01

    Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by clinical symptoms of vascular thrombosis and/or pregnancy morbidity in the presence of autoimmune antiphospholipid antibodies (aPL). Current laboratory APS criteria include the presence of at least one of the three relevant aPL: lupus anticoagulant, anticardiolipin antibodies and anti-β2 glycoprotein I antibodies. Therefore, patients could have a single aPL pattern or combinations of aPL. Evidence arising from clinical experience indicates that patients having the highest aPL titer and simultaneous aPL detected by different tests have a worse prognosis and a higher probability of recurrence of the APS clinical features. In recent years, an emerging role of multiple aPL positivity in the identification of high-risk patients with aPL/APS is evident. This paper will review the current knowledge on the clinical relevance of having single or multiple aPL positivity.

  4. A case of seronegative catastrophic antiphospholipid antibody syndrome.

    Science.gov (United States)

    Shreders, Amanda; Bar, Michael

    2013-02-01

    Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare syndrome associated with multiorgan failure that carries a high mortality rate. It has been defined previously by the presence of autoantibodies in a patient with acute multiorgan failure as a result of small vessel occlusion by multiple thrombi. We report a patient who meets all criteria of CAPS except for persistent seronegativity.

  5. Anesthetic management in a case of antiphospholipid antibody syndrome.

    Science.gov (United States)

    Mikkiliineni, Venkata Rama Rao; Panidapu, Nagarjuna; Parasa, Mrunalini; Shaik, Mastan Saheb

    2015-01-01

    Antiphospholipid antibody (APLA) syndrome is one of the most common thrombocytophilias but, unfortunately, goes unrecognized most often. It is an auto-immune disorder in which thrombotic events and a recurrent fetal loss occur in the presence of antibodies to phospholipids. It is the most common acquired hyper-coagulable state. There is a limited literature on peroperative management of patients with this syndrome. We report a case of APLA syndrome in a parturient due to its rarity and complexity.

  6. Primary antiphospholipid antibody syndrome with adrenal hemorrhage in a child : a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Lee, Soo Hyun; Kim, Hyun Joo; Yoo, Han Wook; Yoon, Chong Hyun [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-11-01

    Primary antiphospholipid antibody syndrome is a disease that is clinically diagnosed if a patient suffers recurrent thromboses, stroke, recurrent fetal loss, livedo reticularis, and thrombocytopenia, without evidence of systemic lupus erythematosus or other connective diseases. Adrenal hemorrhage in a patient with primary antiphospholipid antibody syndrome is a rarely recognized, but potentially catastrophic disorder. We recently encountered bilateral adrenal hemorrhaging in a child with antiphospholipid antibody syndrome and casem as well as reviewing the literature.

  7. Bridging therapy in antiphospholipid syndrome and antiphospholipid antibodies carriers: case series and review of the literature.

    Science.gov (United States)

    Raso, Samuele; Sciascia, Savino; Kuzenko, Anna; Castagno, Irene; Marozio, Luca; Bertero, Maria Tiziana

    2015-01-01

    Peri-operative management of patients on warfarin involves assessing and balancing individual risks for thromboembolism and bleeding. The timing of warfarin withdrawal and a tailored pre/postoperative management (including the substitution of heparin in place of warfarin, the so-called bridging therapy) is critical in patients with prothrombotic conditions. The antiphospholipid syndrome (APS) is the most common cause of acquired thrombophilia. In this particular subset of patients, as the risk of thrombosis is higher than in general population, bridging therapy can represent a real challenge for treating physicians. Only few studies have been designed to address this topic. We aim to report our experience and to review the available literature in the peri-procedural management of APS and antiphospholipid antibody-positive patients, reporting adverse events and attempting to identify potential risk factor associated with thrombosis or bleeding complications.

  8. Update on anti-phospholipid antibodies in SLE: the Hopkins' Lupus Cohort.

    Science.gov (United States)

    Petri, M

    2010-04-01

    Anti-phospholipid antibodies are common in patients in the Hopkins' Lupus Cohort: 47% have anti-cardiolipin, 32.5% anti-beta(2)-glycoprotein I and 26% lupus anticoagulant (by dRVVT confirmatory testing). Systemic lupus erythematosus patients with the lupus anticoagulant at baseline have a 50% chance of a deep venous thrombosis/pulmonary embolus in the next 20 years. Anti-phospholipid antibodies differ in their association with thrombosis: the lupus anticoagulant is most strongly associated with arterial and venous thrombosis and is the only anti-phospholipid antibody associated with myocardial infarction. Anti-phospholipid antibodies are not associated with atherosclerosis.

  9. Antiphospholipid antibody syndrome secondary to trimethoprim/sulfamethoxazole.

    Science.gov (United States)

    Silverberg, Jonathan I; Votava, Henry J; Smith, Barry L

    2012-09-01

    Antiphospholipid antibody syndrome (APS) results from autoantibodies to cell surface phospholipids or phospholipid-binding proteins resulting in clotting anomalies and can have devastating sequelae, including stroke, deep venous thrombosis, pulmonary embolism, and recurrent spontaneous abortions. However, cutaneous manifestations are the first sign of APS in up to 41% of patients. We present a case report of APS that developed several days after taking trimethoprim/sulfamethoxazole. The clinical and pathological features of this unique presentation, differential diagnoses, and treatments are discussed.

  10. Stroke in an Infant; Its Association with Antiphospholipid Antibody and Acquired Protein C and S Deficiencies

    Directory of Open Access Journals (Sweden)

    Soroor Inaloo Mohammad Ghofrani

    2004-06-01

    Full Text Available We present the first reported case of antiphospholipid syndrome with stroke in an Iranian boy (7-month-old who had two ischemic strokes within a period of 2 months. Serum anticardiolipid antibody was positive and the patient had low levels of protein S and C. This case emphasizes the importance of antiphospholipid antibody in children with unexplained ischemic stroke.

  11. Multiple antiphospholipid antibodies positivity and antiphospholipid syndrome criteria re-evaluation.

    Science.gov (United States)

    Forastiero, R

    2014-10-01

    The antiphospholipid syndrome (APS) is characterized by the presence of aPL and thrombosis and/or pregnancy morbidity. The last APS laboratory classification criteria include the presence of at least one of the antiphospholipid antibodies (aPL) [lupus anticoagulant (LA), anticardiolipin (aCL) and/or anti-β2 glycoprotein I antibodies (aβ2GPI)] and introduced the concept of subclassification of APS patients into two different categories of aPL assay positivity (combination or single aPL). Several studies have recently shown that the risk for thrombosis increases with each additional aPL detected. We found that the presence of IgG antibodies to β2GPI and/or prothrombin increased thrombotic risk in patients with LA and/or aCLin a prospective study. Various studies have recently demonstrated that patients with triple positivity (LA, aCL and aβ2GPI) are at the highest risk for venous and arterial thrombosis and for obstetric complications. In retrospective but also in prospective studies the rate of thrombotic recurrence was high in subjects with triple positivity even while on anticoagulant therapy. In addition, the occurrence of a first thrombotic event in asymptomatic carriers of triple positivity was higher than in those with single aPL positivity. The inclusion of the detection of antibodies against domain I of β2GPI and/or antibodies to prothrombin would probably help to further identify more clinically relevant aPL. Based on the last findings, there are some proposals to consider only patients with triple positivity as definite APS (thrombotic and obstetric).

  12. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies

    DEFF Research Database (Denmark)

    Schreiber, K

    2016-01-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial...... of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from...

  13. 14th International Congress on Antiphospholipid Antibodies: task force report on antiphospholipid syndrome treatment trends.

    Science.gov (United States)

    Erkan, Doruk; Aguiar, Cassyanne L; Andrade, Danieli; Cohen, Hannah; Cuadrado, Maria J; Danowski, Adriana; Levy, Roger A; Ortel, Thomas L; Rahman, Anisur; Salmon, Jane E; Tektonidou, Maria G; Willis, Rohan; Lockshin, Michael D

    2014-06-01

    Antiphospholipid Syndrome (APS) is characterized by vascular thrombosis and/or pregnancy morbidity occurring in patients with persistent antiphospholipid antibodies (aPL). The primary objective of the APS Treatment Trends Task Force, created as part of the 14th International Congress on aPL, was to systematically review the potential future treatment strategies for aPL-positive patients. The task force chose as future clinical research directions: a) determining the necessity for controlled clinical trials in venous thromboembolism with the new oral direct thrombin or anti-factor Xa inhibitors pending the results of the ongoing rivaroxaban in APS (RAPS) trial, and designing controlled clinical trials in other forms of thrombotic APS; b) systematically analyzing the literature as well as aPL/APS registries, and creating specific registries for non-warfarin/heparin anticoagulants; c) increasing recruitment for an ongoing primary thrombosis prevention trial, and designing secondary thrombosis and pregnancy morbidity prevention trials with hydroxychloroquine; d) determining surrogate markers to select patients for statin trials; e) designing controlled studies with rituximab and other anti-B-cell agents; f) designing mechanistic and clinical studies with eculizumab and other complement inhibitors; and g) chemically modifying peptide therapy to improve the half-life and minimize immunogenicity. The report also includes recommendations for clinicians who consider using these agents in difficult-to-manage aPL-positive patients.

  14. 14th International Congress on Antiphospholipid Antibodies Task Force. Report on antiphospholipid syndrome laboratory diagnostics and trends.

    Science.gov (United States)

    Bertolaccini, Maria Laura; Amengual, Olga; Andreoli, Laura; Atsumi, Tatsuya; Chighizola, Cecilia B; Forastiero, Ricardo; de Groot, Philip; Lakos, Gabriella; Lambert, Marc; Meroni, Pierluigi; Ortel, Thomas L; Petri, Michelle; Rahman, Anisur; Roubey, Robert; Sciascia, Savino; Snyder, Melissa; Tebo, Anne E; Tincani, Angela; Willis, Rohan

    2014-09-01

    Current classification criteria for definite Antiphospholipid Syndrome (APS) require the use of three laboratory assays to detect antiphospholipid antibodies (aCL, anti-β2GPI and LA) in the presence of at least one of the two major clinical manifestations (i.e. thrombosis or pregnancy morbidity) of the syndrome. However, several other autoantibodies shown to be directed to other proteins or their complex with phospholipids have been proposed to be relevant to APS but their clinical utility and their diagnostic value remains elusive. This report summarizes the findings, conclusions and recommendations of the "APS Task Force 3-Laboratory Diagnostics and Trends" meeting that took place during the 14th International Congress on Antiphospholipid Antibodies (APLA 2013, September 18-21, Rio de Janeiro, RJ, Brazil).

  15. Primary antiphospholipid antibody syndrome: neuroradiologic findings in 11 patients

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jung Hoon; Choi, Choong Gon; Choi, Soo Jung; Lee, Ho Kyu; Suh, Dae Chul [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2000-03-01

    To describe the neuroradiologic findings of primary antiphospholipid antibody syndrome (PAPS). During a recent two-year period, abnormally elevated antiphospholipid antibodies were detected in a total of 751 patients. In any cases in which risk factors for stroke were detected - hypertension, diabetes mellitus, hyperlipidemia, smoking, and the presence of SLE or other connective tissue diseases - PAPS was not diagnosed. Neuroradiologic studies were performed in 11 of 32 patients with PAPS. We retrospectively reviewed brain CT (n = 7), MR (n = 8), and cerebral angiography (n = 8) in 11 patients with special attention to the presence of brain parenchymal lesions and cerebral arterial or venous abnormalities. CT or MR findings of PAPS included nonspecific multiple hyper-intensity foci in deep white matter on T2-weighted images (5/11), a large infarct in the territory of the middle cerebral artery (4/11), diffuse cortical atrophy (2/11), focal hemorrhage (2/11), and dural sinus thrombosis (1/11). Angiographic findings were normal (5/8) or reflected either occlusion of a large cerebral artery (2/8) or dural sinus thrombosis (1/8). Neuroradiologic findings of PAPS are nonspecific but in young or middle- aged adults who show the above mentioned CT or MR findings, and in whom risk factors for stroke are not present, the condition should be suspected.

  16. Mechanisms of Disease : antiphospholipid antibodies - from clinical association to pathologic mechanism

    NARCIS (Netherlands)

    de Laat, Bas; Mertens, Koen; de Groot, Philip G.

    2008-01-01

    The discovery that antiphospholipid antibodies recognize plasma proteins that bind to phospholipids rather than recognizing phospholipids themselves has been a major advance in research into antiphospholipid syndrome (APS). It is now established that beta(2)-glycoprotein I (beta(2)GPI) is the most i

  17. Discrimination of a lupus anticoagulant caused by antiphospholipid antibodies or rivaroxaban using taipan venom time

    NARCIS (Netherlands)

    Van Os, G.M.; De Laat, B.; Kamphuisen, P.W.; Meijers, J.C.; de Groot, P.G.

    2011-01-01

    The antiphospholipid syndrome (APS) is an autoimmune disease associated with the presence of antiphospholipid antibodies (APL) and the occurrence of thrombosis and pregnancy complications. One of the assays to detect APL is based on the prolongation of phospho-lipid dependent coagulation assays caus

  18. Antiphospholipid antibodies predict progression of abdominal aortic aneurysms.

    Directory of Open Access Journals (Sweden)

    Christina Duftner

    Full Text Available Antiphospholipid antibodies (aPLs frequently occur in autoimmune and cardiovascular diseases and correlate with a worse clinical outcome. In the present study, we evaluated the association between antiphospholipid antibodies (aPLs, markers of inflammation, disease progression and the presence of an intra-aneurysmal thrombus in abdominal aortic aneurysm (AAA patients. APLs ELISAs were performed in frozen serum samples of 96 consecutive AAA patients and 48 healthy controls yielding positive test results in 13 patients (13.5% and 3 controls (6.3%; n.s.. Nine of the 13 aPL-positive AAA patients underwent a second antibody testing >12 weeks apart revealing a positive result in 6 cases. APL-positive patients had increased levels of inflammatory markers compared to aPL-negative patients. Disease progression was defined as an increase of the AAA diameter >0.5 cm/year measured by sonography. Follow-up was performed in 69 patients identifying 41 (59.4% patients with progressive disease. Performing multipredictor logistic regression analysis adjusting for classical AAA risk factors as confounders, the presence of aPLs at baseline revealed an odds ratio of 9.4 (95% CI 1.0-86.8, p = 0.049 to predict AAA progression. Fifty-five patients underwent a computed tomography in addition to ultrasound assessment indicating intra-aneurysmal thrombus formation in 82.3%. Median thrombus volume was 46.7 cm3 (1.9-377.5. AAA diameter correlated with the size of the intra-aneurysmal thrombus (corrcoeff = 0.721, p<0.001, however neither the presence nor the size of the intra-aneurysmal thrombus were related to the presence of aPLs. In conclusion, the presence of aPLs is associated with elevated levels of inflammatory markers and is an independent predictor of progressive disease in AAA patients.

  19. 14th International Congress on Antiphospholipid Antibodies Task Force report on obstetric antiphospholipid syndrome.

    Science.gov (United States)

    de Jesus, Guilherme R; Agmon-Levin, Nancy; Andrade, Carlos A; Andreoli, Laura; Chighizola, Cecilia B; Porter, T Flint; Salmon, Jane; Silver, Robert M; Tincani, Angela; Branch, D Ware

    2014-08-01

    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 the literature available regarding the association of aPL with obstetric manifestations present in actual classification criteria (recurrent early miscarriage, fetal death, preeclampsia and placental insufficiency) and the quality of the evidence that treatment(s) provide benefit in terms of avoiding recurrent adverse obstetric outcomes. The association of infertility with aPL and the effectiveness of the treatment of patients with infertility and positive aPL was also investigated. This report presents current knowledge and limitations of published studies regarding pregnancy morbidity, infertility and aPL, identifying areas that need better investigative efforts and proposing how critical flaws could be avoided in future studies, as suggested by participants of the Task Force. Except for fetal death, there are limitations in the quality of the data supporting the association of aPL with obstetric complications included in the current APS classification criteria. Recommended treatments for all pregnancy morbidity associated to APS also lack well-designed studies to confirm its efficacy. APL does not seem to be associated with infertility and treatment does not improve the outcomes in infertile patients with aPL. In another section of the Task Force, Dr. Jane Salmon reviewed complement-mediated inflammation in reproductive failure in APS, considering new therapeutic targets to obstetric APS (Ob APS).

  20. Outcomes and treatment of obstetrical antiphospholipid syndrome in women with low antiphospholipid antibody levels.

    Science.gov (United States)

    Mekinian, Arsene; Loire-Berson, Priscille; Nicaise-Roland, Pascale; Lachassinne, Eric; Stirnemann, Jerome; Boffa, Marie-Claire; Chollet-Martin, Sylvie; Carbillon, Lionel; Fain, Olivier

    2012-06-01

    Our objective was to determine whether there is a relationship between low antiphospholipid (aPL) antibody levels and the obstetrical complications of antiphospholipid syndrome (APS) and to analyze the impact of conventional APS treatment in patients with low aPL levels. To this end, we retrospectively reviewed the files of all patients referred to our unit (2003-2010) for unexplained pregnancy morbidity, with an aPL test result. We compared patients with APS confirmed by Sapporo criteria (Group 1) with patients with APS-like obstetrical complications with an aPL titer below the intermediate titer (Group 2). Overall, 57 patients were included (25 in Group 1; 32 in Group 2). Obstetrical events were recurrent spontaneous abortion <10th week of gestation (n=9 patients in Group 1; n=13 patients in Group 2), fetal death (n=11 and 16, respectively), preeclampsia (n=5 in Group 1; n=6 in Group 2). The total number of obstetrical events per patient was very similar before APS treatment (3 [1-8] in Group 1; 3 [1-6] in Group 2) and decreased significantly after APS treatment to 0 [0-2] and 0 [0-2], respectively (p<0.05). The incidence of premature births and the characteristics of neonates were similar in the two groups. In this study, treatment of patients with low aPL levels and APS-like obstetrical events was associated with outcomes similar to those found in otherwise normal women with recurrent miscarriage or other adverse events. However, properly designed treatment trials would be required to prove the benefit of such treatments.

  1. An approach to differential diagnosis of antiphospholipid antibody syndrome and related conditions.

    Science.gov (United States)

    Emmi, Giacomo; Silvestri, Elena; Squatrito, Danilo; Ciucciarelli, Lucia; Cameli, Anna Maria; Denas, Gentian; D'Elios, Mario Milco; Pengo, Vittorio; Emmi, Lorenzo; Prisco, Domenico

    2014-01-01

    The antiphospholipid antibody syndrome is a systemic, acquired, immune-mediated disorder characterized by episodes of venous, arterial, or microcirculation thrombosis and/or pregnancy abnormalities, associated with the persistent presence of autoantibodies, confirmed at least in two occasions 12 weeks apart, directed to molecular complexes consisting of phospholipids and proteins. Antiphospholipid antibody syndrome should always be considered as a potential diagnosis especially for young patients presenting with a history of thrombotic events, in particular when they occur without any obvious external trigger or any inherited thrombophilic mutation (even if 2006 criteria do not exclude antiphospholipid antibody syndrome in patients with other inherited or acquired prothrombotic conditions), or for women with recurrent pregnancy losses or later fetal deaths. Many other disorders are able to mimic antiphospholipid antibody syndrome, so a broad range of alternative diagnoses should be investigated and ruled out during clinical workup.

  2. An Approach to Differential Diagnosis of Antiphospholipid Antibody Syndrome and Related Conditions

    Directory of Open Access Journals (Sweden)

    Giacomo Emmi

    2014-01-01

    Full Text Available The antiphospholipid antibody syndrome is a systemic, acquired, immune-mediated disorder characterized by episodes of venous, arterial, or microcirculation thrombosis and/or pregnancy abnormalities, associated with the persistent presence of autoantibodies, confirmed at least in two occasions 12 weeks apart, directed to molecular complexes consisting of phospholipids and proteins. Antiphospholipid antibody syndrome should always be considered as a potential diagnosis especially for young patients presenting with a history of thrombotic events, in particular when they occur without any obvious external trigger or any inherited thrombophilic mutation (even if 2006 criteria do not exclude antiphospholipid antibody syndrome in patients with other inherited or acquired prothrombotic conditions, or for women with recurrent pregnancy losses or later fetal deaths. Many other disorders are able to mimic antiphospholipid antibody syndrome, so a broad range of alternative diagnoses should be investigated and ruled out during clinical workup.

  3. Acute adrenal insufficiency due to primary antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Kishore Kumar Behera

    2013-01-01

    Full Text Available Introduction: We report a case of acute adrenal insufficiency (AAI in a patient with antiphospholipid syndrome (APS. Case Report: A 44-year-old female patient presented to us with acute abdominal pain associated with recurrent vomiting and giddiness. On examination, her blood pressure was 80/50 mm Hg. Systemic examination was normal. Further evaluation revealed hypocortisolemia with elevated plasma adrenocorticotropin hormone indicative of primary adrenal insufficiency. Her abdominal computed tomography scan showed features of evolving bilateral adrenal infarction. Etiological work-up revealed prolonged activated thromboplastin time, which didn′t correct with normal plasma, her anti-cardiolipin antibody and lupus anticoagulant were also positive. She was diagnosed to have APS with adrenal insufficiency and she was started on intravenous steroids and heparin infusion. Conclusion: AAI due to the APS can present with acute abdominal pain followed by hypotension. A high index of suspicion is needed to make the correct diagnosis and to initiate appropriate treatment.

  4. Cutaneous Vasculitis in a Patient with Antiphospholipid Antibody Syndrome.

    Science.gov (United States)

    Sheth, Khushboo; Parke, Ann

    2016-02-01

    Antiphospholipid antibody syndrome (APS) is an acquired thrombophilia, caused by autoantibodies to anticardiolipin (aCL), or antibeta 2 glycoprotein I, or the presence of lupus anticoagulant (LA) in plasma. It is characterized by recurrent venous and/or arterial thrombi and/or pregnancy related morbidities. We present the case of a 52-year-old female with long-standing APS, who developed cutaneous vasculitis following a common cold. Most of the cutaneous manifestations of APS have been found to be thrombotic on histopathology without evidence of perivascular inflammation. Vasculitis is usually seen in APS patients with coexistent Systemic Lupus Erythematosus (SLE). However, our patient had evidence of vasculitis on skin biopsy and did not have SLE. Though rare, this is a disease process which must be considered in patients with primary APS which must be closely monitored for other vasculitic complications of APS, particularly diffuse alveolar hemorrhage.

  5. Pregnancies in women with systemic lupus erythematosus and antiphospholipid antibodies.

    Science.gov (United States)

    Schreiber, K

    2016-04-01

    Systemic lupus erythematosus (SLE) has preponderance in women in their childbearing years; consequently pregnancy has always been an important issue of concern for the patient and the treating physician. Based upon numerous reports on successful pregnancy outcomes in the past decades, the initial advice against pregnancy in the 1950s has been replaced by a common understanding that women with SLE often have successful pregnancy outcomes, and clinicians therefore advise on pregnancy planning, including possible drug adjustments, timing and close surveillance. The recently published Predictors of Pregnancy Outcome: Biomarkers in Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus (PROMISSE) study, so far the largest multicentre cohort study of pregnant women with underlying stable SLE, has given some important answers to long-discussed questions. Future studies on data collected from the PROMISSE cohort will hopefully identify serological biomarkers, possibly genes, and in addition, give valuable information about underlying disease mechanisms.

  6. An Approach to Differential Diagnosis of Antiphospholipid Antibody Syndrome and Related Conditions

    OpenAIRE

    Giacomo Emmi; Elena Silvestri; Danilo Squatrito; Lucia Ciucciarelli; Anna Maria Cameli; Gentian Denas; Mario Milco D’Elios; Vittorio Pengo; Lorenzo Emmi; Domenico Prisco

    2014-01-01

    The antiphospholipid antibody syndrome is a systemic, acquired, immune-mediated disorder characterized by episodes of venous, arterial, or microcirculation thrombosis and/or pregnancy abnormalities, associated with the persistent presence of autoantibodies, confirmed at least in two occasions 12 weeks apart, directed to molecular complexes consisting of phospholipids and proteins. Antiphospholipid antibody syndrome should always be considered as a potential diagnosis especially for young pati...

  7. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients : Report of a Task Force at the 13th International Congress on Antiphospholipid Antibodies

    NARCIS (Netherlands)

    Ruiz-Irastorza, G.; Cuadrado, M. J.; Ruiz-Arruza, I.; Brey, R.; Crowther, M.; Derksen, R.; Erkan, D.; Krilis, S.; Machin, S.; Pengo, V.; Pierangeli, S.; Tektonidou, M.; Khamashta, M.

    2011-01-01

    The antiphospholipid syndrome (APS) is defined by the presence of thrombosis and/or pregnancy morbidity in combination with the persistent presence of circulating antiphospholipid antibodies: lupus anticoagulant, anticardiolipin antibodies and/or anti-beta 2-glycoprotein I antibodies in medium to hi

  8. A novel mechanism of thrombosis in antiphospholipid antibody syndrome.

    Science.gov (United States)

    Vlachoyiannopoulos, Panayiotis G; Routsias, John G

    2010-11-01

    Antiphospholipid antibody syndrome (APS) is an autoimmune thrombophilia mediated by autoantibodies directed against phospholipid-binding plasma proteins, mainly β2 Glycoprotein I (β2GPI)-a plasma apolipoprotein and prothrombin (PT). A subgroup of these antibodies termed "Lupus Anticoagulant" (LA) elongate in vitro the clotting times, this elongation not corrected by adding normal plasma in the detection system. The exact mechanism by which these autoantibodies induce thrombosis is not well understood. Resistance to natural anticoagulants such as protein C, impaired fibrinolysis, activation of endothelial cells to a pro-coagulant phenotype and activation of platelets, are among the mechanisms partially supported by experimental evidence. Artificially dimerized β2GPI binds tightly to platelet membrane activating them. We search for mechanisms of natural dimerization of β2GPI by proteins of the platelet membranes and found that platelet factor 4 (PF4) assembled in homotetramers binds two molecules of β2GPI and this complex is recognized by anti-β2GPI antibodies, the whole complexes being thrombogenic in terms of activating platelets as confirmed by p38MAP kinase phosphorylation and thromboxane B2 production. Of note PF4/heparin complexes are also immunogenic triggering the production of anti-PF4/heparin antibodies which activate also platelets (the so-called "heparin-induced thrombocytopenia and thrombosis syndrome", HITT). The anti-β2GPI antibodies activate platelets by their F(ab)2, while the anti-PF4/heparin by their Fc fragments. Thus PF4 is a common denominator in the pathogenesis of APS and HITT which share also clinical characteristics such as thrombocytopenia and thrombosis.

  9. The significance of antiphospholipid antibodies in pregnant women with chronic hypertension

    NARCIS (Netherlands)

    Zeeman, GG; Alexander, JM; McIntire, DD; Leveno, KJ

    2004-01-01

    The objective of this study was to perform antiphospholipid antibody screening in women with chronic hypertension to assess whether the presence of such antibodies is associated with adverse pregnancy outcome. Serum for anticardiolipin antibodies and lupus anticoagulant was obtained in pregnant wome

  10. [Antiphospholipid antibody syndrome in pediatric neurosurgery: a hemostasis problem].

    Science.gov (United States)

    Bocquet, R; Blanot, S; Dautzenberg, M D; Pierre-Kahn, A; Carli, P

    1999-11-01

    The case of a 11-year-old boy under anticoagulant therapy for a familial antiphospholipid antibody syndrome (SAAPF), who underwent surgery for a cerebrovascular malformation responsible for an intracerebral haematoma, is reported. Antivitamins K (AVK) were changed for unfractioned heparin (HNF), three days before. Heparin was discontinued two hours prior to surgery to obtain a normal peroperative coagulation. A vascular dural fistula was removed without any haemostatic problem. The neurological status rapidly returned to normal and tomodensitometry at day 1 showed a normal intracranial status. Heparin was readministered at h 16. Thrombocytopenia occurred at day 4 of heparin treatment. The change for a low weight molecular heparinoid, danaparoid (Orgaran), normalized the platelet count. The platelets aggregation tests were negative during thrombopenia. However, the test for antibodies against the PF4-heparin complex with the Elisa technique, was in favour of a heparin induced thrombocytopenia (TIH). In spite of its anecdotic occurrence due to cumulative thrombotic risks from the association of immunologic disorders (TIH and SAAPF), this case report underlines the value but also the risks of anticoagulant therapy in neurosurgery, when patients are at high risk for thrombosis.

  11. Recurrent miscarriages in women not fulfilling classification criteria for antiphospholipid antibody syndrome.

    Science.gov (United States)

    Proietta, M; Ferrero, S; Ferri, L; Cifani, N; Bruno, G; Del Porto, F

    2014-01-01

    Obstetric antiphospholipid antibody syndrome (APS), is well defined by classification criteria. It is well known that women with APS should receive prophylactic anticoagulation therapy with subcutaneous low weight heparin all throughout pregnancy and in the first 6 weeks postpartum. However, the optimal treatment for pregnant women having positive anti-phospholipid antibodies, but not fulfilling classification criteria for APS is still unclear. In this retrospective study we report pregnancy outcomes of 10 patients affected by recurrent miscarriages and positive anti-cardiolipin or aβ2GP1 antibodies with titers ranging from 10 to 20 GPL/MPL demonstrated at least twice before pregnancy.

  12. Bilateral Internal Carotid Artery Occlusion Associated with the Antiphospholipid Antibody Syndrome

    Directory of Open Access Journals (Sweden)

    Pria Anand

    2014-03-01

    Full Text Available A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies.

  13. Bilateral internal carotid artery occlusion associated with the antiphospholipid antibody syndrome.

    Science.gov (United States)

    Anand, Pria; Mann, Sharan K; Fischbein, Nancy J; Lansberg, Maarten G

    2014-01-01

    A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies.

  14. Primary antiphospholipid antibody syndrome and autoimmune haemolytic anaemia--a rare combination.

    Science.gov (United States)

    Suhail, Saera; Baig, Muhammad Shakil; Humail, Syed Mujahid; Riaz, Amir

    2009-06-01

    Primary Antiphospholipid Antibody Syndrome (PAPS) and Autoimmune haemolytic anemia (AIHA) is a very rare combination. Antiphospholipid Antibody Syndrome (APS) with underlying SLE, however, has a well documented association with Coomb's positive Auoimmune Haemolytic Anaemia. We describe a young girl with PAPS presenting with deep venous thrombosis, livedo reticularis and features of AIHA. The patient was refractory to treatment for 5 years however, her condition improved dramatically with anticoagulants, corticosteroid therapy and the addition of hydroxychloroquine and azathioprin. We have also discussed hydroxychloroquine therapy in PAPS which is not yet fully established and the probability of this patient developing other autoimmune disorders in future.

  15. Antibodies Against Annexin V and Prothrombin, Their Correlation with Other Anti-phospholipid Antibodies in Recurrent Pregnancy Loss

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    Objective To study the findings of serum antibodies against annexin V, prothrombin,ph-inositol, ph-acid, ph-ethanolamine, ph-serine, ph-glycerol, cardiolipin, and beta2-glycoprotein I and analyze the trophoblast annexin V receptorsMethods Sera from 156 patients aged 26-41 years with recurrent pregnancy loss (3-7 times) were investigated. Eighty-four fertile healthy women aged 24-38 years were included in a control group. ELISA methods were used for detecting a panel of sera anti-phospholipid antibodies. Immunolocalization of annexin Vreceptors in 143trophoblast specimens of 156patients was investigated by the immunofluorescence technique using Annexin V-FITC, Apoptosis and Annexin V-CY3 commercial kits.Results Positivity for anti-phospholipid antibodies mainly against ph-serine, phethanolamine, and ph-inositol was found together in 80. 8% (126 out of 156 patients),anti-prothrombin antibodies in 12% (18), and anti-annexin Vantibodies in 13. 5%(21) women. No significant levels of anti-phospholipid antibodies were found in 6controls. Placenta immunohistopathology also exhibited some changes manifested by the presence of apoptotic and necrotic cells in trophoblast, and very few microthrombotization in some intervillous spaces.Conclusion Our detailed study demonstrated the prevalence of majority of antiphospholipid antibodies as a high risk factor for repeated reproductive failure. Very low microthrombosis in placentas could be explained by the changes of haemocoagulation properties out of uterus.

  16. Antibodies to age-β2 glycoprotein I in patients with anti-phospholipid antibody syndrome.

    Science.gov (United States)

    Sorice, M; Buttari, B; Capozzi, A; Profumo, E; Facchiano, F; Truglia, S; Recalchi, S; Alessandri, C; Conti, F; Misasi, R; Valesini, G; Riganò, R

    2016-05-01

    Anti-phospholipid antibody syndrome (APS) is a systemic autoimmune disease characterized clinically by arterial and/or venous thromboses, recurrent abortions or fetal loss and serologically by the presence of 'anti-phospholipid antibodies' (aPL). The main target antigen of the antibodies is β2 glycoprotein I (β2 GPI). Post-translational oxidative modifications of the protein have been widely described. In this study we aimed to analyse sera reactivity to glucose-modified β2 GPI (G-β2 GPI). Sera collected from 43 patients with APS [15 primary APS (PAPS) and 28 APS associated with systemic lupus erythematosus (SLE) (SAPS)], 30 with SLE, 30 with rheumatoid arthritis (RA) and 40 healthy subjects were analysed by an enzyme-linked immunosorbent assay (ELISA) using a G-β2 GPI. Nine of 15 consecutive PAPS out-patients (60%) and 16 of 28 SAPS (57.1%) showed serum antibodies [immunoglobulin (Ig)G class] against G-β2 GPI (anti-G-β2 GPI) by ELISA. The occurrence of anti-G-β2 GPI was significantly higher in APS patients compared to patients suffering from SLE. No RA patients or control healthy subjects resulted positive for anti-G-β2 GPI. Of note, aG-β2 GPI prompted to identify some APS patients (four PAPS and seven SAPS), who were negative in the classical anti-β2 GPI test. Moreover, in APS patients, anti-G-β2 GPI titre was associated significantly with venous thrombosis and seizure in APS patients. This study demonstrates that G-β2 GPI is a target antigen of humoral immune response in patients with APS, suggesting that β2 GPI glycation products may contain additional epitopes for anti-β2 GPI reactivity. Searching for these antibodies may be useful for evaluating the risk of clinical manifestations.

  17. Hepatic infarction in a pregnant woman with antiphospholipid syndrome and triple antibody positivity: A case report focusing on catastrophic antiphospholipid syndrome.

    Science.gov (United States)

    Kim, Ji-Hye; Yee, Cheonga; Kuk, Jin-Yi; Choi, Suk-Joo; Oh, Soo-Young; Roh, Cheong-Rae; Kim, Jong-Hwa

    2016-09-01

    Pregnant women with antiphospholipid syndrome (APS) carry a high risk of arterial or venous thrombosis. Such thrombotic conditions occur more frequently in patients with triple positivity to antiphospholipid antibodies or with high antibody titers. Hepatic infarction is a rare complication in pregnant women with APS, and it sometimes mimics HELLP syndrome. This report describes a preeclamptic pregnant woman with APS who had high titers of three antiphospholipid antibodies. She experienced severe epigastric pain with elevated liver enzymes; in addition, she had tachycardia and tachypnea. The clinical findings suggested hepatic infarction and pulmonary thromboembolism, a partial manifestation of catastrophic APS. Therefore, she underwent emergent cesarean section at 25+2 weeks of gestation. After the delivery, her laboratory test indicated HELLP-like features, and computed tomography confirmed hepatic infarction and pulmonary micro-thromboembolism. Here, we report a case of a partial manifestation of catastrophic APS in a pregnant woman with triple antibody positivity, including a brief literature review.

  18. From antibody to clinical phenotype, the black box of the antiphospholipid syndrome: pathogenic mechanisms of the antiphospholipid syndrome.

    Science.gov (United States)

    Du, Vivian X; Kelchtermans, Hilde; de Groot, Philip G; de Laat, Bas

    2013-09-01

    The antiphospholipid syndrome (APS) is diagnosed by the combination of vascular thrombosis and/or pregnancy morbidity and the detection of antiphospholipid antibodies (aPLs) in plasma. In the last few years, a great effort has been made to unravel the mechanism by which aPLs cause thrombosis and a vast amount of mechanisms have been proposed. aPLs were proposed to induce a prothrombotic state by influencing the cellular blood compartment, the plasma compartment, the vascular wall and even metabolic pathways beyond the hemostatic system. However, due to the diversity in the mechanisms and the differences in the methodology, the focus of the mechanistical studies in this field seems to be largely diffused. It is hard to imagine that aPLs can exert such a diversity of effects, resulting in either thrombosis and/or pregnancy morbidity and the relationship between aPLs and the clinical manifestations remains to be a mysterious "black box". In an attempt to get insight in what takes place inside the black box, we have analyzed 126 mechanistical studies on aPLs and discussed differences in the type of antibodies that were used, the involvement of beta2-glycoprotein I (β2GPI), and the criteria used to diagnose APS patients.

  19. Antiphospholipid Antibody Syndrome: Raised Intracranial Pressure Without Cerebral Venous Sinus Thrombosis.

    Science.gov (United States)

    Rudich, Danielle S; Yun, Samuel H; Liebling, Anne; Silbert, Jonathan E; Moeckel, Gilbert W; Lesser, Robert L

    2015-12-01

    Antiphospholipid antibody syndrome (APS) has been reported to cause elevated intracranial pressure, but usually this is due to cerebral venous sinus thrombosis (CVST). We present a 36-year old man with APS with elevated intracranial pressure with neuro-ophthalmic, renal and hematological involvement without identifiable CVST.

  20. Real world experience with antiphospholipid antibody tests : how stable are results over time?

    NARCIS (Netherlands)

    Erkan, D; Derksen, WJM; Kaplan, [No Value; Sammaritano, L; Pierangeli, SS; Roubey, R; Lockshin, MD

    2005-01-01

    Objective: To determine the stability and the degree of variation of antiphospholipid antibody (aPL) results over time in a large cohort of well evaluated aPL positive patients; and to analyse factors contributing to aPL variation and the validity of aPL in a real world setting in which aPL tests ar

  1. [Clinical significance of antiphospholipid antibody measured by EliA anticardiolipin antibodies and anti-β2Glycoprotein I antibodies in antiphospholipid syndrome].

    Science.gov (United States)

    Fujieda, Yuichiro; Shida, Haruki; Oku, Kenji; Bohgaki, Toshiyuki; Amengual, Olga; Horita, Tetsuya; Yasuda, Shinsuke; Atsumi, Tatsuya

    2014-01-01

    Anticardiolipin antibodies (aCL-IgG/IgM) and anti-β2-glycoprotein I antibodies (aβ2GPI-IgG/IgM) are laboratory tests included in the current classification criteria for definite antiphospholipid syndrome (APS). However, not all of these assays have been commercially available in Japan. We investigated the efficacy of aCL-IgG/IgM and aβ2GPI-IgG/IgM assays using fluorescence enzyme immunoassay (Phadia:EliA(TM)) for the diagnosis of APS in Japan. This study comprised 229 sera from patients (100 with APS and 129 without APS). The diagnosis of APS was made according to Sydney revised Sapporo criteria. EliA(TM)Cardiolipin and EliA(TM)β2-Glycoprotein (Phadia AB. Uppsala Sweden) were used to detect aCL IgG/M and aβ2GPI IgG/M, respectively. Sensitivity, specificity and area under the receiver operating characteristic curve (AUC) were as follows; aCL-IgG (45%, 94%, 0.80), aCL-IgM (20%, 94%, 0.54), aβ2GPI-IgG (33%, 98%, 0.88) and aβ2GPI-IgM (16%, 99%, 0.64) respectively. aCL-IgM, aβ2GPI-IgG or aβ2GPI-IgM were detected in 10 patients (18%) with aCL-IgG negative. The use of Phadia:EliA(TM)antiphospholipid antibodies assays improve the diagnostic yield of thrombotic risk in APS patients.

  2. Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.

    Science.gov (United States)

    Galland, Joris; Mohamed, Shirine; Revuz, Sabine; de Maistre, Emmanuel; de Laat, Bas; Marie, Pierre-Yves; Zuily, Stéphane; Lévy, Bruno; Regnault, Véronique; Wahl, Denis

    2016-07-01

    Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare. Within a few days, catastrophic antiphospholipid syndrome was diagnosed with heart, liver and kidney involvement. The patient recovered under pulse steroids, intravenous heparin and intravenous immunoglobulins.

  3. Seronegative Antiphospholipid Syndrome with Anti-phosphatidylethanolamine Antibody in a Boy.

    Science.gov (United States)

    Asano, Takeshi; Narazaki, Hidehiko; Kaizu, Kiyohiko; Kuwabara, Kentaroh; Fujino, Osamu; Itoh, Yasuhiko

    2015-01-01

    Antiphospholipid syndrome (APS) is an autoimmune disease caused by antiphospholipid antibodies. At our institution, APS is diagnosed on the basis of the Sapporo criteria, which consist of thrombosis and recurrent pregnancy-related complications and the following laboratory findings: the presence of lupus anticoagulant, anticardiolipin antibody, or anti-β2 glycoprotein 1 antibody. However, we sometimes treat patients we strongly suspect of having APS but who do not satisfy the laboratory criteria. To accommodate such suspected cases, a subtype of APS termed seronegative APS has been proposed. Here, we report on a man with chronic thromobocytopenic purpura since the age of 3 years and multiple cerebral infarctions since the age of 14 years who finally received a diagnosis of seronegative APS with positive antiphosphatidylethanolamine antibodies.

  4. Transmetatarsal amputation in the setting of antiphospholipid antibody syndrome.

    Science.gov (United States)

    McLeod, Jacob M; Brantigan, Charles O; Alix, Kristen; Kruse, Dustin L; Stone, Paul A

    2013-01-01

    Antiphospholipid syndrome is a hypercoagulable disease that can present foot and ankle surgeons with a unique challenge in treating patients who present with thrombosis and ischemia despite having normal pedal pulses. Appropriate perioperative management is imperative in these patients, because limb- and life-threatening complications can occur postoperatively, despite aggressive anticoagulation. We present the case of a 46-year-old male who underwent a transmetatarsal amputation and, despite aggressive therapy, developed a myriad of complications postoperatively. At 10 months postoperatively, the patient was doing well in an accommodative orthotic with minimal pain while receiving continued aggressive therapy and follow-up examinations by a number of specialists to treat his antiphospholipid syndrome.

  5. Eculizumab prevents recurrent antiphospholipid antibody syndrome and enables successful renal transplantation.

    Science.gov (United States)

    Lonze, B E; Zachary, A A; Magro, C M; Desai, N M; Orandi, B J; Dagher, N N; Singer, A L; Carter-Monroe, N; Nazarian, S M; Segev, D L; Streiff, M B; Montgomery, R A

    2014-02-01

    Renal transplantation in patients with antiphospholipid antibodies has historically proven challenging due to increased risk for thrombosis and allograft failure. This is especially true for patients with antiphospholipid antibody syndrome (APS) and its rare subtype, the catastrophic antiphospholipid antibody syndrome (CAPS). Since a critical mechanism of thrombosis in APS/CAPS is one mediated by complement activation, we hypothesized that preemptive treatment with the terminal complement inhibitor, eculizumab, would reduce the extent of vascular injury and thrombosis, enabling renal transplantation for patients in whom it would otherwise be contraindicated. Three patients with APS, two with a history of CAPS, were treated with continuous systemic anticoagulation together with eculizumab prior to and following live donor renal transplantation. Two patients were also sensitized to human leukocyte antigens (HLA) and required plasmapheresis for reduction of donor-specific antibodies. After follow-up ranging from 4 months to 4 years, all patients have functioning renal allografts. No systemic thrombotic events or early graft losses were observed. While the appropriate duration of treatment remains to be determined, this case series suggests that complement inhibitors such as eculizumab may prove to be effective in preventing the recurrence of APS after renal transplantation.

  6. The Significance of Anti-Beta-2-Glycoprotein I Antibodies in Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Anna Brusch

    2016-06-01

    Full Text Available Antiphospholipid syndrome (APS is a thrombophilic disorder that classically presents with vascular thrombosis and/or obstetric complications. APS is associated with antiphospholipid antibodies: a heterogeneous group of autoantibodies that are directed against membrane phospholipids in complex with phospholipid-binding proteins. Beta-2-glycoprotein I (B2GPI binds anionic phospholipids and is considered to be the predominant antigen in APS and antibodies against B2GPI (anti-B2GPI are recognised in the laboratory criteria for APS diagnosis. This review focuses on the part played by anti-B2GPI in the pathogenesis of APS, their associations with different clinical phenotypes of the disorder and new avenues for refining the diagnostic potential of anti-B2GPI testing.

  7. Bioprosthetic mitral valve thrombosis complicating antiphospholipid antibody syndrome, successfully treated with thrombolysis.

    Science.gov (United States)

    Chamsi-Pasha, Mohammed A; Alyousef, Tareq; Sayyed, Samer

    2014-10-01

    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.

  8. Arterial thrombosis in the antiphospholipid syndrome

    NARCIS (Netherlands)

    Urbanus, R.T

    2008-01-01

    The antiphospholipid syndrome (APS) is a non-inflammatory autoimmune disease that mainly affects young women. The syndrome is characterized by recurrent thrombosis or pregnancy morbidity in association with the persistent serological presence of antiphospholipid antibodies. Antiphospholipid antibodi

  9. Anesthetic management of right atrial mass removal and pulmonary artery thrombectomy in a patient with primary antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Rawat SKS

    2010-01-01

    Full Text Available Antiphospholipid antibody syndrome (APLAS characterises a clinical condition of arterial and venous thrombosis associated with phospholipids directed antibodies. APLAS occurs in 2% of the general population. However, one study demonstrated that 7.1% of hospitalised patients were tested positive for at least one of the three anticardiolipin antibody idiotype. Antiphospholipid antibodies often inhibit phospholipids dependent coagulation in vitro and interfere with laboratory testing of hemostasis. Therefore, the management of anticoagulation during cardiopulmonary bypass can be quite challenging in these patients. Here, we present a case of right atrial mass removal and pulmonary thrombectomy in a patient of APLAS.

  10. Confirmation of antiphospholipid antibody positivity: a year’s results in a cohort of 113 patients

    Directory of Open Access Journals (Sweden)

    A. Ruffatti

    2011-06-01

    Full Text Available Objective: To evaluate the confirmation rate of antiphospholipid antibodies (aPL, to analyze their behaviour at confirmation time, and to study the clinical value of their confirmation. Methods: Blood samples from 380 subjects, enrolled in this study from June 1, 2007 to May 31, 2008, were tested for anti-cardiolipin (aCL and anti-beta2glycoprotein (aβ2GPI antibodies using an ELISA method and for Lupus anticoagulant (LA using a series of clotting tests. The samples of the 113 subjects resulting positive at the first testing time were assayed again to confirm antiphospholipid positivity. Results: aPL positivity was confirmed in 67 out of the 113 subjects (59.3%. Medium-high antibody levels of all, except IgM aCL, aPL/ELISA had a significantly higher confirmation rate with respect to that in subjects with low levels. The confirmation rate in the category I antibody patients (multiple positivity was higher than that in the category II antibody subjects (single positivity. LA positivity was confirmed only when it was associated to other aPL. The cut-off of 40 GPL produced a confirmation rate equal to that resulting from a 99th percentile cut-off. Confirmation of aPL positivity made it possible for us to confirm the diagnosis of antiphospholipid syndrome (APS in 8 out of the 113 subjects originally resulting positive (7,1%. APS clinical features were vascular thrombosis in 4 of these and pregnancy morbidity in the other 4. Conclusions: Our data emphasize aPL positivity confirmation selectivity, and medium-high antibody levels and category I antibodies (multiple positivity had the best confirmation rates.

  11. The obstetric antiphospholipid syndrome

    NARCIS (Netherlands)

    Derksen, R. H. W. M.; de Grootb, Ph. G.

    2008-01-01

    The association of persistent presence of circulating antiphospholipid antibodies and thromboembolic events, (recurrent) pregnancy loss or both is termed antiphospholipid syndrome. Pregnancies in women with the syndrome should be regarded as at high-risk for complications. Optimal management consist

  12. Lack of Association between Anti-Phospholipid Antibodies (APLA) and Attention Deficit/Hyperactivity Disorder (ADHD) in Children

    OpenAIRE

    Yael Leitner; Isaac Vinograd; Yehuda Shoenfeld; Miriam Katz; Yair Levy; Shay Bujanover

    2003-01-01

    Numerous studies have shown the pathological influence anti-phospholipid antibodies (APLA) have on the physiology of the single neuron as well as the function of the entire human nervous system. The influence is well demonstrated in the antiphospholipid syndrome (APS). This syndrome is characterized by a triad of arterial or venous thrombotic events, recurrent fetal loss and thrombocytopenic purpura. The syndrome exhibits different neurological pathologies such as: chorea, seizures, transvers...

  13. Implications of Antiphospholipid and Antineutrophilic Cytoplasmic Antibodies in the Context of Postinfectious Glomerulonephritis

    Science.gov (United States)

    Leifer, Daniel

    2017-01-01

    While antineutrophil cytoplasmic antibody (ANCA) positivity has been documented in some patients with postinfectious glomerulonephritis (PIGN) and is associated with more severe disease, antiphospholipid antibodies (APA) are not known to be a common occurrence. We describe a child with severe acute kidney injury who was noted to have prolonged positivity of both ANCA and APA; a renal biopsy showed noncrescentic immune complex mediated glomerulonephritis with subepithelial deposits compatible with PIGN. He recovered without maintenance immunosuppressive therapy and at last follow-up had normal renal function. We discuss the cooccurrence and implications of ANCA and APA in children with PIGN. PMID:28255306

  14. Obstetric antiphospholipid syndrome.

    Science.gov (United States)

    Esteve-Valverde, E; Ferrer-Oliveras, R; Alijotas-Reig, J

    2016-04-01

    Obstetric antiphospholipid syndrome is an acquired autoimmune disorder that is associated with various obstetric complications and, in the absence of prior history of thrombosis, with the presence of antiphospholipid antibodies directed against other phospholipids, proteins called cofactors or PL-cofactor complexes. Although the obstetric complications have been related to the procoagulant properties of antiphospholipid antibodies, pathological studies of human placenta have shown the proinflammatory capacity of antiphospholipid antibodies via the complement system and proinflammatory cytokines. There is no general agreement on which antiphospholipid antibodies profile (laboratory) confers the greatest obstetric risk, but the best candidates are categories I and IIa. Combined treatment with low doses of aspirin and heparin achieves good obstetric and maternal outcomes. In this study, we also review the therapeutic possibilities in refractory cases, although the likelihood of progressing to other autoimmune diseases is low. We briefly comment on incomplete obstetric antiphospholipid syndrome, also known as antiphospholipid antibody-mediated pregnancy morbidity syndrome.

  15. The relevance of "non-criteria" clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features.

    Science.gov (United States)

    Abreu, Mirhelen M; Danowski, Adriana; Wahl, Denis G; Amigo, Mary-Carmen; Tektonidou, Maria; Pacheco, Marcelo S; Fleming, Norma; Domingues, Vinicius; Sciascia, Savino; Lyra, Julia O; Petri, Michelle; Khamashta, Munther; Levy, Roger A

    2015-05-01

    The purpose of this task force was to critically analyze nine non-criteria manifestations of APS to support their inclusion as APS classification criteria. The Task Force Members selected the non-criteria clinical manifestations according to their clinical relevance, that is, the patient-important outcome from clinician perspective. They included superficial vein thrombosis, thrombocytopenia, renal microangiopathy, heart valve disease, livedo reticularis, migraine, chorea, seizures and myelitis, which were reviewed by this International Task Force collaboration, in addition to the seronegative APS (SN-APS). GRADE system was used to evaluate the quality of evidence of medical literature of each selected item. This critical appraisal exercise aimed to support the debate regarding the clinical picture of APS. We found that the overall GRADE analysis was very low for migraine and seizures, low for superficial venous thrombosis, thrombocytopenia, chorea, longitudinal myelitis and the so-called seronegative APS and moderate for APS nephropathy, heart valve lesions and livedo reticularis. The next step can be a critical redefinition of an APS gold standard, for instance derived from the APS ACTION registry that will include not only current APS patients but also those with antiphospholipid antibodies not meeting current classification criteria.

  16. Anti-phospholipid antibodies in patients with Plasmodium falciparum malaria

    DEFF Research Database (Denmark)

    Jakobsen, P H; Morris-Jones, S D; Hviid, L;

    1993-01-01

    Plasma levels of antibodies against phosphatidylinositol (PI), phosphatidylcholine (PC) and cardiolipin (CL) were measured by enzyme-linked immunosorbent assay (ELISA) in patients from malaria endemic area of Sudan and The Gambia. Some Sudanese adults produced IgM antibodies against all three types...... of phospholipids (PL) during an acute Plasmodium falciparum infection. The anti-PL antibody titre returned to preinfection levels in most of the donors 30 days after the disease episode. IgG titres against PI, PC and CL were low. In Gambian children with malaria, IgM antibody titres against PI and PC were...... significantly higher in those with severe malaria than in those with mild malaria. These results show that a proportion of malaria patients produce anti-PL antibodies during infection and that titres of these antibodies are associated with the severity of disease....

  17. 'Criteria' aPL tests : Report of a Task Force and preconference workshop at the 13th International Congress on Antiphospholipid Antibodies, Galveston, Texas, April 2010

    NARCIS (Netherlands)

    Pierangeli, S. S.; de Groot, P. G.; Dlott, J.; Favaloro, E.; Harris, E. N.; Lakos, G.; Ortel, T.; Meroni, P. L.; Otomo, K.; Pengo, V.; Tincani, A.; Wong, R.; Roubey, R.

    2011-01-01

    Current classification criteria for definite antiphospholipid syndrome (APS) mandate the use of one or more of three positive 'standardized' laboratory assays to detect antiphospholipid antibodies (aPL) (viz: anticardiolipin [aCL] IgG and IgM; anti-beta(2)glycoprotein I [anti-beta(2)GPI] antibodies

  18. EDTA-dependent pseudothrombocytopenia. Association with antiplatelet and antiphospholipid antibodies.

    Science.gov (United States)

    Bizzaro, N; Brandalise, M

    1995-01-01

    In a study of 88 patients with EDTA-dependent pseudothrombocytopenia (PTCP), EDTA-dependent antiplatelet antibodies were seen in the sera of 72 (81.8%) patients (44 IgM, 25 IgG, and 3 IgA). The same sera also were tested for anticardiolipin antibodies (aCL), and 56 (63.6%) patients had sera that also were reactive for aCL (33 IgM, 21 IgG, and 2 IgA). The 16 patients who were negative for antiplatelet antibodies also were negative for aCL antibody. Overall concordance between antiplatelet and aCL antibodies was 82.9%; the correlation between antiplatelet and aCL antibody isotype distribution was 82.1%. Following cardiolipin absorption, most of the PTCP-sera were negative for antiplatelet activity, and no longer reproduced platelet clumping when incubated with normal blood. This finding showed that the antiplatelet antibodies cross-reacted with negatively charged phospholipids. However, after absorption on normal platelets, complete inhibition of aCL activity was observed in 34 (60.7%), and partial inhibition in 14 of the 56 patients who were aCL positive. These findings support the hypothesis that antibody subpopulations (naturally occurring autoantibodies) directed against negatively charged phospholipids can bind to antigens modified by EDTA on the platelet membrane, and may be responsible for PTCP genesis.

  19. Successful treatment of life-threatening Evans syndrome due to antiphospholipid antibody syndrome by rituximab-based regimen: a case with long-term follow-up.

    Science.gov (United States)

    Rückert, A; Glimm, H; Lübbert, M; Grüllich, C

    2008-08-01

    An association of antiphospholipid antibody syndrome with antibodies directed against either phospholipids or plasma proteins strongly suggest that B-cell dysfunction may be involved in its pathogenesis. Antiphospholipid antibody syndrome with autoimmune cytopenias shows a poor response rate to conventional treatment with anticoagulants, glucocorticosteroids, immunosuppressive agents, intravenous immunoglobulin or plasmapheresis. We report a case of life-threatening antiphospholipid antibody syndrome with Evans syndrome receiving successful multimodal treatment including anti-CD20 monoclonal antibody rituximab with long-term follow-up.

  20. CEREBRAL SINUS THROMBOSIS IN A CASE OF ANTI-PHOSPHOLIPID ANTIBODY SYNDROME WITHOUT ASSOCIATED CONNECTIVE TISSUE INVOLVEMENT

    Directory of Open Access Journals (Sweden)

    Biswarup

    2014-12-01

    Full Text Available : The association of antiphospholipid antibodies with vascular thrombotic episodes is well established. In absence of other connective tissue disease such an association is very rare & known as the primary antiphospholipid antibody syndrome. Cerebral venous sinus thrombosis is associated with hypercoaguable states and a number of immune-mediated conditions. However the report of cerebral venous sinus thrombosis with antiphospholipid antibodies alone is limited. Here a case presenting with painful bilateral ophthalmoplegia with bilateral optic disc edema (due to raised intra cranial tension showing positive lupus anticoagulant in serum and right central venous sinus (transverse and sigmoid thrombosis on MRI and MR venogram is reported which showed clinical improvement with anticoagulant therapy

  1. Antiphospholipid antibodies and multiple organ failure in critically ill cancer patients

    Directory of Open Access Journals (Sweden)

    Jorge I. F. Salluh

    2009-02-01

    Full Text Available OBJECTIVES: To describe the clinical outcomes and thrombotic events in a series of critically ill cancer patients positive for antiphospholipid (aPL antibodies. DESIGN: Retrospective case series study. SETTING: Medical-surgical oncologic intensive care unit (ICU. PATIENTS AND PARTICIPANTS: Eighteen patients with SIRS/sepsis and multiple organ failure (MOF and positive for aPL antibodies, included over a 10-month period. INTERVENTIONS: None MEASUREMENTS AND RESULTS: aPL antibodies and coagulation parameters were measured up to 48 hours after the occurrence of acrocyanosis or arterial/venous thrombotic events. When current criteria for the diagnosis of aPL syndrome were applied, 16 patients met the criteria for "probable" and two patients had a definite diagnosis of APL syndrome in its catastrophic form (CAPS. Acrocyanosis, arterial events and venous thrombosis were present in eighteen, nine and five patients, respectively. Sepsis, cancer and major surgery were the main precipitating factors. All patients developed MOF during the ICU stay, with a hospital mortality rate of 72% (13/18. Five patients were discharged from the hospital. There were three survivors at 90 days of follow-up. New measurements of lupus anticoagulant (LAC antibodies were performed in these three survivors and one patient still tested positive for these antibodies. CONCLUSIONS: In this small series of patients, we observed a high frequency of auto-antibodies and micro- and macro-vascular thrombotic events in critically ill cancer patients. The coexistence of sepsis or SIRS and aPL antibodies was often associated with MOF and death. More studies are necessary to determine the pathophysiological significance of antiphospholipid antibodies in severely ill cancer patients.

  2. Autosplenectomy Causing Catastrophic Pneumococcal Meningitis in a Patient with Lupus/Antiphospholipid Antibody Syndrome.

    Science.gov (United States)

    Sheth, Khushboo; Snyder, Aaron; Wu, Ulysses; Lahiri, Bimalin; Grover, Prashant

    2016-01-01

    We present the case ofa26-year-old female who presented to the hospital with pneumococcal meningitis. A review of her records showed atrophic spleen, and a hypercoagulable workup was positive for Systemic Lupus Erythematous (SLE)/Antiphospholipid Antibody Syndrome (APS). An autosplenectomy from thrombotic occlusion of the splenic artery made her susceptible to pneumococcal meningitis. Autoimmune conditions, particularly SLE and APS, are important causes of hypercoagulable states in a young population, and earlier detection of these conditions and appropriate treatment helps to decrease morbidity and mortality among these patients.

  3. Mitral bioprosthetic valve stenosis in a patient with antiphospholipid antibody syndrome and systemic lupus erythematosus.

    Science.gov (United States)

    Morisaki, Akimasa; Hirai, Hidekazu; Sasaki, Yasuyuki; Hosono, Mitsuharu; Sakaguchi, Masanori; Nakahira, Atsushi; Seo, Hiroyuki; Suehiro, Shigefumi

    2012-12-01

    A 45-year-old woman with antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus was admitted because of severe dyspnea. She had undergone mitral valve replacement (MVR) using a Mosaic bioprosthesis for infective endocarditis 9 years previously. She developed congestive heart failure secondary to mitral bioprosthetic valve stenosis resulting from relatively early structural valve deterioration. She underwent a second MVR using a mechanical valve prosthesis. The explanted bioprosthesis showed marked pannus formation and mineralization with fibrin thrombus formation, especially on the outflow surfaces of the leaflets. After the second operation, she was discharged without APS-related thromboembolic events under meticulous anticoagulant and antiplatelet therapies.

  4. Tissue factor in antiphospholipid antibody-induced pregnancy loss:a pro-inflammatory molecule

    OpenAIRE

    Girardi, G.; MACKMAN, N.

    2008-01-01

    Fetal loss in patients with antiphospholipid antibodies (aPL) has been ascribed to thrombosis of placental vessels. However, we have shown that inflammation, specifically complement activation with generation of the anaphylotoxin C5a, is an essential mediator of fetal injury. We have analysed the role of tissue factor (TF) in a mouse model of aPL-induced pregnancy loss. TF is the major cellular activator of the coagulation cascade but also has cell signaling activity. Mice that received aPL-I...

  5. Lupus erythematosus panniculitis presenting with initial periorbital swelling and positive antiphospholipid antibodies: A rare association

    Directory of Open Access Journals (Sweden)

    Jyotsna Oak

    2013-01-01

    Full Text Available Lupus erythematosus panniculitis (LEP, an unusual form of chronic cutaneous lupus erythematosus, is characterized by chronic inflammation and fibrosis of subcutaneous tissue. Clinically, it presents as subcutaneous nodules on common locations such as forehead, cheeks, proximal limbs and buttocks. Ulceration of the nodules may occur in certain cases. Very few case studies have reported the occurrence of early solitary periorbital involvement, highlighting the need for a high index of suspicion in such cases. We report here a case having generalized extensive LEP with initial manifestation of a solitary periorbital swelling, autoimmune hemolytic anemia, and associated antiphospholipid antibodies positivity.

  6. Rituximab induces resolution of recurrent diffuse alveolar hemorrhage in a patient with primary antiphospholipid antibody syndrome.

    Science.gov (United States)

    Scheiman Elazary, A; Klahr, P P; Hershko, A Y; Dranitzki, Z; Rubinow, A; Naparstek, Y

    2012-04-01

    Diffuse alveolar hemorrhage (DAH) is a rare manifestation of primary antiphospholipid antibody syndrome (APS). We describe a patient with primary APS and refractory recurrent episodes of DAH. The patient was admitted 15 times due to recurrent episodes of DAH in a period of 18 months. Multiple immunosuppressive drugs did not improve his condition. Two years after his presentation, he was treated with rituximab (two doses of 1 g, 2 weeks apart). Six months later, the attacks of DAH have gradually disappeared. In a follow-up of more than 2 years after he received rituximab, the patient has had no further admissions due to DAH. Levels of antiphospholipid antibodies were measured during follow-up of 4 years. Anti-β2 glycoprotein IgG titer decreased to normal 6 months after therapy but anticardiolipin (aCL) antibody titer increased. We conclude that rituximab caused a dramatic clinical response in this patient. Anti-β2 glycoprotein IgG correlated better with the clinical response in this patient than aCL.

  7. Antibodies to Phosphatidylserine/Prothrombin Complex in Antiphospholipid Syndrome: Analytical and Clinical Perspectives.

    Science.gov (United States)

    Peterson, Lisa K; Willis, Rohan; Harris, E Nigel; Branch, Ware D; Tebo, Anne E

    2016-01-01

    Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by thrombosis and/or pregnancy-related morbidity accompanied by persistently positive antiphospholipid antibodies (aPL). Current laboratory criteria for APS classification recommend testing for lupus anticoagulant as well as IgG and IgM anticardiolipin, and beta-2 glycoprotein I (anti-β2GPI) antibodies. However, there appears to be a subset of patients with classical APS manifestations who test negative for the recommended criteria aPL tests. While acknowledging that such patients may have clinical features that are not of an autoimmune etiology, experts also speculate that these "seronegative" patients may test negative for relevant autoantibodies as a result of a lack of harmonization and/or standardization. Alternatively, they may have aPL that target other antigens involved in the pathogenesis of APS. In the latter, autoantibodies that recognize a phosphatidylserine/prothrombin (PS/PT) complex have been reported to be associated with APS and may have diagnostic relevance. This review highlights analytical and clinical attributes associated with PS/PT antibodies, taking into consideration the performance characteristics of criteria aPL tests in APS with specific recommendations for harmonization and standardization efforts.

  8. Thrombotic risk assessment in antiphospholipid syndrome: the role of new antibody specificities and thrombin generation assay.

    Science.gov (United States)

    Sciascia, Savino; Baldovino, Simone; Schreiber, Karen; Solfietti, Laura; Radin, Massimo; Cuadrado, Maria J; Menegatti, Elisa; Erkan, Doruk; Roccatello, Dario

    2016-01-01

    Antiphospholipid syndrome (APS) is an autoimmune condition characterized by the presence of antiphospholipid antibodies (aPL) in subjects presenting with thrombosis and/or pregnancy loss. The currently used classification criteria were updated in the international consensus held in Sidney in 2005. Vascular events seem to result of local procoagulative alterations upon triggers influence (the so called "second-hit theory"), while placental thrombosis and complement activation seem to lead to pregnancy morbidity. The laboratory tests suggested by the current classification criteria include lupus anticoagulant, a functional coagulation assay, and anticardiolipin and anti-β2-glycoprotein-I antibodies, generally detected by solid phase enzyme-linked immunosorbent assay. The real challenge for treating physicians is understanding what is the actual weight of aPL in provoking clinical manifestations in each case. As thrombosis has a multi-factorial cause, each patient needs a risk-stratified approach. In this review we discuss the role of thrombotic risk assessment in primary and secondary prevention of venous and arterial thromboembolic disease in patients with APS, focusing on new antibody specificities, available risk scoring models and new coagulation assays.

  9. Elevated levels of antibodies against phosphatidylserine/prothrombin complex and/or cardiolipin associated with infection and recurrent purpura in a child: a forme fruste of antiphospholipid syndrome?

    OpenAIRE

    Kinoshita, Yuri; Mayumi, Nobuko; Inaba, Motoyuki; Igarashi, Touru; Katagiri, Ichigen; KAWANA, SEIJI

    2015-01-01

    Antiphospholipid syndrome is an autoimmune disorder characterized by the occurrence of venous and arterial thrombosis, as well as morbidity in pregnancy, in the presence of anti-phospholipid antibodies. The diagnosis of antiphospholipid syndrome is usually established based on clinical and laboratory findings by strictly following the 2006 Sapporo classification. However, the diagnosis remains challenging owing to the ongoing debates on the serological criteria. We report a case we describe a...

  10. Elevated levels of antibodies against phosphatidylserine/prothrombin complex and/or cardiolipin associated with infection and recurrent purpura in a child: a forme fruste of antiphospholipid syndrome?

    Science.gov (United States)

    Kinoshita, Yuri; Mayumi, Nobuko; Inaba, Motoyuki; Igarashi, Touru; Katagiri, Ichigen; Kawana, Seiji

    2015-07-15

    Antiphospholipid syndrome is an autoimmune disorder characterized by the occurrence of venous and arterial thrombosis, as well as morbidity in pregnancy, in the presence of anti-phospholipid antibodies. The diagnosis of antiphospholipid syndrome is usually established based on clinical and laboratory findings by strictly following the 2006 Sapporo classification. However, the diagnosis remains challenging owing to the ongoing debates on the serological criteria. We report a case we describe as forme fruste antiphospholipid syndrome in which these criteria were not fulfilled. Purpura appeared repeatedly in a female infant starting from the age of 6 months and following episodes of upper respiratory infections and vaccinations. The levels of anti-cardiolipin IgG antibodies and anti-phosphatidylserine/prothrombin complex antibodies were elevated in accordance with these events. Histopathological evaluation revealed multiple small vessel thrombi in the dermis and adipose tissue. After 2 weeks of treatment with aspirin and heparin, the cutaneous symptoms subsided. Infection has long been associated with antiphospholipid syndrome, and anti-phosphatidylserine/prothrombin antibodies are considered a new marker for the diagnosis of antiphospholipid syndrome. Forme fruste antiphospholipid syndrome should be considered even if the antiphospholipid syndrome diagnostic criteria are not completely fulfilled, especially in the presence of elevated levels of anti-phosphatidylserine/prothrombin antibodies and known preceding infections.

  11. Recurrent early pregnancy loss and antiphospholipid antibodies: where do we stand?

    Science.gov (United States)

    Wong, L F; Porter, T F; de Jesús, G R

    2014-10-01

    Evidence from basic science studies supports a causative relationship between antiphospholipid antibodies (aPL) and recurrent early miscarriage (REM) (prior to 10 weeks of gestation). However, human studies have not consistently found a relationship between aPL and REM. Members of the Obstetric Task Force of the 14th International Congress on Antiphospholipid Antibodies performed a literature review of the association of aPL and REM and searched for clinical trials in women with REM who tested positive for aPL. Of the 46 studies that investigated the relationship between aPL and REM, 27 found a positive association, seven found no association, and the remaining 12 papers could not report an association (lack of control group). The main identified problems for such conflicting results were varying definitions of REM (two or three abortions, not necessarily consecutive; different gestational age at which pregnancy losses occurred); analysis of patients with previous fetal death (>10 weeks) in the same group of REM; and different definitions of "positive aPL" (cutoffs not following international recommendations; small number of studies confirmed persistence of positive aPL after six to 12 weeks). The 10 identified randomized trials with proposed treatments for women with REM who test positive for aPL also had heterogeneous inclusion criteria, with only one trial limited to subjects who would meet the current criteria for antiphospholipid syndrome (APS) by both clinical and laboratory criteria. Against this background, we conclude that the association between REM and aPL remains inconclusive and that the findings of treatment trials are at best inconsistent and at worst misleading. More convincing data are critically needed. Studies that identify, or at least stratify, according to international consensus criteria and include standardized core laboratory testing results are crucial if we are to establish an evidence-based association between aPL and REM and treatment

  12. Antiglutamate Receptor Antibodies and Cognitive Impairment in Primary Antiphospholipid Syndrome and Systemic Lupus Erythematosus

    Science.gov (United States)

    Gerosa, Maria; Poletti, Barbara; Pregnolato, Francesca; Castellino, Gabriella; Lafronza, Annalisa; Silani, Vincenzo; Riboldi, Piersandro; Meroni, Pier Luigi; Merrill, Joan T.

    2016-01-01

    Systemic lupus erythematosus (SLE) and antiphospholipid syndrome have an increased risk to develop cognitive impairment. A possible role for antiphospholipid antibodies (aPL) and antiglutamate receptor (anti-NMDA) antibodies in the pathogenesis of neurological manifestations of these two conditions, have been suggested. In particular, the role of anti-NMDA antibodies in the pathogenesis of neuropsychiatric SLE is supported by several experimental studies in animal models and by the finding of a correlation between anti-NMDA positivity in cerebrospinal fluid and neurological manifestations of SLE. However, data from the literature are controversial, as several studies have reported a correlation of these antibodies with mild cognitive impairment in SLE, but more recent studies have not confirmed this finding. The synergism between anti-NMDA and other concomitant autoantibodies, such as aPL, can be hypothesized to play a role in inducing the tissue damage and eventually the functional abnormalities. In line with this hypothesis, we have found a high incidence of at least one impaired cognitive domain in a small cohort of patients with primary APS (PAPS) and SLE. Interestingly, aPL were associated with low scoring for language ability and attention while anti-NMDA titers and mini-mental state examination scoring were inversely correlated. However, when patients were stratified according to the presence/absence of aPL, the correlation was confirmed in aPL positive patients only. Should those findings be confirmed, the etiology of the prevalent defects found in PAPS patients as well as the synergism between aPL and anti-NMDA antibodies would need to be explored. PMID:26870034

  13. Management of women with recurrent pregnancy losses and antiphospholipid antibody syndrome.

    Science.gov (United States)

    Kwak-Kim, Joanne; Agcaoili, Maria Socorro L; Aleta, Lara; Liao, Aihua; Ota, Kuniaki; Dambaeva, Svetlana; Beaman, Kenneth; Kim, Joon Woo; Gilman-Sachs, Alice

    2013-06-01

    Antiphospholipid antibodies (aPL) have been associated with recurrent pregnancy losses (RPL) and other obstetrical complications. The diagnostic criteria for the classical antiphospholipid antibody syndrome (APS) have been utilized for the detection of obstetrical APS in women with RPL. However, laboratory findings and immunopathology of obstetrical APS are significantly different from those of classical APS. In addition, many women with RPL who have positive aPL do not have symptoms consistent with the current APS criteria. The induction of a proinflammatory immune response from trophoblasts and complement activation by aPL rather than thromboembolic changes has been reported as a major immunopathological feature of obstetrical APS. Heparin treatment has been reported to be effective in prevention of early pregnancy loss with APS but not for the late pregnancy loss or complications. The complex effects of heparin may explain the limited efficacy of heparin treatment in RPL. New diagnostic criteria for obstetrical APS are needed urgently, and new therapeutic approaches should be explored further.

  14. Antiphospholipid antibodies promote leukocyte-endothelial cell adhesion and thrombosis in mice by antagonizing eNOS via beta 2GPI and apoER2

    NARCIS (Netherlands)

    Ramesh, Sangeetha; Morrell, Craig N.; Tarango, Cristina; Thomas, Gail D.; Yuhanna, Ivan S.; Girardi, Guillermina; Herz, Joachim; Urbanus, Rolf T.; de Groot, Philip G.; Thorpe, Philip E.; Salmon, Jane E.; Shaul, Philip W.; Mineo, Chieko

    2011-01-01

    In antiphospholipid syndrome (APS), antiphospholipid antibodies (aPL) binding to beta 2 glycoprotein I (beta 2GPI) induce endothelial cell-leukocyte adhesion and thrombus formation via unknown mechanisms Here we show that in mice both of these processes are caused by the inhibition of eNOS In studie

  15. Impact of Persistent Antiphospholipid Antibodies on Risk of Incident Symptomatic Thromboembolism in Children : A Systematic Review and Meta-Analysis

    NARCIS (Netherlands)

    Kenet, Gili; Aronis, Sofia; Berkun, Yackov; Bonduel, Mariana; Chan, Anthony; Goldenberg, Neil A.; Holzhauer, Susanne; Iorio, Alfonso; Journeycake, Janna; Junker, Ralf; Male, Christoph; Manco-Johnson, Marilyn; Massicotte, Patti; Mesters, Rolf; Monagle, Paul; van Ommen, Heleen; Rafini, Leslie; Simioni, Paolo; Young, Guy; Nowak-Goettl, Ulrike

    2011-01-01

    The aim of this study was to estimate the impact of antiphospholipid (aPL) antibodies on the risk of incident thromboembolism (TE; arterial and venous) in children via meta-analysis of published observational studies. A systematic search of electronic databases (Medline, EMBASE, OVID, Web of Science

  16. LEVELS OF ANTIPHOSPHOLIPID ANTIBODY, ERYTHROCYTE SEDIMENTATION RATE AND PLATELETS AMONGST MIGRAINE PATIENTS IN NORTH-EASTERN, NIGERIA

    Directory of Open Access Journals (Sweden)

    Timothy Samuel Yerima

    2011-04-01

    Full Text Available Migraine is mostly mis-diagnosed, and even when correctly diagnosed does not receive desired attention. This study was aimed at assessing the levels of antiphospholipid antibody, erythrocyte sedimentation rate and platelets during migraine attack so as to provide physicians and allied healthcare professionals with guidelines for the diagnosis and subsequent management of migraine in clinical practice. One hundred consecutive adult (18 years and above patients that met the International Headache Society diagnostic criteria for migraine who attended the Neurology Clinic of the Department of Medicine, University of Maiduguri Teaching Hospital, Maiduguri from May, 2009 to December, 2010 and from whom informed consent was obtained were evaluated for this disorder. General, physical and neurological examinations were also conducted. Samples were taken for haematological and immunological analyses before and after acute therapy. Acute migraine attack caused a statistical significant increase in the levels of antiphospholipid antibody, erythrocyte sedimentation rate and platelets among the migraineurs studied (p<0.001. Acute migraine therapy significantly reduced the levels of laboratory parameters studied among migraineurs with either moderate or severe attack (p<0.001. Based on this study, acute migraine attack was found to increase the levels of antiphospholipid antibody, erythrocyte sedimentation rate and platelets above hospital reference value and acute therapy was able to significantly reduce their levels. It is suggested that antiphospholipid antibody, erythrocyte sedimentation rate and platelets should be included as a marker for migraine detection.

  17. Immunoglobulin G4-positive multi-organ lymphoproliferative syndrome with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Kawakami, Nobuyo; Kawai, Kazuhiro; Baba, Naoko; Ohshima, Kouichi; Kanekura, Takuro

    2012-07-01

    We report immunoglobulin (Ig)G4-positive multi-organ lymphoproliferative syndrome (IgG4(+) -MOLPS) with antiphospholipid antibody syndrome (APS) in a 56-year-old Japanese man presenting with purpuric patches on his legs. Skin biopsy revealed leukocytoclastic vasculitis. Laboratory tests demonstrated high levels of serum IgG and IgG4, hypocomplementemia and anticardiolipin antibody. Echography of the lower limbs and pulmonary scintigraphy showed a thrombus in the left soleal vein and multiple emboli in the basal part of both inferior pulmonary arteries. Computed tomography revealed systemic lymphadenopathy. Histologically, there was reactive paracortical hyperplasia with proliferation of histiocytes and infiltration of IgG4-positive plasma cells. We made a diagnosis of IgG4(+) -MOLPS with APS. To our knowledge, this complication has not been reported previously.

  18. Catastrophic antiphospholipid antibody syndrome in a young woman in the postpartum period.

    Science.gov (United States)

    Jacobson, Tatiana B; Kolade, Victor O; Kapadia, Avni S

    2010-01-01

    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 trimester abortion and one live birth complicated by intrauterine growth retardation. She was found to have several focal brain infarcts, exudative retinal detachment, bilateral adrenal hemorrhage, renal insufficiency, hypertension and subsequently hypotension. Positive anticardiolipin antibodies, lupus anticoagulant, and anti-B2 glycoprotein-I, as well as deranged coagulation profile and PTT mixing studies aided in the diagnosis of catastrophic antiphospholipid antibody syndrome. Anticoagulation and high-dose intravenous steroids led to significant improvement in the patient's condition, including her vision.

  19. Strategies for managing heparin therapy in patients with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Mehta, Trupti P; Smythe, Maureen A; Mattson, Joan C

    2011-12-01

    Antiphospholipid antibody syndrome (APS) is a common acquired thrombophilia. The diagnosis of APS is based on both clinical and laboratory criteria. The clinical criteria include vascular thrombosis or pregnancy morbidity. The laboratory criteria include a positive test for lupus anticoagulant, anticardiolipin antibodies, or anti-β(2)-glycoprotein I (anti-β(2)GPI) antibodies on two or more occasions at least 12 weeks apart. Antiphospholipid antibodies with lupus anticoagulant activity may prolong phospholipid-dependent coagulation tests such as the activated partial thromboplastin time (aPTT) and the activated clotting time (ACT). This prolongation adds a level of complexity to monitoring heparin therapy in patients with APS who have thrombosis. A literature search of the PubMed database was conducted for relevant articles published from 1995-April 2011. The usual management approach in nonsurgical patients with APS is to switch to low-molecular-weight heparin. In patients in whom heparin remains the agent of choice, management options include monitoring heparin antifactor Xa levels, determining an individualized therapeutic aPTT range, targeting an aPTT goal of 2 times the baseline aPTT, or using an aPTT reagent insensitive to lupus anticoagulant. An algorithm for anticoagulation management in nonsurgical patients with APS who require heparin is provided. The strategies to monitor intraoperative heparin in patients undergoing cardiac surgery include measuring heparin concentrations by an automated protamine titration device, targeting twice the baseline ACT, using preoperative in vitro heparin-ACT titration curves, and measuring heparin antifactor Xa levels. The available published case reports on the use of these strategies are reviewed. Each institution should determine an approach to managing heparin in patients with APS that best meets its needs and resources.

  20. [Mononeuritis multiplex due to thrombotic ischemia of primary antiphospholipid antibody syndrome without vasculitis: an autopsy case report].

    Science.gov (United States)

    Takahashi, Masatoshi; Katada, Fumiaki; Sato, Susumu; Shibayama, Hidehiro; Fukutake, Toshio; Murayama, Shigeo

    2015-01-01

    The patient was a 78-year-old man. Three years before admission, he developed transient peripheral neuropathy and purpura, and at admission, he presented with livedo reticularis of both his lower extremities and with mononeuritis multiplex. Vasculitis was not observed, and antiphospholipid antibodies were detected. The nerve and skin biopsies revealed no inflammation; axonal degeneration accompanied by thrombi was found in his arterioles and venules. Based on these findings, he was diagnosed with ischemic peripheral neuropathy due to primary antiphospholipid syndrome. Administration of anticoagulant therapy resulted in an improvement in symptoms; however, two months later, a relapse occurred, and the patient contracted an infection while undergoing immunosuppressive therapy. The infection became fulminant, and the patient succumbed to multiple organ failure. The autopsy revealed a systemic arterial and venous embolism; however, no vasculitis was observed. Antiphospholipid syndrome, which is responsive to antithrombotic treatment, should be considered as a differential diagnosis of mononeuritis multiplex.

  1. Anticardiolipine antibodies in skin and muscle eluates of patients with primary and secondary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Z S Alekberova

    2004-01-01

    Full Text Available Objective. To detect anticardiolipin antibodies (АСА, anti-p2-GPl antibodies, C3 and C4 complement components in immune complexes including those containing АСА in skin and muscle eluates of pts with systemic lupus erythematosus (SLE and antiphospholipid syndrome (APS. Material and methods . In 7 pts (6 female and I male, 2 with primary APS, 3 with SLE+APS and 2 with SLE skin and muscle biopsies were taken. 6 from 7 pts had thrombotic complications. Eluates were obtained from frozen skin and skeletal muscle biopsies (size was 1,5x0,5 and 0,5x0,5 respectively. Because of small size of biopsies it was not possible to use traditional methods of tissue pounding such as sharp homogenization of tissues in homogenizers with pulverizing and subsequent process of freezing-unfreezing which lead to large protein loss and make impossible serological tissue analysis. Application of acid eluates method by T.E.W. Feltkamp and J,H. Boode of own modification allowed to minimize tissue protein loss and perform serological tissue analysis. Results. Serum of all 7 pts contained antiphospholipid antibodies - IgG-ACA in 3, combination of IgG- und IgM-ACA in 5. In 5 from 7 eluates lgG АСА exceeded 0,109 OO units were revealed. They contained СЗ, C4 and different protein products mostly immunoglobulines. Anti-(I2GP1 antiboddie;. were absent. Conclusion. For the first time presence of АСА in tissues of APS pts was showed which may be of particular interest in studying morphogenesis of local tissue disturbances with participation of immune complexes containing АСА.

  2. Genetics Home Reference: antiphospholipid syndrome

    Science.gov (United States)

    ... blood vessels. This clotting tendency is known as thrombophilia. In antiphospholipid syndrome , the thromboses can develop in ... Obstetrical Anti-Phospholipid Antibody Syndrome March of Dimes: Thrombophilias National Blood Clot Alliance ClinicalTrials.gov (1 link) ...

  3. Isolated IgA anti- β2 glycoprotein I antibodies in patients with clinical criteria for antiphospholipid syndrome.

    Science.gov (United States)

    Ruiz-García, Raquel; Serrano, Manuel; Martínez-Flores, José Ángel; Mora, Sergio; Morillas, Luis; Martín-Mola, María Ángeles; Morales, José M; Paz-Artal, Estela; Serrano, Antonio

    2014-01-01

    Seronegative antiphospholipid syndrome (SNAPS) is an autoimmune disease present in patients with clinical manifestations highly suggestive of Antiphospholipid Syndrome (APS) but with persistently negative consensus antiphospholipid antibodies (a-PL). IgA anti-β 2 Glycoprotein I (aB2-GPI) antibodies are associated with APS. However, they are not currently considered to be laboratory criteria due to the heterogeneity of published works and the use of poor standardized diagnostic systems. We have aimed to assess aPL antibodies in a group of patients with clinical manifestations of APS (C-APS) to evaluate the importance of the presence of IgA aB2GPI antibodies in APS and its relation with other aPL antibodies. Only 14% of patients with C-APS were positive for any consensus antibody, whereas the presence of isolated IgA aB2GPI antibodies was found in 22% of C-APS patients. In patients with arterial thrombosis IgA aB2GPI, antibodies were the only aPL antibodies present. Serologic profile in primary APS (PAPS) is different from systemic autoimmune disorders associated APS (SAD-APS). IgA aB2GPI antibodies are more prevalent in PAPS and IgG aB2GPI antibodies are predominant in SAD-APS. The analysis of IgA aB2GPI antibodies in patients with clinical manifestations of PAPS might avoid underdiagnosed patients and provide a better diagnosis in patients with SAD-APS. Laboratory consensus criteria might consider including analysis of IgA aB2GPI for APS diagnosis.

  4. Isolated IgA Anti-β2 Glycoprotein I Antibodies in Patients with Clinical Criteria for Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Raquel Ruiz-García

    2014-01-01

    Full Text Available Seronegative antiphospholipid syndrome (SNAPS is an autoimmune disease present in patients with clinical manifestations highly suggestive of Antiphospholipid Syndrome (APS but with persistently negative consensus antiphospholipid antibodies (a-PL. IgA anti-β2 Glycoprotein I (aB2-GPI antibodies are associated with APS. However, they are not currently considered to be laboratory criteria due to the heterogeneity of published works and the use of poor standardized diagnostic systems. We have aimed to assess aPL antibodies in a group of patients with clinical manifestations of APS (C-APS to evaluate the importance of the presence of IgA aB2GPI antibodies in APS and its relation with other aPL antibodies. Only 14% of patients with C-APS were positive for any consensus antibody, whereas the presence of isolated IgA aB2GPI antibodies was found in 22% of C-APS patients. In patients with arterial thrombosis IgA aB2GPI, antibodies were the only aPL antibodies present. Serologic profile in primary APS (PAPS is different from systemic autoimmune disorders associated APS (SAD-APS. IgA aB2GPI antibodies are more prevalent in PAPS and IgG aB2GPI antibodies are predominant in SAD-APS. The analysis of IgA aB2GPI antibodies in patients with clinical manifestations of PAPS might avoid underdiagnosed patients and provide a better diagnosis in patients with SAD-APS. Laboratory consensus criteria might consider including analysis of IgA aB2GPI for APS diagnosis.

  5. Systemic lupus erythematosis with antiphospholipid antibody syndrome: A mimic of Buerger′s disease

    Directory of Open Access Journals (Sweden)

    Vasugi Zoya

    2006-01-01

    Full Text Available This case report is about a past smoker who presented with history of recurrent ulcers and digital gangrene with claudication pain of the left foot for the past fifteen years. Clinical examination and angiogram showed disease involving the peripheral vessels of lowervlimb. This patient had been labeled as Buerger′s disease 15 years ago based on clinical and demographic profile of the illness. We felt that the progression of the disease despite the patient having stopped smoking 15 years ago along with the presence of elevated inflammatory markers in the blood with proteinuria was not in keeping with the nature of the disease. Furthur evaluation revealed that the patient had systemic lupus erythematosus with antiphospholipid antibody syndrome. This case highlights the need for a careful search for diseases, which can mimic Buerger′s disease in young smokers who present with peripheral vascular disease and who have an atypical clinical presentation or progression.

  6. Bilateral optic neuritis in pediatric systemic lupus erythematosus associated with antiphospholipid antibodies and neuromyelitis optica immunoglobulin.

    Science.gov (United States)

    Wei, Wenxin; Zerfoss, Erica; Ashker, Lamees; Cantore, William A

    2010-05-21

    The authors report a case of a 16-year-old girl with a history of systemic lupus erythematosus who developed bilateral acute optic neuritis. Systemic lupus erythematosus can present with a vast array of neurological and ophthalmic complications, with optic neuritis being a rare but devastating manifestation and the major cause of blindness in these patients. The patient presented with an acute unilateral visual deficit that progressed to bilateral visual loss with no light perception over the course of days. Treatment included high-dose steroids, cyclophosphamide, intravenous immunoglobulin, and eventually rituximab. Furthermore, the patient was also seropositive for both antiphospholipid and neuromyelitis optica antibodies, which can have implications on prognosis and treatment options.

  7. Clinical and neuroimaging correlates of antiphospholipid antibodies in multiple sclerosis: a preliminary study

    Directory of Open Access Journals (Sweden)

    Gonzalez-Toledo Eduardo

    2007-10-01

    Full Text Available Abstract Background The presence of antiphospholipid antibodies (APLA in multiple sclerosis (MS patients has been reported frequently but no clear relationship between APLA and the clinical and neuroimaging features of MS have heretofore been shown. We assessed the clinical and neuroimaging features of MS patients with plasma APLA. Methods A consecutive cohort of 24 subjects with relapsing-remitting (RR MS were studied of whom 7 were in remission (Rem and 17 in exacerbation (Exc. All subjects were examined and underwent MRI of brain. Patients' plasma was tested by standard ELISA for the presence of both IgM and IgG antibodies using a panel of 6 targets: cardiolipin (CL, β2 glycoprotein I (β2GPI, Factor VII/VIIa (FVIIa, phosphatidylcholine (PC, phosphatidylserine (PS and phosphatidylethanolamine (PE. Results In exacerbation up to 80% of MS subjects had elevated titers of IgM antibodies directed against the above antigens. However, in remission, less than half of MS patients had elevated titers of IgM antibodies against one or more of the above antigens. This difference was significant, p Conclusion The findings of this preliminary study show that increased APLA IgM is associated with exacerbations of MS. Currently, the significance of this association in pathogenesis of MS remains unknown. However, systematic longitudinal studies to measure APLA in larger cohorts of patients with relapsing-remitting MS, particularly before and after treatment with immunomodulatory agents, are needed to confirm these preliminary findings.

  8. Anti-Phospholipid Antibodies in Patients Undergoing Total Joint Replacement Surgery

    Directory of Open Access Journals (Sweden)

    Melissa Simpson

    2012-01-01

    Full Text Available Background. Patients undergoing joint replacement remain at increased risk for venous thromboembolism (VTE compared to other types of surgery, regardless of thromboprophylactic regimen. The pathophysiologic processes rendering this group of patients at risk for VTE are multifactorial. Procedure-specific and patient-specific exposures play a role in the postoperative development of VTE, including the development of anti-phospholipid antibodies (aPL. Methods. We measured three aPL (anti-cardiolipin, anti-β2 glycoprotein, and lupus anticoagulant in 123 subjects undergoing total knee or hip arthroplasty to describe the presence of these antibodies preoperatively and to describe the rate of postoperative seroconversion among those people who were negative preoperatively. Postoperative antibodies were measured at day 7, 14, and 21. Results. The prevalence of aPL antibodies in the preoperative period was 44%, positive subjects were more likely to be smokers (P=0.05 and were less likely to have undergone a previous arthroplasty procedure (P=0.002. Subjects seroconverted in a 21 day postoperative period at a rate of 79%. Conclusions. These pilot data suggest that the prevalence of aPL in this population both preoperatively and postoperatively is higher than previously expected. Further studies are needed to describe aPL in a larger population and to establish their clinical significance in populations undergoing joint replacement surgeries.

  9. A Case Report of Antiphospholipid Antibody Syndrome%抗磷脂抗體綜合徵1例

    Institute of Scientific and Technical Information of China (English)

    張雪飛; 李楚峰

    2003-01-01

    The antiphospholipid syndrome is a disorder of the immune system that is characterized by arterial and venous thrombosis, recurrent miscarriage and presents with antiphospholipid antibodies that have been reported in approximately 2-4% of the general population. (Western country). Antiphospholipid antibodies have also been detected in over half of patients with immune disease such as Systemic Lupus Erythematosus.Here is a case report of 16 years old female presents with fever, palpitation and right lower limb edema. After investigation, she is finally diagnosed as antiphospholipid syndrome and Systemic Lupus Erythematosus.%抗磷脂抗體綜合徵是免疫系統失常導致的疾病,臨床少見.其特徵為動靜脈的栓塞,反覆流産和抗磷脂抗體的出現.在西方國家,此抗體的出現約為人口的2-4%.對於免疫性疾病病患如紅斑狼瘡,若半數血中可測出抗磷脂抗體.現報導一宗相關病例,一名十六歲女性,表現為發熱、心悸及右下肢浮腫,經臨床和實驗室檢查,最後確診為抗磷脂抗體綜合徵和紅斑狼瘡.

  10. Antiphospholipid Antibodies Bind ATP: A putative Mechanism for the Pathogenesis of Neuronal Dysfunction

    Directory of Open Access Journals (Sweden)

    J. Chapman

    2005-01-01

    Full Text Available Antiphospholipid antibodies (aPL generated in experimental animals cross-react with ATP. We therefore examined the possibility that aPL IgG from human subjects bind to ATP by affinity column and an enzyme linked immunosorbent assay (ELISA. Sera with high levels of aPL IgG were collected from 12 patients with the antiphospholipid syndrome (APS. IgG fractions from 10 of 12 APS patients contained aPL that could be affinity-bound to an ATP column and completely eluted with NaCl 0.5 M. A significant (>50% inhibition of aPL IgG binding by ATP 5 mM was found in the majority. Similar inhibition was obtained with ADP but not with AMP or cAMP. All the affinity purified anti-ATP antibodies also bound β2-glycoprotein-I (β2-GPI, also known as apolipoprotein H suggesting that, similar to most pathogenic aPL, their binding depends on this serum cofactor. We further investigated this possibility and found that the binding of β2-GPI to the ATP column was similar to that of aPL IgG in that most was reversed by NaCl 0.5 M. Furthermore, addition of β2-GPI to aPL IgG significantly increased the amount of aPL binding to an ATP column. We conclude that aPL IgG bind ATP, probably through β2-GPI. This binding could interfere with the normal extracellular function of ATP and similar neurotransmitters.

  11. A case report of a pregnancy-related death caused by primary antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Sun Y

    2014-11-01

    Full Text Available Yingjian Sun,1 Manhua Cui,1 Wanan Zhu,2 Weiling Xu,2 Na Li2 1Department of Obstetrics and Gynecology, The Second Hospital of Jilin University, Changchun, Jilin, People's Republic of China; 2Department of Obstetrics and Gynecology, The First Hospital of Jilin University, Changchun, Jilin, People's Republic of China Abstract: Primary antiphospholipid antibody syndrome (APS is a rare clinical event in the People's Republic of China. As APS is easily neglected or misdiagnosed, a delayed treatment can result. The patient reported here was a 32-year-old female who died by systemic venous thrombosis on day 11 after a cesarean section delivery. Luckily, the baby survived. A blood test demonstrated that the patient's platelets were decreased at 19 weeks of gestation. Anti-cardolipin antibody and antiß2GP1 (anti-ß2-glycoprotein-I antibody were positive at 36 weeks and 2 days of gestation. This patient was diagnosed with APS. Unfortunately, as physicians, we could not provide proper treatment as the patient's relatives were concerned that the proposed treatment would have negative effects on the infant's health. This clinical case strongly suggests that physicians need to appreciate that APS is a very serious condition, especially for pregnant women, and that proper treatment should be provided as early as possible to avoid a bad outcome, despite the fact that a cure for this disease is not currently available. Keywords: APS, thrombosis, Hughes syndrome

  12. A case report of a pregnancy-related death caused by primary antiphospholipid antibody syndrome.

    Science.gov (United States)

    Sun, Yingjian; Cui, Manhua; Zhu, Wanan; Xu, Weiling; Li, Na

    2014-01-01

    Primary antiphospholipid antibody syndrome (APS) is a rare clinical event in the People's Republic of China. As APS is easily neglected or misdiagnosed, a delayed treatment can result. The patient reported here was a 32-year-old female who died by systemic venous thrombosis on day 11 after a cesarean section delivery. Luckily, the baby survived. A blood test demonstrated that the patient's platelets were decreased at 19 weeks of gestation. Anti-cardolipin antibody and antiβ2GP1 (anti-β2-glycoprotein-I antibody) were positive at 36 weeks and 2 days of gestation. This patient was diagnosed with APS. Unfortunately, as physicians, we could not provide proper treatment as the patient's relatives were concerned that the proposed treatment would have negative effects on the infant's health. This clinical case strongly suggests that physicians need to appreciate that APS is a very serious condition, especially for pregnant women, and that proper treatment should be provided as early as possible to avoid a bad outcome, despite the fact that a cure for this disease is not currently available.

  13. Is aorto-arteritis a manifestation of primary antiphospholipid antibody syndrome?

    Science.gov (United States)

    Dhaon, P; Das, S K; Saran, R K; Parihar, A

    2011-12-01

    A 23 year old female presented with dyspnea on exertion and absent pulses in the left upper limb. She had prior history of two first trimester abortions and pre-eclampsia with premature delivery. A Doppler examination had revealed left subclavian and axillary artery thrombosis for which she had been given warfarin six months previously. She was admitted and investigated. Patient had low positive aCL IgG antibody, positive antibeta2gp1 antibody, negative lupus anticoagulant and negative ANA. Patient had cardiomegaly and her echocardiography showed severe aortic regurgitation, moderate mitral regurgitation and moderate pulmonary artery hypertension with poor ejection fraction with normal aortic root. A diagnosis of primary antiphospholipid antibody syndrome with valvular involvement with dilated cardiomyopathy was entertained. A CT angiogram of the aorta revealed narrowing and irregularity of the aorta and its multiple branches suggestive of type III Takayasu's arteritis. Temporal relationship suggests development of aorto-arteritis secondary to APS but simultaneous presence of both these disorders in this patient cannot be ruled out.

  14. Antiphospholipid Antibody Syndrome Associated with Graves' Disease Presenting As Inferior Vena Cava Thrombosis with Bilateral Lower Limb DVT.

    Science.gov (United States)

    Jain, Ankur

    2014-01-01

    We report a case of a 60-year-old lady who presented with bilateral lower limb swelling and a thyroid swelling with clinical features consistent with thyrotoxicosis. Investigations revealed the presence of a thrombus in bilateral external, internal iliac veins, and inferior vena cava extending up to its infrahepatic part. Hormone profile and radioiodine uptake scan confirmed the diagnosis of Graves' disease. Further workup revealed the presence of antiphospholipid antibodies (confirmed after a repeat test at 12 weeks). The patient was treated with antithyroid drugs and anticoagulants. The patient improved with normalization of thyroid function and partial recanalization of the infrahepatic part of inferior vena cava. Hyperthyroidism has been implicated as a potential hypercoagulable state; however, the association of Graves' disease with antiphospholipid antibody syndrome is limited to isolated case reports. This case highlights a new mechanism underlying hypercoagulability associated with Graves' disease.

  15. Possible Effect of Extended Use of Hormonal Contraception on Increased Levels of Antiphospholipid Antibodies in Infertile Women

    OpenAIRE

    2015-01-01

    Purpose: Increased levels of antiphosholipid antibodies (aPLs) are associated with the autoimmune disorder antiphospholipid syndrome (APS) and are known to play a role in infertility. We investigated the possible effect of prolonged use of hormonal contraception (HC) on autoimmunity after discontinuing HC in women with infertility problems. Material and Methods: We analyzed hormonal status including ovulation and the humoral autoimmune response to eight phospholipids detected by ELISA in 1190...

  16. THE STUDY OF PRODUCTION AND MECHANISM OF ANTIPHOSPHOLIPID ANTIBODIES IN PATIENTS WITH CORONARY HEART DISEASE

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective To assess whether there was strong association between antiphospholipid antibodies(APA) and coronary heart disease(CHD), to study the environmental factors of APA production and APA pathogenic mechanism in patients with CHD.Methods Blood samples from 76 patients with CHD and 30 controls were tested for anticardiolipin antibodies IgG(ACA-IgG),human cytomegalovirus IgG,IgM(HCMV-IgG,IgM) by enzyme-link immunosorbant assay(ELISA) and 6-keto-PGF1a,endothelin(ET) by radioimmunoassay(RIA).Results A total of 27 patients(35.53%) were ACA positive in 76, as compared to 2 of 30(6.67%) healthy individuals, P<0.05. There was no difference in ACA among acute myocardial infarction(AMI,39.13%), old myocardial infarction(OMI,26.53%), unstable angina pectoris(UA,38.40%), P>0.05. The number of ACA positive subjects was higher in HCMV infection patients with CHD than no HCMV infectious patients with CHD. There was no PGI2 and ET level difference between ACA-IgG positive and negative CHD.Conclusion There are strong association between APA and CHD. The HCMV infection may be an environmental factor of APA production in CHD patients with raised ACA. The alteration of PGI2 and ET are not the pathogenic mechanism of ACA in patients with CHD.

  17. Post-surgical hemorrhagic infarction of the adrenal gland as the first clinical manifestation of antiphospholipid syndrome after 43 years of antibody-positivity.

    Science.gov (United States)

    Haselboeck, Johanna; Ringl, Helmut; Mueller, Catharina; Pabinger, Ingrid; Winkler, Stefan

    2013-11-01

    We report on a male patient who tested positive for antiphospholipid antibodies for 43 years without thromboembolic manifestation of antiphospholipid syndrome (APS). He has been followed up in a prospective cohort study since 2001. Following his second hip replacement surgery, the patient developed acute adrenal failure due to bilateral hemorrhagic infarction. Prophylactic anticoagulation, surgery, or an immunological reaction to blood transfusion may have triggered this late and unusually located primary manifestation of APS in our patient.

  18. The effects of lupus and antiphospholipid antibody syndrome on foetal outcomes.

    Science.gov (United States)

    Nalli, C; Iodice, A; Andreoli, L; Lojacono, A; Motta, M; Fazzi, E; Tincani, A

    2014-05-01

    Systemic lupus erythematosus (SLE) is a multi-organ autoimmune disease that primarily affects women of childbearing-age. Antiphospholipid syndrome (APS) is a systemic autoimmune disorder defined by the occurrence of venous and arterial thrombosis, often multiple, and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). Recently, the long-term outcome of children born to patients with lupus and APS has become a major topic of interest both to patients and physicians. One of the major problems related to maternal disease is preterm delivery with all the consequences that this condition may bring. Prematurity may also be due to the presence of aPL; however, aPL do not generally display any thrombotic potential on neonates. Another complication may be neonatal lupus (NL), mediated by the presence of maternal antibodies (anti-Ro/SSA and anti-La/SSB). In addition, behaviour and neuropsychological outcomes have also been a matter of interest, but there are currently few data available. Beyond the biological influence of both maternal disease and autoimmune background, it is important to focus on the possible influence of maternal chronic illness on the neuropsychological development of her children. Whether aPL exposure could have a direct effect on brain development is still being debated. In children of mothers with APS, language delays have been noted and learning disabilities were described with a higher rate than the general age-school population. Several studies were performed on children born to lupus mothers: even if maternal lupus does not seem to impair intelligence levels, it may increase the occurrence of learning disabilities and particularly dyslexia in male children. To the best of our knowledge, no studies are available on the long-term outcome of children born to mothers with lupus or APS and particularly regarding the development of autoimmune diseases. Nevertheless, common experience of experts in the field is that these children do

  19. B-cell directed therapies in antiphospholipid antibody syndrome--new directions based on murine and human data.

    Science.gov (United States)

    Khattri, Saakshi; Zandman-Goddard, Gisele; Peeva, Elena

    2012-08-01

    The increased awareness of the role of humoral immunophysiology in antiphospholipid syndrome (APS) has aroused interest in B cells as therapeutic targets in this disease. This paper reviews the literature on B cell directed therapies in human and experimental APS. The clinical data is limited to B cell depletion with rituximab and comprises case reports and case series. Murine studies include use of modulators of B cell function such as belimumab and abatacept. In both human and murine studies, B cell directed therapies appeared to have clinical and serologic beneficial effects including a decrease in the antiphospholipid antibody titers after treatment. Randomized controlled clinical trials are needed to determine whether B cell depletors and/or B cell modulators can be effective agents for treating patients with APS.

  20. Controversies concerning the antiphospholipid syndrome in obstetrics.

    Science.gov (United States)

    Camarena Cabrera, Dulce María Albertina; Rodriguez-Jaimes, Claudia; Acevedo-Gallegos, Sandra; Gallardo-Gaona, Juan Manuel; Velazquez-Torres, Berenice; Ramírez-Calvo, José Antonio

    Antiphospholipid antibody syndrome is a non-inflammatory autoimmune disease characterized by recurrent thrombotic events and/or obstetric complications associated with the presence of circulating antiphospholipid antibodies (anticardiolipin antibodies, anti-β2 glycoprotein-i antibodies, and/or lupus anticoagulant. Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with recurrent miscarriage, stillbirth, fetal growth restriction and premature birth. The diversity of the features of the proposed placental antiphospholipid antibodies fingerprint suggests that several disease processes may occur in the placentae of women with antiphospholipid antibody syndrome in the form of immune responses: inflammatory events, complement activation, angiogenic imbalance and, less commonly, thrombosis and infarction. Because of the disparity between clinical and laboratory criteria, and the impact on perinatal outcome in patients starting treatment, we reviewed the aspects of antiphospholipid antibody syndrome related to obstetric complications and seronegative antiphospholipid antibody syndrome, and their treatment in obstetrics.

  1. Antiphospholipid antibody syndrome: the flow cytometric annexin A5 competition assay as a diagnostic tool.

    Science.gov (United States)

    Tomer, A; Bar-Lev, S; Fleisher, S; Shenkman, B; Friger, M; Abu-Shakra, M

    2007-10-01

    The mechanism underlying hypercoagulability in antiphospholipid antibody syndrome (APS) is uncertain. Here, we present a flow-cytometric assay (FCA) based on the hypothesis that anti-platelet-anionic-phospholipid autoantibodies (aPL) interfere with the activity of the natural anticoagulant protein annexin A5, thereby accelerating platelet procoagulant activity. This study assessed the clinical utility of the feasible FCA, which demonstrates the competition of the patient's aPL with the binding of annexin A5 to the platelet-anionic-phospholipids, in the diagnosis of APS. Sixty-two (94%) of 66 APS patients, 20 (51%) of 39 patients with systemic lupus erythematosus and two (4%) of 49 healthy individuals were positive by FCA. Compared with the anticardiolipin (aCL) assay, the relative sensitivity was 82% and the specificity 73.3%. However, 19 (25%) aCL-negative patients were positive by FCA; 12 were positive for lupus-anticoagulant (LA). Compared with LA assay, the relative sensitivity was 85% and the specificity 72.2%. However, 21 (26%) LA-negative patients were FCA-positive, 12 were positive for aCL. The FCA was particularly sensitive for APS patients with arterial (97.0%) and gestational vascular complications (100%) with overall sensitivity of 95% and specificity of 97%. Our findings suggest that the FCA is practical, sensitive and specific for the detection of clinically relevant aPL in the diagnosis of APS.

  2. β2GP1, Anti-β2GP1 Antibodies and Platelets: Key Players in the Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Yik C. Ho

    2016-05-01

    Full Text Available Anti-beta 2 glycoprotein 1 (anti-β2GP1 antibodies are commonly found in patients with autoimmune diseases such as the antiphospholipid syndrome (APS and systemic lupus erythematosus (SLE. Their presence is highly associated with increased risk of vascular thrombosis and/or recurrent pregnancy-related complications. Although they are a subtype of anti-phospholipid (APL antibody, anti-β2GP1 antibodies form complexes with β2GP1 before binding to different receptors associated with anionic phospholipids on structures such as platelets and endothelial cells. β2GP1 consists of five short consensus repeat termed “sushi” domains. It has three interchangeable conformations with a cryptic epitope at domain 1 within the molecule. Anti-β2GP1 antibodies against this cryptic epitope are referred to as ‘type A’ antibodies, and have been suggested to be more strongly associated with both vascular and obstetric complications. In contrast, ‘type B’ antibodies, directed against other domains of β2GP1, are more likely to be benign antibodies found in asymptomatic patients and healthy individuals. Although the interactions between anti-β2GP1 antibodies, β2GP1, and platelets have been investigated, the actual targeted metabolic pathway(s and/or receptor(s involved remain to be clearly elucidated. This review will discuss the current understanding of the interaction between anti-β2GP1 antibodies and β2GP1, with platelet receptors and associated signalling pathways.

  3. Occult pulmonary mucosa-associated lymphoid tissue lymphoma presenting as catastrophic antiphospholipid antibody syndrome.

    Science.gov (United States)

    Regunath, Hariharan; Shortridge, James; Raza, Shahzad; Nistala, Puja; Huffman, Brandon M; Wang, Michael X; Xiang, Dong

    2013-11-01

    Catastrophic antiphospholipid antibody syndrome (CAPS) is characterized by fulminant thrombosis of the arterial and venous beds of multiple organ systems over a relatively short period of time and with a high mortality rate. Mucosa-associated lymphoid tissue (MALT) lymphoma of the lung has never been reported as a causative or precipitating factor for CAPS in the CAPS registry database. The present study describes a rare case of pulmonary MALT lymphoma of the lung that presented as CAPS. A 19-year-old Hispanic female presented with shortness of breath and abdominal pain. Computed tomography (CT) scans of the chest and abdomen revealed multiple portal vein thromboses and bilateral pulmonary nodules. Within one week of presentation, the patient developed a straight sinus thrombosis and upper extremity deep vein thrombosis, which led to shortness of breath. A biopsy of the lung nodule revealed MALT lymphoma. The present case illustrates a rarely reported pulmonary MALT lymphoma presenting as CAPS in a young female. The patient was successfully treated with 90 mg/m(2) bendamustine on days one and two and rituximab 375 mg/m(2) on day one of each 28-day cycle. Complete remission of the lung nodules was observed following three cycles of treatment, as visualized by positron emission tomography (PET)/CT scan. Fondaparinux was identified as a feasible anticoagulation drug of choice for this case. At seven months post-treatment, the patient continues to be stable with no further evidence of thrombosis and is currently undergoing rituximab maintenance therapy every six months for two years. A repeat lupus anticoagulant antibody assay turned and remained negative during the clinical follow-up period. A prompt diagnosis and early aggressive treatment is potentially curative and may dramatically decrease the mortality risk. Future studies should explore the role of rituximab in the management of CAPS-associated B-cell lymphoid malignancies.

  4. Clinical value of antibodies to lysobisphosphatidic acid in patients with primary antiphospholipid sindrome

    Directory of Open Access Journals (Sweden)

    S. Giunco

    2011-06-01

    Full Text Available To assess the clinical value of anti-lysobisphosphatidic acid (anti-LBPA antibodies in patients with primary antiphospholipid syndrome (APS, the sera of 140 primary APS patients were tested and compared with those of 70 control subjects affected with rheumatic systemic diseases (n. 24 or autoimmune thyroiditis (n. 46. Anti-LBPA anticardiolipin (aCL and anti-β2 Glycoprotein I (anti-β2GPI antibodies were determined using a “home made” ELISA method. Lupus anticoagulant (LA was assessed using a series of clotting tests in accordance with the literature. IgG anti-LBPA was significantly prevalent in primary APS (p=0.000 with a sensitivity of 58.6% and a specificity of 92.9%. IgM anti-LBPA showed a significant frequency in primary APS (p=0.000 with a sensitivity of 28.6% and a specificity of 97.1%. Anti-LBPA’s sensitivity and specificity for APS were lower or equal to those of aCL and anti-β2GPI. The prevalence of anti-LBPA in the different clinical and laboratory subsets of APS was lower than those of aCL and anti- β2GPI. It is interesting to observe that both IgG and IgM anti-LBPA were never found alone. The comparison between anti-LBPA and LA showed that the former had a higher sensitivity but a lower specificity. In conclusion, in view of our results anti-LBPA cannot at present be considered a further tool to be utilized to diagnose APS and to differentiate the different clinical and laboratory subsets of this disease.

  5. Migraine in SLE: role of antiphospholipid antibodies and Raynaud’s phenomenon

    Directory of Open Access Journals (Sweden)

    Serena D'Agostini

    2011-09-01

    Full Text Available Objectives: To determine the role of antiphospholipid antibodies (aPL and of Raynaud’s phenomenon (RP in the development of migraine in patients with systemic lupus erythematosus (SLE. Methods: 50 unselected SLE patients and 20 rheumatoid arthritis (RA controls underwent an interview to define the presence of migraine according to the guidelines of the International Headache Society (1988. Serological tests for aPL were performed in all patients. SLE patients were divided according to positivity for RP and/or aPL into 4 subsets: R-/aPL-, R-/aPL+, R+/aPL- and R+/aPL+. Data were analysed using Fisher’s exact test, Chi-square test and U Mann-Whitney test. Results: SLE and RA patients were similar for demographic and clinical features; aPL positivity was found in a greater proportion of SLE patients versus RA controls (68% vs 25%, p=0.0036. 31 of the 50 lupic patients (62% and 7 of the 20 RA controls (35% suffered from migraine (OR=3, CI:1-8.9. Among SLE and RA patients, migraine was associated with aPL positivity (p=0.027 and p=0.019. Analysing the combined effect of aPL and RP on migraine, in R+/aPL+ patients we detected an higher frequency of migraine (85.7% with respect to the patients negative for these two features (27%, p=0.0051, OR=16, CI:2.2-118 and to the patients positive only for aPL (65%, p=0.0031, OR=6.2, CI:1.2-32. Conclusions: Migraine in SLE and RA associates with aPL positivity. The simultaneous presence of RP increases by 2,5 times the probability of having migraine, suggesting that cerebral vasospasm might be more common in patients with peripheral vasospasm, given the presence of aPL.

  6. Platelets and the antiphospholipid syndrome

    NARCIS (Netherlands)

    Urbanus, R. T.; Derksen, R. H. W. M.; de Groot, P. G.

    2008-01-01

    The antiphospholipid syndrome is a non-inflammatory autoimmune disease characterised by the presence of antiphospholipid antibodies in the plasma of patients with venous or arterial thrombosis or recurrent complications of pregnancy. The strong relation between the presence of antibodies against ani

  7. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis

    Directory of Open Access Journals (Sweden)

    Izumi M

    2011-12-01

    Full Text Available Manabu Izumi, Shoko Teraoka, Keisuke Yamashita, Kenji Matsumoto, Tomohiro Muronoi, Yoshimitsu Izawa, Chikara Yonekawa, Masaki Ano, Masayuki SuzukawaDepartment of Emergency and Critical Care Medicine, Jichi Medical University, Tochigi, JapanAbstract: A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.Keywords: intra-aortic thrombus, antiphospholipid antibody syndrome, spinal cord infarction

  8. Spontaneous Coronary Artery Dissection in a Male Patient with Takayasu's Arteritis and Antiphospholipid Antibody Syndrome.

    Science.gov (United States)

    Gerede, Demet Menekşe; Yüksel, Bağdagül; Tutar, Eralp; Küçükşahin, Orhan; Uzun, Cağlar; Atasoy, Kayhan Çetin; Düzgün, Nurşen; Bengisun, Uğur

    2013-01-01

    We present a case of a 34-year-old male who presented to the emergency ward with fever and abdominal pain. The diagnosis of Takayasu's arteritis and also antiphospholipid syndrome was made during an imaging workup of deep-vein thrombosis. A spontaneous coronary artery dissection was revealed in coronary CT angiography requested for chest pain and dyspnea. The patient was treated medically and discharged on close followup. The concurrence of spontaneous coronary artery dissection with antiphospholipid syndrome and Takayasu's arteritis has not been reported in the previous literature. The possibility of a spontaneous coronary artery dissection should be considered in patients presenting with both diseases.

  9. Are the current attempts at standardization of antiphospholipid antibodies still useful? Emerging technologies signal a shift in direction.

    Science.gov (United States)

    Andreoli, Laura; Rizzini, Silvia; Allegri, Flavio; Meroni, Pierluigi; Tincani, Angela

    2008-06-01

    The pathogenic role of antiphospholipid antibodies (aPL) has been widely established over past years in several experimental models and clinical studies. Accordingly, the detection of aPL by immunoassays (anticardiolipin antibodies; anti-beta2 glycoprotein I antibodies) has become a routine practice in the clinical workup of patients with systemic autoimmune diseases. aPL are mostly assayed using commercial ELISA kits, whose performance has not been found to be sufficiently concordant among the different manufacturers. In the past years, collaborative groups have spent considerable effort to reach some form of standardization but this process is still ongoing. Such lack of standardization has recently become even more crucial, as manufacturers have had to face an increasing demand for fully automated tests for aPL, like those test systems that have been developed for other autoantibodies (e.g., antinuclear antibodies, anti-ENA antibodies). We therefore report our recent experience with two newly developed automated methods for anticardiolipin antibodies testing. In particular, we discuss the results obtained using routine samples, as we believe that these better reflect the "real-life" situation in which those automated methods will operate. We also mention other emerging technologies in the field of aPL detection.

  10. Antibodies to phosphatidylserine/prothrombin complex in suspected antiphospholipid syndrome in the absence of antibodies to cardiolipin or Beta-2-glycoprotein I.

    Science.gov (United States)

    Sanfelippo, M J; Joshi, A; Schwartz, Sl; Meister, J A; Goldberg, J W

    2013-11-01

    Antibodies to phosphatidylserine/prothrombin (aPS/PT) complex were measured in 728 serum specimens from patients suspected of having antiphospholipid syndrome (APS), but without diagnostic elevations in the levels of antibodies to cardiolipin or Beta-2 Glycoprotein 1 (β2-GP1). Of the 728 specimens, 41 had elevated levels of aPS/PT. Thrombotic events occurred in 11 of the 22 patients with accessible medical histories. Six of the patients with accessible medical records also had laboratory evidence of the lupus anticoagulant. The identification of aPS/PT in patients without evidence of antibodies to cardiolipin, β2-GP1, or the lupus anticoagulant can contribute to the identification of APS in patients that may go undetected with current testing methods.

  11. 抗磷脂抗体检测的现状及展望%Current status and prospects of antiphospholipid antibodies tests

    Institute of Scientific and Technical Information of China (English)

    张蜀澜; 李永哲

    2014-01-01

    抗磷脂抗体(APLs)是辅助诊断抗磷脂综合征(APS)的重要实验室指标,是血栓形成和合并妊娠时的主要危险因素之一.但是由于APLs存在显著异质性,检测方法标准化程度较低,不同实验室间检测结果一致性较差,一定程度上限制了APLs检测的临床应用和对于APS诊治结果的进一步认识.因此,亟须建立APLs检测的标准化体系,提高检测方法的特异性、敏感性和重复性,实施设计合理的APLs检测结果大规模的临床评估.%Antiphospholipid antibodies (APLs) are important for the diagnosis of antiphospholipid syndrome (APS),especially for predicting the risk of thrombosis and pathological pregnancy.However,the heterogeneity of antiphospholipid antibodies,lacking of standardization and significant interlaboratory variation binder the clinical application of APLs and better understanding of APS diagnosis and treatment.Therefore,it is urgent to establish a standardize system for antiphospholipid antibodies test and to improve the performance of the test and perform well-designed clinical evaluation.

  12. Antiphospholipid Antibodies in Women Undergoing In Vitro Fertilization Treatment: Clinical Value of IgA Anti-β2glycoprotein I Antibodies Determination

    Directory of Open Access Journals (Sweden)

    Odile Paulmyer-Lacroix

    2014-01-01

    Full Text Available Implantation failure could be related to antiphospholipid antibodies (aPL. We retrospectively analyzed the usefulness of aPL determination in women undergoing IVF. Conventional aPL of the antiphospholipid syndrome, lupus anticoagulant (LA, anticardiolipin antibodies (aCL, anti-β2glycoprotein I (aβ2GPI antibodies, and IgG and IgM isotypes as well as IgA isotype were analyzed in women presenting with at least two implantation failures after in vitro fertilization (IVF. In a population of 40 IVF patients, a total prevalence of 20% (8/40 of aPL was found, significantly different from that of the control population (100 healthy blood donors, P<0.0005. Among the panels of aPL tested, aβ2GPI IgA antibodies were the most prevalent (62.5% 5/8, significantly higher in IVF patients (12.5%, 5/40 than in controls (1%, 1/100 (P=0.01. No difference according to the numbers of IVF attempts and success of embryo implantation was found between aPL positive and negative IVF patients. In contrast, no accomplished pregnancy with full-term live birth was observed in aPL positive IVF patients. Altogether our data led us to propose aPL assessment, in particular aβ2GPI IgA antibodies, in support of IVF treated women. In a perspective way, an early aPL detection could be the basis for defining novel therapeutic strategy.

  13. A novel dimeric inhibitor targeting Beta2GPI in Beta2GPI/antibody complexes implicated in antiphospholipid syndrome.

    Directory of Open Access Journals (Sweden)

    Alexey Kolyada

    Full Text Available BACKGROUND: β2GPI is a major antigen for autoantibodies associated with antiphospholipid syndrome (APS, an autoimmune disease characterized by thrombosis and recurrent pregnancy loss. Only the dimeric form of β2GPI generated by anti-β2GPI antibodies is pathologically important, in contrast to monomeric β2GPI which is abundant in plasma. PRINCIPAL FINDINGS: We created a dimeric inhibitor, A1-A1, to selectively target β2GPI in β2GPI/antibody complexes. To make this inhibitor, we isolated the first ligand-binding module from ApoER2 (A1 and connected two A1 modules with a flexible linker. A1-A1 interferes with two pathologically important interactions in APS, the binding of β2GPI/antibody complexes with anionic phospholipids and ApoER2. We compared the efficiency of A1-A1 to monomeric A1 for inhibition of the binding of β2GPI/antibody complexes to anionic phospholipids. We tested the inhibition of β2GPI present in human serum, β2GPI purified from human plasma and the individual domain V of β2GPI. We demonstrated that when β2GPI/antibody complexes are formed, A1-A1 is much more effective than A1 in inhibition of the binding of β2GPI to cardiolipin, regardless of the source of β2GPI. Similarly, A1-A1 strongly inhibits the binding of dimerized domain V of β2GPI to cardiolipin compared to the monomeric A1 inhibitor. In the absence of anti-β2GPI antibodies, both A1-A1 and A1 only weakly inhibit the binding of pathologically inactive monomeric β2GPI to cardiolipin. CONCLUSIONS: Our results suggest that the approach of using a dimeric inhibitor to block β2GPI in the pathological multivalent β2GPI/antibody complexes holds significant promise. The novel inhibitor A1-A1 may be a starting point in the development of an effective therapeutic for antiphospholipid syndrome.

  14. A Novel Dimeric Inhibitor Targeting Beta2GPI in Beta2GPI/Antibody Complexes Implicated in Antiphospholipid Syndrome

    Energy Technology Data Exchange (ETDEWEB)

    A Kolyada; C Lee; A De Biasio; N Beglova

    2011-12-31

    {beta}2GPI is a major antigen for autoantibodies associated with antiphospholipid syndrome (APS), an autoimmune disease characterized by thrombosis and recurrent pregnancy loss. Only the dimeric form of {beta}2GPI generated by anti-{beta}2GPI antibodies is pathologically important, in contrast to monomeric {beta}2GPI which is abundant in plasma. We created a dimeric inhibitor, A1-A1, to selectively target {beta}2GPI in {beta}2GPI/antibody complexes. To make this inhibitor, we isolated the first ligand-binding module from ApoER2 (A1) and connected two A1 modules with a flexible linker. A1-A1 interferes with two pathologically important interactions in APS, the binding of {beta}2GPI/antibody complexes with anionic phospholipids and ApoER2. We compared the efficiency of A1-A1 to monomeric A1 for inhibition of the binding of {beta}2GPI/antibody complexes to anionic phospholipids. We tested the inhibition of {beta}2GPI present in human serum, {beta}2GPI purified from human plasma and the individual domain V of {beta}2GPI. We demonstrated that when {beta}2GPI/antibody complexes are formed, A1-A1 is much more effective than A1 in inhibition of the binding of {beta}2GPI to cardiolipin, regardless of the source of {beta}2GPI. Similarly, A1-A1 strongly inhibits the binding of dimerized domain V of {beta}2GPI to cardiolipin compared to the monomeric A1 inhibitor. In the absence of anti-{beta}2GPI antibodies, both A1-A1 and A1 only weakly inhibit the binding of pathologically inactive monomeric {beta}2GPI to cardiolipin. Our results suggest that the approach of using a dimeric inhibitor to block {beta}2GPI in the pathological multivalent {beta}2GPI/antibody complexes holds significant promise. The novel inhibitor A1-A1 may be a starting point in the development of an effective therapeutic for antiphospholipid syndrome.

  15. Fluoroscopy-guided thrombolysis of mechanical mitral valve thrombosis in a young female with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Ikram, Sohail; Pant, Sadip; Hussain, Zeeshan; Brown, Lorrel

    2015-05-07

    Prosthetic valve thrombosis (PVT) is a rare but potentially fatal complication of mechanical valve prosthesis. The differential diagnoses for prosthetic valve obstruction includes pannus formation, prosthetic valve dehiscence, prosthetic valve endocarditis, chordae entrapment, patient-prosthesis mismatch and primary device failure. Establishing a diagnosis requires an understanding of prosthetic valve haemodynamics and careful correlation of clinical and imaging findings. Definitive therapy must be individualised based on various patient-specific factors. We present a case of mechanical mitral PVT in a young woman with antiphospholipid antibody syndrome, and outline the diagnostic and therapeutic approach utilised for successful treatment. The success and complication rates of various therapeutic strategies are also discussed, and highlight the need for individualised decision-making rather than a one-size-fits-all approach to PVT.

  16. Technetium-99m-ECD SPECT in antiphospholipid antibody syndrome: a drastic improvement in brain perfusion by antiplatelet therapy

    Energy Technology Data Exchange (ETDEWEB)

    Tokumaru, Sunao; Yoshikai, Tomonori; Uchino, Akira; Kudo, Sho [Dept. of Radiology, Saga Medical School (Japan); Matsui, Makoto; Kuroda, Yasuo [Dept. of Neurology, Saga Medical School (Japan)

    2001-12-01

    We present a case of antiphospholipid antibody syndrome (APS) with repeated transient ischemic attacks (TIAs). Magnetic resonance imaging showed multiple cerebral infarcts and ischemic changes in the cerebral white matter. Cerebral angiographies showed no abnormalities. Technetium-99m-ethyl cysteinate dimer (Tc-99m-ECD) brain SPECT showed multiple decreased perfusion areas, which were more extensive than the lesions demonstrated on MRI. After treatment with an antiplatelet agent, the patient subsequently recovered from the TIAs. Although no interval changes were observed by MRI after therapy, follow-up Tc-99m-ECD SPECT revealed a marked improvement in brain perfusion. This is the first imaging report of remarkable post-therapy improvement in brain perfusion in APS cases. (orig.)

  17. The IgM isotype of anti-annexin A5 antibodies and multiple positivity of conventional antiphospholipid antibodies: increasing the number of clinical manifestations of primary antiphospholipid syndrome.

    Science.gov (United States)

    Bećarević, Mirjana; Stojanović, Ljudmila; Ignjatović, Svetlana; Dopsaj, Violeta

    2016-05-01

    We evaluated the importance of anti-annexin A5 antibodies (aanxA5 Abs) for clinical (thrombosis and/or recurrent pregnancy loss) and serologic (presence of antiphospholipid antibodies: lupus anticoagulant (LA), anticardiolipin (aCL), and anti-β2 glycoprotein I (aβ2GPI) antibodies) features of patients with primary antiphospholipid syndrome (PAPS). Our study included 70 patients with PAPS according to the international consensus criteria for APS. The mean age of the analyzed patients was 45.97 ± 12.72. The disease duration above 5 years was present in 31/70 of patients. Concentrations of analyzed antibodies were measured by ELISA. Cutoff values were set in accordance to the manufacturers' recommendations. History of recurrent pregnancy loss was associated with double positivity for aanxA5 IgM and LA (χ (2) = 4.000, P = 0.046) and triple positivity for aanxA5 IgM + LA + aβ2GPI IgM (χ (2) = 4.168, P = 0.041). Venous thromboses were associated with triple positivity for aanxA5 IgM + aCLIgG + aβ2GPI IgM (χ (2) = 3.965, P = 0.046). The IgG isotype of aanxA5 Abs was in positive correlation with aCL Abs of the IgG (r = 0.310, P = 0.009) and IgM (r = 0.254, P = 0.034) isotype. The presence of the clinical manifestations of PAPS is increasing with the number of positive conventional aPL and the IgM aanxA5 Abs tests. This new combination of Abs is beneficial even when the number of patients with positivity for aanxA5 Abs is low. This is important in further detection of patients prone to recurrence of thrombotic episodes.

  18. Antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Pavlović Dragan M.

    2010-01-01

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disease with recurrent thromboses and pregnancy complications (90% are female patients that can be primary and secondary (with concomitant autoimmune disease. Antiphospholipid antibodies are prothrombotic but also act directly with brain tissue. One clinical and one laboratory criterion is necessary for the diagnosis of APS. Positive serological tests have to be confirmed after at least 12 weeks. Clinical picture consists of thromboses in many organs and spontaneous miscarriages, sometimes thrombocytopaenia and haemolytic anaemia, but neurological cases are the most frequent: headaches, stroke, encephalopathy, seizures, visual disturbances, Sneddon syndrome, dementia, vertigo, chorea, balism, transitory global amnesia, psychosis, transversal myelopathy and Guillain-Barre syndrome. About 50% of strokes below 50 years of age are caused by APS. The first line of therapy in stroke is anticoagulation: intravenous heparin or low-weight heparins. In chronic treatment, oral anticoagulation and antiplatelet therapy are used, warfarin and aspirin, mostly for life. In resistant cases, corticosteroids, intravenous immunoglobulins and plasmapheresis are necessary. Prognosis is good in most patients but some are treatment-resistant with recurrent thrombotic events and eventually death.

  19. Risk of thrombosis in patients with primary immune thrombocytopenia and antiphospholipid antibodies: A systematic review and meta-analysis.

    Science.gov (United States)

    Moulis, Guillaume; Audemard-Verger, Alexandra; Arnaud, Laurent; Luxembourger, Cécile; Montastruc, François; Gaman, Amelia Maria; Svenungsson, Elisabet; Ruggeri, Marco; Mahévas, Matthieu; Gerfaud-Valentin, Mathieu; Brainsky, Andres; Michel, Marc; Godeau, Bertrand; Lapeyre-Mestre, Maryse; Sailler, Laurent

    2016-03-01

    Antiphospholipid antibodies (aPL) are common in ITP, but their role for the occurrence of ITP-related thrombosis is controversial. We performed a systematic review and a meta-analysis to investigate the risk of thrombosis associated with lupus anticoagulant (LA), anticardiolipin (aCL) and anti-β2GP-I antibodies in primary ITP. The literature search was run on Medline, Cochrane and ISI Web of Science from January 1st 1980 to December 31st 2014. Unpublished studies were searched in meeting abstracts. The main analysis assessed the risk of all thromboses (arterial or venous) associated with the presence of LA, aCL or anti-β2GP-I antibodies. Random-effect models were used to calculate odds ratios (OR) and their 95% confidence intervals (CI). Searches in electronic databases retrieved 776 citations. Twelve additional studies from unpublished literature were added. Eventually, 10 cohort studies totalizing 1574 patients were included in the analysis. The pooled OR for the risk of all thromboses associated with LA was 6.11, 95% CI [3.40-10.99]; it was 2.14, 95% CI [1.11-4.12] with aCL. The ORs were similar when stratifying on the type of thrombosis (arterial vs. venous). Only two studies assessed the risk of thrombosis associated with anti-β2GP-I antibody positivity; consequently, no pooled OR was computed for these antibodies. This meta-analysis highly suggests that LA positivity, and to a less extent aCL antibodies, are associated with an enhanced risk of thrombosis in primary ITP patients. Further prospective studies are needed to identify the factors associated with the risk of thrombosis among LA patients before assessing prevention strategies.

  20. Antiphospholipid syndrome and kidney disease.

    Science.gov (United States)

    Bienaimé, Frank; Legendre, Christophe; Terzi, Fabiola; Canaud, Guillaume

    2017-01-01

    The antiphospholipid syndrome is a common autoimmune disease caused by pathogenic antiphospholipid antibodies, leading to recurrent thrombosis and/or obstetrical complications. Importantly for nephrologists, antiphospholipid antibodies are associated with various renal manifestations including large renal vessel thrombosis, renal artery stenosis, and a constellation of intrarenal lesions that has been termed antiphospholipid nephropathy. This last condition associates various degrees of acute thrombotic microangiopathy, proliferative and fibrotic lesions of the intrarenal vessels, and ischemic modifications of the renal parenchyma. The course of the disease can range from indolent nephropathy to devastating acute renal failure. The pejorative impact of antiphospholipid antibody-related renal complication is well established in the context of systemic lupus erythematous or after renal transplantation. In contrast, the exact significance of isolated antiphospholipid nephropathy remains uncertain. The evidence to guide management of the renal complications of antiphospholipid syndrome is limited. However, the recent recognition of the heterogeneous molecular mechanisms underlying the progression of intrarenal vascular lesions in antiphospholipid syndrome have opened promising tracks for patient monitoring and targeted therapeutic intervention.

  1. Thromboprophylaxis in carriers of antiphospholipid antibodies (APL) without previous thrombosis: "Pros" and "Cons".

    Science.gov (United States)

    Ceccarelli, Fulvia; Chighizola, Cecilia; Finazzi, Guido; Meroni, Pier Luigi; Valesini, Guido

    2012-06-01

    The presence of anti-phospholipid (aPL) is necessary but not sufficient to induce a thrombotic event. The "second hit" hypothesis suggested that an additional trigger may be needed to develop a vascular event in aPL carriers. In this article, pro and con of primary thromboprophylaxis in aPL carriers is deeply discussed, concluding that univocal data are not available, due to conflicting results of available clinical trials. However, in clinical practice the primary thromboprophylaxis is not indicated in all unselected asymptomatic aPL carriers, and the best strategy begin with the assessment of the peculiar risk profile of the subject. Thus, it is mandatory to eliminate modifiable prothrombotic risk factors (i.e. smoking, oral contraceptive), to treat the irreversible risk factors (i.e. hypertension, diabetes) and to introduce an aggressive prophylaxis with subcutaneous LMWH in high-risk situations (i.e. surgical procedures with prolonged immobilization). A different evaluation should be addressed to aPL carriers with a concomitant autoimmune disease that are considered as an additional pro-thrombotic risk factor. Similarly, concomitant positivity for more than one anti-phospholipid test confer a stronger risk of developing the thrombotic manifestations. Specific trials with larger cohorts of patients are needed to better clarify this issue.

  2. Renal involvement in primary antiphospholipid syndrome.

    Science.gov (United States)

    Marcantoni, Carmelita; Emmanuele, Carmela; Scolari, Francesco

    2016-08-01

    Antiphospholipid syndrome is an autoimmune disorder characterized by recurrent venous or arterial thrombosis and/or pregnancy-related problems associated with persistently elevated levels of antiphospholipid antibodies. The kidney is a major target organ in both primary and secondary antiphospholipid syndrome. This review describes several aspects of the renal involvement in the primary form of the syndrome, in particular the histological pattern of the so-called antiphospholipid syndrome nephropathy (APSN). APSN is a vascular nephropathy characterized by small vessel vaso-occlusive lesions associated with fibrous intimal hyperplasia of interlobular arteries, recanalizing thrombi in arteries and arterioles, and focal atrophy, a constellation of morphological lesions suggestive of primary antiphospholipid syndrome.

  3. Fibrillary glomerulonephritis with small fibrils in a patient with the antiphospholipid antibody syndrome successfully treated with immunosuppressive therapy

    Directory of Open Access Journals (Sweden)

    Tagboto Senyo

    2007-05-01

    Full Text Available Abstract Background Fibrillary glomerulonephritis is a rare cause of progressive renal dysfunction, often leading to the need for dialysis within a few years. The role of immunosuppressive treatment is still uncertain although this has been tried with variable success. Case presentation A 56 year old woman with the antiphospholipid antibody syndrome (IgM anticardiolipin antibodies was seen in the nephrology clinic with haematuria, proteinuria, and worsening renal function. A renal biopsy demonstrated a mesangial proliferative glomerulonephritis on light microscopy and smaller fibrils (10.6–13.8 nm in diameter than is usual for fibrillary glomerulonephritis (typically 18–22 nm on electron microscopy. Amyloidosis was excluded following detailed evaluation. On account of rapidly worsening renal failure she was started on cyclophosphamide and prednisolone which led to the partial recovery and stabilization of her renal function. Conclusion This case highlights the need for routine electron microscopy in native renal biopsies, where the differential diagnosis is wide and varied and the light and immunofluorescence microscopic findings may be non specific.

  4. Sensibility and specificity for pregnancy morbidity of anti-b2-glycoprotein I antibodies in antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    S. Todesco

    2011-09-01

    Full Text Available Objective: This study aimed to evaluate the sensitivity and specificity of the anti-b2-glycoprotein I antibodies for pregnancy morbidity in the antiphosoplipid syndrome (APS. Methods: 335 women were recruited and on the basis of their clinical features were subdivided into 2 groups homogenous for number and age. The first (study group contained the women whose pregnancy complications satisfied the classification criteria for APS. The second (control group was made up of women with pregnancy complications not included in the classification criteria for APS. Anti-b2-GPI, anticardiolipin antibodies (aCL and lupus anticoagulants (LA were determined in all of these women. Results: The only antiphospholipid antibodies occurring with a significant frequency (p=0,00 in the women with pregnancy criteria for APS were the IgG anti-b2-GPI and the IgG aCL present respectively in 23,92% and in 27,60% of the women. Its association was found to be significant (p=0,000. The distribution of the different levels of positivity of the IgG and IgM anti-b2 GPI in the patients of the study and control groups was not significantly different. The highest sensitivity for pregnancy complications was that of the IgG aCL and of the IgG anti-b2 GPI whose difference was not statistically significant. The comparison of the specificity of the IgG and IgM anti-b2 GPI with that of the IgG and IGM aCL was not statistically significant. Conclusions: The importance of determining the IgG anti-b2 GPI as part of routine laboratory testing of women with pregnancy complications typical of APS was confirmed. Together with IgG aCL these antibodies have proved to be the most sensitive and specific markers of pregnancy complications in APS.

  5. Recurrent, spontaneous esophageal ruptures associated with antiphospholipid antibody syndrome: report of a case.

    Science.gov (United States)

    Naitoh, Hiroshi; Fukuchi, Minoru; Kiriyama, Shinsuke; Fukasawa, Takaharu; Tabe, Yuichi; Yamauchi, Hayato; Yoshida, Tomonori; Saito, Kana; Hagiwara, Kei; Kuwano, Hiroyuki

    2014-01-01

    A 52-year-old man was admitted to our hospital with a spontaneous esophageal rupture (Boerhaave syndrome) and was successfully treated. Eight years after the first incident, he was readmitted with a recurrent rupture. Recurrence of Boerhaave syndrome is extremely rare, with only 7 cases reported in the English literature. During treatment, the patient was also diagnosed with antiphospholipid syndrome (APS). Although APS is known to cause a variety of symptoms due to vascular thrombosis, recurrence of Boerhaave syndrome, coincident with APS, has never been reported. The pathogenesis of Boerhaave syndrome has not been clearly determined. This report serves to increase awareness of the risk of APS, which results in an increased risk of spontaneous rupture of the esophagus.

  6. Antibody profile of pregnant women with antiphospholipid syndrome and pregnancy outcome after treatment with low dose aspirin and low-weight-molecular heparin.

    Science.gov (United States)

    Glasnović, Marija; Bosnjak, Ivica; Vcev, Aleksandar; Soldo, Ivan; Kosuta, Maja; Lenz, Bahrija; Glasnović-Horvatić, Elizabeta; Soldo-Butković, Silva; Mićunović, Nikola

    2007-03-01

    The aim of the research was to show our diagnostic and therapeutic experience with antiphospholipid syndrome (APS) in pregnant women. 36 pregnant women suspect on APS were included in the study: 32 with primary antiphospholipd syndrome (PAPS) and 4 with secondary antiphospholipid syndrome (SAPS). All pregnant women received low-molecular-weight-heparin (LMWH) and low dose aspirin (LDA) therapy. Control group represented 26 women with SAPS and previous bad reproductive anamnesis. Average pregnancy lasted 37.06 +/- 0.707 weeks. LMWH and LDA therapy was successful in 97.22%. Lupus anticoagulant (LA) was found to be more frequent in PAPS group (71.87%). Anticardiolipin antibodies (aCL) were found to be more frequent in SAPS (26.66%). For three patients (3.37%), PAPS was diagnosed due to a fact that they had positive antibeta2-glycoproteinl (antibeta-GP1). To make APS diagnosis, it is of great importance to search for all antiphospholipid antibodies. LMWH and low dose of acetylsalicylic acid should be the first choice therapy.

  7. Management of antiphospholipid syndrome.

    Science.gov (United States)

    Del Papa, Nicoletta; Vaso, Nikoleta

    2010-08-01

    The antiphospholipid syndrome (APS) is an autoimmune disorder presenting with tissue injury in various organs related to large- or small-vessel thrombosis associated with antiphospholipid and antiprotein/phospholipid complex antibodies. Although the pathophysiology, diagnosis, and clinical scenario may seem clear and straightforward, a more detailed examination reveals a more complex and uncertain picture related to the management of APS. This article reviews the current situation relating to APS therapy by evaluating the different clinical features of the syndrome ranging from thrombosis to pregnancy complications together with new strategies and pharmacological approaches.

  8. Successful management of aortic thrombi resulting in spinal cord infarction in a patient with antiphospholipid antibody syndrome and acute cholecystitis.

    Science.gov (United States)

    Izumi, Manabu; Teraoka, Shoko; Yamashita, Keisuke; Matsumoto, Kenji; Muronoi, Tomohiro; Izawa, Yoshimitsu; Yonekawa, Chikara; Ano, Masaki; Suzukawa, Masayuki

    2011-01-01

    A 74-year-old man with coronary artery disease was suffering from acute nonobstructive cholecystitis and was admitted to a nearby hospital. Dual antiplatelet (aspirin and ticlopidine) therapy was discontinued before preparation for surgical resection of the gall bladder. During his time in hospital he was aware of lumbar pain and weakness in both legs. He was transferred to our hospital for further evaluation and therapy. Diffuse intra-aortic thrombi were revealed by computed tomography with contrast media, and magnetic resonance imaging showed spinal cord infarction. However, computed tomography scans of the descending aorta obtained 4 months before admission exhibited no signs of atherosclerotic plaques or intra-aortic thrombi. Laboratory data suggest that antiphospholipid antibody syndrome might have caused these acute multiple intra-arterial thrombi. By restarting dual antiplatelet therapy and increasing the dose of heparin (from 10,000 IU/day to 15,000 IU/day) we successfully managed the patient's clinical condition and symptoms. It is important to understand that stopping antiplatelet therapy may rapidly grow thrombi in patients with a hypercoagulative state.

  9. 抗磷脂抗体与反复流产%Antiphospholipid Antibody and Recurrent Abortion

    Institute of Scientific and Technical Information of China (English)

    鲁爽; 马旭

    2006-01-01

    流产是常见的妊娠并发症,其病因较为复杂,自身免疫因素被认为是导致流产的重要因素之一。在一些发生不明原因反复流产的妇女体内,常常会分离到一些自身抗体,其中以抗磷脂抗体(antiphospholipid antibody,APA)最为普遍。抗磷脂综合征(antiphospholipid syndrome,APS)是近十几年来临床上新发现的一种非器宫特异性的自身免疫性疾病,其主要特征是与其体内的APA密切相关,其临床症状主要包括血栓形成、习惯性流产、血小板减少、溶血性贫血、肢端顽固性溃疡等。这些症状可单一出现,也可多个并存。本文主要讨论APA的特点,致流产的机理研究、临床研究进展和主要治疗原则。

  10. Effect of Low Molecular Weight Heparins (LMWHs on antiphospholipid Antibodies (aPL-mediated inhibition of endometrial angiogenesis.

    Directory of Open Access Journals (Sweden)

    Silvia D'Ippolito

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity in the presence of circulating antiphospholipid antibodies (aPL. Different pathogenic mechanisms for aPL-mediated pregnancy failure have been proposed. In particular a direct effect of aPL on both maternal and fetal side of the placental tissue has been reported, since their reactivity with β2-glycoprotein I (β2GPI makes them adhere to trophoblast and human endometrial endothelial cell (HEEC membranes. β2GPI can be recognized by aPL that, once bound, interfere with both trophoblast functions and with the HEEC differentiation.APS patients can be successfully treated with Low Molecular Weight Heparin (LMWH. Recent reports suggest that LMWH acts through mechanisms alternative to its well known anticoagulant effect, because of its ability to bind β2GPI. In our previous studies, we showed that LMWH is able to reduce the aPL binding to trophoblasts and restore cell invasiveness and differentiation. So far, however, no study has described its effects on endometrial angiogenesis.The aim of our research was to evaluate whether two LMWHs, tinzaparin and enoxaparin, have an effect on the aPL-inhibited endometrial angiogenesis. This prompted us to investigate: (i in vitro HEEC angiogenesis through a Matrigel assay; (ii VEGF secretion by ELISA; (iii matrix metalloproteinase-2 (MMP-2 activity by gelatin zymography; (iv Nuclear Factor-κB (NF-κB DNA binding activity by colorimetric assay; (v STAT-3 activation by a sandwich-ELISA kit. Furthermore, using an in vivo murine model we investigated the LMWHs effects on angiogenesis.We demonstrated that the addition of LMWHs prevents aPL-inhibited HEEC angiogenesis, both in vitro and in vivo, and is able to restore the aPL inhibited NF-κB and/or STAT-3 activity, the VEGF secretion and the MMPs activity.The demonstration of a beneficial role for LMWHs on the aPL-inhibited HEEC angiogenesis

  11. [Catastrophic antiphospholipid syndrome].

    Science.gov (United States)

    Wisłowska, Małgorzata

    2015-01-01

    Catastrophic antiphospholipid syndrome is the most dangerous form of the antiphospholipid syndrome, which is characterized by rapid onset of thrombosis in small vessels of many organs and intravascular coagulation, thrombocytopenia and hemolytic anemia. The syndrome develops over a short period of time with acute multi-organ failure, including kidney, respiratory, cardiovascular, central nervous system and adrenal glands, often associated with disseminated thrombotic microangiopathy. The catastrophic antiphospholipid syndrome involves three or more systems, organs and/or tissues, the development of symptoms must occur within less than one week, it is necessary to confirm the histopathological vascular occlusion in at least one organ or tissue, and laboratory confirmation of the presence of antiphospholipid antibodies in the serum on two occasions over an interval of 12 weeks. This syndrome is characterized by a high mortality despite the use of optimal treatment. Early diagnosis and aggressive treatment of patients with catastrophic antiphospholipid syndrome is essential to save the life of these patients. In the last 10 years, the mortality in this disease decreased from 50% to 30% with simultaneous treatment with anticoagulants, corticosteroids, plasmapheresis and immunoglobulins.

  12. Antibodies to endothelial cells and to beta 2-glycoprotein I in the antiphospholipid syndrome: prevalence and isotype distribution.

    Science.gov (United States)

    Navarro, M; Cervera, R; Teixidó, M; Reverter, J C; Font, J; López-Soto, A; Monteagudo, J; Escolar, G; Ingelmo, M

    1996-06-01

    The aim of this study was to analyse the prevalence and isotype distribution of antibodies to endothelial cells (aEC) and to beta 2-glycoprotein I (a beta 2GPI) in the antiphospholipid syndrome (APS). Fifteen patients with an APS [nine associated with systemic lupus erythematosus (SLE) and six "primary'] and 15 with SLE without an APS were prospectively studied. The aEC were determined by an enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein and the a beta 2GPI by ELISA using highly purified beta 2GPI. A positive titre of aEC was detected in 20 out of 30 patients (67%), but in none of the control group. Ten patients had both IgG and IgM isotypes, five had IgG only and five had only IgM. Thirteen patients with the APS (87%) were found to have a positive titre of aEC, while only seven with SLE but without a history of APS (47%) had aEC (P < 0.05). Nine patients with the APS (60%) had a positive titre of a beta 2GPI (four had both IgG and IgM isotypes, one had IgG only and four had only IgM), while none of the patients without an APS (0%) had these antibodies (P < 0.001). A significant association was also found between the presence of aPL and aEC (P < 0.05), as well as between aPL and a beta 2GPI (P < 0.001). Both aEC and a beta 2GPI can be found in the APS. This reinforces the theory that APS represents a complex autoimmune disorder in which several autoantibodies co-exist with aPL.

  13. A CASE OF STROKE IN YOUNG ADULT SECONDARY TO ANTIPHOSPHOLIPID ANTIBODY SYNDROME

    Directory of Open Access Journals (Sweden)

    Aravinda

    2013-10-01

    Full Text Available Anti phospholipid antibody syndrome is autoantibody - mediated acquired thromb ophilia characterized by recurrent arterial or venous thrombosis. It may occur alone (Primary or associated with other autoimmune disorders ( secondary. We report a case of ischemic stroke in a young individual caused by anti phospholipid antibody syndrom e

  14. Antiphospholipid Syndrome with Antiβ2glicoprotein-1 Antibodies as the Cause of Recurrent Tibial Vein Thrombosis in SAPHO syndrome.

    Science.gov (United States)

    Przepiera-Będzak, Hanna; Brzosko, Marek

    2016-12-01

    The antiphospholipid antibody syndrome is defined by the presence of antiphospholipid antibodies in patients with recurrent venous or arterial thromboembolism (1). SAPHO syndrome is a rare disease, characterized by specific clinical manifestations of synovitis, acne pustulosis, hyperostosis, and osteitis. It is a disease that manifests with a combination of osseous and articular manifestations associated with skin lesions (2). Venous thrombosis complicating SAPHO syndrome seems to be uncommon with an unclear pathogenesis (3-9). Coexistence of antiphospholipid syndrome and SAPHO syndrome was not previously mentioned in literature. A 33-year-old white woman was diagnosed with SAPHO syndrome at the age of 31. The patient was previously diagnosed with polycystic ovary syndrome and depressive syndrome. She was treated with sulfasalazin (2 g daily) and methotrexate (20 mg weekly). Seven months before admission to our department she experienced an episode of deep vein thrombosis of the left leg, successfully treated with subcutaneous enoxaparin sodium (40 mg daily) that was continued for the following 6 months as secondary prophylaxis. Pustular skin changes on palmar surface of the hands and plantar surface of the feet (characteristic for palmo-plantar pustulosis), tenderness of sterno-clavicular joints, swelling and restricted motion of both wrists, and pain on motion in both elbows, shoulders, knees, and ankles were found on physical examination. There was also a moderate amount of effusion in her left knee. There was a 3-centimeter difference between the circumferences of the shins. The level of C reactive protein was increased (6.21 mg/L). The patient was positive for antiβ2glicoprotein-1 (anti-β2G-1) antibodies. Tests for anticardiolipin antibodies (aCL), antiannexin V antibodies, antiphosphatidylserine antibodies (aPS), and antiprothrombin antibodies (aPT) were negative. Prothrombin time, activated partial thromboplastin time, and D-dimer level were normal, and

  15. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  16. A case of catastrophic antiphospholipid antibody syndrome complicated with systemic lupus erythematosus, double positive for anti-cardiolipin/β₂ glycoprotein I and anti-phosphatidylserine/prothrombin autoantibodies.

    Science.gov (United States)

    Hirakawa, Eri; Saito, Kazuyoshi; Hirata, Shintaro; Atsumi, Tatsuya; Koike, Takao; Tanaka, Yoshiya

    2012-09-01

    A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β(2)-glycoprotein I (β(2)GPI) were simultaneously detected, indicating that the different pathways of both PS/PT and CL/β(2)GPI might be associated with the radical manifestation of CAPS.

  17. Non-traumatic carotid dissection and stroke associated with anti-phospholipid antibody syndrome: Report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    Kluger Benzi

    2008-01-01

    Full Text Available Young adults with stroke frequently do not have any of the traditional risk factors associated with stroke, prompting a search for other mechanical and hypercoagulable causes. The authors report a young man presenting with stroke and subsequently diagnosed with a carotid dissection. Recurrent strokes while on heparin prompted a search for a second etiology and the patient was found to have antiphospholipid antibody syndrome. Although these conditions may be coincidental, we propose that their interaction was significant in this patient′s presentation. Other reports of this association will also be reviewed.

  18. The obstetric outcome following treatment in a cohort of patients with antiphospholipid antibody syndrome in a tertiary care center

    Directory of Open Access Journals (Sweden)

    V Dadhwal

    2011-01-01

    Full Text Available Background: Antiphospholipid antibody syndrome (APAS is regarded as the most frequently acquired risk factor for thrombophilia. The obstetric manifestations of APAS include early or late pregnancy losses and complications like preeclampsia and fetal growth restriction. Its timely diagnosis and treatment can improve maternal and neonatal outcome. Aims: To study the pregnancy outcome of patients with APAS treated with heparin and aspirin. Settings and Design: This was a retrospective study of pregnancy outcome in 42 consecutive women with APAS, treated with heparin and aspirin. Materials and Methods: The case records of 42 diagnosed cases of APAS with pregnancy, over a 3-year period, were studied. The pregnancy outcome in this group was compared before and after treatment with heparin and low-dose aspirin in terms of abortions, intrauterine deaths and live birth rate. The outcome of the present pregnancy in terms of fetal and maternal complications was analyzed. Results: The mean age and average parity of women with APAS were 30.1±4.1 years and 3.2±1.2, respectively. Among the treated patients of APAS, 13 (30.9% had preeclampsia and 9 (21.4% had intrauterine growth restriction (IUGR. There were 2 (4.7% intrauterine deaths, 4 (9.5% missed abortions and 3 (7.1% abruptio placentae. Women with APAS had a live birth rate of 4.6% before treatment and 85.7% in the index pregnancy after treatment. Conclusion: Treatment of pregnant women with APAS results in marked improvement in the live birth rate (4.6-85.7%. However, complications like preeclampsia and IUGR occur even after treatment, requiring strict monitoring and timely delivery.

  19. Systemic Lupus Erythematosus and Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Aleksandra Plavsic

    2014-09-01

    Full Text Available Antiphospholipid syndrome is an autoimmune disorder defined as association of vascular thrombosis and/or pregnancy complications with presence of antiphospholipid antibodies (lupus anticoagulant, anticardiolipin and anti-β2 glycoprotein I. It is the most common cause of acquired thrombophilia, and can occur as an independent entity or in relation with other diseases, especially systemic lupus erythematosus. Presence of antiphospholipid syndrome in systemic lupus erythematosus is additional vaso occlusive factor in already present inflammation, bringing further risk for thrombotic events. Clinical and serological manifestations of antiphospholipid syndrome and systemic lupus erythematosus are very similar, so possible connection for these two autoimmune disorders is assumed.

  20. Antiphospholipid Syndrome Clinical Research Task Force Report

    NARCIS (Netherlands)

    Erkan, D.; Derksen, R.; Levy, R.; Machin, S.; Ortel, T.; Pierangeli, S.; Roubey, R.; Lockshin, M.

    2011-01-01

    The Antiphospholipid Syndrome (APS) Clinical Research Task Force (CRTF) was one of six Task Forces developed by the 13(th) International Congress on Antiphospholipid Antibodies (aPL) organization committee with the purpose of: a) evaluating the limitations of APS clinical research and developing gui

  1. Thrombotic risk assessment in antiphospholipid syndrome the role of new antibody specificities and thrombin generation assay

    DEFF Research Database (Denmark)

    Sciascia, Savino; Baldovino, Simone; Schreiber, Karen

    2016-01-01

    . Vascular events seem to result of local procoagulative alterations upon triggers influence (the so called "second-hit theory"), while placental thrombosis and complement activation seem to lead to pregnancy morbidity. The laboratory tests suggested by the current classification criteria include lupus...... anticoagulant, a functional coagulation assay, and anticardiolipin and anti-β2-glycoprotein-I antibodies, generally detected by solid phase enzyme-linked immunosorbent assay. The real challenge for treating physicians is understanding what is the actual weight of aPL in provoking clinical manifestations in each...

  2. Clinical Application of Revised Laboratory Classification Criteria for Antiphospholipid Antibody Syndrome: Is the Follow-Up Interval of 12 Weeks Instead of 6 Weeks Significantly Useful?

    Directory of Open Access Journals (Sweden)

    Sang Hyuk Park

    2016-01-01

    Full Text Available Background. According to revised classification criteria of true antiphospholipid antibody syndrome, at least one of three antiphospholipid antibodies should be present on two or more occasions at least 12 weeks apart. However, it can be inconvenient to perform follow-up tests with interval of 12 weeks. We investigated clinical application of follow-up tests with interval of 12 weeks. Method. Totals of 67, 199, and 332 patients tested positive initially for the lupus anticoagulants confirm, the anti-β2 glycoprotein-I antibody, and the anti-cardiolipin antibody test, respectively, from Jan 2007 to Jul 2009. We investigated clinical symptoms of patients, follow-up interval, and results of each test. Results. Among patients with initial test positive, 1.5%–8.5% were subjected to follow-up tests at interval of more than 12 weeks. Among 25 patients with negative conversion in tests, patients with interval of more than 12 weeks showed clinical symptom positivity of 33.3%, which was higher than that of 12.5% with 6–12 weeks. Among 34 patients with persistent test positive, clinical symptoms positivity trended to be more evident in patients at interval of 6–12 weeks (47.4% versus 26.7%, P=0.191 than more than 12 weeks. Conclusion. Less than 10% of patients with initial test positive had follow-up tests at interval of more than 12 weeks and the patients with persistent test positive at interval of more than 12 weeks showed trends toward having lower clinical symptoms than 6–12 weeks. More research is needed focused on the evidence that follow-up test at interval of more than 12 weeks should be performed instead of 6 weeks.

  3. Clinical Application of Revised Laboratory Classification Criteria for Antiphospholipid Antibody Syndrome: Is the Follow-Up Interval of 12 Weeks Instead of 6 Weeks Significantly Useful?

    Science.gov (United States)

    Park, Sang Hyuk; Jang, Seongsoo; Park, Chan-Jeoung; Chi, Hyun-Sook

    2016-01-01

    Background. According to revised classification criteria of true antiphospholipid antibody syndrome, at least one of three antiphospholipid antibodies should be present on two or more occasions at least 12 weeks apart. However, it can be inconvenient to perform follow-up tests with interval of 12 weeks. We investigated clinical application of follow-up tests with interval of 12 weeks. Method. Totals of 67, 199, and 332 patients tested positive initially for the lupus anticoagulants confirm, the anti-β 2 glycoprotein-I antibody, and the anti-cardiolipin antibody test, respectively, from Jan 2007 to Jul 2009. We investigated clinical symptoms of patients, follow-up interval, and results of each test. Results. Among patients with initial test positive, 1.5%-8.5% were subjected to follow-up tests at interval of more than 12 weeks. Among 25 patients with negative conversion in tests, patients with interval of more than 12 weeks showed clinical symptom positivity of 33.3%, which was higher than that of 12.5% with 6-12 weeks. Among 34 patients with persistent test positive, clinical symptoms positivity trended to be more evident in patients at interval of 6-12 weeks (47.4% versus 26.7%, P = 0.191) than more than 12 weeks. Conclusion. Less than 10% of patients with initial test positive had follow-up tests at interval of more than 12 weeks and the patients with persistent test positive at interval of more than 12 weeks showed trends toward having lower clinical symptoms than 6-12 weeks. More research is needed focused on the evidence that follow-up test at interval of more than 12 weeks should be performed instead of 6 weeks.

  4. Apheresis and intravenous immunoglobulins used in addition to conventional therapy to treat high-risk pregnant antiphospholipid antibody syndrome patients. A prospective study.

    Science.gov (United States)

    Ruffatti, Amelia; Favaro, Maria; Hoxha, Ariela; Zambon, Alessandra; Marson, Piero; Del Ross, Teresa; Calligaro, Antonia; Tonello, Marta; Nardelli, Giovanni B

    2016-06-01

    Pregnant women with triple antibody positive antiphospholipid syndrome (APS) who have had thrombosis or a history of early, severe pregnancy complications are generally considered at high risk of pregnancy loss. The objectives of this study were to investigate the efficacy and safety of a relatively new treatment protocol used in addition to conventional therapy in high-risk pregnant patients affected with primary APS. The study's two inclusion criteria were: (1) the presence of triple antiphospholipid positivity, (2) previous thrombosis and/or a history of one or more early, severe pregnancy complications. Eighteen pregnancies occurring between 2002 and 2015 in 14 APS patients, (mean age 34.8±3.6 SD) were monitored. All 14 (100%) patients had triple antiphospholipid positivity. In addition, six of them (42.8%) had a history of thrombosis, four (28.6%) had one or more previous early and severe pregnancy complications, and four (30.8%) met both clinical study criteria. The study protocol included weekly plasmapheresis or immunoadsorption and fortnightly 1g/kg intravenous immunoglobulins. Seventeen of the pregnancies (94.4%) produced live neonates, all born between the 26th and 37th weeks of gestation (mean 33.1±3.5 SD). One female (5.5%), born prematurely at 24 weeks, died of sepsis a week after birth. There were two cases (11.1%) of severe pregnancy complications. No treatment side effects were registered. Given the high live birth rate and the safety associated to it, the study protocol described here could be taken into consideration by medical teams treating high-risk APS pregnant patients.

  5. Immunotherapy in antiphospholipid syndrome.

    Science.gov (United States)

    Lopez-Pedrera, Ch; Aguirre, M A; Ruiz-Limon, P; Pérez-Sánchez, C; Jimenez-Gomez, Y; Barbarroja, N; Cuadrado, M J

    2015-08-01

    Antiphospholipid syndrome (APS) is a disorder characterized by the association of arterial or venous thrombosis and/or pregnancy morbidity with the presence of antiphospholipid antibodies (anticardiolipin antibodies, lupus anticoagulant antibodies, and/or anti-β2-glycoprotein I antibodies). Thrombosis is the major manifestation in patients with aPLs, but the spectrum of symptoms and signs associated with aPLs has broadened considerably, and other manifestations, such as thrombocytopenia, non-thrombotic neurological syndromes, psychiatric manifestations, livedo reticularis, skin ulcers, hemolytic anemia, pulmonary hypertension, cardiac valve abnormality, and atherosclerosis, have also been related to the presence of those antibodies. Several studies have contributed to uncovering the basis of antiphospholipid antibody pathogenicity, including the targeted cellular components, affected systems, involved receptors, intracellular pathways used, and the effector molecules that are altered in the process. Therapy for thrombosis traditionally has been based on long-term oral anticoagulation; however, bleeding complications and recurrence despite high-intensity anticoagulation can occur. The currently accepted first-line treatment for obstetric APS (OAPS) is low-dose aspirin plus prophylactic unfractionated or low-molecular-weight heparin (LMWH). However, in approximately 20% of OAPS cases, the final endpoint, i.e. a live birth, cannot be achieved. Based on all the data obtained in different research studies, new potential therapeutic approaches have been proposed, including the use of new oral anticoagulants, statins, hydroxychloroquine, coenzyme Q10, B-cell depletion, platelet and TF inhibitors, peptide therapy or complement inhibition among others. Current best practice in use of these treatments is discussed.

  6. Platelt receptors involved in the antiphospholipid syndrome

    NARCIS (Netherlands)

    Pennings, M.T.T.

    2007-01-01

    The antiphospholipid syndrome (APS) is a non-inflammatory autoimmune disease clinically characterized by the occurrence of either venous or arterial thrombosis or the presence of specific pregnancy complications. Serological criteria are the persistent presence of antibodies directed against cardiol

  7. Antibodies Against β2-Glycoprotein I Complexed With an Oxidised Lipoprotein Relate to Intima Thickening of Carotid Arteries in Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    P. R. J. Ames

    2006-01-01

    Full Text Available To explore whether antibodies against β2-glycoprotein I (β2GPI complexed to 7-ketocholesteryl-9-carboxynonanoate (oxLig-1 and to oxidised low-density lipoproteins (oxLDL relate to paraoxonase activity (PONa and/or intima media thickness (IMT of carotid arteries in primary antiphospholipid syndrome (PAPS. As many as 29 thrombotic patients with PAPS, 10 subjects with idiopathic antiphospholipid antibodies (aPL without thrombosis, 17 thrombotic patients with inherited thrombophilia and 23 healthy controls were investigated. The following were measured in all participants: β2GPI−oxLDL complexes, IgG anti-β2GPI−oxLig-1, IgG anti-β2GPI−oxLDL antibodies (ELISA, PONa, (para-nitrophenol method, IMT of common carotid (CC artery, carotid bifurcation (B, internal carotid (IC by high resolution sonography. β2GPI−oxLDL complex was highest in the control group (p < 0.01, whereas, IgG anti-β2GPI−oxLig1 and IgG anti-β2GPI−oxLDL were highest in PAPS (p < 0.0001. In healthy controls, β2GPI−oxLDL complexes positively correlated to IMT of the IC (p = 0.007 and negatively to PONa after correction for age (p < 0.03. PONa inversely correlated with age (p = 0.008. In PAPS, IgG anti-2GPI−oxLig-1 independently predicted PONa (p = 0.02 and IMT of B (p = 0.003, CC, (p = 0.03 and of IC (p = 0.04. In PAPS, PONa inversely correlated to the IMT of B, CC and IC (p = 0.01, 0.02 and 0.003, respectively. IgG anti-2GPI−oxLig-1 may be involved in PAPS related atherogenesis via decreased PON activity.

  8. Catastrophic antiphospholipid syndrome presenting as fever of unknown origin

    Directory of Open Access Journals (Sweden)

    Fatma I Al-Beladi

    2012-01-01

    Full Text Available Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies with characteristic clinical manifestation, which include venous, arterial thrombosis, thrombotic microangiopathy, and recurrent fetal loss. The syndrome can be secondary to many causes including systemic lupus erythematosus (SLE or "primary" antiphospholipid syndrome (APLS. We report a case of a man with catastrophic antiphospholipid syndrome (CAPS, which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis.

  9. Severe antiphospholipid syndrome and cardiac surgery: Perioperative management.

    Science.gov (United States)

    Mishra, Pankaj Kumar; Khazi, Fayaz Mohammed; Yiu, Patrick; Billing, John Stephen

    2016-06-01

    Antiphospholipid syndrome is an antiphospholipid antibody-mediated prothrombotic state leading to arterial and venous thrombosis. This condition alters routine in-vitro coagulation tests, making results unreliable. Antiphospholipid syndrome patients requiring cardiac surgery with cardiopulmonary bypass present a unique challenge in perioperative anticoagulation management. We describe 3 patients with antiphospholipid syndrome who had successful heart valve surgery at our institution. We have devised an institutional protocol for antiphospholipid syndrome patients, and all 3 patients were managed according to this protocol. An algorithm-based approach is recommended because it improves team work, optimizes treatment, and improves patient outcome.

  10. Catastrophic antiphospholipid syndrome presenting as fever of unknown origin.

    Science.gov (United States)

    Al-Beladi, Fatma I

    2012-01-01

    Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies with characteristic clinical manifestation, which include venous, arterial thrombosis, thrombotic microangiopathy, and recurrent fetal loss. The syndrome can be secondary to many causes including systemic lupus erythematosus (SLE) or "primary" antiphospholipid syndrome (APLS). We report a case of a man with catastrophic antiphospholipid syndrome (CAPS), which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis.

  11. 'Criteria' aPL tests: report of a task force and preconference workshop at the 13th International Congress on Antiphospholipid Antibodies, Galveston, Texas, April 2010.

    Science.gov (United States)

    Pierangeli, S S; de Groot, P G; Dlott, J; Favaloro, E; Harris, E N; Lakos, G; Ortel, T; Meroni, P L; Otomo, K; Pengo, V; Tincani, A; Wong, R; Roubey, R

    2011-02-01

    Current classification criteria for definite antiphospholipid syndrome (APS) mandate the use of one or more of three positive 'standardized' laboratory assays to detect antiphospholipid antibodies (aPL) (viz: anticardiolipin [aCL] IgG and IgM; anti-β(2)glycoprotein I [anti-β(2)GPI] antibodies IgG and IgM; and/or a lupus anticoagulant [LAC]), when at least one of the two major clinical manifestations (thrombosis or pregnancy losses) are present. Although, efforts of standardization for these 'criteria' aPL tests have been conducted over the last 27 years, reports of inconsistencies, inter-assay and inter-laboratory variation in the results of aCL, LAC, and anti-β(2)GPI, and problems with the interpretation and the clinical value of the tests still exist, which affect the consistency of the diagnosis of APS. A Task Force of scientists and pioneers in the field from different countries, subdivided in three working groups, discussed and analyzed critical questions related to 'criteria' aPL tests in an evidence-based manner, during the 13(th) International Congress on Antiphospholipid Antibodies (APLA 2010, April 13-16, 2010, Galveston, TX). These included: review of the standardization and the need for international consensus protocol for aCL and anti-β(2)GPI tests; the use of monoclonal and/or polyclonal standards in the calibration curve of those tests; and the need for establishment of international units of measurement for anti-β(2)GPI tests. The group also reviewed the recently updated guidelines for LAC testing, and analyzed and discussed the possibility of stratification of 'criteria' aPL tests as risk factors for APS, as well as the clinical value of single positive vs. multiple aPL positivity. The group members presented, discussed, analyzed data, updated and re-defined those critical questions at a preconference workshop that was open to congress attendees. This report summarizes the findings, conclusions, and recommendations of this Task Force.

  12. Antiphospholipid Syndrome Novel Therapies

    Directory of Open Access Journals (Sweden)

    Mohamad Bittar

    2014-07-01

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disease characterised by arterial and/or venous thrombosis, recurrent pregnancy loss, and persistently positive antiphospholipid antibodies (aPLs. It could be life-threatening as in the case of catastrophic APS where multi-organ failure is observed. APS morbidities are thought to be the result of a combination of thrombotic and inflammatory processes. Over the past decades, the mainstay of therapy of APS has been anticoagulation. As new mechanisms of pathogenesis are being unravelled with time, novel targeted immunomodulatory therapies are being proposed as promising agents in the treatment of APS. In this article, we present an overview of new pathogenetic mechanisms in APS as well as novel antithrombotic and immunomodulatory therapies.

  13. Repeated renal infarction in native and transplanted kidneys due to left ventricular thrombus formation caused by antiphospholipid antibody syndrome.

    Science.gov (United States)

    Scully, Paul; Leckstroem, Daniel C; McGrath, Andrew; Chambers, John; Goldsmith, David J

    2013-01-01

    Antiphospholipid syndrome can be a feature of several underlying conditions, such as lupus, but it can also occur idiopathically. Diagnosis usually comes after investigation of recurrent venous or arterial thromboses, emboli, or hypertension/proteinuria where the kidney is involved and is usually confirmed by laboratory testing. We describe a case of a man with a myocardial infarction who developed mural thrombus in an akinetic left ventricular segment but then who recurrently embolized first to one of his native kidneys and then later to a transplanted kidney. Although the clinical behavior was typical of antiphospholipid syndrome, it took numerous laboratory assays over many years until finally the problem was confirmed and life-long warfarin therapy instituted.

  14. Spontaneous Coronary Artery Dissection in a Male Patient with Takayasu’s Arteritis and Antiphospholipid Antibody Syndrome

    Directory of Open Access Journals (Sweden)

    Demet Menekşe Gerede

    2013-01-01

    Full Text Available We present a case of a 34-year-old male who presented to the emergency ward with fever and abdominal pain. The diagnosis of Takayasu’s arteritis and also antiphospholipid syndrome was made during an imaging workup of deep-vein thrombosis. A spontaneous coronary artery dissection was revealed in coronary CT angiography requested for chest pain and dyspnea. The patient was treated medically and discharged on close followup. The concurrence of spontaneous coronary artery dissection with antiphospholipid syndrome and Takayasu’s arteritis has not been reported in the previous literature. The possibility of a spontaneous coronary artery dissection should be considered in patients presenting with both diseases.

  15. Repeated renal infarction in native and transplanted kidneys due to left ventricular thrombus formation caused by antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Scully P

    2013-01-01

    Full Text Available Paul Scully,1 Daniel C Leckstroem,1 Andrew McGrath,2 John Chambers,3 David J Goldsmith11Nephrology Department, 2Radiology Department, 3Cardiology Department, King's Health Partners, Academic Health Sciences Centre, London, United KingdomAbstract: Antiphospholipid syndrome can be a feature of several underlying conditions, such as lupus, but it can also occur idiopathically. Diagnosis usually comes after investigation of recurrent venous or arterial thromboses, emboli, or hypertension/proteinuria where the kidney is involved and is usually confirmed by laboratory testing. We describe a case of a man with a myocardial infarction who developed mural thrombus in an akinetic left ventricular segment but then who recurrently embolized first to one of his native kidneys and then later to a transplanted kidney. Although the clinical behavior was typical of antiphospholipid syndrome, it took numerous laboratory assays over many years until finally the problem was confirmed and life-long warfarin therapy instituted.Keywords: antiphospholipid therapy, emboli, infarction, kidney, kidney transplant

  16. Antibody specific epitope prediction-emergence of a new paradigm.

    Science.gov (United States)

    Sela-Culang, Inbal; Ofran, Yanay; Peters, Bjoern

    2015-04-01

    The development of accurate tools for predicting B-cell epitopes is important but difficult. Traditional methods have examined which regions in an antigen are likely binding sites of an antibody. However, it is becoming increasingly clear that most antigen surface residues will be able to bind one or more of the myriad of possible antibodies. In recent years, new approaches have emerged for predicting an epitope for a specific antibody, utilizing information encoded in antibody sequence or structure. Applying such antibody-specific predictions to groups of antibodies in combination with easily obtainable experimental data improves the performance of epitope predictions. We expect that further advances of such tools will be possible with the integration of immunoglobulin repertoire sequencing data.

  17. Anticorpos antinucleossomo e síndrome antifosfolipídica: estudo observacional Antinucleosome antibodies and primary antiphospholipid syndrome: an observational study

    Directory of Open Access Journals (Sweden)

    Alexandre Wagner Silva de Souza

    2012-06-01

    Full Text Available OBJETIVO: Avaliar a associação entre a presença de anticorpos antinucleossomo (anti-NCS e a síndrome antifosfolipídica primária (SAFP e o posterior desenvolvimento de lúpus eritematoso sistêmico (LES. MATERIAIS E MÉTODOS: Trinta e seis mulheres com o diagnóstico de SAFP foram avaliadas prospectivamente para manifestações de doenças reumáticas autoimunes e para a presença de anticorpos antifosfolípides, anticorpos antinucleares e anti-NCS/cromatina. RESULTADOS: Após um período médio de seguimento de 45,7 meses, anticorpos anti-NCS/cromatina foram detectados em apenas uma paciente (2,8%, que desenvolveu manifestações de LES tais como poliartrite, linfopenia, neurite óptica, lesões compatíveis com esclerose múltipla em substância branca cerebral e perfil de autoanticorpos altamente sugestivo de LES. CONCLUSÃO: A frequência de anticorpos anti-NCS/cromatina é baixa em pacientes com SAFP, e sua presença pode associar-se ao desenvolvimento de manifestações de LES.OBJECTIVE: To study the association of anti-nucleosome (anti-NCS antibodies in primary antiphospholipid syndrome (APS and the development of systemic lupus erythematosus (SLE during follow-up. MATERIALS AND METHODS: Thirty-six women with primary APS were evaluated prospectively for clinical features of systemic autoimmune diseases and for the presence of antiphospholipid antibodies, antinuclear antibodies and anti-NCS/chromatin antibodies. RESULTS: After a mean follow-up period of 45.7 months, anti-NCS/chromatin antibodies were detected in only one patient (2.8%, who developed features of SLE including polyarthritis, lymphopenia, optic neuritis, multiple sclerosis-like lesions, and an autoantibody profile suggestive of SLE. CONCLUSION: The frequency of anti-NCS/chromatin antibodies in primary APS patients is very low, and they may be associated with the development of SLE manifestations.

  18. The Role of TLR4 on B Cell Activation and Anti-β2GPI Antibody Production in the Antiphospholipid Syndrome

    Science.gov (United States)

    2016-01-01

    High titer of anti-β2-glycoprotein I antibodies (anti-β2GPI Ab) plays a pathogenic role in antiphospholipid syndrome (APS). Numerous studies have focused on the pathological mechanism in APS; however, little attention is paid to the immune mechanism of production of anti-β2GPI antibodies in APS. Our previous study demonstrated that Toll-like receptor 4 (TLR4) plays a vital role in the maturation of bone marrow-derived dendritic cells (BMDCs) from the mice immunized with human β2-glycoprotein I (β2GPI). TLR4 is required for the activation of B cells and the production of autoantibody in mice treated with β2GPI. However, TLR4 provides a third signal for B cell activation and then promotes B cells better receiving signals from both B cell antigen receptor (BCR) and CD40, thus promoting B cell activation, surface molecules expression, anti-β2GPI Ab production, and cytokines secretion and making B cell functioning like an antigen presenting cell (APC). At the same time, TLR4 also promotes B cells producing antibodies by upregulating the expression of B-cell activating factor (BAFF). In this paper, we aim to review the functions of TLR4 in B cell immune response and antibody production in autoimmune disease APS and try to find a new way for the prevention and treatment of APS. PMID:27868072

  19. Prevalence of deep venous thrombosis in the lower limbs and the pelvis and pulmonary embolism in patients with positive antiphospholipid antibodies

    Energy Technology Data Exchange (ETDEWEB)

    Kinuya, Keiko; Kakuda, Kiyoshi; Matano, Sadaya; Sato, Shigehiko; Sugimoto, Tatsuho [Tonami General Hospital, Toyama (Japan); Asakura, Hidesaku; Kinuya, Seigo; Michigishi, Takatoshi; Tonami, Norihisa

    2001-12-01

    Antiphospholipid antibodies (AA) are immunoglobulins that cross-react with phospholipid on cell membrane, and are therefore associated with a hypercoagulable state manifested by arterial/venous thromboses. We aimed to determine the prevalence of deep venous thrombosis in the lower limbs and the pelvic region (DVT) and pulmonary embolism (PE) in patients with positive AA. Sixty-six patients (48 female, 18 male) with positive lupus anticoagulant (LA) and/or positive anticardiolipin antibody (aCL) underwent radionuclide (RN) venography with 370 MBq of {sup 99m}Tc-MAA. Pulmonary perfusion scintigraphy was performed in 58 patients. Fifteen patients had positive LA and positive aCL (LA+/aCL+), 33 patients had positive LA only (LA+/aCL-) and 18 patients had positive aCL only (LA-/aCL+). Forty-three patients were diagnosed with primary antiphospholipid syndrome (APS) and 19 were diagnosed with APS associated with SLE. DVT was detected in 21 of 66 patients (32%). Patients with LA+/aCL+ showed higher prevalence of DVT (53%) as compared to LA+/aCL- (27%) and LA-/aCL+ (22%). PE was found in 13 of 58 patients (22%). The prevalence of PE was higher in patients with positive aCL (33% in LA+/aCL+; 36% in LA-/aCL+) than in patients with negative aCL (10%). Because of the high prevalence of DVT and PE in patients with AA, RN scintigraphy must be recommended in screening for these clinical troubles. These results indicate that the prevalence of DVT and PE may vary in subgroups of AA. (author)

  20. Antiphospholipid-related chorea

    Directory of Open Access Journals (Sweden)

    Silvio ePeluso

    2012-10-01

    Full Text Available Chorea is a movement disorder which may be associated with immunologic diseases, in particular in the presence of antiphospholipid antibodies (aPL. Choreic movements have been linked to the isolated presence of plasmatic aPL, or to primary or secondary antiphospholipid syndrome. The highest incidence of aPL-related chorea is detected in children and females. The presentation of chorea is usually subacute and the course monophasic. Choreic movements can be focal, unilateral, or generalized. High plasmatic titers of aPL in a choreic patient can suggest the diagnosis of aPL related-chorea; neuroimaging investigation does not provide much additional diagnostic information. The most relevant target of aPL is β2-glycoprotein I, probably responsible for the thrombotic manifestations of antiphospholipid syndrome. Etiology of the movement disorder is not well understood but a neurotoxic effect of aPL has been hypothesized, leading to impaired basal ganglia cell function and development of neuroinflammation. Patients affected by aPL-related chorea have an increased risk of thrombosis and should receive antiplatelet or anticoagulant treatment.

  1. Superior vena cava syndrome due to intravascular thrombosis in a patient with rheumatoid arthritis without antiphospholipid antibody syndrome: Is rheumatoid arthritis a separate hypercoagulable state

    Directory of Open Access Journals (Sweden)

    Pramila Dharmshaktu

    2014-03-01

    Full Text Available We report a 60 year male with long history of joint pain later diagnosed as rheumatoid arthritis (RA who presented with dyspnoea and swelling over neck& upper chest. A clinical diagnosis of superior vena cava (SVC syndrome was made. Patient fulfilled criteria for definite rheumatoid arthritis supported with positive serology. Contrast enhanced computerized tomography (CECT scan of chest revealed thrombosis in SVC. Patient was investigated for the cause of SVC thrombosis. Anti nuclear antibody (ANA test was negative. Anti cardiolipin antibody was done to rule out antiphospholipid antibody (APLA syndrome which has a known association with rheumatoid arthritis to cause intravascular thrombosis but was negative. Digital rectal examination (DRE and prostate specific antigen (PSA levels were normal. Further investigations as a part of thrombophilia work up were normal. There are case reports where RA is associated with SVC syndrome but only when it is associated mediastinal lymphadenopathy or SVC thrombosis due to APLA Syndrome. This case suggests RA per se as hypercoagulable state.

  2. [Apheresis in antiphospholipid syndrome (APS)].

    Science.gov (United States)

    De Silvestro, Giustina; Tison, Tiziana; Marson, Piero

    2012-01-01

    Antiphospholipid syndrome (APS) is a rare clinical disorder characterized by thromboembolic manifestations and/or obstetric complications. Along with the clinical symptoms and signs, serum antiphospholipid antibodies have to be detected. APS can be primary, i.e., without any concomitant disorders, or secondary to other autoimmune diseases, particularly systemic lupus erythematosus. Criteria for the diagnosis of APS have been clearly established. Hyperacute APS (or catastrophic antiphospholipid syndrome), often with a poor prognosis, must meet four criteria: involvement of three or more organs, rapid evolution of clinical manifestations, microangiopathic occlusion of small blood vessels at biopsy, and presence of antiphospholipid antibodies. The rationale for apheresis treatment is the removal of pathogenetic antibodies involved in the development of tissue damage. Our experience includes 23 patients, in particular 15 women treated for 19 pregnancies. According to the National Guidelines Program, the effectiveness of apheresis in catastrophic syndrome has a level of evidence of V/VI, with a strength of recommendation A; in highrisk pregnancy it has a level of evidence of V with a strength of recommendation B. It will be necessary to better define the prognosis of various categories of pregnant patients with APS, as well as useful laboratory parameters to monitor its clinical course and anticipate any complications of pregnancy.

  3. Avaliação da pesquisa de anticorpos antifosfolipídios para o diagnóstico da síndrome antifosfolípide Evaluation of antiphospholipid antibodies testing for the diagnosis of antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Paula Gonçalves Perches

    2009-06-01

    Full Text Available OBJETIVO: Determinar a prevalência de anticoagulante lúpico (LAC e dos isótipos de anticardiolipina (ACL e suas eventuais associações clínicas. PACIENTES E MÉTODOS: Estudo retrospectivo que avaliou manifestações clínicas e laboratoriais em indivíduos que apresentaram positividade para anticorpos antifosfolipídios no Hospital Edmundo Vasconcelos entre março de 2005 e junho de 2006. RESULTADOS: Cento e seis indivíduos (média de idade 42,2 ± 14,1 anos, 84% do sexo feminino foram incluídos no estudo. A prevalência de trombose foi de 17,9% (19/106 e de morbidade gestacional foi de 12,3% (13/106. O diagnóstico de Síndrome Antifosfolípide (SAF foi feito em 23,6% (25/106, sendo primária em 68% (17/25 e secundária em 32% (8/25. A prevalência de ACL foi de 97,1% (103/106 e de LAC foi de 11,4% (5/44 dos exames realizados. ACL isótipos IgM, IgG e IgA foram encontrados em 100%, 23,3% e 4,9% dos 103 soros ACL positivos, respectivamente. Para o diagnóstico de SAF, a ACL IgM apresentou sensibilidade de 92% e especificidade de 1,2%, enquanto a ACL IgG teve sensibilidade de 40% e especificidade de 82,5%. A ausência de ACL IgG teve alto valor preditivo negativo (81,4% para SAF. O LAC apresentou sensibilidade de 18,7% e especificidade de 92,8%. A curva Receiver Operating Characteristic (ROC demonstrou maior área abaixo da curva para ACL IgG e LAC. CONCLUSÃO: Em amostra aleatória de indivíduos com anticorpos antifosfolipídios positivos, ACL IgG e LAC apresentaram maior especificidade para o diagnóstico de SAF, que se caracterizou pela maior prevalência de trombose.OBJECTIVE: To evaluate the prevalence of lupus anticoagulant (LAC and isotypes of anticardiolipin (ACL antibodies and its possible clinical associations. PATIENTS AND METHODS: A retrospective study analyzed clinical and laboratorial manifestations in individuals who showed positive antiphospholipid antibodies followed-up at Hospital Edmundo Vasconcelos from March 2005 to

  4. Antiphospholipid Syndrome and Kidney Involvement: New Insights

    Directory of Open Access Journals (Sweden)

    José A. Martínez-Flores

    2016-07-01

    Full Text Available Antiphospholipid syndrome is an autoimmune disorder characterized by vascular thromboses and pregnancy morbidity associated with antiphospholipid antibodies: lupus anticoagulant, IgG or IgM anticardiolipin or anti-beta 2-glycoprotein I. The kidney is one of the major target organs in antiphospholipid syndrome (APS. However, beyond the known involvement of the kidney in primary and associated APS, we may be observing a new form of APS within the context of renal failure. This review describes the classical kidney manifestations of APS and provides new considerations to be taken into account.

  5. Anti-prothrombin (aPT) and anti-phosphatidylserine/prothrombin (aPS/PT) antibodies and the risk of thrombosis in the antiphospholipid syndrome. A systematic review.

    Science.gov (United States)

    Sciascia, Savino; Sanna, Giovanni; Murru, Veronica; Roccatello, Dario; Khamashta, Munther A; Bertolaccini, Maria Laura

    2014-02-01

    Antibodies to prothrombin are detected by directly coating prothrombin on irradiated ELISA plates (aPT) or by using the phosphatidylserine/prothrombin complex as antigen (aPS/PT). Although these antibodies have both been associated with antiphospholipid syndrome (APS) and a correlation between the two assays have been reported, it seems that aPT and aPS/PT belong to different populations of autoantibodies. It was our objective to systematically review the available evidence on aPT and aPS/PT antibodies and the risk of thrombosis in APS. Medline-reports published between 1988 and 2013 investigating aPT and aPS/PT as a risk factor for thrombosis were included. Whenever possible, antibody isotype(s) and site of thrombosis were analysed. This systematic review is based on available data from more than 7,000 patients and controls from 38 studies analysing aPT and 10 aPS/PT. Antibodies to prothrombin (both aPT and aPS/PT) increased the risk of thrombosis (odds ratio [OR] 2.3; 95% confidence interval [CI] 1.72-3.5). aPS/PT seemed to represent a stronger risk factor for thrombosis, both arterial and/or venous than aPT (OR 5.11; 95%CI 4.2-6.3 and OR 1.82; 95%CI 1.44-2.75, respectively). In conclusion, routine measurement of aPS/PT (but not aPT) might be useful in establishing the thrombotic risk of patients with previous thrombosis and/or systemic lupus erythematosus. Their inclusion as laboratory criteria for the APS should be indisputably further explored.

  6. Rheumatic Fever Associated with Antiphospholipid Syndrome: Systematic Review

    OpenAIRE

    2014-01-01

    Objective. To evaluate the clinical associations between rheumatic fever and antiphospholipid syndrome and the impact of coexistence of these two diseases in an individual. Methods. Systematic review in electronics databases, regarding the period from 1983 to 2012. The keywords: “Rheumatic Fever,” “Antiphospholipid Syndrome,” and “Antiphospholipid Antibody Syndrome” are used. Results. were identified 11 cases described in the literature about the association of rheumatic fever and antiphospho...

  7. 抗磷脂综合征中抗TFPI抗体表达及临床意义%The Expression of Anti-TFPI Antibody in Antiphospholipid Syndrome and Its Clinical Signiifcance

    Institute of Scientific and Technical Information of China (English)

    孙传银; 林进

    2014-01-01

    Objective:To study the expression of anti-TFPI antibody in antiphospholipid syndrome and its clinical significance.Methods:The anticardiolipin antibody and anti-β2GPI antibody were determined with the ELISA method after collecting plasma/serum of 26 patients with primary antiphospholipid syndrome and 24 antiphospholipid antibody positive asymptomatic patients.At the same time,determined the lupus anticoagulant,recorded the patients’ clinical data,and determined anti-TFPI antibody(IgG+IgM) with the ELISA method.In addition,serum of 40 healthy people taking medical examination was collected as reference value of anti-TFPI antibody.Results:The expression rate of anti-TFPI antibody in patients with antiphospholipid syndrome was 30.77%,significantly higher than that of the normal control(P < 0.05).The incidence of thrombosis of the positive group of anti-TFPI antibody signiifcantly increased compared with that of the negative group(P < 0.05).There was no significant positive correlation between the anti-β2GPI antibody and the anti-TFPI antibody(r = 0.15).Conclusion:The higher expression of the anti-TFPI antibody in patients with antiphospholipid antibodies showed that the anti-TFPI antibodies might have some effects on thrombosis.At the same time,the antibody would help to diagnose antiphospholipid syndrome in some potential patients.%目的:研究国内抗磷脂综合征患者体内抗组织因子途径抑制物(TFPI)抗体的表达情况及其临床意义。方法:通过收集26例原发性抗磷脂综合征患者及24例抗磷脂抗体阳性无症状者的血浆/血清,ELISA法测定其抗心磷脂抗体、抗β2GPI抗体,同时测定狼疮抗凝物并记录患者的临床资料, ELISA法测定抗TFPI抗体(IgG+IgM)。另外随机收集40名健康体检者血清,作为抗TFPI抗体参考值。结果:抗磷脂综合征患者抗TFPI抗体表达率(30.77%)较正常对照组明显升高(P<0.05),抗TFPI抗体阳性组血栓发生率

  8. [Antiphospholipid syndrome diagnosis: an update].

    Science.gov (United States)

    Visseaux, Benoit; Masliah-Planchon, Julien; Fischer, Anne-Marie; Darnige, Luc

    2011-01-01

    The antiphospholipid syndrome (APS) is characterized by arterial and/or venous thrombosis and pregnancy morbidity in association with the persistent presence of autoantibodies called antiphospholipid antibodies (APAs). APAs are a heterogeneous group of circulating autoantibodies that can be detected either by phospholipid-dependent coagulation test for lupus anticoagulant (LA) or ELISA test for anticardiolipin and anti-β2GPI antibodies. In 2006, the revised criteria for the diagnosis of APS introduce the anti-β2GPI antibodies as a new biological criterion and highlight the necessity to increase the interval between two positive APA test from 6 to 12 weeks. However, despite these updated criteria, the diagnosis of APS remains challenging and we proposed here to make an overview of the latest evolution in the diagnosis of this syndrome.

  9. The Diagnosis and Treatment Progress of Pathology Pregnancy Related to Antiphospholipid Antibody%抗磷脂抗体相关病理妊娠诊治进展

    Institute of Scientific and Technical Information of China (English)

    罗相如

    2013-01-01

    抗磷脂抗体(aPL)是一组能与含有磷脂结构的抗原物质发生反应的自身抗体.通过促进血栓形成、损伤滋养细胞功能、影响免疫平衡等不同致病途径最终累及子宫及胎盘功能,导致多种病理妊娠(如复发性流产、子痫前期、胎儿生长受限、HELLP综合征等)的发生.aPL相关的病理妊娠,如复发性流产、早产、重度子痫前期等作为aPL综合征的临床诊断标准之一,对aPL阳性引起病理妊娠患者的治疗主要采用抑制血栓形成和抑制免疫反应的方法.%Anti-phospholipid antibody is a kind of autoantibodies which acts with antigens containing phospholipid structure. It affects the functions of uterus and placenta by promoting thrombosis, damaging the functions of trophoblast or impacting immunological balance,resulting in multiple pathological gestations such as recurrent abortion,preeclampsia,fetal growth restriction, and HELLP syndrome, etc.. Anti-phosphorlipid antibody relevant pathological gestation like recurrent spontaneous abortion, premature delivery, severe preeclampsia is one of the clinical diagnostic standards of antiphospholipid antibody syndrome. Inhibiting thrombosis and suppressing autoimmune reactions are the principal methods in the treatment of pathological gestations caused by anti-phosphorlipid antibody.

  10. The influence of antiphospholipid antibody with pregnancy%抗磷脂抗体对妊娠的影响

    Institute of Scientific and Technical Information of China (English)

    李萍; 朱付凡

    2007-01-01

    自身免疫性疾病或免疫功能异常影响妊娠,在自身免疫疾病中,抗磷脂综合征(antiphospholipid syndrome APS)占了一定比例.本文就抗磷脂抗体的性质进行详细阐述.血栓形成是APS的主要病理基础和突出临床表现,随着研究深入,APA对早孕期滋养细胞的影响越来越受到重视,而APA导致不良妊娠结局的作用机制,目前仍有争论.本文还介绍了APS最新的治疗进展,为今后的APS发病机制研究及临床治疗带来更多的启示.

  11. Antiphospholipid syndrome presenting as cerebral venous sinus thrombosis: a case series and a review.

    Science.gov (United States)

    Shlebak, Abdul

    2016-04-01

    The cerebral venous sinus system is a rare site for venous thrombosis except in patients with antiphospholipid syndrome. We describe three patients presenting with cerebral venous thrombosis in association with other thrombotic sites in two patients and as an only site in one patient. Antiphospholipid syndrome has varied clinical manifestations but the defining feature is the persistent presence of antiphospholipid antibodies. In this report we will review the clinical and laboratory diagnostic criteria and the management of patients with antiphospholipid syndrome.

  12. A rare combination of thrombotic thrombocytopenic purpura and antiphospholipid syndrome.

    Science.gov (United States)

    Viner, Maya; Murakhovskaya, Irina

    2016-11-24

    Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system. In the literature, there have only been nine cases of thrombotic thrombocytopenic purpura associated with primary antiphospholipid syndrome. Seven of the nine cases suffered from one or multiple strokes, a common feature in antiphospholipid syndrome, but an uncommon finding in thrombotic thrombocytopenic purpura. We introduce the possibility of an association between thrombotic thrombocytopenic purpura and the presence of antiphospholipid antibodies. Systematic testing of ADAMTS13 activity and anti-ADAMTS13 antibodies in patients who present with neurological symptoms and thrombocytopenia, in the presence of antiphospholipid antibodies, may help with the diagnosis of the rare thrombotic thrombocytopenic purpura-antiphospholipid syndrome combination.

  13. Sneddon's syndrome: case report and review of its relationship with antiphospholipid syndrome

    OpenAIRE

    2012-01-01

    The Sneddon's syndrome is a rare disorder characterized by the occurrence of cerebrovascular disease associated with livedo reticularis. The antiphospholipid syndrome is the most frequent type of acquired thrombophilia, defined by the occurrence of thrombosis or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. Approximately 80% of Sneddon's syndrome patients have an antiphospholipid antibody marker. These antibodies may play a pathogenetic role in some...

  14. Successful pregnancy outcome in grade IV lupus nephritis and secondary antiphospholipid antibody syndrome with recurrent pregnancy failures - challenging achievement of motherhood

    Directory of Open Access Journals (Sweden)

    Kaliki Hymavathi Reddy

    2016-12-01

    Full Text Available Systemic lupus erythematosus (SLE is a chronic multisystem autoimmune disease that occurs predominantly in women of childbearing age. The risk of complications and adverse fetal outcomes in pregnant women with lupus is high viz., increased risks of preterm birth, hypertensive diseases of pregnancy and lupus flares both during pregnancy and in the postpartum period. An additional association with Antiphospholipid antibody (APLA syndrome is expected to multiply the pregnancy complications. Though improved understanding of the disease nature and greater number of therapeutic options in the treatment of SLE, made the medical community regard these patients with less trepidation, the risk of significant morbidity to both the mother and the fetus still exist. We report an interesting case of grade IV Lupus nephritis (LN with secondary APLA syndrome and h/o recurrent pregnancy failures for twenty times but had a successful pregnancy and delivery in the 21st attempt though pregnancy was absolutely contraindicated in view of her medical illness. Many complications were encountered during her pregnancy which could be successfully tackled and a live male baby was delivered by Caesarean section.

  15. 'Non-criteria' aPL tests: report of a task force and preconference workshop at the 13th International Congress on Antiphospholipid Antibodies, Galveston, TX, USA, April 2010.

    Science.gov (United States)

    Bertolaccini, M L; Amengual, O; Atsumi, T; Binder, W L; de Laat, B; Forastiero, R; Kutteh, W H; Lambert, M; Matsubayashi, H; Murthy, V; Petri, M; Rand, J H; Sanmarco, M; Tebo, A E; Pierangeli, S S

    2011-02-01

    Abstract: Current classification criteria for definite APS recommend the use of one or more of three positive standardized laboratory assays, including anticardiolipin antibodies (aCL), lupus anticoagulant (LA), and antibodies directed to β(2)glycoprotein I (anti-β(2)GPI) to detect antiphospholipid antibodies (aPL) in the presence of at least one of the two major clinical manifestations (i.e., thrombosis or pregnancy morbidity) of the syndrome. Several other autoantibodies shown to be directed to phospholipids and/or their complexes with phospholipids and/or to proteins of the coagulation cascade, as well as a mechanistic test for resistance to annexin A5 anticoagulant activity, have been proposed to be relevant to APS. A task force of worldwide scientists in the field discussed and analyzed critical questions related to 'non-criteria' aPL tests in an evidence-based manner during the 13th International Congress on Antiphospholipid Antibodies (APLA 2010, 13-16 April 2010, Galveston, Texas, USA). This report summarizes the findings, conclusions, and recommendations of this task force.

  16. Obstetric antiphospholipid syndrome.

    Science.gov (United States)

    Galarza-Maldonado, Claudio; Kourilovitch, Maria R; Pérez-Fernández, Oscar M; Gaybor, Mariana; Cordero, Christian; Cabrera, Sonia; Soroka, Nikolai F

    2012-02-01

    Antiphospholipid syndrome (APS) in pregnancy has a serious impact on maternal and fetal morbidity. It causes recurrent pregnancy miscarriage and it is associated with other adverse obstetric findings like preterm delivery, intrauterine growth restriction, preeclampsia, HELLP syndrome and others. The 2006 revised criteria, which is still valid, is used for APS classification. Epidemiology of obstetric APS varies from one population group to another largely due to different inclusion criteria and lack of standardization of antibody detection methods. Treatment is still controversial. This topic should include a multidisciplinary team and should be individualized. Success here is based on strict control and monitoring throughout pregnancy and even in the preconception and postpartum periods. Further research in this field and unification of criteria are required to yield better therapeutic strategies in the future.

  17. Catastrophic primary antiphospholipid syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Dong Hun; Byun, Joo Nam [Chosun University Hospital, Gwangju (Korea, Republic of); Ryu, Sang Wan [Miraero21 Medical Center, Gwangju (Korea, Republic of)

    2006-09-15

    Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distension rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction.

  18. Microangiopatia livedóide associada à síndrome do anticorpo antifosfolípide (SAF Livedoid microangiopathy associated to antiphospholipid antibody syndrome (APS

    Directory of Open Access Journals (Sweden)

    Carla Munhoz Sanches

    2005-12-01

    argues against vasculitis, favouring a thrombotic process. Livedoid microangiopathy attacks mainly young and middle-aged women; can be idiopathic, or associated with coagulation alterations including the factor V Leiden mutation, protein C deficiency, increased plasmatic homocysteine, fibrinolysis abnormalities, platelet activation and antiphospholipid antibody syndrome (APS. We describe a case of a patient with livedoid microangiopathy associated with the presence of APS with multiple ulcers in the lower limbs who showed a clinical improvement only after total anticoagulation with warfarin and association with danazol. Livedoid vasculitis can represent an initial clinical manifestation of a group of diseases which cause occlusive vasculopathy; so, every patient should be investigated for the presence of antiphospholipid antibody or of another cause of thrombophilia.

  19. Patient-level analysis of five international cohorts further confirms the efficacy of aspirin for the primary prevention of thrombosis in patients with antiphospholipid antibodies.

    Science.gov (United States)

    Arnaud, Laurent; Mathian, Alexis; Devilliers, Hervé; Ruffatti, Amelia; Tektonidou, Maria; Forastiero, Ricardo; Pengo, Vittorio; Lambert, Marc; Lefevre, Guillaume; Martinez-Zamora, Maria Angeles; Balasch, Juan; Wahl, Denis; Amoura, Zahir

    2015-03-01

    We performed an individual patient meta-analysis to determine whether aspirin has a significant protective effect on the risk of first thrombosis among patients with antiphospholipid antibodies (aPL). Five international cohort studies with available individual patient-level data, reporting on primary prophylaxis with continuous treatment with low-dose aspirin in patients with aPL were included. The main outcome was the occurrence of a first thrombotic in patients with aPL treated with low-dose aspirin compared to those not treated with low-dose aspirin. Pooled Hazard Ratios (HRs) and 95%CIs were calculated using frailty models. We pooled data from 497 subjects and 79 first thrombotic events (3469 patient-years of follow-up). After adjustment on cardiovascular risk factors, aPL profiles, and treatment with hydroxychloroquine, the HR for the risk of a first thrombosis of any type in aPL carriers treated with low-dose aspirin versus those not treated with aspirin was 0.43 (95%CI 0.25-0.75). Subgroup analysis showed a protective effect of aspirin against arterial (HR: 0.43 [95%CI: 0.20-0.93]) but not venous (HR: 0.49 [95%CI: 0.22-1.11]) thrombosis. Subgroup analysis according to underlying disease revealed a protective effect of aspirin against arterial thrombosis for systemic lupus erythematosus (SLE) (HR: 0.43 [95%CI: 0.20-0.94]) and asymptomatic aPL carriers (HR: 0.43 [95%CI 0.20-0.93]). We found no independent protective effect of hydroxychloroquine. This individual patient data meta-analysis shows that the risk of first thrombotic event as well of first arterial thrombotic event is significantly decreased among SLE patients and asymptomatic aPL individuals treated by low-dose aspirin.

  20. Reversible cerebral vasoconstriction syndrome (RCVS) in antiphospholipid antibody syndrome (APLA): the role of centrally acting vasodilators. Case series and review of literature.

    Science.gov (United States)

    Gupta, Sarthak; Zivadinov, Robert; Ramasamy, Deepa; Ambrus, Julian L

    2014-12-01

    Reversible cerebral vasoconstriction syndrome (RCVS) is Raynaud's phenomenon of the brain. Changes in neurological function are dependent upon which areas of the brain are deprived of normal blood flow. Antiphospholipid antibody syndrome (APLA) is a common cause of Raynaud's phenomenon that can occur anywhere in the body, including the brain. Management of CNS vasospasm generally involves the use of centrally acting calcium channel blockers, which have been shown to relieve the associated headaches and transient neurological symptoms associated with it. Three patients with APLA and RCVS from our clinics are illustrated. It is demonstrated that the use of centrally acting calcium channel-blocking drugs, such as nimodipine, which prevent and reverse CNS vasospasm, led to clinical improvement in our patients over the course of 5-9 years. All of them had MRIs done at the initiation of therapy and 5-9 years after being on therapy. MRI measures of T2 lesion volumes (LVs) and number were obtained. All three patients had a good response in controlling clinical symptoms related to CNS vasospasm, Raynaud's phenomenon, visual disturbances, confusion, headaches, and hearing loss. There was also a resolution in the MRI findings of these patients. This case series of three patients shows a clinical improvement and decrease in T2 LV and number in patients with APLA and Raynaud's syndrome on centrally acting calcium channel blockers. RCVS is much more common than that currently appreciated. APLA is the common cause of RCVS. Further studies are needed to determine the optimal methods to diagnose RCVS and optimal therapies to treat it.

  1. Successful pregnancy after rituximab in a women with recurrent in vitro fertilisation failures and anti-phospholipid antibody positive.

    LENUS (Irish Health Repository)

    Ng, C T

    2012-02-01

    We report a case of successful pregnancy after rituximab in a patient with a history of in vitro fertilisation (IVF) failures and positive anti-cardiolipin antibody (ACA). Following a course of rituximab, her ACA became negative and she successfully conceived with IVF treatment. This is the first case in literature describing the use of rituximab therapy in this clinical scenario.

  2. [Multiple sclerosis associated with antiphospholipid syndrome: diagnostic and therapeutic difficulties].

    Science.gov (United States)

    Ahbeddou, N; Ait Ben Haddou, E; Hammi, S; Slimani, C; Regragui, W; Benomar, A; Yahyaoui, M

    2012-01-01

    Strokes are the main neurological manifestation of antiphospholipid syndrome. Other clinical presentations are possible and may mimic classic symptoms of multiple sclerosis (MS). A 46-year-old woman, with a history of two miscarriages, presented four subacute neurological episodes (optic neuritis, right facial paralysis, paraparesis of the thigh, and right brachial monoparesis). Using McDonald criteria, the diagnosis of multiple sclerosis was retained. Because of the occurrence of thrombocytopenia during a final relapse, we reconsidered the diagnosis of MS. Search for antiphospholipid antibodies was positive. All clinical manifestations and complementary tests were compatible with the diagnosis of antiphospholipid syndrome associated with multiple sclerosis. Given the great similarity of clinical, radiological and biological findings in the two diseases, non-thrombotic neurological manifestations of antiphospholipid syndrome can be difficult to distinguish from MS associated with antiphospholipid syndrome.

  3. Development of cerebral venous sinus thrombosis in an aplastic anemia patient with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    CHEN Jian-hua; YOU Xin; QIAN Min

    2010-01-01

    @@ Aplastic anemia (AA) is an acquired disorder in which bone marrow fails to produce or release sufficient amounts of blood cell. Antiphospholipid syndrome (APS)is an autoimmune disease characterised by recurrent arterial or venous thrombosis, pregnancy morbidity and the persistence of positive antiphospholipid antibodies (aPL), including anticardiolipin antibody (ACL) and lupus anticoagulant (LA).

  4. Successful treatment of post-exertion acute myocardial infarction by primary angioplasty and stenting in a patient with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Musuraca, Gerardo; Imperadore, Ferdinando; Terraneo, Clotilde; De Girolamo, Piergiuseppe; Cemin, Claudio; Bonmassari, Roberto; Vergara, Giuseppe

    2004-01-01

    Antiphospholipid syndrome is a disorder characterized by arterial and venous thromboses, thrombocytopaenia and stroke. Acute myocardial infarction is rarely associated with this syndrome. The treatment of these patients is a clinical challenge. This report is about a patient with antiphospholipid syndrome presenting with an acute myocardial infarction after an exercise test. The infarct-related coronary artery was successfully revascularized by primary angioplasty and stenting without any major bleeding complications. We think that the physical exertion could have favoured acute coronary thrombosis in this particular setting.

  5. Antiphospholipid syndrome: A diagnostic challenge.

    Science.gov (United States)

    Mallhi, R S; Kushwaha, Neerja; Chatterjee, T; Philip, J

    2016-12-01

    The antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilic disorder that is characterized by thrombosis (venous, arterial and microvascular) and obstetric morbidity due to a diverse family of antibodies against phospholipid-binding proteins present in plasma. The term antiphospholipid antibody is actually a misnomer as the antibodies are not against the phospholipid per se, but target the plasma protein co-factors, which bind to anionic PLs. The exact etiology has not been elucidated and is multifactorial. The initial guidelines for the diagnosis of APS were laid down in Sapporo, 1999, which were subsequently revised as the Sydney Consensus Conference criteria in 2006. Major changes were the inclusion of β2GPI as independent laboratory criteria, addition of ischemic stroke and transient cerebral ischemia as established clinical criteria and the requirement of repeating the test after 12 weeks. The laboratory tests recommended are coagulation assays, which study the effect of lupus anticoagulant on the clotting time and immunological assays, mostly ELISAs to detect IgG and IgM antibodies against cardiolipin and/or β2 glycoprotein I. For the diagnosis of APS, at least one clinical criterion and one laboratory criterion should be present. Limitations pertaining to the standardization, reproducibility and robustness of the currently recommended diagnostic tests still remain. Despite elaborate guidelines and syndrome defining criteria, the diagnosis of APS still remains a challenge. A greater interaction between the clinicians and the laboratory professionals is necessary for arriving at the correct diagnosis as a misdiagnosis of APS can have grave consequences.

  6. Antiphospholipid syndrome: A case study

    Energy Technology Data Exchange (ETDEWEB)

    Davies, T. [Royal Adelaide Hospital, Adelaide, SA (Australia). Department of Nuclear Medicine

    1998-03-01

    Full text: A forty-two-year-old male presented to the Royal Adelaide Hospital with symptoms of increasing shortness of breath, swelling in both ankles, petechial rash and blood in his sputum. Initial investigations showed cardiomegaly, right ventricular hypertrophy, patchy lung infiltrates, a platelet count of 1500 and a clotting time of 60 seconds. A V/Q scan indicated a high probability of pulmonary embolism. Further investigations showed that the patient was positive for lupus anticoagulant and cardiolipin antibodies. A diagnosis of primary antiphospholipid syndrome was made. The patient``s high risk of strokes and hemorrhaging prompted investigation by a {sup 99}mTc-HMPAO brain scan. Further V/Q scans were performed to follow up the initial finding of multiple pulmonary embolism and a R-L shunt study was performed to investigate a left subclavian murmur. The patient was admitted for four weeks and began treatment which included cyclaphosphamide, corticosteroids and plasmaphoresis and was discharged when stable. Over the next six months he was re admitted three times for relapse of antiphospholipid syndrome. On his fourth admission he collapsed and died five hours after admission. Cause of death was due to cardiac arrhythmia secondary to severe right ventricular hypertrophy and dilation. The effects of antiphospholipid syndrome was believed to be responsible for this outcome.

  7. Primary antiphospholipid syndrome presenting as renal vein thrombosis and membranous nephropathy.

    Science.gov (United States)

    Chaturvedi, Swasti; Brandao, Leonardo; Geary, Denis; Licht, Christoph

    2011-06-01

    Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by thrombotic events and the presence of circulating antiphospholipid antibodies. Large vessel involvement in the form of thrombosis/stenosis and thrombotic microangiopathy is a commonly described renal finding. However, non-thrombotic glomerulopathies are increasingly being recognized in patients with antiphospholipid syndrome. We report a rare occurrence of both renal vein thrombosis and membranous nephropathy in a previously healthy adolescent male. Investigations revealed persistently positive antiphospholipid antibodies in the absence of an underlying systemic autoimmune disorder or malignancy. Our patient responded favourably to anti-proteinuric therapy and anticoagulation with complete resolution of proteinuria and a nearly occlusive thrombus.

  8. 原发性抗磷脂抗体综合征合并肺血栓栓塞%Pulmonary thromboembolism complicating primary antiphospholipid antibody syndrome

    Institute of Scientific and Technical Information of China (English)

    高金明

    2005-01-01

    @@ 抗磷脂抗体综合征(antiphospholipid syndrome,APS)是指血清抗磷脂抗体(APL)阳性,临床上有静脉或动脉血栓形成、大于3次的习惯性流产、血小板减少中任一症状者.

  9. Cerebrospinal fluid and serum antiphospholipid antibodies in multiple sclerosis, Guillain-Barré syndrome and systemic lupus arythematosus

    Directory of Open Access Journals (Sweden)

    Paulo E. Marchiorji

    1990-12-01

    Full Text Available Immuneglobulins isotypes (IgG and IgM for myelin basic protein (MBP, cerebrosides (CER, gangliosides (GANG and cardiolipin (CARD were detected in the cerebrospinal fluid (CSF from 33 patients with multiple sclerosis (MS, 18 with Guillain-Barré syndrome (GBS and 30 with systemic lupus erythematosus (SLE. In MS patients occurred positive and significant levels of IgG-MBP in 51,5% (p<0.05 and IgM-MBP in only 18.2%, IgG-CARD in 46.2%, as long as CER and GANG were detected in almost 20%. From serum samples of MS patients 20.6% presented IgG-MBP, while 53% showed positive levels foi IgM-MBP. The CSF analysis of patients with GBS showed that 56.3% revealed IgG-MBP (p<0.05, 53% for IgM-MBP. 3&.5% for IgG-CER and 23% for IgM-CER, while 50% of patients had IgG-CARD, as long -as 31% also had IgG-GANG. The serum evaluation from 14 patients showed that 18.8% had positive concentrations of IgG-MBP and 56.3% presented IgM-MBP (p<0.05 Except for 50% of patients with SLE who presented positive CSF levels of IgG-CARD. only 24.1% had positive levels of IgG-MBP. We believe that the presence of antiphosphohoid antibodies in CSF of the above mentioned diseases occurred as immune epiphenomena, but their appearance would permit the maintenance of and perpetuate the immune event.

  10. β2糖蛋白I、抗β2糖蛋白I抗体与抗磷脂综合征%β2-glycoproteinⅠ,anti-β2-glycoproteinⅠantibody and antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    张寅; 刘湘源; 邓晓莉

    2011-01-01

    @@ β2糖蛋白I(β2-glycoprotein I,β2GP I)作为抗磷脂抗体(antiphospholipid antibody,aPL)的主要靶抗原,在抗磷脂综合征(antiphospholipid syndrome,APS)的血栓形成过程中发挥重要作用.虽然抗β2GP I抗体较抗心磷脂抗体(anticardiolipinantibody,aCL)对APS的诊断具有更高的特异性,但抗β2GP I抗体与抗心磷脂抗体联合检测更利于APS的诊断.本文对β2GP I的结构、生理功能及其与抗β2GP I抗体的结合、抗β2GP I抗体诱发血栓形成的机制等方面进行较详细的阐述.

  11. Cardiovascular Risk Factors in the Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Felipe Freire da Silva

    2014-01-01

    Full Text Available A major cause of morbidity and mortality in the context of the antiphospholipid syndrome (APS is the occurrence of thrombotic events. Besides the pathogenic roles of antiphospholipid antibodies (aPL, other risk factors and medical conditions, which are conditions for traditional risk of an individual without the APS, can coexist in this patient, raising their risk of developing thrombosis. Therefore, the clinical and laboratory investigation of comorbidities known to increase cardiovascular risk in patients with antiphospholipid antibody syndrome is crucial for the adoption of a more complete and effective treatment. Experimental models and clinical studies show evidence of association between APS and premature formation of atherosclerotic plaques. Atherosclerosis has major traditional risk factors: hypertension, diabetes mellitus, obesity, dyslipidemia, smoking, and sedentary lifestyle that may be implicated in vascular involvement in patients with APS. The influence of nontraditional risk factors as hyperhomocysteinemia, increased lipoprotein a, and anti-oxLDL in the development of thromboembolic events in APS patients has been studied in scientific literature. Metabolic syndrome with all its components also has been recently studied in antiphospholipid syndrome and is associated with arterial events.

  12. The mosaic of "seronegative" antiphospholipid syndrome.

    Science.gov (United States)

    Conti, Fabrizio; Capozzi, Antonella; Truglia, Simona; Lococo, Emanuela; Longo, Agostina; Misasi, Roberta; Alessandri, Cristiano; Valesini, Guido; Sorice, Maurizio

    2014-01-01

    In the clinical practice it is possible to find patients with clinical signs suggestive of antiphospholipid syndrome (APS), who are persistently negative for the laboratory criteria of APS, that is, anti-cardiolipin antibodies (aCL), anti-β2-GPI antibodies and lupus anticoagulant. Therefore, it was proposed for these cases the term of seronegative APS (SN-APS). In order to detect autoantibodies with different methodological approaches, sera from 24 patients with SN-APS were analysed for anti-phospholipid antibodies using TLC immunostaining, for anti-vimentin/cardiolipin antibodies by enzyme-linked immunosorbent assay (ELISA), and for anti-annexin V and anti-prothrombin antibodies by ELISA and dot blot. Control groups of our study were 25 patients with APS, 18 with systemic lupus erythematosus (SLE), and 32 healthy controls. Results revealed that 13/24 (54.2%) SN-APS sera were positive for aCL (9 of whom were also positive for lysobisphosphatidic acid) by TLC immunostaining, 11/24 (45.8%) for anti-vimentin/cardiolipin antibodies, 3/24 (12.5%) for anti-prothrombin antibodies, and 1/24 (4.2%) for anti-annexin V antibodies. These findings suggest that in sera from patients with SN-APS, antibodies may be detected using "new" antigenic targets (mainly vimentin/cardiolipin) or methodological approaches different from traditional techniques (mainly TLC immunostaining). Thus, SN-APS represents a mosaic, in which antibodies against different antigenic targets may be detected.

  13. Fitting and flailing: recognition of paediatric antiphospholipid syndrome.

    Science.gov (United States)

    Freeman, H; Patel, J; Fernandez, D; Sharples, P; Ramanan, A V

    2014-02-01

    Antiphospholipid syndrome (APS) is a systemic autoimmune condition where the presence of antiphospholipid antibodies is thought to predispose to thrombotic events. It is uncommon in the paediatric population, but current diagnostic criteria are based on adult population studies, making assessment of its true paediatric prevalence difficult. We present two cases of paediatric APS, who presented with primary neurological events, and discuss approaches to diagnosis, interpretation of screening investigations, including antinuclear antibodies (ANA), anti-extractable nuclear antigen (ENA) antibodies and lupus anticoagulant. Possible approaches to the management of paediatric APS are discussed.

  14. Cutaneous necrosis associated with the antiphospholipid syndrome and mycosis fungoides.

    Science.gov (United States)

    Hill, V A; Whittaker, S J; Hunt, B J; Liddell, K; Spittle, M F; Smith, N P

    1994-01-01

    The development of extensive cutaneous necrosis in a patient with tumour-stage mycosis fungoides is described. Skin biopsies showed a lymphomatous infiltrate, and thrombosis of dermal blood vessels. Investigation revealed the presence of anticardiolipin antibodies, a lupus anticoagulant, and low free protein S, which contributed to a prothrombotic state. Antiphospholipid antibodies have been detected in non-Hodgkin's lymphoma, but clinical manifestations are uncommon. Such autoantibodies may be produced by neoplastic lymphoid cells. The frequency with which antiphospholipid antibodies occur in mycosis fungoides is currently unknown.

  15. CARDIAC TRANSPLANTATION IN YOUNG PATIENT WITH DILATED CARDIOMYOPATHY AND SECONDARY ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    E. V. Shlyakhto

    2013-01-01

    Full Text Available Patients with congestive heart failure have an increased incidence of thromboembolic events. The choice of me- dical management in patients with antiphospholipid antibodies and generalized thrombosis due to hypercoagula- bility is complex issue. We report heart transplant outcome in 15 years old patient with dilated cardiomyopathy and secondary anti-phospholipid syndrome. 

  16. Familial antiphospholipid syndrome presenting as bivessel arterial occlusion in a 17-year-old girl.

    Science.gov (United States)

    Jelušić, Marija; Starčević, Katarina; Vidović, Mandica; Dobrota, Savko; Potočki, Kristina; Banfić, Ljiljana; Anić, Branimir

    2013-05-01

    This article presents a case of a 17-year-old girl with primary antiphospholipid syndrome developing subacute signs of hand and leg ischaemia caused by radiologically verified radial and popliteal artery occlusion. She is successfully treated with a thrombolytic agent (alteplase) and recovers completely. Her laboratory results came positive for all three subtypes of antiphospholipid antibodies. This kind of antiphospholipid syndrome presentation is a very rare entity in itself. Shortly afterwards her mother is diagnosed with primary antiphospholipid syndrome as well. A familial form of antiphospholipid syndrome is suspected. Combination of a familial antiphospholipid syndrome presenting as bivessel arterial thrombosis is a unique case, to the best of our knowledge, never described in the literature before.

  17. Catastrophic Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Rawhya R. El-Shereef

    2016-01-01

    Full Text Available This paper reports one case of successfully treated patients suffering from a rare entity, the catastrophic antiphospholipid syndrome (CAPS. Management of this patient is discussed in detail.

  18. Anticorpos antifosfolípides em 66 pacientes com infarto cerebral entre 15 e 40 anos Antiphospholipid antibodies in 66 patients with cerebral infarction between 15 and 40 years old

    Directory of Open Access Journals (Sweden)

    José Ibiapina Siqueira Neto

    1996-12-01

    Full Text Available Os anticorpos antifosfolípides (aFLs constituem grupo heterogêneo de imunoglobulinas que tem sido relacionado com alterações na coagulabilidade. Indivíduos com títulos elevados teriam maior probabilidade de desenvolver tromboses de repetição, tanto arterial como venosa, e por conseguinte infarto cerebral (IC. Os testes para detecção mais utilizados em estudos clínicos são o inibidor lúpico e a anticardiolipina. Têm-se relatado maiores percentuais de positividade nesses testes em pacientes jovens com IC. Neste estudo procuramos investigar a prevalência desses anticorpos em pacientes com IC entre 15 e 40 anos em nosso Serviço. Examinamos 66 pacientes para presença de aFLs e obtivemos 16,65% de resultados positivos. Confirmamos diagnóstico de síndrome do anticorpo antifosfolípide primária em três (4,55% casos. Concluímos que a pesquisa de rotina para aFLs em pacientes jovens com IC está indicada neste grupo de pacientes, mas correlacioná-los com o episódio isquêmico nem sempre é possível.The antiphospholipid antibodies (aPLs are a heterogenous group of immunoglobulins that have been related with alterations in blood coagulability in recent years. Patients with elevated titers of these antibodies have a high probability to develop thrombotic events, including cerebral infarct (CI. The tests currently used to detect these antibodies are the lupus anticoagulant and ELISA for anticardiolipin antibodies which have a larger proportion of positivity among young patients with CI. In our study we tested 66 patients with cerebral infarcts whose ages ranged from 15 to 40 years for the presence of lupus anticoagulant and anticardiolipin antibodies. The results showed that eleven (16.65% patients were positive for aPLs and three (4.55% of them fulfilled the diagnostic criteria for primary antiphospholipid syndrome. These data point out to the importance of investigating aPLs in young patients with CI and its high prevalence in this

  19. Anticorpos antifosfolípides em mulheres com antecedentes de perdas gestacionais: estudo caso-controle Antiphospholipid antibodies in women with recurrent pregnancy loss: a case-control study

    Directory of Open Access Journals (Sweden)

    Olívia Lúcia Nunes Costa

    2005-06-01

    Full Text Available OBJETIVO: determinar a prevalência de anticorpos antifosfolípides em mulheres com antecedentes de perdas gestacionais na população obstétrica em geral e verificar se os anticorpos antifosfolípides representam fator de risco para perdas gestacionais na população estudada. MÉTODOS: foi realizado um estudo caso-controle prospectivo com mulheres grávidas e não grávidas, atendidas numa maternidade pública entre março de 2003 e junho de 2004. As mulheres foram divididas em dois grupos de acordo com o passado obstétrico; 100 mulheres com antecedentes de perdas gestacionais de acordo com a definição estabelecida para o diagnóstico da síndrome antifosfolipídica e que não apresentassem outros fatores relacionados ao insucesso gestacional; 150 mulheres saudáveis com antecedentes de duas ou mais gestações bem sucedidas. A determinação do anticoagulante lúpico (AL foi feita mediante os testes de TTPA, dRVVT de triagem e dRVVT confirmatório. Para a pesquisa dos anticorpos anticardiolipina (aCL classes IgG e IgM foi utilizado o teste de ELISA. e os resultados semiquantitativos expressos em unidades GPL e MPL. RESULTADOS: o anticoagulante lúpico estava presente em 5% das pacientes-caso e 2% dos controles (p=0,27. Os anticorpos aCL IgG estavam presentes em 18% das pacientes-caso e 8,7% das pacientes-controle (p=0,028; OR=2,3; IC 95%=1-53. Na classe IgM, 5% de positividade para os casos e 1% para os controles (p=0,21. CONCLUSÕES: os anticorpos antifosfolípides (AL e/ou aCL IgG e/ou IgM foram mais prevalentes nas mulheres com perdas gestacionais (28% que na população obstétrica em geral (17%. As mulheres com aCL IgG têm duas vezes mais chance de ter perdas gestacionais que a população obstétrica em geral.OBJECTIVE: To determine the prevalence of antiphospholipid antibodies in women with pregnancy loss and verify if such antibodies represent a risk factor for pregnancy failure. METHODS: We performed a case-control study with

  20. Antiphospholipid Syndrome and the Kidney.

    Science.gov (United States)

    Sciascia, Savino; Baldovino, Simone; Schreiber, Karen; Solfietti, Laura; Roccatello, Dario

    2015-09-01

    The antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis, pregnancy morbidity, and the persistent presence of circulating antiphospholipid antibodies (aPL). APS has been described as primary APS when it occurs in the absence of any features of other autoimmune disease, and as secondary in the presence of other autoimmune diseases, mainly systemic lupus erythematosus (SLE). There is a well-known link between SLE and APS; 40% of SLE patients have aPL, and, in turn, some, but only a minority of patients with APS, eventually will develop features of SLE. Because SLE and APS can target the kidneys independently or at the same time, discriminating between inflammatory or thrombotic lesions is crucial in planning therapy. We provide an overview of the renal manifestations associated with the presence of aPL in patients with SLE, and discuss the impact of aPL in selected scenarios such as lupus nephritis, end-stage renal disease, and pregnancy.

  1. Emerging Therapies in Antiphospholipid Syndrome.

    Science.gov (United States)

    Andrade, Danieli; Tektonidou, Maria

    2016-04-01

    Antiphospholipid syndrome (APS) is a hypercoagulable state characterized by arterial and venous thromboses and pregnancy morbidity in the presence of antiphospholipid antibodies. Although warfarin remains the main therapeutic choice in APS, there is still concern about its efficacy, safety, and patient compliance. Patients with refractory APS to conventional therapy as well as patients with non-classical manifestations of APS may have alternative treatment approaches. APS pathogenesis has been further elucidated over the past years identifying new molecules as potential new treatment targets. This review summarizes available data from in vitro and animal models and clinical studies on the role of new potential treatment approaches including new oral anticoagulants and immunoregulatory agents: direct thrombin or factor Xa inhibitors, hydroxychloroquine, statins, B cell inhibition, complement inhibition, peptide therapy, nuclear factor κB and p38 mitogen-activated kinase inhibitors, defibrotide, abciximab, mTOR inhibitor, and other potential targets. Large multicenter prospective studies of well-characterized APS patients are needed to assess the efficacy and safety profile of these potential treatment alternatives.

  2. What have we learned about antiphospholipid syndrome from patients and antiphospholipid carrier cohorts?

    Science.gov (United States)

    Pengo, Vittorio; Banzato, Alessandra; Bison, Elisa; Bracco, Alessia; Denas, Gentian; Ruffatti, Amelia

    2012-06-01

    Venous or arterial thrombosis or pregnancy morbidity in the presence of circulating antiphospholipid antibodies (aPL) define the antiphospholipid syndrome (APS). In terms of accepted APS criteria, aPL are detected by one coagulation test (lupus anticoagulant; LAC) and two immunoassays (anticardiolipin antibodies and anti-β2-glycoptrotein I antibodies). In patients with APS, a single positive test carries a much lower risk of thrombosis recurrence or new pregnancy loss than does multiple (or triple) positivity. The same holds true for aPL carriers, namely subjects with laboratory tests but without clinical criteria for APS. Thus, very different risk categories exist among patients with APS as well as in carriers of aPL. Triple positivity apparently identifies the pathogenic autoantibody (antidomain I-II of β2-glycoptrotein I); it is in this category of patients that trials on new therapeutic strategies should focus.

  3. Management of Antiphospholipid Antibody Syndrome A Systematic Review%抗磷脂抗体综合征的诊治系统综述

    Institute of Scientific and Technical Information of China (English)

    Wendy Lim; Mark A. Crowther; John W. Eikelboom; 周佳鑫

    2007-01-01

    背景:抗磷脂抗体是指可与磷脂结合蛋白发生作用的抗体.抗磷脂抗体综合征(antiphospholipid antibody syndrome,APS)涉及抗磷脂抗体与血栓形成风险或病态妊娠之间的联系.APS患者反复发生动、静脉血栓形成或流产的危险增加.目的:在抗磷脂抗体阳性或者APS患者中,对血栓形成危险的治疗证据进行系统评估.证据收集:于MEDLINE(1966年至2005年11月)和Cochrane图书馆电子数据库(2005年)中检索相关随机试验、随机试验汇总分析以及于抗磷脂抗体阳性或APS患者中进行的血栓形成危险治疗前瞻性队列研究.对检出研究的参考文献亦予检索.研究的选择基于临床相关性.证据综合:在抗磷脂抗体阳性的患者中,既往未发生栓塞事件的健康者新发血栓的绝对危险较低(每年<1%);既往无血栓形成事件但反复流产的女性新发血栓的危险中度升高(每年10%);有静脉血栓形成史的患者在停止抗凝治疗6个月内危险最高(每年>10%).与安慰剂或未治疗对照组相比,无论抗磷脂抗体是否为阳性,中等强度华法令抗凝治疗(调整目标INR为2.0~3.0)均可使复发静脉血栓形成的危险降低80%~90%,并且对预防动脉血栓复发也有效.没有高强度华法令抗凝(目标INR>3.0)比中等强度华法令抗凝更为有效的证据.对于抗磷脂抗体阳性且既往有脑卒中的患者而言,阿斯匹林与中等强度华法令预防卒中复发的效果相同.临床试验未涉及的治疗问题或者证据不一的问题包括:预防性抗血栓治疗对无栓塞史的抗磷脂抗体阳性患者的作用,非脑血管动脉血栓形成的最佳治疗,华法令治疗后血栓复发以及抗磷脂抗体阳性并反复流产女性的治疗.结论:对于APS患者而言,中等强度华法令可有效预防静脉血栓复发,对预防动脉血栓形成也可能有效.对于有脑卒中史且抗磷脂抗体阳性的患者,阿斯匹林预防卒中

  4. Complement and thrombosis in the antiphospholipid syndrome.

    Science.gov (United States)

    Oku, Kenji; Nakamura, Hiroyuki; Kono, Michihiro; Ohmura, Kazumasa; Kato, Masaru; Bohgaki, Toshiyuki; Horita, Tetsuya; Yasuda, Shinsuke; Amengual, Olga; Atsumi, Tatsuya

    2016-10-01

    The involvement of complement activation in the pathophysiology of antiphospholipid syndrome (APS) was first reported in murine models of antiphospholipid antibody (aPL)-related pregnancy morbidities. We previously reported that complement activation is prevalent and may function as a source of procoagulant cell activation in the sera of APS patients. Recently, autoantibodies against C1q, a component of complement 1, were reported to be correlated with complement activation in systemic lupus erythematosus. These antibodies target neoepitopes of deformed C1q bound to various molecules (i.e., anionic phospholipids) and induce accelerated complement activation. We found that anti-C1q antibodies are more frequently detected in primary APS patients than in control patients and in refractory APS patients with repeated thrombotic events. The titer of anti-C1q antibodies was significantly higher in refractory APS patients than in APS patients without flare. The binding of C1q to anionic phospholipids may be associated with the surge in complement activation in patients with anti-C1q antibodies when triggered by 'second-hit' biological stressors such as infection. Such stressors will induce overexpression of anionic phospholipids, with subsequent increases in deformed C1q that is targeted by anti-C1q antibodies.

  5. Laboratory diagnosis of antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    A. Ruffatti

    2011-09-01

    Full Text Available Diagnosis of antiphosholipid syndrome (APS is based on laboratory detection of antiphospholipid (aPL antibodies in patients with documented thrombosis or in women with pregnancy morbidity. Recently, both clinical and laboratory criteria were revised on the basis of an international consensus conference held in Sydney (1. The previous international consensus statement of one clinical and one laboratory criterion to diagnose APS was maintained (2 but time-lapse between the previous thromboembolism and laboratory diagnosis should not exceed 5 years. Moreover, laboratory tests should not be performed in the 12 weeks following the event to avoid any interference of the acute phase of the disease. Thus, laboratory evaluation of venous thromboembolism (VTE should not be requested during the hospital stay as tests may be false-positive with no influence on the treatment regimen...

  6. Autonomic neuropathy-in its many guises-as the initial manifestation of the antiphospholipid syndrome.

    Science.gov (United States)

    Schofield, Jill R

    2017-01-24

    Autonomic disorders have previously been described in association with the antiphospholipid syndrome. The present study aimed to determine the clinical phenotype of patients in whom autonomic dysfunction was the initial manifestation of the antiphospholipid syndrome and to evaluate for autonomic neuropathy in these patients. This was a retrospective study of 22 patients evaluated at the University of Colorado who were found to have a disorder of the autonomic nervous system as the initial manifestation of antiphospholipid syndrome. All patients had persistent antiphospholipid antibody positivity and all patients who underwent skin biopsy were found to have reduced sweat gland nerve fiber density suggestive of an autonomic neuropathy. All patients underwent an extensive evaluation to rule out other causes for their autonomic dysfunction. Patients presented with multiple different autonomic disorders, including postural tachycardia syndrome, gastrointestinal dysmotility, and complex regional pain syndrome. Despite most having low-titer IgM antiphospholipid antibodies, 13 of the 22 patients (59%) suffered one or more thrombotic event, but pregnancy morbidity was minimal. Prothrombin-associated antibodies were helpful in confirming the diagnosis of antiphospholipid syndrome. We conclude that autonomic neuropathy may occur in association with antiphospholipid antibodies and may be the initial manifestation of the syndrome. Increased awareness of this association is important, because it is associated with a significant thrombotic risk and a high degree of disability. In addition, anecdotal experience has suggested that antithrombotic therapy and intravenous immunoglobulin therapy may result in significant clinical improvement in these patients.

  7. Using a Contradictory Approach to Treat a Wound Induced by Hematoma in a Patient With Antiphospholipid Antibody Syndrome Using Negative Pressure Wound Therapy: Lessons Learnt.

    Science.gov (United States)

    Jang, Min Young; Hong, Joon Pio; Bordianu, Anca; Suh, Hyun Suk

    2015-09-01

    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). Then as the base showed initial granulation, we changed the NPWT every 4 days. NPWT was used with lower pressure and cyclic mode (-40 to -75 mm Hg) to minimize trauma and to reduce the possibility of bleeding from the wounds. After 2 months of NPWT treatment, all the wounds eventually healed with secondary intension despite the patient's condition with diabetes, hemodialysis, anticoagulant use, and corticosteroid therapy. This report supports the idea that if accompanied by conservative debridement with meticulous bleeding control, application of NPWT in low pressures and close monitoring of the patient, NPWT is possible to use even in wounds of patients with risk for bleeding.

  8. Vasculitis in antiphospholipid syndrome.

    Science.gov (United States)

    Lally, Lindsay; Sammaritano, Lisa R

    2015-01-01

    The major manifestations of antiphospholipid syndrome (APS) are caused by thrombosis within the venous or arterial vasculature, whereas the vascular lesions in systemic vasculitis result from an inflammatory infiltrate in the vessel wall. There is an association between vascular thrombosis and inflammation, however, as vasculitis can occur in APS and thromboembolic complications are seen in systemic vasculitis. Although differentiating between vasculitis and antiphospholipid-associated thrombosis can be difficult, it may be crucial to do so given the different therapeutic implications for immunosuppression or anticoagulation. This article explores the relationship between thrombosis and inflammation as it relates to APS and systemic vasculitis.

  9. Characterization of beta2-glycoprotein I-dependent and -independent "antiphospholipid" antibodies from lupus-prone NZW/BXSB F1 hybrid male mice.

    Science.gov (United States)

    Thiagarajan, P; Le, A; Shapiro, S S

    1997-10-01

    Male (NZW x BXSB)F1 (W/BF1) mice develop a systemic lupus-like syndrome characterized by thrombocytopenia, coronary vascular disease, nephritis, and anticardiolipin antibodies. Three stable hybridoma cell lines secreting monoclonal anticardiolipin antibodies were developed from these mice by fusing their splenic lymphocytes with nonsecreting myeloma cell line, NS-1. Monoclonal antibody A1.17 reacted with cardiolipin in a beta2-Glycoprotein I-dependent manner. The epitope for this antibody consisted of beta2-glycoprotein I bound to cardiolipin or immobilized on plastic plates. Other anionic phospholipid-binding proteins, such as prothrombin or annexin V, had no significant effect in the reactivity of these antibodies. The specificity is similar to the autoimmune anticardiolipin antibodies described in patients with systemic lupus erythematosus and other infectious diseases. In contrast, monoclonal antibodies A1.72 and A1.84 reacted with cardiolipin in the absence of beta2-glycoprotein I. Beta2-glycoprotein I, either in the fluid phase or bound to cardiolipin, inhibited the binding of these antibodies. The specificity of the latter two antibodies was similar to that described in patients with syphilis and allied disorders. Both types of antibodies had lupus anticoagulant properties. Thus lupus-prone male (NZW x BXSB)F1 (W/BF1) mice develop both beta2-glycoprotein I-dependent and beta2-glycoprotein I-independent anticardiolipin antibodies.

  10. Twenty-two years of failure to set up undisputed assays to detect patients with the antiphospholipid syndrome

    NARCIS (Netherlands)

    de Groot, Philip G.; Derksen, Ronald H. W. M.; de Laat, Bas

    2008-01-01

    The antiphospholipid syndrome is defined by the persistent presence of antiphospholipid antibodies in plasma of patients with a history of thrombosis and/or pregnancy morbidity. From the definition in 1985 onwards, confusion has arisen concerning who has the syndrome and who has not. Although the cl

  11. [Antiphospholipid syndrome in nephrology. Kidney damage and practical aspects of the management].

    Science.gov (United States)

    Dekeyser, Manon; Zuily, Stéphane; Champigneulle, Jacqueline; Eschwège, Valérie; Frimat, Luc; Perret-Guillaume, Christine; Wahl, Denis

    2014-02-01

    The antiphospholipid syndrome is a thrombophilia characterized by the combination of arterial and/or venous thrombotic events or obstetric clinical events, associated with persistent presence of antiphospholipid antibodies. In this syndrome, thromboses may affect all of the vascular tree, renal damage is frequently associated with a specific antiphospholipid syndrome nephropathy. We propose in this review to provide updated recommendations on the management of antiphospholipid syndrome in nephrology. Treatment is based on long-term anticoagulant therapy with or without antiplatelet agents according to clinical events. The use of a conventional nephroprotection must not be forgotten (strict control of blood pressure with drugs blocking the renin-angiotensin-aldosterone system). Catastrophic antiphospholipid syndrome is an extremely severe complication which can threaten the vital prognosis of the patient. This justifies particular surveillance, as well as prevention in high-risk situations. We also illustrate the difficulties of long-term management in these patients, both in dialysis or kidney transplantation.

  12. Acute Unilateral Blindness from Superior Ophthalmic Vein Thrombosis: A Rare Presentation of Nephrotic Syndrome from Class IV Lupus Nephritis in the Absence of Antiphospholipid or Anticardiolipin Syndrome

    Science.gov (United States)

    Baidoun, Firas; Issa, Rommy; Al-Turk, Bashar

    2015-01-01

    Patients with systemic lupus erythematosus (SLE) are at high risk of arterial and venous thrombosis secondary to anti-phospholipid antibodies. Herein, we are presenting an interesting case of venous thrombosis in a patient with SLE in the absence of anti-phospholipid antibodies. PMID:26858847

  13. Endocardite de Libman-Sacks, anticorpos antifosfolípides e trombose arterial no lúpus ertitematoso sistêmico: relato de caso Libman-Sacks endocarditis, antiphospholipid antibodies and arterial thrombosis in systemic lupus erythematosus: case report

    Directory of Open Access Journals (Sweden)

    Raul Amorim Marques

    2010-12-01

    Full Text Available Relato de caso de paciente de 38 anos, feminina, com lúpus eritematoso sistêmico (LES que apresentou evento tromboembólico arterial agudo em membro inferior direito. A investigação evidenciou a presença de anticorpos antifosfolípides e vegetação asséptica em válvula mitral, endocardite de Libman-Sacks (eLS. São discutidas as possíveis causas de eventos tromboembólicos arteriais no LES, com ênfase nas recomendações atuais para diagnóstico e tratamento da eLSCase report of a 38-year-old female patient with systemic lupus erythematosus (SLE who presented an acute arterial thromboembolic event in the right lower limb. Investigation showed the presence of antiphospholipid antibodies and sterile vegetation in the mitral valve, Libman-Sacks endocarditis (LSE. Possible causes of thromboembolic events in SLE are discussed, with emphasis on current recommendations for diagnosis and treatment of LSE

  14. ANTIPHOSPHOLIPID SYNDROME: DIAGNOSIS AND CLINICAL MANIFESTATIONS (A LECTURE

    Directory of Open Access Journals (Sweden)

    Tat’yana M Reshetnyak

    2014-01-01

    Full Text Available The lecture provides information about the etiology and pathogenesis of antiphospholipid syndrome (APS and genetic susceptibility to its development. The most recent international diagnostic criteria for APS and its variants have been reported. This syndrome can affect multiple organ systems depending on localization of thrombosis; therefore, nowadays the problem of APS is multidisciplinary. Clinical manifestations of APS are rather general (thrombosis of different localization; thus, the diagnosis can be verified only in the case of presence of antiphospholipid antibodies. The differential diagnosis of APS is discussed.

  15. [The pathogenesis, diagnosis and treatment of antiphospholipid syndrome].

    Science.gov (United States)

    Ohmura, Kazumasa; Oku, Kenji; Atsumi, Tatsuya

    2014-07-01

    Antiphospholipid syndrome (APS) is a clinical condition characterized by recurrent thrombotic events and/or pregnancy morbidity associated with the persistence of antiphospholipid antibodies (aPLs). It is the one of the most common diseases in acquired thrombophilia. In approximately 50 % of APS patients, systemic lupus erythematosus coexist. APS is diagnosed by the 2006 Sydney revised Sapporo criteria. The pathogenic processes of APS have not been fully elucidated. Multiple mechanisms have been proposed, including interference with hemostatic reactions, activation of endothelial cells and complement activation. The treatment of APS is virtually the preventive therapy such as long-term anticoagulation and the role of corticosteroid or immunosuppressive agents is limited.

  16. The role of β2-glycoprotein I (β2GPI) carbohydrate chains in the reactivity of anti-β2GPI antibodies from patients with primary antiphospholipid syndrome and in the activation and differentiation of U937 cells.

    Science.gov (United States)

    Hernández-Ramírez, Diego F; Olivares-Martínez, Elizabeth; Núñez-Álvarez, Carlos A; Chavelas, Eneas A; García-Hernández, Enrique; Gómez-Hernández, Gregoria; Llorente, Luis; Cabral, Antonio R

    2014-10-10

    Several studies have shown that conformational changes of β(2)-glycoprotein I (β(2)GPI) when bound to negatively charged components expose cryptic epitopes and subsequent binding of anti-β(2)GPI from patients with antiphospholipid syndrome (APS). However, the role of the carbohydrate chains of β(2)GPI in this anti-β(2)GPI reactivity is poorly understood. We therefore studied the reactivity and inhibition of anti-β(2)GPI antibodies from APS patients with native, partially glycosylated β(2)GPI (pdβ(2)GPI; without sialic acid) and completely deglycosylated β(2)GPI (cdβ(2)GPI). To determine the potential biologic importance of these glycoforms and their interaction with anti-β(2)GPI in vitro, stimulation assays were performed with the U937 cell line. Circular dichroism (CD) and fluorescence analysis of the three β(2)GPI forms were also studied. We found an increased reactivity of anti-β(2)GPI against pdβ(2)GPI and cdβ(2)GPI compared to native β(2)GPI. Both deglycosylated β(2)GPI isoforms showed higher inhibition of the anti-β(2)GPI reactivity than the native protein in soluble-phase. Likewise, the antibody/β(2)GPI/glycoform complexes increased the synthesis of IL-6, IFNγ and TNFα and the expression of HLA-DR, CD14 and CD11c in U937 cells. CD and fluorescence studies of the glycoforms yielded considerable changes in the fluorescence signals. Our work suggests that the partial or complete removal of the carbohydrate chains uncover cryptic epitopes present in β(2)GPI. The differentiation and increased synthesis of pro-inflammatory cytokines by U937 cells in vitro may have pathogenetic implications.

  17. [Systemic lupus erythematosus and antiphospholipid syndrome: How to manage pregnancy?].

    Science.gov (United States)

    Guettrot-Imbert, G; Le Guern, V; Morel, N; Vauthier, D; Tsatsaris, V; Pannier, E; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Pregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

  18. Antiphospholipid syndrome in dermatology: An update

    Directory of Open Access Journals (Sweden)

    Rai Reena

    2010-01-01

    Full Text Available Antiphospholipid syndrome (APS is characterized by the presence of antiphospholipid antibodies, recurrent thrombosis, and fetal loss. Antiphospholipid antibodies are a family of autoantibodies that recognize various combinations of phospholipids, phospholipid-binding proteins, or both. APS can occur in the absence of underlying or associated disease (primary APS or in combination with other diseases (secondary APS. The exact pathogenic mechanism by which these antibodies cause thrombosis is not known; however, several hypotheses, such as activation of platelet and endothelial cells and interference with the coagulation system, have been proposed. Diagnosis is based on the presence of at least one clinical and laboratory criterion each, according to International Consensus Statement on preliminary classification criteria. However, APS can be diagnosed in individuals even in the absence of some of the classification criteria. Clinical manifestations involve different organs and systems such as the blood vessels, central nervous system, skin, kidneys, gastrointestinal tract, heart, and placenta. The unifying mechanism of all these manifestations is thrombosis, either arterial or venous. Skin manifestations are varied and although not included in the diagnostic criteria, may be the presenting feature of this syndrome. Therefore all dermatologists should investigate the possibility of APS when cutaneous findings are related to venous or arterial thrombosis. The risk of thrombosis cannot be predicted, and therefore treatment is not initiated until a thrombotic event occurs. Indefinite anticoagulation is prescribed once a thrombotic event occurs. Prognosis depends on the severity of the clinical manifestations and so, knowledge of the presentation of this disease is important for early detection and prompt treatment to prevent life-threatening consequences of this catastrophic disease process.

  19. [2016 review on catastrophic antiphospholipid syndrome].

    Science.gov (United States)

    Costedoat-Chalumeau, Nathalie; Coutte, Laetitia; Le Guern, Véronique; Morel, Nathalie; Leroux, Gaelle; Paule, Romain; Mouthon, Luc; Piette, Jean-Charles

    2016-12-01

    The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement. The vital prognosis is frequently engaged. CAPS is often precipitated by infectious diseases, surgical procedures and/or withdrawal or modification of the anticoagulation. CAPS overall mortality rate has decreased and is currently below 30%. The main differential diagnoses are other thrombotic microangiopathies, and heparin-induced thrombocytopenia. The treatment of CAPS consists of the association of anticoagulation and steroids, plus plasma exchange and/or intravenous immunoglobulins. Cyclophosphamide is added only in patients with active systemic lupus erythematosus. The potential contribution of some additional therapies (rituximab, eculizumab or sirolimus) needs to be assessed. The prevention of CAPS is essential and is based upon the adequate management of the perioperative period when surgery cannot be avoided, the prompt treatment and the prevention with immunization of infections and the education of patients with antiphospholipid syndrome, especially for the management of oral anticoagulants.

  20. Is It Antiphospholipid Syndrome?

    Directory of Open Access Journals (Sweden)

    Maria Chiara Ditto

    2010-01-01

    Full Text Available The diagnosis of bacterial endocarditis remains a challenge, as nearly half of cases develop in the absence of preexistent heart disease and known risk factors. Not infrequently, a blunted clinical course at onset can lead to erroneous diagnoses. We present the case of a 47-year-old previously healthy man in which a presumptive diagnosis of antiphospholipid syndrome was made based on the absence of echocardiographically detected heart involvement, a negative blood culture, normal C-reactive protein (CRP levels, a positive lupus anticoagulant (LAC test, and evidence of splenic infarcts. The patient eventually developed massive aortic endocarditic involvement, with blood cultures positive for Streptococcus bovis, and was referred for valvular replacement. This case not only reminds us of the diagnostic challenges of bacterial endocarditis, but also underlines the need for a critical application of antiphospholipid syndrome diagnostic criteria.

  1. Anti-beta2 glycoprotein 1 and the anti-phospholipid syndrome.

    LENUS (Irish Health Repository)

    Keane, Pearse A

    2012-02-03

    PURPOSE: To describe a patient who presented with bilateral retinal vascular occlusion and the use of anti-beta2 glycoprotein 1 (GPI) antibody testing in the diagnosis of antiphospholipid syndrome. DESIGN: Observational case report. METHODS: Hematological investigations were performed on a 49-year-old man who presented with rapid onset of bilateral severe central retinal vein occlusion. RESULTS: Lupus anticoagulant and anticardiolipin antibody testing was negative. Markedly raised titers of anti-beta2 GPI antibodies were detected on two separate occasions. CONCLUSIONS: The raised titers of anti-beta2 GPI antibodies were considered to strongly suggest an underlying diagnosis of the antiphospholipid syndrome.

  2. [Primary antiphospholipid syndrome and cerebrovascular disturbances].

    Science.gov (United States)

    Kalashnikova, L A

    2005-01-01

    Neurological, including cecbrovascular, disorders frequently emerge in primary antiphospholipid syndrome (PAS). Clinical peculiarities of PAS were studied in 113 patients with cerebrovascular disturbances. Its had mainly ischemic patogenesis. Structure of cerebrovascular disorders was as follows: stroke (33% cases), transient ischemic lesions (10%), its combination (57%), thrombosis of brain venous sinuses (3%), vascular dementia (27%). Besides it were found epileptic seizures, peripheral neuropathy, headache, chorea and some symptoms of myasthenia, parkinsonism, multiple sclerosis and psychotic disorders. In all cases antibodies to phospholipids have been detected. Secondary prophylaxis includes regular use of anticoagulants and small doses of aspiriny.

  3. Diagnosis and classification of the antiphospholipid syndrome.

    Science.gov (United States)

    Gómez-Puerta, Jose A; Cervera, Ricard

    2014-01-01

    The antiphospholipid syndrome (APS) is defined by the occurrence of venous and arterial thromboses, often multiple, and recurrent fetal losses, frequently accompanied by a moderate thrombocytopenia, in the presence of antiphospholipid antibodies (aPL). Some estimates indicate that the incidence of the APS is around 5 new cases per 100,000 persons per year and the prevalence around 40-50 cases per 100,000 persons. The aPL are positive in approximately 13% of patients with stroke, 11% with myocardial infarction, 9.5% of patients with deep vein thrombosis and 6% of patients with pregnancy morbidity. The original classification criteria for the APS were formulated at a workshop in Sapporo, Japan, in 1998, during the 8th International Congress on aPL. The Sapporo criteria, as they are often called, were revised at another workshop in Sydney, Australia, in 2004, during the 11th International Congress on aPL. At least one clinical (vascular thrombosis or pregnancy morbidity) and one laboratory (anticardiolipin antibodies, lupus anticoagulant or anti-β2-glycoprotein I antibodies) criterion had to be met for the classification of APS.

  4. Antiphospholipid Syndrome--Not a Noninflammatory Disease.

    Science.gov (United States)

    de Groot, Philip G; Urbanus, Rolf T

    2015-09-01

    The autoimmune disease antiphospholipid syndrome (APS) is characterized by thrombosis or pregnancy morbidity in patients with persistent antiphospholipid antibodies (aPLs). Although inflammation is not a key feature of the clinical presentation of the syndrome, there are indications that the inflammatory response plays an important role in APS. The major antigen of aPLs, the plasma protein β2-glycoprotein I, is involved in clearance of microparticles and in the innate immune response. In light of these physiological functions, the formation of antibodies against the protein is easily understood, as antibodies might augment the clearance reaction. In addition, inflammatory mediators are thought to play a role in the activation of leukocytes and the induction of endothelial dysfunction in APS. Moreover, evidence for a role of complement activation in the pathogenesis of the syndrome is accumulating. This review will provide an overview of current knowledge on the physiological function of β2-glycoprotein I, the formation of autoantibodies against β2-glycoprotein I and will explore the contribution of inflammation to the clinical manifestations of APS.

  5. Recent advances in understanding antiphospholipid syndrome

    Science.gov (United States)

    Bertolaccini, Maria Laura; Sanna, Giovanni

    2016-01-01

    Antiphospholipid syndrome (APS), also known as Hughes Syndrome, is a systemic autoimmune disease characterized by thrombosis and/or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. A patient with APS must meet at least one of two clinical criteria (vascular thrombosis or complications of pregnancy) and at least one of two laboratory criteria including the persistent presence of lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and/or anti-b2 glycoprotein I (anti-b2GPI) antibodies of IgG or IgM isotype at medium to high titres in patient’s plasma. However, several other autoantibodies targeting other coagulation cascade proteins (i.e. prothrombin) or their complex with phospholipids (i.e. phosphatidylserine/prothrombin complex), or to some domains of β2GPI, have been proposed to be also relevant to APS. In fact, the value of testing for new aPL specificities in the identification of APS in thrombosis and/or pregnancy morbidity patients is currently being investigated. PMID:28105326

  6. Recent advances in understanding antiphospholipid syndrome.

    Science.gov (United States)

    Bertolaccini, Maria Laura; Sanna, Giovanni

    2016-01-01

    Antiphospholipid syndrome (APS), also known as Hughes Syndrome, is a systemic autoimmune disease characterized by thrombosis and/or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. A patient with APS must meet at least one of two clinical criteria (vascular thrombosis or complications of pregnancy) and at least one of two laboratory criteria including the persistent presence of lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and/or anti-b2 glycoprotein I (anti-b2GPI) antibodies of IgG or IgM isotype at medium to high titres in patient's plasma. However, several other autoantibodies targeting other coagulation cascade proteins (i.e. prothrombin) or their complex with phospholipids (i.e. phosphatidylserine/prothrombin complex), or to some domains of β2GPI, have been proposed to be also relevant to APS. In fact, the value of testing for new aPL specificities in the identification of APS in thrombosis and/or pregnancy morbidity patients is currently being investigated.

  7. Antiphospholipid syndrome: a clinical and laboratorial challenge

    Directory of Open Access Journals (Sweden)

    Luci Maria Santana Dusse

    2014-01-01

    Full Text Available Antiphospholipid syndrome (APS is an acquired autoimmune thrombophilia characterized by the presence of a heterogeneous family of antibodies that bind to plasma proteins with affinity for phospholipid surfaces. The two major protein targets of antiphospholipid antibodies are prothrombin and β2-glycoprotein I (β2GPI. APS leads to aprothrombotic state, and it is characterized by the occurrence of arterial, venous or microvascular thrombosis or recurrent fetal loss. The diagnosis of APS is based on a set of clinical criteria and the detection of lupus anticoagulant (LA, anticardiolipin antibodies (ACA or anti-β2GPI in plasma. Although laboratory tests are essential for APS diagnosis, these tests have limitations associated with the robustness, reproducibility and standardization. The standardization of diagnostic tests for detection of APLAs has been a challenge and a variety of results have been obtained using different commercial kits and in-house techniques. An increased sensitivity of the ELISA kits for detection of ACA effectively has contributed to APS diagnosis. However, the lack of specificity associated with a high number of false-positive results is a clinical and laboratorial challenge, since such results may lead to mistaken clinical decisions, such as prescription of oral anticoagulant, leading to the risk of hemorrhaging. Furthermore, clinicians are often unfamiliar with these tests and have difficulty interpreting them, requiring interaction between clinical and laboratory professionals in order to ensure their correct interpretation.

  8. Hemolysis, hemorrhage, headache, and hidden abortion: imaging findings in antiphospholipid syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Mahnken, A.H.; Brandenburg, V.M.; Haage, P.; Guenther, R.W. [Dept. of Diagnostic Radiology, Univ. Hospital, Univ. of Technology, Aachen (Germany); Frank, R.D. [Dept. of Nephrology and Immunology, Univ. of Technology, Aachen (Germany)

    2003-12-01

    Antiphospholipid antibodies are associated with arterial and venous thromboses, recurrent pregnancy loss, and organ infarction. Any vascular region can be affected. We present a 20-year-old woman suffering from secondary antiphospholipid syndrome with a unique combination of multifocal venous thromboses, pulmonary embolism, spontaneous abortion, and splenic infarction. Diversity of clinical symptoms and diagnostic imaging modalities are discussed with emphasis on cross-sectional imaging. The syndrome should be suspected in patients with thromboses and organ infarctions of otherwise undetermined etiology. (orig.)

  9. 抗磷脂抗体综合征与早发型子痫前期的相关性%Correlation between antiphospholipid antibody syndrome and the early onset of preeclampsia

    Institute of Scientific and Technical Information of China (English)

    陈先侠; 唐志霞; 孟祥莲; 董革; 张俊强; 陈晓宇

    2015-01-01

    Objective To investigate the correlation between antiphospholipid antibody syndrome and the early onset of preeclampsia. Methods From May 2010 to July 2013, one hundred and threecases in-patient treatment of the early onset preeclampsia were enrolled in this study. The maternal serum anticardiolipin antibodies(ACA)and anti-β2-glycoproteinⅠantibody (Aβ2-GPⅠ) were detected by ELISA method. 58 cases of pregnant women were randomly divided into the routine treatment group (30 cases) and the anticoagulant therapy group (28 cases). Results ACA positive predictive value of the early onset preeclampsia value was 3.9%. No significant difference was found in the prolonged anticoagulation of early onset preeclampsia time between the control group and the treatment group. Conclusion ACA may not be used to predict the early onset preeclampsia. Anticoagulation therapy can′t extend the early onset preeclampsia time and improve the outcome of pregnancy.%目的:探讨抗磷脂抗体综合征与早发型子痫前期的相关性。方法:选取本院2010年5月至2013年7月住院治疗的103例早发型子痫前期孕妇为研究对象,酶联免疫吸附法(ELISA)检测抗心磷脂抗体(anticardiolipin antibodies, ACA)和抗β2-糖蛋白抗体(anti-β2-glycoproteinⅠantibody, Aβ2-GPⅠ),计算阳性预测值,58例期待治疗孕妇随机分为常规治疗组(30例)和增加抗凝治疗组(28例)。结果:ACA预测早发型子痫前期的阳性预测值为3.9%,两组相比,抗凝治疗组在延长早发型子痫前期期待治疗时间上差异无显著性(P >0.05)。结论:尚不能认为ACA与早发型子痫前期具有相关性,且抗凝治疗不能延长早发型子痫前期期待治疗时间,不能明显改善妊娠结局。

  10. Trombose da artéria renal e síndrome do anticorpo antifosfolípide: um relato de caso Renal arterial thrombosis and the antiphospholipid antibody syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Célia S. Macedo

    2001-12-01

    a history of abdominal pain, pallor, lethargy, and anuria for 36 hours. On physical examination, the patient showed malnutrition, high blood pressure, moderate edema, and hypochondrial pain. Laboratory findings included: urea=112mg/dl, serum creatinine= 4.5 mg/dl, blood pH= 7.47, blood bicarbonate= 12.8 mmol/L, K=7.2 mEq/L. Peritoneal dialysis was started and maintained for 11 days. After 7 weeks, the patient still needed anti-hypertensive drugs and the renal function was still abnormal. Renal biopsy was performed and revealed renal infarction. The result of Doppler ultrasonography revealed absent renal blood flow on the right side. Renal arteriography showed total occlusion of the right renal artery. Results for collagen diseases were negative. A right nephrectomy was performed and the blood pressure was controlled. The child was hospitalized again at 5 years and 8 months old with episodes of absence seizures and abdominal and precordial pain. Anticardiolipin antibody test was positive. The child is now 7 years old, asymptomatic, with negative anticardiolipin antibody, and has been under regular follow-up. COMMENTS: children with arterial thrombosis should be investigated for a possible association with the antiphospholipid antibody syndrome even in the absence of collagen disease.

  11. Frequency of antiphospholipid antibodies in patients with infectious diseases using three different ELISA methods Freqüência de anticorpos antifosfolípides em pacientes com doenças infecciosas usando três diferentes testes de ELISA

    Directory of Open Access Journals (Sweden)

    Mittermayer Barreto Santiago

    2006-02-01

    Full Text Available OBJECTIVE: The standard enzyme-linked immunosorbent assay (ELISA for anticardiolipin (aCL antibodies is the most important test for the diagnosis of antiphospholipid syndrome (APS. However, the test is also positive in some infectious diseases and other non-related syndromes. It has been suggested that the detection of antibodies to a mixture of phospholipids or to beta2-glycoprotein I (beta2-GP I has higher specificity for APS than the standard aCL ELISA. The aim of the present work is to compare the diagnostic specificity of three different antiphospholipid (aPL assays in patients with infectious diseases. METHODS: Antiphospholipid antibodies were searched by three ELISA techniques, namely standard aCL, APhL® ELISA kit and anti-beta2-GP I, in sera of patients with infectious diseases, including syphilis (69, leptospirosis (33 and visceral leishmaniasis (30. RESULTS: The frequency of positivity of IgG aPL in patients with syphilis, leptospirosis and Kala-azar was 13/69 (19%, 9/33 (27% and 2/30 (6%, respectively, using standard ELISA, versus only 1/69 (1.4%, 0/33 (0% and 0/30 (0% positivity by the APhL® ELISA kit. The positivity of the isotype IgM aPL was 10/69 (14%, 4/33 (12% and 1/30 (3%, respectively, by the standard ELISA, and 1/69 (1.4%, 0/33 (0% and 0/30 (0% by the APhL® ELISA kit. The presence of significant levels of IgG anti-beta2GPI was observed in 14/69 cases of syphilis (20%, 6/33 cases of leptospirosis (18% and 16/30 cases of Kala-azar (53%. The APhL® ELISA kit had superior performance showing the highest specificity: 97% (95% CI: 92%-99% for IgG compared to 81% (95% CI: 74%-87% for standard ELISA and 72% (95% CI: 64%-79% for anti-beta2 GPI assay. CONCLUSIONS: The APhL® ELISA kit proved to be significantly more specific than the aCL standard ELISA and the anti-beta2GPI ELISA, and it should be used to help in the diagnosis and confirmation of APS.OBJETIVO: O ensaio de enzyme-linked immunosorbent assay (ELISA para a pesquisa de

  12. The role of anti-annexin A2 antibodies in antiphospholipid syndrome%抗膜联蛋白A2抗体在抗磷脂综合征中的作用

    Institute of Scientific and Technical Information of China (English)

    敖文; 郑辉; 陈晓微; 申艳; 杨程德

    2009-01-01

    Objective This study has explored the role of antibody against annexin A2 in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Methods Using purified recombinant annexin A2, IgG anti-annexin A2 antibody was measured by ELISA in 101 APS patients, 41 SLE patients with thrombosis, 124 SLE patients without thrombosis and 120 healthy controls. Results The positive rate of IgG anti-annexin A2 antibody in APS patients and SLE patients with thrombosis was 21.8%, 26.8%, respectively, they were all significantly higher than in SLE patients without thrombosis (6.5%). IgG anti-annexin A2 antibody was associated with thrombosis and/or pregnancy morbidity (P<0.01). Conclusion Anti-annexin A2 antibody is associated with thrombosis and/or pregnancy mnrbidity. It suggests that anti-annexin A2 antibody may be helpful in identifying in some potential AIRS.%目的 研究抗膜联蛋白A2抗体在抗磷脂综合征(APS)、系统性红斑狼疮(SLE)的血栓/病态妊娠中的可能作用.方法 先用分子克隆方法表达纯化出重组膜联蛋白A2,然后以重组膜联蛋白A2为抗原,采用酶联免疫吸附试验(ELISA)法分别检测了,101例APS患者,41例SLE合并血栓患者,124例无血栓的SLE患者及120名健康人的血清中IgG型抗膜联蛋白A2抗体水平.结果 APS组和SLE合并血栓组的IgC型抗膜联蛋白A2抗体阳性率分别为21.8%,26.8%,均品著高于单纯SLE组(6.5%)(P值均<0.0.).IgG型抗膜联蛋白A2抗体与血栓/病态妊娠有关联(P<0.01).IgG型抗膜联蛋白A2抗体对血栓/病态妊娠诊断的敏感性、特异性、预测值分别为0.232、0.935、0.805.结论 IgG型抗膜联蛋白A2抗体与APS和SLE患者的血栓/病态妊娠表现相关,将有助于一些潜在的APS患者的诊断.

  13. 抗磷脂抗体对妊娠丢失筛查的临床价值%Clinical value of antiphospholipid antibody in the screening for pregnancy loss

    Institute of Scientific and Technical Information of China (English)

    张浩如

    2013-01-01

    Objective To investigate the relationship between history of pregnancy loss and antiphospholipid antibodies (APA),including anticardiolipin (ACA) and lupus anti-coagulant antibodies (LA).Methods One hundred and fifty patients with history of unexplained pregnancy loss as study group and 120 normal nonpregnant women as control group.The study group was further divided into three subgroups:embryo growth arrest (n =36),stillbirth (n =44),and recurrent abortion (n =70)levels of serum APA(including ACA and LA) were determined repectively and analyzed.Results The positive rates of APA,ACA,LA in the study group were significantly higher than those in control group (P < 0.05).The above significance was true in both stillbirth and recurrent abortion groups but not in embryo growth arrest group.Conclusions Levels of serum APA are associated with pregnancy loss,especially about recurrent abortion and stillbirth.We suggest routine screening of serum APA should be performed in patients with history of fetal wastage for the sake of early treatment.%目的 探讨妊娠丢失与抗磷脂抗体(APA)[包括抗心磷脂抗体(ACA)和狼疮抗凝抗体(LA)]的关系.方法 观察组为150例有妊娠丢失史的患者,其中分为胚胎停育组(36例)、死胎组(44例)和复发性流产组(70例).对照组为同期120例正常孕妇,分别测定观察组和对照组静脉血清APA水平,并进行对比分析.结果 整个观察组APA、ACA、LA的阳性率均高于对照组,差异有统计学意义(P<0.05).胚胎停育组与对照组比较,差异无统计学意义(P>0.05).死胎组和复发性流产组的APA、ACA、LA阳性率分别与对照组比较,差异均有统计学意义(P<0.05).结论 APA与妊娠丢失有关,尤其对于复发性流产和死胎者.因此,对有不良孕产史的患者常规筛查APA,有利于尽早对因治疗.

  14. Antiphospholipid syndrome: analysis of dilute Russell's viper venom time titer.

    Science.gov (United States)

    Martinez, Alan P; Cunningham, Mark T

    2016-07-01

    To evaluate the characteristic features of the dilute Russell's viper venom time (DRVVT) titer in the antiphospholipid syndrome (APS). The medical record of 3660 consecutive patients with DRVVT orders between 2006 and 2015 were examined for criteria satisfying the diagnosis of APS. DRVVT titer was studied as a function of titer distribution, titer stability, and clinicopathologic features. Twenty-six patients were diagnosed with APS based on a persistently positive DRVVT and a history of arterial or venous thrombosis. DRVVT titer was mostly of low magnitude (65-77% of patients), was of similar value between initial and repeat testing (mean DRVVT titer 1.40 vs. 1.38; P = 0.858; mean time interval 216 days), and was positively associated with anticardiolipin antibodies (IgG and IgM) and antibeta-2-glycoprotein I antibodies (IgG and IgM) (P antiphospholipid antibody profile in 0 and 62% of patients, respectively (P antiphospholipid antibody profiles.

  15. Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management.

    Science.gov (United States)

    Pinto-Almeida, Teresa; Caetano, Mónica; Sanches, Madalena; Selores, Manuela

    2013-01-01

    Antiphospholipid syndrome is a relatively recent systemic autoimmune disorder defined by thrombotic events and/or obstetric complications in the presence of persistent elevated antiphospholipid antibodies. It\\'s characterized by a wide spectrum of clinical presentations and virtually any organ system or tissue may be affected by the consequences of vascular occlusion. Diagnosis is sometimes difficult and although classification criteria have been published and revised there remain ongoing issues regarding nomenclature, expanding clinical features, laboratory tests and management and much still has to be done. Cutaneous manifestations are common and frequently the first sign of the disease. Although extremely diverse it\\'s important to know which dermatological findings should prompt consideration of antiphospholipid syndrome and the appropriate management for those patients. Much has been debated about when to consider antiphospholipid syndrome and consensus still does not exist, however in spite of being a diagnostic challenge clinicians should know when to look for antiphospholipid antibodies since an early diagnosis is important to prevent further and serious complications. In this article we focus on the cutaneous features that should raise suspicion on the presence of antiphospholipid syndrome and on the complex management of such patients. Many other dermatological signs related to this syndrome have been described in the literature but only occasionally and without consistency or statistic impact and therefore will not be considered here.

  16. Complete resolution of a mitral valve vegetation with anticoagulation in seronegative antiphospholipid syndrome.

    Science.gov (United States)

    Ruan, Yuheng; Bridges, Jonathan S; Kumar, Kapil; Raphael, Jonelle A; Acharjee, Subroto; Welty, Francine K

    2008-12-01

    Antiphospholipid syndrome (APS) is a disorder characterized by recurrent venous or arterial thrombosis and/or fetal loss; involvement of cardiac valves is also seen. A seronegative variant has been described previously. We report a case of a woman with recurrent pregnancy loss, prior strokes, and a negative workup for known antiphospholipid antibodies. During her current pregnancy, she presented with acute stroke and mitral valve vegetation. Her workup for antiphospholipid syndrome and other thrombophilias remained negative even after the stroke. Her mitral valve vegetation resolved completely with aspirin, heparin, and warfarin. We believe this to be the first report of complete resolution of valvular vegetation with antiplatelet and anticoagulant therapy alone in a patient with seronegative antiphospholipid syndrome. Moreover, this appears to be the first report of stroke associated with this condition.

  17. Rheumatic Fever Associated with Antiphospholipid Syndrome: Systematic Review

    Directory of Open Access Journals (Sweden)

    Felipe da Silva

    2014-01-01

    Full Text Available Objective. To evaluate the clinical associations between rheumatic fever and antiphospholipid syndrome and the impact of coexistence of these two diseases in an individual. Methods. Systematic review in electronics databases, regarding the period from 1983 to 2012. The keywords: “Rheumatic Fever,” “Antiphospholipid Syndrome,” and “Antiphospholipid Antibody Syndrome” are used. Results. were identified 11 cases described in the literature about the association of rheumatic fever and antiphospholipid syndrome. Clinical presentation of rheumatic fever was characterized by the predominance of carditis (11/11 and chorea (7/11. Regarding the manifestations of APS, the stroke was observed in 7/11 (63.6%, with one of them having probable embolic origin. Conclusion. The present study brings the information that the association between APS and RF is quite rare, however, is of great clinical importance. Doctors who deal with the RF should include in their differential diagnosis the APS, especially in the presence of stroke in patients with RF and whose echocardiogram does not show intracavitary thrombi.

  18. Antiphospholipid Syndrome and Vascular Ischemic (Occlusive) Diseases: An Overview

    OpenAIRE

    2007-01-01

    Antiphospholipid syndrome (APS) is primarily considered to be an autoimmune pathological condition that is also referred to as "Hughes syndrome". It is characterized by arterial and/or venous thrombosis and pregnancy pathologies in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disease or secondary to a connective tissue disorder, most frequently systemic lupus erythematosus (SLE). Damage to the nervous system is one of the most promin...

  19. Determinación de anticuerpos anti-β2glicoproteína I en pacientes con síndrome antifosfolípido Anti- β2 glycoprotein antibodies in patients with antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Oscar Uribe Uribe

    2004-09-01

    and with the clinical manifestations of the Antiphospholipid Syndrome (APS. In this study 80 women with APS (35 from the Rheumatology Service and 45 with a history of recurrent spontaneous abortion, RSA were included, as well as 5 women with rheumatic diseases but no APS, 27 RSA-women without APS and 20 healthy women in their reproductive age. The presence of IgG and IgM anticardiolipin antibodies (aCL, anti- β2GPI antibodies by ELISA method and lupus anticoagulant by the test of activated partial thromboplastin time was investigated. Additionally the clinical manifestations associated to APS were registered. In the group of women with APS, 25.7% (9/35 of those with rheumatic diseases and 4.4% /2/45 of the ones with RSA were positive for anti- β2GPI while none of the women without APS or the controls had such positive reaction. There was a significant association at titers of 3+ (highly positive between the presence of anti- β2GPI antibodies and IgG and IgM aCL in contrast to anti- β2GPI-negative individu als. The positivity of lupus anticoagulant also correlated with the presence of anti- β2GPI antibodies. There was no significant correlation between any specific clinical manifestation and the presence of anti- β2GPI antibodies. In conclusion, the determination of anti- β2GPI antibodies was highly specific in patients with APS but did not associate with any clinical manifestation of the syndrome.

  20. Síndrome antifosfolípide Antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Jesus Rodriguez Santamaria

    2005-06-01

    Full Text Available Condição adquirida, sistêmica, caracterizada por tromboses recorrentes no sistema arterial, venoso ou ambos, a síndrome antifosfolípide pode ser primária ou secundária, esta última mais associada ao lúpus eritematoso sistêmico e menos freqüentemente a infecções, fármacos e outras doenças. São marcadores sorológicos da síndrome antifosfolípide os anticorpos antifosfolípides anticoagulante lúpico e anticardiolipina. O critério diagnóstico primário inclui trombose arterial ou venosa e morte fetal recorrente. Cerca de 41% dos pacientes apresentam lesões cutâneas como primeiro sinal da síndrome, que também pode provocar livedo reticular, ulcerações cutâneas, vasculite livedóide, entre outras manifestações. Seu controle consiste principalmente no tratamento e profilaxia da trombose com anticoagulantes e antiagregantes plaquetários.Antiphospholipid syndrome is an acquired multisystem disorder characterized by recurrent thromboses in the arterial system, venous system, or both. Antiphospholipid syndrome is classified into 2 groups: primary and secondary. Secondary antiphospholipid syndrome is often associated with systemic lupus erythematosus and less frequently with infections, drugs and other diseases. Serologic markers are antiphospholipid antibodies, lupus anticoagulant and anticardiolipin. The primary diagnostic criteria include arterial thrombosis or venous thrombosis and recurrent fetal loss. About 41% of patients with lupus anticoagulant have skin lesions as the first sign of antiphospholipid syndrome. Cutaneous manifestations include livedo reticularis, cutaneous ulceration and livedo vasculitis. The mainstays of prophylaxis and treatment of thrombosis are anticoagulant and antiplatelet agents.

  1. 抗磷脂综合征1例报告%Antiphospholipid syndrome: one case report

    Institute of Scientific and Technical Information of China (English)

    范琰; 刘梅林

    2011-01-01

    @@ 抗磷脂综合征(antiphospholipid syndrome,APS)是由抗磷脂抗体(antiphospholipid antibody,APL)(包括狼疮抗凝物和抗心磷脂抗体)引起的自身免疫性疾病,临床上以血栓形成、习惯性流产及血小板减少为主要表现.

  2. A Case of Antiphospholipid Syndrome Refractory to Secondary Anticoagulating Prophylaxis after Deep Vein Thrombosis-Pulmonary Embolism

    OpenAIRE

    Gu, Kang Mo; Shin,Jong Wook; Park, In Won

    2014-01-01

    Antiphospholipid syndrome (APS) is an acquired systemic autoimmune disorder characterized by a combination of clinical criteria, including vascular thrombosis or pregnancy morbidity and elevated antiphospholipid antibody titers. It is one of the causes of deep vein thrombosis and pulmonary embolism that can be critical due to the mortality risk. Overall recurrence of thromboembolism is very low with adequate anticoagulation prophylaxis. The most effective treatment to prevent recurrent thromb...

  3. PREVENTION OF THROMBOSES IN ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    Lyubov Valeryevna Kondratyeva

    2009-01-01

    Patients with antiphospholipid (aPL antibodies and venous thromboses need long-term moderate-intensity warfarin therapy. Patients with ischemic strokes without other indications for the use of anticoagulants may be given either warfarin or ASA. In the latter case, there is no need for laboratory control or an individual dose adjustment. The primary prevention of thromboses in the presence of aPL is also performed with ASA. When pregnancy occurs, women with obstetric manifestations of APS may be given small-dose ASA in combination with heparins. To reduce the risk of hemorrhages, warfarin dosage adjustment is initiated with the minimum doses (<5 mg/day. Novel ASA formulations, such as ASA with the unabsorbed antacid magnesium hydroxide, have been developed to prevent gastrointestinal tract complications.

  4. Non-stroke Central Neurologic Manifestations in Antiphospholipid Syndrome.

    Science.gov (United States)

    Yelnik, Cécile M; Kozora, Elizabeth; Appenzeller, Simone

    2016-02-01

    Thrombotic manifestations of antiphospholipid syndrome (APS) are well known, and various non-stroke neuro-psychiatric manifestations (NPMs) have also been consistently described, but their place in APS remains unclear. Some syndromes, such as migraine or cognitive dysfunction, are frequently described in APS, whereas others, like seizure, multiple sclerosis-like symptoms, transverse myelitis, movement disorders, or psychiatric symptoms, are rarely found. Overlap with other autoimmune diseases, in particular with systemic lupus erythematosus, the lack of large sample size prospective studies, and discrepancies in antiphospholipid antibody (aPL) determinations complicate the study of the relationship between those disorders and aPL/APS. This review article aimed to summarize updated data on pathophysiologic, epidemiologic, and radiologic findings about non-stroke NPM described in primary APS and aPL-positive patients without overlap of other autoimmune diseases.

  5. Takayasu's arteritis and primary antiphospholipid syndrome presenting as hypertensive urgency.

    Science.gov (United States)

    Yang, Andrew; Nayeemuddin, Mohammed; Prasad, Bhanu

    2016-01-18

    A 33-year-old Caucasian man was admitted to the hospital with chest pain and hypertensive urgency. Physical examination revealed widespread arterial bruits and marked difference in blood pressure between the upper limbs. Vascular imaging showed widespread narrowing in multiple vascular territories. He met the established American College of Rheumatology criteria for Takayasu's arteritis. His resistant hypertension was considered to be a consequence of bilateral renal artery stenosis and he subsequently underwent sequential stenting of his renal arteries leading to improvement in blood pressure and reduction in the number of antihypertensive medications. Subsequent imaging revealed progression of aortic thrombus in the setting of an elevated erythrocyte sedimentation rate, and persistently elevated antiphospholipid antibodies fulfilling diagnostic criteria for primary antiphospholipid syndrome, requiring initiation of immunosuppression and anticoagulation.

  6. The role of infectious diseases in the catastrophic antiphospholipid syndrome.

    Science.gov (United States)

    Garcia-Carrasco, M; Mendoza-Pinto, C; Macias-Diaz, S; Vazquez de Lara, F; Etchegaray-Morales, I; Galvez-Romero, J L; Mendez-Martinez, S; Cervera, R

    2015-11-01

    Catastrophic antiphospholipid syndrome (CAPS), also called "Asherson syndrome", is a variant of the antiphospholipid syndrome (APS) that occurs in less than 1% of APS cases. The etiology of CAPS is uncertain; however, several triggering factors have been recognized. The most common of these are infectious diseases, particularly those of the respiratory tract. CAPS pathogenesis is incompletely understood, but several theories have been proposed, such as the molecular mimicry theory, which describes the production of anti-β2-glycoprotein I (GP1) antibody in response to infection. The process is complex and involves the activation of Toll-like receptor 4 (TLR-4), which triggers a cytokine storm, followed by endothelial alterations that induce a procoagulant state.

  7. Antiphospholipid syndrome: 30 years and our contribution.

    Science.gov (United States)

    Koike, Takao

    2015-02-01

    In 1983, Graham Hughes first described the concept of antiphospholipid syndrome (APS). In 1984, we described the enzyme-linked immunosorbent assay (ELISA) system which directly detected circulating aCL in patients with systemic lupus erythematosus (SLE) who revealed biological false positive serological test for syphilis. In 1990, three groups, including our group, independently reported the necessity of a cofactor for the binding of autoimmune anticardiolipin antibodies (aCL) to the solid phase phospholipids. β2-glycoprotein I (β2GPI) was identified as this cofactor. In 1994,the epitope for aCL was shown to develop when β2GPI is adsorbed on polyoxygenated polystyrene plates. In 2000, we described antiprothrombin antibodies bind to prothrombin exposed to immobilized phosphatidylserine and established a phosphatidylserine dependent monoclonal antiprothrombin antibody. In 2004, a novel role of nicked β2GPI was identified in the negative feedback pathway of extrinsic fibrinolysis. Nicked β2GPI was found to bind angiostatin 4.5 and to attenuate its antiangiogenic property. In 2004, we demonstrated that the p38 MAPK pathway mediates induction of the TF gene in stimulated with human monoclonal anti- β2GPI antibodies. Very recently, β2GPI was identified as a complement regulator. The cross-link between complement activation and prothrombotic status in patients with APS has been drawn much attention. Genetic factors are hypothesized to play a role in the susceptibility to APS based on several family studies in patients with antiphospholipid antibodies (aPL) and/or clinical manifestations of APS. The genetics of β2GPI has been extensively studied. 247 Val/Leu polymorphism can affect the conformational change of β2-GPI and the exposure of the epitopes for aCL. We found that 247 Val was correlated with anti-β2-GPI production in patients with primary APS, and 247 Val may be important for β2-GPI antigenicity. STAT4 SNP in Japanese patients with SLE and/or APS. T

  8. The Mosaic of “Seronegative” Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Fabrizio Conti

    2014-01-01

    Full Text Available In the clinical practice it is possible to find patients with clinical signs suggestive of antiphospholipid syndrome (APS, who are persistently negative for the laboratory criteria of APS, that is, anti-cardiolipin antibodies (aCL, anti-β2-GPI antibodies and lupus anticoagulant. Therefore, it was proposed for these cases the term of seronegative APS (SN-APS. In order to detect autoantibodies with different methodological approaches, sera from 24 patients with SN-APS were analysed for anti-phospholipid antibodies using TLC immunostaining, for anti-vimentin/cardiolipin antibodies by enzyme-linked immunosorbent assay (ELISA, and for anti-annexin V and anti-prothrombin antibodies by ELISA and dot blot. Control groups of our study were 25 patients with APS, 18 with systemic lupus erythematosus (SLE, and 32 healthy controls. Results revealed that 13/24 (54.2% SN-APS sera were positive for aCL (9 of whom were also positive for lysobisphosphatidic acid by TLC immunostaining, 11/24 (45.8% for anti-vimentin/cardiolipin antibodies, 3/24 (12.5% for anti-prothrombin antibodies, and 1/24 (4.2% for anti-annexin V antibodies. These findings suggest that in sera from patients with SN-APS, antibodies may be detected using “new” antigenic targets (mainly vimentin/cardiolipin or methodological approaches different from traditional techniques (mainly TLC immunostaining. Thus, SN-APS represents a mosaic, in which antibodies against different antigenic targets may be detected.

  9. How Is Antiphospholipid Antibody Syndrome Treated?

    Science.gov (United States)

    ... thinners," are used to stop blood clots from forming. They also may keep existing blood clots from getting larger. These medicines are taken as either a pill, an injection under the skin, or through a needle or tube inserted into a vein (called intravenous, or IV, ...

  10. Primary antiphospholipid syndrome presenting as antiphospholipid syndrome nephropathy: a case report

    OpenAIRE

    Abeysekera, Rajitha Asanga; Wazil, Abdul Wahid Mohomad; Nanayakkara, Nishantha; Ratnatunga, Neelakanthi VI; Fernando, Kaushal Maithree; Thinnarachchi, Jalitha

    2015-01-01

    Introduction Primary antiphospholipid syndrome can be a difficult diagnosis in the absence of typical clinical features. We describe an unusual presentation of primary antiphospholipid syndrome mimicking vasculitis for which the only diagnostic clue on initial presentation was antiphospholipid syndrome nephropathy. Case presentation A 29-year-old Sri Lankan woman presented with features mimicking vasculitis with no obvious clinical features of antiphospholipid syndrome. Classical symptoms of ...

  11. [Hepatic hemorrhagic infarction in eclampsia and HELLP Syndrome associated with the antiphospholipid syndrome].

    Science.gov (United States)

    Enriquez, R; Gutierrez, A; Sirvent, A E; Saez, J; Palacios, F; Cabezuelo, J B

    1999-01-01

    A 33 year-old woman developed eclampsia with HELLP syndrome. Laboratory results revealed lupus anticoagulant and anticardiolipin antibodies. Imaging tests showed liver and spleen infarctions. The patients was given enoxaparin and supportive care and there was a good evolution. We discuss some aspects about liver infarction and its association with toxemia of pregnancy and the antiphospholipid syndrome.

  12. 抗磷脂抗体综合征肠系膜血管血栓形成的特点%The characteristics of mesenteric vascular thrombosis in patients with antiphospholipid antibody syndrome

    Institute of Scientific and Technical Information of China (English)

    史旭华; 郑毅

    2008-01-01

    Objective To investigate the characteristics of mesenteric vascular thrombosis (MVT) in patients with antiphospholipid antibody syndrome (APS).Methods The cases reports about MVT in patients with APS were searched in Pubmed and Chinese biomedical database (1983.1-2007.7) and then were analyzed.Results There were 13 males and 8 females in 21 patients.The average age was (37±17) years (5months~69 years).Three cases (14%) had a history of deep venous thrombosis and 4 (19%) had spontaneous abortions.The course of disease was 4 hours to 4 months.The clinical manifestations included abdominal pain 18 (86%),hemafecia or melaena 4 (19%),vomiting 3 (14%),diarrhea 2 (10%),hematemesis 2(10%).Physical signs included abdominal tenderness in 10(48%),peritoneal irritation signs in 5 (24%),shifting dullness in 3 (14%) anddecreased bowel sounds in 3 (14%).Mesenteric vascular thrombosis were detected through B uhrasonography (3/10,33%),abdominal CT (9/13,69%),MRI (4/4,100%),Doppler ultrasound (4/4,100%),angiography (6/6,100%).Eighteen cases (86%) had positive anti-cardiophospholipin antibody and 14 (67%) were IgG-subtype.Lupus anticoagulants were detected in 4 (19%).Sixteen cases reee-ived exploratory laparotomy,lsehemia or necrosis of intestine were found in 9(56%).In 21 cases,superior mesenteric vein thrombosis,suprior mesenteric artery thrombosis,inferior mesenteric vein thrombosis,inferior mesenteric artery thrombosis were discovered in 17 (81%),4 (19%),0 (0%),1 (5%)patients respectively.Portal vein was also involved in 7 (33%) cases who had superior mesenteric vein thrombosis.Conclusion Superior mesenteric vein is usually involved in patients with APS who have MVT.MVTs are always occurr in middle-age male patients.Some patients have deep vein thrombosis or spontaneous abortion before MVT.The disease may be fulminant or had is insidious in onset.Abdominal pain and intestinal obstruction are the most common manifestations.IgG-subtype anticardiophospholipin antibodiesare the

  13. Seronegative antiphospholipid syndrome.

    Science.gov (United States)

    Nayfe, Rabih; Uthman, Imad; Aoun, Jessica; Saad Aldin, Ehab; Merashli, Mira; Khamashta, Munther A

    2013-08-01

    APS is an autoimmune disease that leads to arterial and/or venous thrombosis, recurrent pregnancy loss and persistently positive aPLs. Patients with clinical manifestations highly suggestive of APS but persistently negative conventional aPLs are classified as having seronegative APS. Ongoing research has revealed the existence of non-criteria antibodies proposed to be relevant to APS and that can be potentially included in the disease's classification criteria. We present a literature review on the most promising antibodies of this heterogeneous aPL family, which includes antibodies to a zwitterionic phospholipid, namely phosphatidylethanolamine, phospholipid-binding plasma proteins, phospholipid-protein complexes and anionic phospholipids other than cardiolipin. Although these molecules can increase the diagnostic yield of APS, their clinical relevance is still debatable and needs to be confirmed by interlaboratory efforts toward standardizing diagnostic tools, in addition to experimental data and larger longitudinal studies.

  14. Antibody

    Science.gov (United States)

    An antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples ... microorganisms (bacteria, fungi, parasites, and viruses) and chemicals. Antibodies may be produced when the immune system mistakenly ...

  15. Incidence, pathophysiology, and clinical manifestations of antiphospholipid syndrome.

    Science.gov (United States)

    Brock, Clifton O'neill; Brohl, Andrew Scott; Običan, Sarah Gloria

    2015-09-01

    Antiphospholipid syndrome (APLS) is a complex systemic disease with a wide variety of clinical manifestations. In the obstetric population, recurrent early pregnancy loss, fetal loss, and thrombosis are hallmarks of the disease. Patients with APLS have developed one or more pathogenic auto-antibodies directed against plasma and cell surface proteins. These antibodies are characterized by their affinity for anionic phospholipids. Interactions between APLS antibodies and their protein targets influence a wide variety of biological systems and signaling pathways, including monocytes, platelets, the complement system, and endothelial cells. While much research is currently directed at understanding the mechanisms involved in this autoimmune disease, the key clinical presentation is the hypercoagulable state resulting in thrombosis occurring in essentially any arterial or venous location, as well as numerous obstetrical complications. Treatment of APLS is generally directed at preventing thrombosis and poor pregnancy outcomes by ameliorating the hypercoagulable state.

  16. Segmental small bowel necrosis associated with antiphospholipid syndrome: a case report.

    Science.gov (United States)

    Wang, Qun-Ying; Ye, Xiao-Hua; Ding, Jin; Wu, Xiao-Kang

    2015-04-07

    Antiphospholipid syndrome is a multi-system disease characterized by the formation of thromboembolic complications and/or pregnancy morbidity, and with persistently increased titers of antiphospholipid antibodies. We report the case of a 50-year-old, previously healthy man who presented with fever and new-onset, dull abdominal pain. A contrast-enhanced computed tomography scan showed segmental small bowel obstruction, for which an emergency laparotomy was performed. Histopathologic examination of resected tissues revealed multiple intestinal and mesenteric thromboses of small vessels. Laboratory tests for serum antiphospholipid (anticardiolipin IgM) and anti-β2-glycoprotein I antibodies were positive. Despite proactive implementation of anticoagulation, steroid, and antibiotic therapies, the patient's condition rapidly deteriorated, and he died 22 d after admission. This case highlights that antiphospholipid syndrome should be suspected in patients with unexplainable ischemic bowel and intestinal necrosis presenting with insidious clinical features that may be secondary to the disease, as early diagnosis is critical to implement timely treatments in order to ameliorate the disease course.

  17. Neurological manifestations in patients with antiphospholipid syndrome.

    Directory of Open Access Journals (Sweden)

    Masoud Etemadifar

    2013-12-01

    Full Text Available Anti-phospholipids syndrome (APS is considered a non inflammatory auto-immune disease with a significant thrombophilic risk with varied clinical manifestations. The purpose of the current study was to investigate the frequency of thrombotic and non-thrombotic events in patients with APS.In this retrospective study, 102 definite APS subjects were recruited (2007-2011 at Alzahra Hospital, Isfahan, Iran. The patients were referred to Multiple Sclerosis Clinic with the diagnosis of definite APS according to 2006 Sydney's criteria. Disorders associated with APS such as pregnancy complication, vascular thrombosis and livedo reticularis (LR were assessed. Neurological signs and symptoms such as cognitive dysfunction were recorded. Data analyses were performed using SPSS software and P < 0.05 were considered to be statistically significant.Our findings showed that majority of female gender, higher rate of ischemic thrombotic stroke and high miscarriage lied in a large number of APS patients.Overall recurrent miscarriage is a common complication among (antiphospholidpid antibody aPL patients. Furthermore, ischemic stroke is the second common neurological manifestations of APS patients.

  18. Antiphospholipid syndrome; its implication in cardiovascular diseases: a review

    Directory of Open Access Journals (Sweden)

    Goudevenos John

    2010-11-01

    Full Text Available Abstract Antiphospholipid syndrome (APLS is a rare syndrome mainly characterized by several hyper-coagulable complications and therefore, implicated in the operated cardiac surgery patient. APLS comprises clinical features such as arterial or venous thromboses, valve disease, coronary artery disease, intracardiac thrombus formation, pulmonary hypertension and dilated cardiomyopathy. The most commonly affected valve is the mitral, followed by the aortic and tricuspid valve. For APLS diagnosis essential is the detection of so-called antiphospholipid antibodies (aPL as anticardiolipin antibodies (aCL or lupus anticoagulant (LA. Minor alterations in the anticoagulation, infection, and surgical stress may trigger widespread thrombosis. The incidence of thrombosis is highest during the following perioperative periods: preoperatively during the withdrawal of warfarin, postoperatively during the period of hypercoagulability despite warfarin or heparin therapy, or postoperatively before adequate anticoagulation achievement. Cardiac valvular pathology includes irregular thickening of the valve leaflets due to deposition of immune complexes that may lead to vegetations and valve dysfunction; a significant risk factor for stroke. Patients with APLS are at increased risk for thrombosis and adequate anticoagulation is of vital importance during cardiopulmonary bypass (CPB. A successful outcome requires multidisciplinary management in order to prevent thrombotic or bleeding complications and to manage perioperative anticoagulation. More work and reporting on anticoagulation management and adjuvant therapy in patients with APLS during extracorporeal circulation are necessary.

  19. Delineating the deranged immune system in the antiphospholipid syndrome.

    Science.gov (United States)

    van den Hoogen, Lucas L; van Roon, Joël A G; Radstake, Timothy R D J; Fritsch-Stork, Ruth D E; Derksen, Ronald H W M

    2016-01-01

    The antiphospholipid syndrome (APS) is a systemic autoimmune disease that is characterized serologically by the presence of antiphospholipid antibodies (aPL) and clinically by vascular thrombosis and obstetric complications. The protein β2 glycoprotein I (β2GPI) is identified as the most important autoantigen in this syndrome. Activation of endothelial cells, thrombocytes and placental tissue by anti-β2GPI antibodies relates to the clinical manifestations of APS. This review describes genetic and environmental factors in relation to APS and summarizes the current knowledge on abnormalities in components of both the innate and adaptive immune system in APS. The role of dendritic cells, T-cells, B-cells, monocytes, neutrophils and NK-cells as well as the complement system in APS are discussed. Several gaps in our knowledge on the pathophysiology of APS are identified and a plea is made for future extensive immune cell profiling by a systems medicine approach in order to better unravel the pathogenesis of APS, to gain more insight in the role of the immune system in APS as well as having the potential to reveal biomarkers or novel therapeutic targets.

  20. A REPORT OF CENTRAL RETINAL ARTERY OCCLUSION (CRAO , IN YOUNG MALES IN ITS INITIAL MANIFESTATION, AS PRIMAR Y ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    Rani

    2013-05-01

    Full Text Available ABSTRACT: AIM: To report a case of Central Retinal Artery Occlusi on (CRAO in young males in its initial manifestation as Primary Antiphospholipid Syndrome. METHODS: 32 year healthy male, with abrupt sudden painless loss of vision in r ight eye since 48 hours, with Grade 2 Relative afferent pupillary defect, visual acuity of hand movements in OD and 6/18 in OS. Fundoscopy disclosed signs compatible of central reti nal artery occlusion confirmed with FFA. Carotid Doppler imaging and echocardiography was done to determine the source. RESULTS: Antiphospholipid antibody cofactor, beta2-glycoprotein 1 antibodies, IgM, was positive with titre of more than 94 un its/ml on two occasions, 1 2 weeks apart, with normal range being less than 20 units/ml for each isotope (IgG, IgM, or IgA .According to the 2006 revised Sapporo criteria Antiphospholipid syndrome was diagnosed. Thor ough examination excluded other system involvement. Immunological studies excluded other systemic disorders. CONCLUSIONS: In literature, prevalence of CRAO is 0.85% for every 100000 and prevalence of Antiphospholipid Syndrome in patients showing a major retinal vascula r obstruction is 5% - 33%. Antiphospholipid syndrome should be ruled out in every young patient who presents with Central retinal artery occlusion. Association must be considered, as Central retinal artery occlusion could be the initial manifestation of ant iphospholipid syndrome with high risk of recurrence.

  1. Genetic aspects of the antiphospholipid syndrome: An update.

    Science.gov (United States)

    Sebastiani, Gian Domenico; Iuliano, Annamaria; Cantarini, Luca; Galeazzi, Mauro

    2016-05-01

    Studies on the immunogenetic predisposition to antiphospholipid syndrome (APS) and on other non-genetic and epigenetic factors are summarised and discussed. Family studies suggest a genetic predisposition to APS. It appears that this genetic predisposition is in part accounted for by the HLA system, the most consistent associations being those with DR4 and DRw53. Furthermore, it appears that lupus anticoagulant (LA) and anticardiolipin (aCL) antibodies are both associated with the same HLA antigens. Population studies suggest that HLA genes have a role in conferring susceptibility to develop primary APS, with some differences in different ethnic groups. Other genes, outside the MHC, give their contribution to the development of this autoimmune syndrome, such as IRF5, STAT4 and those related to inherited thrombophilia--factor V Leiden and G20210A prothrombin polymorphisms. Finally, post-transcriptional modifications of anti-beta2GPI antibodies could be implicated too.

  2. Catastrophic antiphospholipid syndrome: task force report summary.

    Science.gov (United States)

    Cervera, R; Rodríguez-Pintó, I

    2014-10-01

    The Task Force on Catastrophic Antiphospholipid Syndrome (CAPS) aimed to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of CAPS. This article summarizes the main aspects of its final report.

  3. Renal artery thrombosis and hypertension in a 13 year old girl with antiphospholipid syndrome.

    Science.gov (United States)

    Ostuni, P A; Lazzarin, P; Pengo, V; Ruffatti, A; Schiavon, F; Gambari, P

    1990-01-01

    The case of a 13 year old girl with renal artery thrombosis and hypertension is described. A cerebrovascular accident and a probable occlusion of the superior mesenteric artery also occurred. Very high levels of 'lupus anticoagulant', anticardiolipin antibodies as well as false positive Venereal Disease Research Laboratory tests were repeatedly shown. Moreover, the patient fulfilled at least four classification criteria for systemic lupus erythematosus, but only a slight positivity for antinucleolar antibodies was present. The striking relation between antiphospholipid antibody levels and clinical events and the treatment of this complex syndrome are discussed. Images PMID:2108619

  4. Epilepsy as part of systemic lupus erythematosus and systemic antiphospholipid syndrome (Hughes syndrome).

    Science.gov (United States)

    Cimaz, R; Meroni, P L; Shoenfeld, Y

    2006-01-01

    The antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies (aPL), demonstrated by ELISAs for antibodies against phospholipids and associated phospholipid-binding cofactor proteins and/or a circulating lupus anticoagulant (LA) together with diverse systemic clinical manifestations such as thrombosis, and recurrent spontaneous abortions. According to the criteria set out in Sydney the only neurological manifestations that can be suitable as APS classification criteria are ischemic events (stroke and transient ischemic attacks). However, other neurological manifestations, including seizures in particular, have been repeatedly reported in APS patients. The present review will summarize recent research on the association of aPL, as well as other autoantibodies, with seizure disorders, with or without concomitant SLE.

  5. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus.

    Science.gov (United States)

    Tincani, A; Rebaioli, C B; Taglietti, M; Shoenfeld, Y

    2006-10-01

    Cardiac involvement is one of the main complications substantially contributing to the morbidity and mortality of patients suffering from systemic autoimmune diseases. All the anatomical heart structures can be affected, and multiple pathogenic mechanisms have been reported. Non-organ-specific autoantibodies have been implicated in immune complex formation and deposition as the initial triggers for inflammatory processes responsible for Libman-Sacks verrucous endocarditis, myocarditis and pericarditis. Anti-phospholipid antibodies have been associated with thrombotic events in coronary arteries, heart valve involvement and intra-myocardial vasculopathy in the context of primary and secondary anti-phospholipid syndrome. Antibodies-SSA/Ro and anti-SSB/La antigens play a major pathogenic role in affecting the heart conduction tissue leading to the electrocardiographic abnormalities of the neonatal lupus syndrome and have been closely associated with endocardial fibroelastosis.

  6. Avaliação clínico-laboratorial de pacientes com síndrome antifosfolípide primária segundo a frequência de anticorpos antinucleares (FAN Hep-2 Clinical and laboratory evaluation of patients with primary antiphospholipid syndrome according to the frequency of antinuclear antibodies (ANA Hep-2

    Directory of Open Access Journals (Sweden)

    Jozélio Freire de Carvalho

    2010-06-01

    Full Text Available OBJETIVO: Avaliar a frequência de manifestações clínicas e laboratoriais em pacientes com síndrome antifosfolípide primária (SAFP com anticorpos antinucleares positivos (FAN Hep-2+, comparados àqueles com esses anticorpos negativos (FAN Hep-2 -. PACIENTES E MÉTODOS: Estudo transversal em 58 pacientes (82,8% mulheres com SAFP. Foram avaliados os dados demográficos, clínicos, comorbidades, medicações e anticorpos antifosfolípides. RESULTADOS: Dos 58 pacientes incluídos no estudo, vinte (34,5% apresentaram presença de FAN Hep-2. Comparando-se o grupo de pacientes FAN Hep-2+ com aqueles FAN Hep-2 -, verificou-se que ambos os grupos de pacientes com SAFP não diferiram estatisticamente em relação aos dados demográficos, bem como em relação ao tempo de doença. Em relação às manifestações clínicas e laboratoriais, o grupo com FAN Hep-2 + apresentou maior frequência de trombose venosa profunda (85 versus 52,6%, P = 0,04, uma frequência estatística e significativamente maior de anticardiolipina IgG (85 versus 52,6%, P = 0,02 e uma tendência para anticardiolipina IgM (80% versus 52,6%, P = 0,05, bem como maiores medianas desses anticorpos [33 (0-128 versus 20 (0-120 GPL, P = 0,008] e [33 (0-120 versus 18,5 (0-120 MPL, P = 0,009]. Tal diferença não foi observada no que se refere a outras manifestações da SAF, presença de comorbidades, estilo de vida e uso de medicações. CONCLUSÃO: Pacientes com SAFP que apresentam FAN Hep-2+ têm maior frequência de trombose venosa profunda e anticardiolipinas IgG e IgM.OBJECTIVE: To evaluate the frequency of clinical and laboratory manifestations in patients with primary antiphospholipid syndrome (PAPS with positive antinuclear antibodies (ANA Hep-2+ compared to those in whom this antibody is negative (ANA Hep-2-. PATIENTS AND METHODS: This is a transversal study with 58 patients (82.8% females with PAPS. Demographic and clinical data, comorbidities, medications, and

  7. Patient with antiphospholipid syndrome accompanied by pre-eclampsia who developed hellp syndrome and eclampsia after abortion

    Institute of Scientific and Technical Information of China (English)

    WANG Yong-qing; NIU Ji-hong; WANG Jia-lue; YE Rong-hua; ZHAO Yang-yu

    2012-01-01

    Antiphospholipid syndrome(APS)refers to a group of clinical symptoms and signs caused by antiphospholipid antibody(aPLA).We reported a rare case of poor outcome of a pregnant woman with APS.The pregnant woman had APS,hemolytic anemia,elevated liver function and low platelet count(HELLP)syndrome,and eclampsia and had a poor outcome from a second pregnancy.She was treated with antispasmodics,sedatives,and anti-hypertensive agents,along with anticoagulant therapy and infusion of immunoglobulin.APS during pregnancy often makes pregnancy even more complex and risky.Obstetricians should carry out anticoagulation treatment throughout the perinatal period.

  8. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): thrombocytopenia and skin manifestations.

    Science.gov (United States)

    Cervera, R; Tektonidou, M G; Espinosa, G; Cabral, A R; González, E B; Erkan, D; Vadya, S; Adrogué, H E; Solomon, M; Zandman-Goddard, G; Shoenfeld, Y

    2011-02-01

    The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyze the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations, and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analyzed on thrombocytopenia and skin manifestations, and presents the recommendations elaborated by the Task Force after this analysis.

  9. Transient Antiphospholipid Syndrome Associated with Primary Cytomegalovirus Infection: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Tsuyoshi Nakayama

    2014-01-01

    Full Text Available Viral infection is known to induce transient autoimmunity in humans. Acute cytomegalovirus (CMV infection is implicated in occasional thrombosis formation. We here, for the first time, report a 19-year-old female who had an acute CMV infection, leading to a deep venous thrombosis and a pulmonary embolism along with transient appearance of lupus anticoagulant. The pathological role of antiphospholipid antibodies in CMV-mediated thrombosis is discussed.

  10. Síndrome do anticorpo antifosfolípide e endometriose Antiphospholipid syndrome and endometriosis

    Directory of Open Access Journals (Sweden)

    Leonardo Schmidt

    2006-12-01

    Full Text Available Descreve-se um caso de síndrome de Sneddon associada à presença de anticorpos antifosfolípides em uma paciente jovem com endometriose. Os autores analisam a associação destas duas enfermidades.We describe a patient with Sneddon's syndrome associated with antiphospholipid antibodies and endometriosis. The authors analyze the association of these two diseases.

  11. Síndrome do anticorpo antifosfolípide e endometriose Antiphospholipid syndrome and endometriosis

    OpenAIRE

    2006-01-01

    Descreve-se um caso de síndrome de Sneddon associada à presença de anticorpos antifosfolípides em uma paciente jovem com endometriose. Os autores analisam a associação destas duas enfermidades.We describe a patient with Sneddon's syndrome associated with antiphospholipid antibodies and endometriosis. The authors analyze the association of these two diseases.

  12. Antiphospholipid syndrome in a pregnant female presenting with severe thrombocytopenia and bleeding.

    Science.gov (United States)

    Mahajan, Kunal; Katyal, Virender; Arya, Suvrat; Shrama, Meha

    2015-01-01

    The antiphospholipid antibody syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies in patients with recurrent venous or arterial thromboembolism or pregnancy morbidity. Antithrombotic therapy is the mainstay of treatment given the high risk of recurrent thromboembolism that characterizes this condition. Despite the prothrombotic nature of APS, thrombocytopenia is present in a proportion of patients, which can complicate management and limit the use of antithrombotic therapy. The mechanism of APS-associated thrombocytopenia is multifactorial and its relation to thrombotic risk is poorly characterized. The presence of thrombocytopenia does not appear to reduce thrombotic risk in patients with APS, who can develop thromboembolic complications necessitating antithrombotic treatment. In these cases, treatment of the thrombocytopenia may be necessary to facilitate administration of antithrombotic agents. We present such a pregnant lady with history of recurrent pregnancy losses who presented with severe thrombocytopenia and bleeding manifestations, who was subsequently diagnosed to have antiphospholipid antibody syndrome. She was initially managed with steroids and when her platelet counts improved, antithrombotic therapy was started. She delivered an uneventful and successful pregnancy outcome without any complications during follow-up.

  13. Antiphospholipid Syndrome in a Pregnant Female Presenting with Severe Thrombocytopenia and Bleeding

    Directory of Open Access Journals (Sweden)

    Kunal Mahajan

    2015-01-01

    Full Text Available The antiphospholipid antibody syndrome (APS is defined by the persistent presence of antiphospholipid antibodies in patients with recurrent venous or arterial thromboembolism or pregnancy morbidity. Antithrombotic therapy is the mainstay of treatment given the high risk of recurrent thromboembolism that characterizes this condition. Despite the prothrombotic nature of APS, thrombocytopenia is present in a proportion of patients, which can complicate management and limit the use of antithrombotic therapy. The mechanism of APS-associated thrombocytopenia is multifactorial and its relation to thrombotic risk is poorly characterized. The presence of thrombocytopenia does not appear to reduce thrombotic risk in patients with APS, who can develop thromboembolic complications necessitating antithrombotic treatment. In these cases, treatment of the thrombocytopenia may be necessary to facilitate administration of antithrombotic agents. We present such a pregnant lady with history of recurrent pregnancy losses who presented with severe thrombocytopenia and bleeding manifestations, who was subsequently diagnosed to have antiphospholipid antibody syndrome. She was initially managed with steroids and when her platelet counts improved, antithrombotic therapy was started. She delivered an uneventful and successful pregnancy outcome without any complications during follow-up.

  14. Postpartum spontaneous colonic perforation due to antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    Kamran Ahmed; Amir Darakhshan; Eleanor Au; Munther A Khamashta; Iraklis E Katsoulis

    2009-01-01

    The antiphospholipid syndrome (APS) is a multi-systemic disease being characterized by the presence of antiphospholipid antibodies that involves both arterial and venous systems resulting in arterial or venous thrombosis, fetal loss, thrombocytopenia, leg ulcers, livedo reticularis, chorea,and migraine. We document a previously unreported case of a 37-year-old female in whom APS was first manifested by infarction and cecal perforation following cesarean section. At laparotomy the underlying cause of colonic perforation was not clear and after resection of the affected bowel an ileo-colostomy was performed. The diagnosis of APS was established during post-operative hospital stay and the patient was commenced on warfarin.Eventually, she made a full recovery and had her stoma reversed after 4 mo. Pregnancy poses an increased risk of complications in women with APS and requires a more aggressive approach to the obstetric care. This should include full anticoagulation in the puerperium and frequent doppler ultrasound monitoring of uterine and umbilical arteries to detect complications such as preeclampsia and placental insufficiency.

  15. Current treatment of antiphospholipid syndrome: lights and shadows.

    Science.gov (United States)

    Espinosa, Gerard; Cervera, Ricard

    2015-10-01

    For patients with antiphospholipid syndrome (APS), the consensus is to treat those who develop thrombosis with long-term oral anticoagulation therapy and to prevent obstetric manifestations by use of aspirin and heparin. These recommendations are based on data from randomized controlled trials and observational studies. Despite this body of knowledge, areas of uncertainty regarding the management of APS exist where evidence is scarce or nonexistent. In other words, for a subset of patients the course of management is unclear. Some examples are patients with 'seronegative' APS, those who do not fulfil the formal (clinical or serological) classification criteria for definite APS, and those with recurrent thrombotic events despite optimal anticoagulation. Other challenges include the treatment of clinical manifestations not included in the classification criteria, such as haematologic manifestations (thrombocytopenia and haemolytic anaemia), neurologic manifestations (chorea, myelitis and multiple sclerosis-like lesions), and nephropathy and heart valve disease associated with antiphospholipid antibodies (aPL), as well as the possible withdrawal of anticoagulation treatment in selected cases of thrombotic APS in which assays for aPL become persistently negative. This Review focuses on the current recommendations for thrombotic and obstetric manifestations of APS, as well as the management of difficult cases. Some aspects of treatment, such as secondary prophylaxis of venous thrombosis, are based on strong evidence--the 'lights' of APS treatment. Conversely, other areas, such as the treatment of non-criteria manifestations of APS, are based only on expert consensus or common sense and remain the 'shadows' of APS therapy.

  16. Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations.

    Science.gov (United States)

    Rangel, M L; Alghamdi, I; Contreras, G; Harrington, T; Thomas, D B; Barisoni, L; Andrews, D; Wolf, M; Asif, A; Nayer, A

    2013-07-01

    Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.

  17. Gangrena de pavilhão auricular como primeira manifestação de síndrome do anticorpo antifosfolípide Gangrene of the auricle as the first sign of antiphospholipid antibody syndrome

    Directory of Open Access Journals (Sweden)

    Erika Bettini de Sá

    2011-12-01

    Full Text Available A síndrome do anticorpo antifosfolípide (SAF, mais comum em mulheres, manifesta-se clinicamente como trombose e/ou abortamentos de repetição. Anemia hemolítica autoimune e manifestações neurológicas, cardíacas e cutâneas são comuns. Relata-se o caso de um paciente do gênero masculino cuja manifestação inicial da doença foi gangrena em pavilhão auricular, e o diagnóstico de SAF se deu por meio de biópsia de pele do membro inferior, que mostrava vasculopatia trombótica, sem evidência de vasculite. Esse resultado é um dos dois critérios maiores que, associados a um critério menor, fecham o diagnóstico dessa doença. Discutem-se neste caso os possíveis diagnósticos diferenciais e como eles se diferenciam da doença em foco, além da importância que a biópsia teve no diagnóstico de SAF nesse indivíduo.Antiphospholipid syndrome (APS, more common in females, manifests clinically as thrombosis and/or recurrent fetal loss. Hemolytic autoimmune anemia and neurological, cardiac and cutaneous manifestations are common. This is the case report of a male patient whose first manifestation of the disease was gangrene of the auricle. The diagnosis of APS was established by biopsy of the lower limb skin, which showed thrombotic vasculopathy with no evidence of vasculitis. This is one of the two major criteria, which, along with a minor criterion, establishes the diagnosis of APS. Possible differential diagnoses are discussed. The importance of the biopsy in the APS diagnosis of this male patient is emphasized.

  18. 36-Year-Old Female with Catastrophic Antiphospholipid Syndrome Treated with Eculizumab: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    Marianna Strakhan

    2014-01-01

    Full Text Available Catastrophic antiphospholipid syndrome (CAPS is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL. CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor.

  19. 抗磷脂抗体及凝血因子Ⅻ缺乏与视网膜静脉阻塞的相关研究%The correlation of antiphospholipid antibody and factor Ⅻ deficiency in patients with retinal vein occlusion

    Institute of Scientific and Technical Information of China (English)

    孙红晶; 李毓敏

    2009-01-01

    目的 观察抗磷脂抗体(APA)及凝血因子Ⅻ(F Ⅻ)缺乏在视网膜静脉阻塞(RVO)发病中的作用.方法 对RVO患者33例(33眼)及正常对照组30例(30眼),采用ELISA法检测抗心磷脂抗体(ACA)(IgG、IgM、IgA);APTT法检测LA;一期法测定FⅫ活性.采用Fisher检验进行统计学分析.结果 RVO组APAs总阳性率24.24%(8/33)与对照组6.67%(2/30)比较差异无统计学意义(P=0.085).RVO组ACA阳性率18.18%(6/33)与对照组(0/30)比较差异有统计学意义(P=0.025).≤50岁组及>50岁组RVO患者LA总阳性率与同年龄对照组相比差异均无统计学意义(P=0.160,P=0.206).RVO组FⅫ缺乏率42.42%(14/33)与对照组13.33%(4/30)比较差异有统计学意义(P=0.013).≤50岁组及>50岁组RVO患者FⅫ缺乏与同年龄对照组比较差异无统计学意义(P=0.206,P=0.052).结论 研究表明ACA阳性及FⅫ缺乏引起的凝血障碍与RVO发病相关.%Objective Antiphospholipid antibody and factor Ⅻ deficiency are among the coagulation disorders that have been implicated in many thrombembolic events. The aim of this study was to investigate the prevalence of antiphospholipid antibodies and factor Ⅻ deficiency in patients with retinal vein occlusion (RVO). Methods The investigation was a case control study. The periphery blood was collected from a cohort of 33 consecutive patients with RVO and 30 age- and gender-matched normal subjects. Anticardiolipin antibody (ACA) was detected by ELISA as binding index (BI) =A value/standard A value. The lupus anticoagulant antibody was examined by APTT test and the activity of factor Ⅻ was detected. This study was approved by The Human Research Ethics Committee of this hospital, and written informed consent was obtained from all the subjects before initiation of any study protocol. Results The total positive rate of APA in RVO group was 24. 24% (8/33), showing a insignificant difference in comparison with control group (6. 67%, 2/30) (P = 0. 085). The positive rate of

  20. The characteristics of bronchoalveolar lavage from a patient with antiphospholipid syndrome who developed acute respiratory distress syndrome.

    Science.gov (United States)

    Nakos, G; Kitsiouli, E; Maneta-Peyret, L; Cassagne, C; Tsianos, E; Lekka, M

    2001-01-01

    The purpose of this study was to investigate the biochemical characteristics as well as the occurrence and specificity of antiphospholipid antibodies in the bronchoalveolar lavage (BAL) fluid from a patient with both antiphospholipid antibodies syndrome (APS) and acute respiratory distress syndrome (ARDS). Proteins, lipids, cells and autoantibodies were determined. Immunoglobulins were purified with affinity chromatography. Autoantibody identification was assessed with enzyme-linked immunosorbent assay (ELISA) and with electrophoresis, followed by immunoblotting and revelation with antihuman IgG-peroxidase conjugate. Antiphospholipid antibodies were found to be present in the BAL fluid as well as in the serum from a patient with APS. Specifically, antiphosphatidylserine and antiphosphatidic acid IgG antibodies in the BAL fluid and antiphosphatidylcholine and anticardiolipin IgG antibodies in the serum were detected at high levels. BAL fluid protein and the percentage of neutrophils were found to be increased. A quantitative as well as qualitative deficiency of surfactant phospholipids was also observed. Antibodies directed against surfactant phospholipids could cause surfactant abnormalities and an inflammatory reaction. These disorders may be one of the causes of the ARDS or a factor in the perpetuation of the inflammation.

  1. 抗磷脂综合征%Antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    杨霁云

    2003-01-01

    @@ 抗磷脂综合征(antiphospholipid syndrome, APS)是指血中抗心脂抗体(anticardiolipin antibody)和(或)狼疮抗凝物(lupus anticoagulant, LA)阳性、伴有血栓形成、血小板减少、习惯性流产的综合征,曾称为抗心脂综合征[1],现多称APS.本征可累及多个系统、器官,且症状复杂,易于误诊或漏诊.初多为成人报道,近年儿科亦多报道[2-5].今就其与临床密切相关的一些问题,综述如下.

  2. Successful Anticoagulation Therapy for Antiphospholipid Syndrome with Mobile Aortic Thrombi

    Science.gov (United States)

    Park, Hyun Oh; Moon, Seong Ho; Kim, Jong Woo; Byun, Joung Hun; Kim, Sung Hwan; Yang, Jun Ho; Lee, Chung-Eun; Kim, Jong-Duk

    2016-01-01

    Hypercoagulable states have been associated with aortic thrombosis. Antiphospholipid syndrome (APS) is one of the commonest types of acquired thrombophilia. We report the case of successful anticoagulation management in an APS patient with mobile thrombi within the aorta. A 58-year-old male patient presented to the emergency department (ED) with right-sided hemiparesis. His first symptoms were noted approximately 12–16 hours before presentation to the ED. Magnetic resonance imaging of the brain showed acute embolic infarction of the left frontal and parietotemporal lobes. Transesophageal echocardiography (TEE) and computed tomography angiography (CTA) demonstrated mobile thrombi attached to the wall of the ascending aorta and aortic arch. The patient was diagnosed with APS based on positivity of anti-beta-2 glycoprotein 1 antibodies, and was initiated on anticoagulation therapy. Repeated TEE and CTA revealed complete resolution of the thrombi after 12 days of treatment; the patient was discharged well. PMID:28042559

  3. Antiphospholipid syndrome and vascular ischemic (occlusive) diseases: an overview.

    Science.gov (United States)

    Atanassova, Penka A

    2007-12-31

    Antiphospholipid syndrome (APS) is primarily considered to be an autoimmune pathological condition that is also referred to as "Hughes syndrome". It is characterized by arterial and/or venous thrombosis and pregnancy pathologies in the presence of anticardiolipin antibodies and/or lupus anticoagulant. APS can occur either as a primary disease or secondary to a connective tissue disorder, most frequently systemic lupus erythematosus (SLE). Damage to the nervous system is one of the most prominent clinical constellations of sequelae in APS and includes (i) arterial/ venous thrombotic events, (ii) psychiatric features and (iii) other non- thrombotic neurological syndromes. In this overview we compare the most important vascular ischemic (occlusive) disturbances (VIOD) with neuro-psychiatric symptomatics, together with complete, updated classifications and hypotheses for the etio-pathogenesis of APS with underlying clinical and laboratory criteria for optimal diagnosis and disease management.

  4. Correlation study on antiphospholipid antibody syndrome, prethrombotic state and the incidence of early onset severe preeclampsia%抗磷脂抗体综合征、血栓前状态与早发型重度子痫前期发病的相关研究

    Institute of Scientific and Technical Information of China (English)

    肖海燕; 席雅娟

    2013-01-01

    目的 探讨抗磷脂抗体综合征、血栓前状态与早发型重度子痫前期发病之前的相关性,寻找早期诊断早发型重度子痫前期的指标.方法 收集我院住院分娩的早发型(早发组)及晚发型重度子痫前期患者(晚发组)各70例.同时随机选取70例同期入院的正常孕妇为对照组.对比各组患者外周血甘油三脂(TG)、胆固醇(TC)、出凝血功能指标以及血清抗心磷脂抗体、抗β2-GPI抗体等表达水平.结果 三组间出凝血功能对比中,早发组抗凝血酶(AT)活性显著低于晚发组及正常对照组,差异均有统计学意义(P<0.05).三组TC、TG及D-二聚体对比中,早发组D-二聚体平均秩次为76.6 mg/L,显著高于晚发组的58.3 mg/L和正常组的54.1 mg/L,差异均具有统计学意义(P<0.05).早发组抗ACA抗体阳性率及抗β2-GPI抗体阳性率显著高于晚发组及正常组,差异有统计学意义(P<0.05).结论 抗凝血酶、D-二聚体、抗ACA抗体阳性率及抗&-GPI抗体可作为早期诊断早发型重度子痫前期的指标.%Objective To study the correlation of antiphospholipid antibody syndrome,prethrombotic state with the incidence of early onset severe preeclampsia,and to investigate the indexes for the early diagnosis of early onset severe preeclampsia.Methods Puerperants in our hospital with early onset severe preeclampsia (the early group,n=70) and late onset severe preeclampsia (the late group,n=70) were collected.At the same time,70 normal pregnant women were randomly selected as the control group.The three groups were compared in glycerol three fat (TG),cholesterol (TC),coagulation index and serum anticardiolipin antibody,anti-β2-GPI antibody levels.Results For coagulation index,the antithrombin (AT) activity in the early group was significantly lower than that in the late group and the control group (P<0.05).For TC,TG and D-dimer,the D-dimer average rank was 76.6 in the early group,significantly higher than 58.3 in

  5. Catastrophic antiphospholipid syndrome in pregnancy, a diagnosis that should not be missed.

    Science.gov (United States)

    Hoayek, Jennifer G; Moussa, Hind N; Rehman, Hina A; Nasab, Susan Hosseini; Blackwell, Sean C; Sibai, Baha M

    2016-12-01

    Catastrophic antiphospholipid syndrome (CAPS) is an accelerated form of the antiphospholipid antibody syndrome resulting in multi-organ ischemia and failure. It is a rare and life-threatening condition that can be easily mistaken with hemolysis elevated liver enzymes low platelets syndrome, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. In order to make a diagnosis, it is required to have multi-organ thrombosis over 1 week affecting at least three organs or systems, and to have positive antiphospholipid antibody on two occasions (6 weeks apart), and histopathologic confirmation of small vessel occlusion. However, due to similarities in clinical and laboratory findings between CAPS and some other obstetric complications, potential misdiagnosis or delay in diagnosis are common, increasing the risk of adverse maternal and perinatal outcomes. In this review we summarized information presented in previous studies, focusing on CAPS related to pregnancy. We reviewed diagnostic criteria, differential diagnosis, and common presentation ranging from malaise, abdominal pain, dyspnea, hypertension, to altered mental status and seizures. We also discussed management in pregnancy and included a detailed algorithm with steps to take. Of note, the most significant reduction in mortality was seen in patients receiving triple therapy which will be discussed in this review.

  6. Prolactin May Not Play a Role in Primary Antiphospholipid (Hughes' Syndrome

    Directory of Open Access Journals (Sweden)

    Manoel Tavares Neves Junior

    2011-01-01

    Full Text Available The relationship between prolactin (PRL and the immune system has been demonstrated in the last two decades and has opened new windows in the field of immunoendocrinology. However, there are scarce reports about PRL in primary antiphospholipid syndrome (pAPS. The objective of this study was to evaluate PRL levels in patients with pAPS compared to healthy controls and to investigate their possible clinical associations. Fifty-five pAPS patients according to Sapporo criteria were age- and sex-matched with 41 healthy subjects. Individuals with secondary causes of hyperprolactinemia (HPRL were excluded; demographic, biometric, and clinical data, PRL levels, antiphospholipid antibodies, inflammatory markers, and other routine laboratory findings were analyzed. PRL levels were similar between pAPS and healthy controls (8.94±7.02 versus 8.71±6.73 ng/mL, P=.876. Nine percent of the pAPS patients and 12.1% of the control subjects presented HPRL (P=.740. Comparison between the pAPS patients with hyper- and normoprolactinemia revealed no significant differences related to anthropometrics, clinical manifestations, medications, smoking, and antiphospholipid antibodies (P>.05. This study showed that HPRL does not seem to play a role in clinical manifestations of the pAPS, differently from other autoimmune rheumatic diseases.

  7. [Diagnostic and therapeutic approach to pregnant women suspect on antiphospholipid syndrome].

    Science.gov (United States)

    Glasnović, Marija; Bosnjak, Ivica; Vcev, Aleksandar; Kosuta, Maja; Lenz, Bahrija; Glasnović-Horvatić, Elizabeta

    2008-01-01

    Antiphospholipid syndrome includes the presence of antiphospholipid antibodies, vascular thrombosis and reproductive function disturbances. The aim was to show our diagnostic and therapeutic experiences. 62 women were included in study, 32 with primary antiphospholipd syndrome (PAPS), and 30 with secondary antiphospholipid syndrome (SAPS). 36 were pregnant and studied prospectively throughout pregnancy and six weeks after the delivery. Lupus-anticoagulant (LA) was positive in 23 patients with PAPS (71.9%), and in 10 patients with SAPS (33.3%). In SAPS group anticardiolipin antibodies (aCL) was positive in 8 patients (26.6%) compared to PAPS group with 3 aCL positive patients (9.4%). Antibeta2glycoprotein1 (antibeta2GP1) was positive in 3 patients with PAPS. Complications in previous pregnancies were in 25 cases (69.4%) spontaneous abortion, in 7 cases (19.4%) preeclampsia with intrauterine growth restriction (IUGR) in 3 patients. In 4 cases the complication was fetal death in utero. Average pregnancy lasted 37.06+/-0.707 weeks. Therapy with low dose aspirin and low-molecular-weight heparin was successful in 97.2%.

  8. Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

    Institute of Scientific and Technical Information of China (English)

    Tatiana; M; Reshetnyak; Natalia; V; Seredavkina; Maria; A; Satybaldyeva; Evgeniy; L; Nasonov; Vasiliy; I; Reshetnyak

    2015-01-01

    The antiphospholipid syndrome(APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of BuddChiari syndrome(BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.

  9. Update on the current recommendations and outcomes in pregnant women with antiphospholipid syndrome.

    Science.gov (United States)

    Chighizola, Cecilia Beatrice; Gerosa, Maria; Trespidi, Laura; Di Giacomo, Alessio; Rossi, Federica; Acaia, Barbara; Meroni, Pier Luigi

    2014-11-01

    Pregnancy morbidity is part of the clinical spectrum of the antiphospholipid syndrome (APS), a chronic autoimmune condition serologically characterized by the persistent positivity of antiphospholipid antibodies (aPL). Antiplatelet and anticoagulant agents are the mainstay of the treatment of obstetric APS. However, there is an ongoing debate about the optimal management of women with most severe aPL-mediated obstetric complications, women not fulfilling APS criteria and those with refractory disease. Unfortunately, the literature cannot provide definite answers to these controversial issues, being flawed by many limitations. The evidence supporting the recommended therapeutic management of different aPL-related obstetrical clinical manifestations is presented, with a critical appraisal of each approach.

  10. Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    B. Routy

    2013-01-01

    Full Text Available Catastrophic antiphospholipid syndrome (CAPS is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported.

  11. Perioperative anticoagulation management in antiphospholipid syndrome.

    Science.gov (United States)

    Ishida, Keiichi; Masuda, Masahisa; Kohno, Hiroki; Tamura, Yusaku; Matsumiya, Goro

    2015-09-01

    Patients with antiphospholipid syndrome are at increased risk of developing thrombotic and hemorrhagic complications after cardiac surgery, and may have abnormal coagulation tests and develop thrombocytopenia after invasive procedures, which can complicate the perioperative management of anticoagulant therapy. We describe a patient with chronic thromboembolic pulmonary hypertension and antiphospholipid syndrome, who presented with prolonged activated partial thromboplastin and activated clotting times, and developed thrombocytopenia after the catheterization workup. We performed pulmonary endarterectomy and successfully managed anticoagulation by restricting heparin use at the time of surgery and monitoring the heparin effect by measuring heparin concentrations during cardiopulmonary bypass.

  12. Manifestações neuropsiquiátricas em crianças e adolescentes com lúpus eritematoso sistêmico juvenil: associação com anticorpos antifosfolípide? Neuropsychiatric manifestations of children and adolescents with juvenile systemic lupus erythematosus: is there an association with antiphospholipid antibodies?

    Directory of Open Access Journals (Sweden)

    Cássia Maria Passarelli Lupoli Barbosa

    2006-10-01

    Full Text Available OBJETIVO: estudar a freqüência de anticorpos antifosfolípide (aFL em pacientes com lúpus eritematoso sistêmico juvenil (LESJ e sua possível associação com manifestações neuropsiquiátricas. MÉTODOS: análise retrospectiva de prontuários de 64 pacientes com LESJ, de acordo com os critérios do American College of Rheumatology (ACR, acompanhados por um período mínimo de seis meses. Foram consideradas manifestações neuropsiquiátricas: cefaléia, convulsão, acidente vascular cerebral (AVC, coréia, neuropatia medular e periférica, além de alterações do comportamento, com ou sem psicose. Duas dosagens de anticorpos anticardiolipina foram realizadas com intervalo de dois meses e foram considerados positivos os títulos de IgG maiores que 20 e de IgM maiores que 12. O anticoagulante lúpico foi dosado em 32 pacientes. A análise estatística foi realizada através do teste de Fisher com nível de significância OBJECTIVE: to study the frequency of antiphospholipid antibodies (aPL in patients with juvenile systemic lupus erythematosus (JSLE and the possible association to neuropsychiatric manifestations. METHODS: retrospective analysis of charts of 64 JSLE patients according to the American College of Rheumatology (ACR classification criteria, followed for at least six months. The neuropsychiatric manifestations were defined by the presence of: headache, seizure, cerebrovascular accident (CVA, chorea, medular or peripheral neuropathy and behavior disturbances with psichosis or not. The aPL were tested in two occasions with an interval of two months. Values greater than 20 for IgG or 12 for IgM were considered as positive. The lupus anticoagulant was tested in 32 patients. The statistical analysis was performed using the Fisher’s exact test with a significance level of 0,05. RESULTS: 38 (59.4% out of 64 JSLE patients had neuropsychiatric manifestations. APL antibodies were presented in 29 patients (45.3%. We did not observe a

  13. 系统性红斑狼疮患者抗磷脂抗体与视网膜血管病关系的研究%Relationship between Retinal Vasculopathy and Antiphospholipid Antibodies in Patients with Systemic Lupus Erythematosus

    Institute of Scientific and Technical Information of China (English)

    刘岩; 熊毅彤; 翁欢; 徐康; 李秋华

    2003-01-01

    目的:研究系统性红斑狼疮(systemic lupus erythematosus,SLE)抗磷脂抗体等血清免疫学标志物与患者视网膜血管病的关系.方法:从2002年6月至2003年1月,对164例SLE患者作最佳矫正视力检查、眼压测量、裂隙灯检查和眼底检查,血清免疫学检查指标包括抗心磷脂抗体(anticardiolipin antibody, ACL)、狼疮抗凝物(lupus anticoagulant,LA)、抗核抗体(antinuclear antibody,ANA)和抗ds-DNA抗体等.结果:164例SLE患者中26例(16%)检查到有视网膜血管病,其中19例(73%)检测到抗磷脂抗体(17例ACL和2例LA).结论:SLE患者易发生视网膜血管病变,抗磷脂抗体的存在可能与其有较高的相关性.

  14. An Outdated Notion of Antibody Specificity is One of the Major Detrimental Assumptions of the Structure-Based Reverse Vaccinology Paradigm, Which Prevented It from Helping to Develop an Effective HIV-1 Vaccine.

    Science.gov (United States)

    Van Regenmortel, Marc H V

    2014-01-01

    The importance of paradigms for guiding scientific research is explained with reference to the seminal work of Karl Popper and Thomas Kuhn. A prevalent paradigm, followed for more than a decade in HIV-1 vaccine research, which gave rise to the strategy known as structure-based reverse vaccinology is described in detail. Several reasons why this paradigm did not allow the development of an effective HIV-1 vaccine are analyzed. A major reason is the belief shared by many vaccinologists that antibodies possess a narrow specificity for a single epitope and are not polyspecific for a diverse group of potential epitopes. When this belief is abandoned, it becomes obvious that the one particular epitope structure observed during the crystallographic analysis of a neutralizing antibody-antigen complex does not necessarily reveal, which immunogenic structure should be used to elicit the same type of neutralizing antibody. In the physical sciences, scientific explanations are usually presented as logical deductions derived from a relevant law of nature together with certain initial conditions. In immunology, causal explanations in terms of a single cause acting according to a law of nature are not possible because numerous factors always play a role in bringing about an effect. The implications of this state of affairs for the rational design of HIV vaccines are outlined. An alternative approach to obtain useful scientific understanding consists in intervening empirically in the immune system and it is suggested that manipulating the system experimentally is needed to learn to control it and achieve protective immunity by vaccination.

  15. Clinical features and pregnancy outcome in antiphospholipid syndrome patients with history of severe pregnancy complications.

    Science.gov (United States)

    Matsuki, Yuko; Atsumi, Tatsuya; Yamaguchi, Koushi; Hisano, Michi; Arata, Naoko; Oku, Kenji; Watanabe, Noriyoshi; Sago, Haruhiko; Takasaki, Yoshinari; Murashima, Atsuko

    2015-03-01

    Abstract Objective. To clarify the clinical significance of antiphospholipid antibody (aPL) profile in patients with obstetric antiphospholipid syndrome (APS). Methods. Clinical records of 13 pregnant patients (15 pregnancies) with obstetrical APS were reviewed over 10 years. Patients who met the Sapporo Criteria fully were studied, whereas those with only early pregnancy loss were excluded. In addition to classical aPL: lupus anticoagulant (LA), anticardiolipin antibody (aCL), and anti-β2-glycoprotein I (aβ2GPI); phosphatidylserine-dependent anti-prothrombin antibody (aPS/PT) and kininogen-dependent anti-phosphatidylethanolamine antibody (aPE) were also examined in each case. Results. Cases were divided into two groups according to patient response to standard treatment: good and poor outcome groups. All cases with poor outcome presented LA, with IgG aβ2GPI and IgG aPS/PT were also frequently observed. IgG aPE did not correlate with pregnancy outcome. Conclusion. aPL profile may predict pregnancy outcome in patients with this subset of obstetric APS.

  16. Fatal antiphospholipid syndrome following endoscopic transnasal-transsphenoidal surgery for a pituitary tumor

    Science.gov (United States)

    Li, Chiao-Zhu; Li, Chiao-Ching; Hsieh, Chih-Chuan; Lin, Meng-Chi; Hueng, Dueng-Yuan; Liu, Feng-Chen; Chen, Yuan-Hao

    2017-01-01

    Abstract Introduction: The fatal type of antiphospholipid syndrome is a rare but life-threating condition. It may be triggered by surgery or infection. Endoscopic transnasal-transsphenoidal surgery is a common procedure for pituitary tumor. We report a catastrophic case of a young woman died of fatal antiphospholipid syndrome following endoscopic transnasal-transsphenoidal surgery. Methods and Result: A 31-year-old woman of a history of stroke received endoscopic transnasal-transsphenoidal surgery for a pituitary tumor. The whole procedure was smooth. However, the patient suffered from acute delirium on postoperative day 4. Then, her consciousness became comatose state rapidly with dilatation of pupils. Urgent magnetic resonance imaging of brain demonstrated multiple acute lacunar infarcts. The positive antiphosphoipid antibody and severe thrombocytopenia were also noted. Fatal antiphospholipid syndrome was diagnosed. Plasma exchange, corticosteroids, anticoagulant agent were prescribed. The hemodynamic condition was gradually stable. However, the consciousness was still in deep coma. The patient died of organ donation 2 months later. Conclusion: If patients have a history of cerebral stroke in their early life, such as a young stroke, the APS and higher risk of developing fatal APS after major surgery should be considered. The optimal management of APS remains controversial. The best treatment strategies are only early diagnosis and aggressive therapies combing of anticoagulant, corticosteroid, and plasma exchange. The intravenous immunoglobulin is prescribed for patients with refractory APS. PMID:28072724

  17. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions.

    Science.gov (United States)

    Cervera, R; Tektonidou, M G; Espinosa, G; Cabral, A R; González, E B; Erkan, D; Vadya, S; Adrogué, H E; Solomon, M; Zandman-Goddard, G; Shoenfeld, Y

    2011-02-01

    The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyse the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analysed on the catastrophic APS, APS nephropathy and heart valve lesions, and presents the recommendations elaborated by the Task Force after this analysis.

  18. Treatment strategies and pregnancy outcomes in antiphospholipid syndrome patients with thrombosis and triple antiphospholipid positivity. A European multicentre retrospective study.

    Science.gov (United States)

    Ruffatti, Amelia; Salvan, Elisa; Del Ross, Teresa; Gerosa, Maria; Andreoli, Laura; Maina, Aldo; Alijotas-Reig, Jaume; De Carolis, Sara; Mekinian, Arsene; Bertero, Maria Tiziana; Canti, Valentina; Brucato, Antonio; Bremme, Katarina; Ramoni, Véronique; Mosca, Marta; Di Poi, Emma; Caramaschi, Paola; Galeazzi, Mauro; Tincani, Angela; Trespidi, Laura; Meroni, Pier Luigi

    2014-10-01

    Previous thrombosis, diagnosis of systemic lupus erythematosus (SLE) and triple antiphospholipid (aPL) antibody positivity have recently been found to be independent factors associated to pregnancy failure during conventional therapy in women with antiphospholipid syndrome (APS). This study aimed to assess the effect of various treatment strategies on pregnancy outcomes in women with APS and the risk factors for pregnancy failure. One hundred ninety-six pregnancies of 156 patients diagnosed with APS were analysed: 118 (60.2%) of these had previous thrombosis, 81 (41.3%) were diagnosed with SLE, and 107 (54.6%) had triple aPL positivity. One hundred seventy-five (89.3%) were treated with conventional therapies (low-dose aspirin [LDA] or prophylactic doses of heparin + LDA or therapeutic doses of heparin + LDA), while 21 (10.7%) were prescribed other treatments in addition to conventional therapy. The pregnancies were classified into seven risk profiles depending on the patients' risk factors - thrombosis, SLE, and triple aPL positivity - and their single, double or triple combinations. It was possible to find significant difference in outcomes correlated to treatments only in the thrombosis plus triple aPL positivity subset, and logistic regression analysis showed that additional treatments were the only independent factor associated to a favourable pregnancy outcome (odds ratio=9.7, 95% confidence interval=1.1-88.9, p-value<0.05). On the basis of this retrospective study, we found that APS pregnant patients with thrombosis and triple aPL positivity treated with additional therapy had a significant higher live-birth rate with respect to those receiving conventional therapy alone.

  19. The role of MSCT angiography in early detection of lower limb arterial lesions in patients with antiphospholipid syndrome.

    Science.gov (United States)

    Saponjski, Jovica; Stojanovich, Ljudmila; Petrovic, Jelena; Saponjski, Dusan

    2017-01-25

    Antiphospholipid syndrome (APS) is an autoimmune disease which is characterized by arterial and venous thromboses, fetal loss, and the presence of antiphospholipid antibodies in the serum. It is characterized by accelerated atherosclerosis. Increased tendency towards thrombosis leads to the occurrence of various vascular events. The objective of our study was to determine if there are subclinical changes on lower limb arteries in APS patients and what the best diagnostic choice for their establishment is. In this study, we analyzed 50 patients with primary antiphospholipid syndrome (PAPS) and 50 patients, who have secondary antiphospholipid syndrome (SAPS). The results were compared to 50 controls. The groups were comparable with respect to age, gender, and traditional risk factors except for the lipid status, since controls had significantly higher levels of cholesterol and triglycerides. Study was conducted on 64-multi-slice computed tomography (64-MSCT), where we analyzed quantitative and morphological characteristics of blood vessel-detected lesions. Patients from the control group had statistically very significant elevated cholesterol and triglyceride levels in regard to the patients with SAPS and PAPS (p tissue (n = 32) and mixed lesions (n = 36) in patients with PAPS than the calcified one (n = 7, p disease progression.

  20. A case of catastrophic antiphospholipid syndrome: first report with advanced cardiac imaging using MRI.

    Science.gov (United States)

    Rosenbaum, A N; Anavekar, N S; Ernste, F C; Mankad, S V; Le, R J; Manocha, K K; Barsness, G W

    2015-10-01

    This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the

  1. Lower Limb Ischemia: Aortoiliac Thrombosis Related to Antiphospholipid Syndrome (APS—Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Arnaldo Toffon

    2013-01-01

    Full Text Available Antiphospholipid syndrome (APS is recognized as one of the main determinants of hypercoagulable conditions. The literature reports the incidence of this syndrome in a third of patients who underwent surgery for peripheral revascularization. Antiphospholipid antibodies are divided into two categories in relation to specific diagnostic tests. The first group is called lupus anticoagulant and consists of immunoglobulins that inhibit the phospholipid dependent coagulation tests in vitro. The second group is defined by their ability to conduct the phospholipid in an ELISA test. The occurrence of thrombotic events in patients with systemic erythematosus lupus (SEL and anticoagulant antibodies was described for the first time in 1963 by Bowie. The discovery of anti-cardiolipin antibodies in antiphospholipid syndrome is due to Harris et al. who described the syndrome. Primitive APS was consequently defined in the absence of further underlying illnesses. In this disease, arterial thrombosis occurs mainly in the brain. Peripheral arteries are affected less frequently. Thrombosis of the great vessels is reported as anecdotal.

  2. 偏头痛相关性脑卒中发病中抗磷脂抗体的作用%Relationship between antiphospholipid antibodies and migraine related cerebral infarction

    Institute of Scientific and Technical Information of China (English)

    刘昌勤; 解翠红; 孙圣刚

    2004-01-01

    目的:研究和探讨偏头痛与缺血性卒中的相关性以及抗磷脂抗体在偏头痛相关性卒中发病中的作用. 方法:运用问卷调查方式统计普通人群及脑梗死患者偏头痛的患病率,将脑梗死患者按有否偏头痛病史分为两组,用 ELISA方法分别测定其血清抗心磷脂抗体( anticardiolipin antibodies , ACA)水平. 结果:①脑梗死患者偏头痛的患病率与普通人群偏头痛的患病率分别为 20.0 %和 6.0%,二者之间差异有显著性意义(χ2=13.2671,P< 0.01).②脑梗死伴有偏头痛史者和无偏头痛史者,其 ACA阳性率分别为 41.7 %和 19.5 %,二者差异具有显著性意义(χ 2=5.0133,P< 0.05). 结论:偏头痛病史与脑梗死具有明显相关性.抗磷脂抗体可能参与偏头痛所相关的脑梗死的发病机制.%AIM:To study the relationship between migraine and cerebral infarction, and also to explore whether anticardiolipin antibodies(ACA) play a role in the mechanisms of migraine-related cerebral infarction. METHODS:Patients with cerebral infarction and control subjects received a questionnaire that is mainly relative to migraine history.Besides,the patients with cerebral infarction also received sera IgG-ACA detection of enzyme-linked immunoadsordent assay(ELISA). RESULTS:Twenty percent patients had a history of migraine,while only 6.0% of the control subjects had the incidence of migraine.They had statistically significant difference(χ 2=13.2671,P< 0.01) .The patients with cerebral infarction were divided into two groups:patients with migraine history and patients without migraine history.The positive rates of ACA in the stroke patients with migraine history and that of those without migraine history were 41.67% and 19.5% respectively. They also had statistically significant difference(χ 2=5.0133,P< 0.05) . CONCLUSION:The study suggested that migraine history was relative to cerebral infarction and ACA might play an important role in the mechanisms of migraine

  3. Neurosurgical management for complicated catastrophic antiphospholipid syndrome.

    Science.gov (United States)

    Drazin, Doniel; Westley Phillips, H; Shirzadi, Ali; Drazin, Noam; Schievink, Wouter

    2014-04-01

    Antiphospholipid syndrome (APS) is an autoimmune condition involving arterial and venous thrombosis. An unusual APS variant, catastrophic antiphospholipid syndrome (CAPS), includes rapid multi-organ failure from widespread small vessel thrombosis. Central nervous system complications arise in one-third of CAPS patients. In rare cases, CAPS co-manifests with cerebellar hemorrhage presenting a neurosurgical emergency. We present a 65-year-old woman with CAPS-related cerebellar hematoma, co-morbid idiopathic thrombocytopenic purpura, deep vein thrombosis and altered mental status, with treatment complicated by thrombocytopenia. The patient suddenly deteriorated, secondary to a cerebellar subdural hematoma, and underwent decompression and excision of the hematoma. After recovery in the intensive care unit, she developed a new spontaneous epidural hematoma requiring additional surgery. Management of these patients is hematologically complex and often requires a multi-disciplinary team of physicians. This patient provides an important learning point for clinicians - consider CAPS when hemorrhage and thrombosis are present.

  4. Moyamoya disease associated with antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Mahmut Abuhandan

    2011-12-01

    Full Text Available Moyamoya (MMD is a disease that often involves the vascular structures of anterior cerebral circulation, particularly the proximal segments of anterior and middle cerebral arteries. The etiology of the disease is unknown. MMD often presents with cerebral ischemia and rarely with cerebral hemorrhage. The pathology is termed Moyamoya syndrome (MMS when the pathological cerebral angiography findings are accompanied by meningitis, neurofibromatosis, neoplasm, Down syndrome or polycystic kidney disease. Autoimmune diseases including Graves’ disease, Behcet’s disease and antiphospholipid syndrome might also lead to the development of MMS. In this manuscript, we presented an interesting case of MMD associated with antiphospholipid syndrome, which is quite a rare cause of acute cerebral infarction in childhood

  5. The coexistence of antiphospholipid syndrome and systemic lupus erythematosus in Colombians.

    Directory of Open Access Journals (Sweden)

    Juan-Sebastian Franco

    Full Text Available OBJECTIVES: To examine the prevalence and associated factors related to the coexistence of antiphospholipid syndrome (APS and systemic lupus erythematosus (SLE in a cohort of Colombian patients with SLE, and to discuss the coexistence of APS with other autoimmune diseases (ADs. METHOD: A total of 376 patients with SLE were assessed for the presence of the following: 1 confirmed APS; 2 positivity for antiphospholipid (aPL antibodies without a prior thromboembolic nor obstetric event; and 3 SLE patients without APS nor positivity for aPL antibodies. Comparisons between groups 1 and 3 were evaluated by bivariate and multivariate analysis. RESULTS: Although the prevalence of aPL antibodies was 54%, APS was present in just 9.3% of SLE patients. In our series, besides cardiovascular disease (AOR 3.38, 95% CI 1.11-10.96, p = 0.035, pulmonary involvement (AOR 5.06, 95% CI 1.56-16.74, p = 0.007 and positivity for rheumatoid factor (AOR 4.68, 95%IC 1.63-14.98, p = 0.006 were factors significantly associated with APS-SLE. APS also may coexist with rheumatoid arthritis, Sjögren's syndrome, autoimmune thyroid diseases, systemic sclerosis, systemic vasculitis, dermatopolymyositis, primary biliary cirrhosis and autoimmune hepatitis. CONCLUSIONS: APS is a systemic AD that may coexist with other ADs, the most common being SLE. Awareness of this polyautoimmunity should be addressed promptly to establish strategies for controlling modifiable risk factors in those patients.

  6. [Pathogenesis and Laboratory Findings in Antiphospholipid Syndrome, Especially Associated with Lupus Anticoagulant].

    Science.gov (United States)

    Ieko, Masahiro; Naito, Sumiyoshi; Yoshida, Mika; Takahashi, Nobuhiko

    2015-10-01

    Antiphospholipid syndrome (APS), an acquired thrombotic condition, is a complex clinical state characterized by the presence of circulating antiphospholipid antibodies in patients with thrombosis or pregnancy morbidity. Revised APS classification criteria are used for diagnosis, which include at least one clinical criterion (thrombosis or pregnancy loss) and at least one of the laboratory criteria [anticardiolipin antibodies, anti-β2GPI antibodies, lupus anticoagulant (LA)]. LA is also an independent risk factor for developing thrombosis, though some LA-positive cases have been reported to have a bleeding symptom. Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare disorder characterized by a bleeding tendency due to low prothrombin activity in patients with LA, and has recently been reported not only in children but also in adults We have encountered LA cases with bleeding and low coagulation factor activities except for prothrombin. Based on our findings, we propose that LA-positive cases with a bleeding symptom and characterized by low coagulation factor activity including prothrombin be termed lupus anticoagulant-associated coagulopathy (LAAC). Furthermore, coagulation factor autoantibodies are often detected in LAAC patients; thus, correct measurement of LA is important to distinguish LAAC patients from those possessing an inhibitor to coagulation factors such as acquired hemophilia A as well as to select the optimal therapeutic strategy.

  7. The Role of Complement Inhibition in Thrombotic Angiopathies and Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Doruk Erkan

    2016-03-01

    Full Text Available Antiphospholipid syndrome (APS is characterized by thrombosis (arterial, venous, small vessel and/or pregnancy morbidity occurring in patients with persistently positive antiphospholipid antibodies (aPL. Catastrophic APS is the most severe form of the disease, characterized by multiple organ thromboses occurring in a short period and commonly associated with thrombotic microangiopathy (TMA. Similar to patients with complement regulatory gene mutations developing TMA, increased complement activation on endothelial cells plays a role in hypercoagulability in aPL-positive patients. In mouse models of APS, activation of the complement is required and interaction of complement (C 5a with its receptor C5aR leads to aPL-induced inflammation, placental insufficiency, and thrombosis. Anti-C5 antibody and C5aR antagonist peptides prevent aPL-mediated pregnancy loss and thrombosis in these experimental models. Clinical studies of anti-C5 monoclonal antibody in aPL-positive patients are limited to a small number of case reports. Ongoing and future clinical studies of complement inhibitors will help determine the role of complement inhibition in the management of aPL-positive patients.

  8. Lung Pathology in Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    T. M. Reshetnyak

    2005-01-01

    Full Text Available Functional lesions of organs depend on the size of a diseased vessel and frequently require the use of intensive therapy methods. The commonest manifestation of antiphospholipid syndrome (APS is deep vein thrombosis of the leg and pulmonary thromboembolism (PTE.Objective: to estimate the frequency of lung lesions in primary APS (PAPS, secondary (in the presence of systemic lupus erythematosus (SLE and catastrophic APS and to assess a relationship between lung pathology and other clinical and laboratory manifestations of the disease.Subjects and methods. The study covered 372 patients followed up at the Institute of Rheumatology, Russian Academy of Medical Sciences, since 1990, of whom 290 and 82 patients had SLE and PAPS, respectively. Among the 290 patients with SLE, there were 96 males and 194 females. At the moment of the study, the patients’ age was 31.2±11.1 years and the duration of the disease was 8.6±7.2 years. The group of patients with PAPS comprised 20 males and 62 females. Their mean age was 35.6±9.9 years and the duration of the disease was 11.9±8.5 years. Thrombotic events were verified only by instrumental studies. Lung pathology was instrumentally confirmed; all the patients underwent lung X-ray study, if required, scintigraphy and computed tomography.Results. Lung lesion associated with the pathology of vessels was revealed in 28% of the examined patients (105/372. There were prevalent patients with PTE, followed by the development of lung infarcts, which was present in 96 (91% of the 105 patients with pulmonary vascular pathology. Autopsy revealed pulmonary microangiopathy was in 12 patients, which was concurrent with focal pneumonia in 7 of them, with pneumonitis and exudative pleuritis in 5. Hemorrhagic alveolitis detected at autopsy in combination with occlusions of the pulmonary arterioles was in 3 patients who had been diagnosed as having thromboembolism of small branches of the pulmonary artery. Thrombosis of the

  9. The chequered history of the antiphospholipid syndrome.

    Science.gov (United States)

    Jayakody Arachchillage, Deepa; Greaves, Mike

    2014-06-01

    Consideration of the chronology of advances in medical knowledge can provide useful insights into the pathogenesis, diagnosis and treatment of diseases. The antiphospholipid syndrome is an enigmatic disorder and this is reinforced by the misleading associated terminology, the adoption of which results directly from early discoveries relating to the condition. Thus the target antigen of the causative autoantibodies in antiphospholipid syndrome does not reside on phospholipid, and the frequently associated lupus anticoagulant is not restricted to subjects with systemic lupus erythematosus and, paradoxically, despite causing prolongation of clotting times in vitro it is associated with a pronounced tendency to thrombosis. Recognition of the antiphospholipid syndrome has its origins in the identification of subjects with so-called biological false-positive serological reactions for syphilis in the middle years of the last century. Since that time there have been considerable advances in our understanding of the pathogenesis of the disease and the clinical manifestations and associations, improved diagnostic accuracy and an evolving evidence base for optimal therapy. However many gaps in our knowledge remain.

  10. New developments in lupus-associated antiphospholipid syndrome

    NARCIS (Netherlands)

    Lockshin, M. D.; Derksen, R. H. W. M.

    2008-01-01

    Systemic lupus erythematosus is the disease in which the antiphospholipid syndrome was first described more than 20 years ago and which is the most frequent underlying disorder in secondary antiphospholipid syndrome. With respect to pathogenic concepts and treatment, the subjects of this review, no

  11. Basilar artery thrombosis in the setting of antiphospholipid syndrome.

    Science.gov (United States)

    Saad, Amin F; Nickell, Larry T; Heithaus, R Evans; Shamim, Sadat A; Opatowsky, Michael J; Layton, Kennith F

    2014-07-01

    Antiphospholipid syndrome is an autoimmune disorder characterized by arterial or venous thrombosis, recurrent first-trimester pregnancy loss, and multiple additional clinical manifestations. We describe a man with severe atherosclerotic basilar artery stenosis and superimposed in situ thrombus who was found to have antiphospholipid syndrome.

  12. Proinflammatory proteins in female and male patients with primary antiphospholipid syndrome: preliminary data.

    Science.gov (United States)

    Bećarević, Mirjana; Ignjatović, Svetlana

    2016-10-01

    The latest classification criteria for the diagnosis of the antiphospholipid syndrome (APS, an autoimmune disease characterized by thromboses, miscarriages and presence of antiphospholipid antibodies (Abs)) emphasized that thrombotic manifestations of APS should be without any signs of an inflammatory process. However, atherosclerosis (a chronic inflammatory response to the accumulation of lipoproteins in the walls of arteries) and APS are characterized by some similar features. We evaluated whether proinflammatory proteins were associated with the features of the primary APS (PAPS). PAPS patients without obstetric complications and with impaired lipid profile were included in the study. Antiphospholipid antibodies, TNF-alpha, and apo(a) were determined by ELISA. Complement components and hsCRP were measured by immunonephelometry. Decreased C3c was observed in female patients with increased titers of IgG anti-β2gpI (χ(2) = 3.939, P = 0.047) and in male patients with increased IgM anticardiolipin Abs (χ(2) = 4.286, P = 0.038). Pulmonary emboli were associated with interleukin (IL)-6 in male (χ(2) = 6.519, P = 0.011) and in female (χ(2) = 10.405, P = 0.001) patients. Cerebrovascular insults were associated with LDL-cholesterol (P = 0.05, 95 % CI: 1.003 - 12.739) in female and with apo(a) (P = 0.016, 95 % CI: 0.000-0.003) in male patients. Older female patients had increased LDL-cholesterol levels and frequency of myocardial infarctions. Proinflammatory proteins were associated with features of primary APS. No real gender differences in regard to proinflammatory protein levels were observed. Premenopausal state of female PAPS patients confers lower cardiovascular risk.

  13. An outdated notion of antibody specificity is one of the major detrimental assumptions of the structure-based reverse vaccinology paradigm which prevented it from helping to develop an effective HIV-1 vaccine

    Directory of Open Access Journals (Sweden)

    Marc H V Van Regenmortel

    2014-11-01

    Full Text Available The importance of paradigms for guiding scientific research is explained with reference to the seminal work of Karl Popper and Thomas Kuhn. A prevalent paradigm, followed for more than a decade in HIV-1 vaccine research, which gave rise to the strategy known as structure-based reverse vaccinology is described in detail. Several reasons why this paradigm did not allow the development of an effective HIV-1 vaccine are analyzed. A major reason is the belief shared by many vaccinologists that antibodies possess a narrow specificity for a single epitope and are not polyspecific for a diverse group of potential epitopes. When this belief is abandoned, it becomes obvious that the one particular epitope structure observed during the crystallographic analysis of a neutralizing antibody-antigen complex does not necessarily reveal which immunogenic structure should be used to elicit the same type of neutralizing antibody.In the physical sciences, scientific explanations are usually presented as logical deductions derived from a relevant law of nature together with certain initial conditions. In immunology, causal explanations in terms of a single cause acting according to a law of nature are not possible because numerous factors always play a role in bringing about an effect. The implications of this state of affairs for the rational design of HIV vaccines are outlined. An alternative approach to obtain useful scientific understanding consists in intervening empirically in the immune system and it is suggested that manipulating the system experimentally is needed to learn to control it and achieve protective immunity by vaccination.

  14. Antiphospholipid Syndrome and Atherosclerosis%抗磷脂综合征与动脉粥样硬化

    Institute of Scientific and Technical Information of China (English)

    张瑗; 刘永华; 钱晓明

    2010-01-01

    @@ 抗磷脂综合征(antiphospholipid syndrome,APS)是一组以反复发作动、静脉血栓形成、习惯性流产和血小板减少为临床表现,伴有抗磷脂抗体(antiphospholipid antibody,APA)阳性的一种非器官特异性自身免疫性疾病.临床又称为抗磷脂-血栓形成综合征.APS分为原发性APS(primary APS,PAPS)、继发性APS(secondary APS,SAPS)和较少见的恶性抗磷脂综合征(catastrophic APS,CAPS),后者表现为短期内进行性广泛血栓形成,造成多器官功能衰竭甚至死亡[1].

  15. Primary biliary cirrhosis--autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

    Science.gov (United States)

    Pamfil, Cristina; Candrea, Elisabeta; Berki, Emese; Popov, Horațiu I; Radu, Pompilia I; Rednic, Simona

    2015-03-01

    Autoimmune liver diseases may be associated with extrahepatic autoimmune pathology. We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels. Immunological tests revealed positive antinuclear (ANA=1:320) and antimitochondrial antibodies (AMA=1:40) with negative anti-smooth muscle and liver kidney microsomal type 1 antibodies. The biopsy was compatible with overlap syndrome type 1. The patient was commenced on immunosuppressive therapy according to standard of care (azathioprine 50mg, ursodeoxycholic acid and prednisone 0.5mg/kg), with moderate biochemical improvement. She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine. At the three-month follow-up, her symptoms subsided and aminotransferases and muscle enzymes normalized. Upon further investigation the patient was diagnosed with autoimmune thyroiditis and antiphospholipid syndrome. To our knowledge, this is the first case of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

  16. Enfermedad celiaca asociada a síndrome antifosfolípido Celiac disease associated with antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    O. Jorge

    2008-02-01

    Full Text Available Introducción: la enfermedad celiaca puede asociarse a patologías de etiología inmunológica. Presentamos su asociación con síndrome antifosfolípido. Caso 1: mujer, 26 años, diagnosticada de enfermedad celiaca. Seis meses después queda embarazada, presentando muerte fetal. Al año siguiente nuevo embarazo. Anticuerpos anticardiolipina IgG: 20 GPL U/ml (valor normal Introduction: celiac disease may be associated with pathologies of immune etiology. We present its association with antiphospholipid syndrome. Case 1: a 26-year-old female was diagnosed with celiac disease. Six months later she became pregnant, and experienced fetal death. The following year she became pregnant again. IgG anticardiolipin antibodies: 20 GPL U/ml (normal value < 11, and IgM anticardiolipin antibodies: 9 MPL U/ml (n. v. < 10. Hematological tests were otherwise uneventful. Medicated with acetylsalicylic acid she had a normal pregnancy. Case 2: a 48-year-old female diagnosed with celiac disease presented with thrombosis in her left lower limb and renal infarction. Hematological tests showed no prothrombotic alterations (antiphospholipid antibodies were not measured. A year and a half later she had thrombosis in a finger of her hand. IgG anticardiolipin antibodies: 10 GPL (n. v. < 13, and IgM anticardiolipin antibodies: 35 MPL (n. v. < 12. Case 3: a 38-year-old female was diagnosed with celiac disease. Some time later she experienced two spontaneous abortions and a transient ischemic cerebral attack. Nowadays, she is in her sixth month of pregnancy. IgM anticardiolipin antibodies: 75 MPL/ml (n. v. up to 20, and IgG anticardiolipin antibodies within normal values. Hematological tests revealed no other prothrombotic alterations. Discussion: antiphospholipid syndrome is characterized by arterial and venous thrombosis, and spontaneous fetal death. Its association with celiac disease has been described in few cases. Celiac disease is associated with spontaneous fetal

  17. Fulminant antiphospholipid antibody syndrome complicated by Aspergillus tracheobronchitis.

    Science.gov (United States)

    Yegneswaran Prakash, Peralam; Pandit, Vinay; Rao, Sugandhi P

    2012-01-01

    Aspergillus fumigatus is a filamentous mold that causes infections in patients who are inmmunocompromised. We report a case of Aspergillus tracheobronchitis in fulminant systemic lupus erythematosus case. Diagnosis with more invasive diagnostic procedures & aggressive antifungal therapy is indicated at early stage.

  18. Investigation of Antiphosphatidyl-Serine Antibody and Antiphosphatidyl-Inositol Antibody in Ischemic Stroke Patients

    Directory of Open Access Journals (Sweden)

    Hirohisa Okuma

    2010-01-01

    Full Text Available Antiphospholipid syndrome is characterized by arterial or venous thrombosis and the presence of antiphospholipid antibodies (aPL. We measured β2-GPI aCL, IgGaCL, LA, antiphosphatidyl-serine antibody (PS, and antiphosphatidyl-inositol antibody (PI in each patient at one month after the onset of stroke. In addition, carotid artery echography was performed in patients positive for PI or PS. Among the 250 patients, 13.6% (34/250 were positive for either PI or PS, and 6.8% (17/250 were positive for both. Carotid artery echography performed on these 34 patients showed that the frequencies of increased intimal-medial thickness (IMT of 1.1 mm or more, plaque, and carotid artery stenosis of 50% or more were all significantly higher in patients positive for antinuclear antibody than those negative for the antibody (P<.05. PI and PS are associated with antinuclear antibody and precipitation of atherosclerosis. Ischemic stroke patients with SLE frequently showed a variety of antiphospholipid-protein antibodies.

  19. Living donor renal transplantation in patients with antiphospholipid syndrome

    Science.gov (United States)

    Choi, Ji Yoon; Jung, Joo Hee; Shin, Sung; Kim, Young Hoon; Han, Duck Jong

    2016-01-01

    Abstract Introduction: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. Clinical Findings: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin. However, he frequently experienced recurrent DVT despite of anticoagulation therapy. Before the surgery, APS was confirmed based on positive results lupus anticoagulant in serological tests. A 40-year-old man with polycystic kidney disease and a history recurrent DVT tested positive for lupus anticoagulant and anticardiolipin antibodies. Lastly, a 42-year-old woman with ESRD was diagnosed with APS 7 years ago. She also developed DVT and tested positive for lupus anticoagulant and anti-B2-glycoprotein 1. The anticoagulation protocol was as follows in all cases: Warfarin was stopped 5 days before living donor renal transplantation and intravenous heparin therapy was started. During surgery, bolus heparin injections (3000 U) were administered to prevent arterial or venous thrombosis. Heparin was substituted with warfarin on postoperative day 4. The third patient (42/F) developed clinical rejection indicated by increased serum creatinine levels and donor-specific antibodies (DSA) and received steroid pulse therapy, plasmapheresis, and rituximab. This treatment restored graft function to within the normal range. The latest graft function in all patients was maintained at normal levels in the outpatient clinic. Conclusions: Living donor renal transplantation may be successful in patients with APS following perioperative anticoagulation therapy. However, because of the high risk of

  20. Significance of fully automated tests for the diagnosis of antiphospholipid syndrome.

    Science.gov (United States)

    Oku, Kenji; Amengual, Olga; Kato, Masaru; Bohgaki, Toshiyuki; Horita, Tetsuya; Yasuda, Shinsuke; Sakamoto, Naoya; Ieko, Masahiro; Norman, Gary L; Atsumi, Tatsuya

    2016-10-01

    Antiphospholipid antibodies (aPLs) can vary both immunologically and functionally, thus it is important to effectively and correctly identify their presence when diagnosing antiphospholipid syndrome. Furthermore, since many immunological/functional tests are necessary to measure aPLs, complete examinations are often not performed in many cases due to significant burden on the testing departments. To address this issue, we measured aPLs defined according to the classification criteria (anticardiolipin antibody: aCL) IgG/IgM and anti-β2 glycoprotein I antibody (aβ2GPI) (IgG/IgM) as well as non-criteria antibodies (aCL IgA, aβ2GPI IgA and aβ2GPI domain I), in a cohort of 211 patients (61 APS, 140 disease controls and 10 healthy individuals). APLs were measured using a fully automated chemiluminescent immunoassay instrument (BIO-FLASH®/ACL AcuStar®) and with conventional ELISA tests. We demonstrated that both sensitivity and accuracy of diagnosis of aCL IgG and aβ2GPI IgG were high, in agreement with the past reports. When multiple aPLs were examined, the accuracy of diagnosis increased. The proportion of APS patients that were positive for 2 or more types of aPLs (47/61, 77%) was higher than that of patients with systemic lupus erythematosus (SLE)(3/37, 9%), those with non-SLE connective tissues diseases (1/53,2%), those with other diseases or healthy volunteers. Based on these findings, it was concluded that the fully automated chemiluminescent immunoassay instrument, which allows the simultaneous evaluation of many types of aPLs, offers clear advantages for a more complete, more rapid and less labor-intensive alternative to running multiple ELISA and could help in better diagnosis for suspected APS patients.

  1. [Diagnosis and potential treatment of antiphospholipid syndrome-related mainly on ischemic stroke].

    Science.gov (United States)

    Okuma, Hirohisa; Kitagawa, Yasuhisa

    2013-11-01

    Antiphospholipid syndrome (APS) was defined in 2006 by an international consensus as an autoimmune disease that manifests clinically as recurrent thrombotic complications or fetal loss and serologically as elevated plasma levels of antiphospholipid antibodies (aPLs). aPLs are a heterogeneous group of antibodies directed against anionic phospholipids, phospholipid-binding plasma proteins, and phospholipid-protein complexes. Standard ELISA for anticardiolipin (aCL) and anti-β2-glycoprotein I antibodies and clotting assays for lupus anticoagulant (LA) are recommended for detecting aPLs. Phosphatidylserine-dependent anti-prothrombin antibody (aPS/PT) assay may also be useful as a confirmatory test for APS. aPLs are an independent risk factor for initial occurence of ischemic stroke, especially in young adults. APS patients with thrombotic stroke frequently have other, often conventional, vascular risk factors. Guidelines issued in 2011 by the American Heart Association and American Stroke Association recommended antiplatelet therapy for patients with cryptogenic ischemic stroke or TIA and who test positive for aPL. In contrast, oral anticoagulants with a target international normalized ratio (INR) of 2.0-3.0 are recommended for patients with ischemic stroke who meet all the criteria for APS. Recently, 3 new anticoagulants for stroke prevention, dabigatran, rivaroxaban, and apixaban, have been studied in phase 3 clinical trials in patients with atrial fibrillation. However, optimal treatment for catastrophic APS is unknown. Current treatment guidelines emphasize the importance of early diagnosis and recommend aggressive therapies to avoid a fatal outcome. Combinations of high doses of intravenous heparin, steroids, and immunoglobulins and/or repeated plasma exchanges can be considered as treatments of choice for this severe condition.

  2. Antiphospholipid syndrome (APS) revisited: Would migraine headaches be included in future classification criteria?

    Science.gov (United States)

    Noureldine, Mohammad Hassan A; Haydar, Ali A; Berjawi, Ahmad; Elnawar, Rody; Sweid, Ahmad; Khamashta, Munther A; Hughes, Graham R V; Uthman, Imad

    2016-07-16

    Headaches have been extensively reported in Antiphospholipid syndrome (APS)/Antiphospholipid antibodies (aPL)-positive patients. The aim of this study was to highlight the prevalence of headaches among APS/aPL-positive patients and discuss its association with laboratory, clinical and imaging findings. We searched the literature through Google Scholar and PubMed for publications on the epidemiology, pathogenesis, laboratory, imaging and clinical findings, and management of headaches in APS/aPL-positive patients. The following keywords were used: Antiphospholipid, Hughes syndrome, anticardiolipin, lupus anticoagulant, anti-β2 glycoprotein I, headache, migraine, tension, and cluster. All reports published between 1969 and 2015 were included. Migraine is the most commonly reported type of headache in APS/aPL-positive patients. Thrombotic and platelet dysfunction hypotheses have been studied to uncover the pathogenic role of aPL in the development of headaches. Several studies are reporting higher levels of aPL in primary and secondary APS migraineurs, but only few reached statistical significance. Migraine patients without clinical signs/symptoms of cerebral infarction rarely show positive imaging findings. Digital subtraction angiography shows promise in demonstrating small vascular lesions otherwise not detected on computed tomography, magnetic resonance imaging, or cerebral angiograms. Although it may be solitary and harmless in many cases, the deleterious effect of migraine on the quality of life of APS patients prompts rapid diagnosis and proper management. An anticoagulation trial is advisable in APS patients with migraine as many cases of severe, refractory migraine resolved with anticoagulation therapy. The profile of migraine headaches discussed in this study permits its candidacy for inclusion in future APS classification criteria.

  3. Pediatric cerebellar stroke associated with elevated titer of antibodies to β2-glycoprotein.

    Science.gov (United States)

    Spalice, Alberto; Del Balzo, Francesca; Perla, Francesco Massimo; Papetti, Laura; Nicita, Francesco; Ursitti, Fabiana; Properzi, Enrico

    2011-06-01

    Antibodies to 2-glycoprotein I (anti-2GPI) have been associated with recurrent thrombosis and pregnancy morbidity. However, the prevalence of anti-2GPI in children suffering from cerebral and cerebellar infarction is unknown. We report on a 10-month-old boy who had an ischemic cerebellar stroke, secondary to antiphospholipid syndrome with high titers of immunoglobulin G anti-2GPI (first titer: 132U) anticardiolipin antibodies and lupus anticoagulant tests were negative. All other causes of infarction were excluded. To our knowledge, this is the first reported case of childhood cerebellar ischemic stroke with only anti-2GPI but no antibodies detectable in standard antiphospholipid assays.

  4. Detection of multiple annexin autoantibodies in a patient with recurrent miscarriages, fulminant stroke and seronegative antiphospholipid syndrome.

    Science.gov (United States)

    Scholz, Philipp; Auler, Markus; Brachvogel, Bent; Benzing, Thomas; Mallman, Peter; Streichert, Thomas; Klatt, Andreas R

    2016-01-01

    Anti-phospholipid syndrome (APS) is one of the main causes for recurrent miscarriages. The diagnosis of APS is based on the occurrence of clinical symptoms such as thrombotic events or obstetric complications as well as the detection of antiphospholipid antibodies directed against β2-glycoprotein I and cardiolipin, or a positive lupus anticoagulant assay. However, there is a subpopulation of patients with clinical symptoms of APS, but the lack of serological markers (seronegative APS). In addition, a large proportion of patients with unexplained recurrent miscarriages exist. These cases may be attributed, at least in part, to a seronegative APS.
The presence of autoantibodies against annexins is potentially associated with APS. Here we used immunoassays and immunoblots to detect autoantibodies directed against annexin A1-5, and A8, respectively, in a patient with a seronegative APS and a history of six recurrent pregnancy losses and fulminant stroke. We found strong IgM isotype antibody reactivity directed against annexin A2 and annexin A8, and moderate to weak IgM isotype antibody reactivity directed against annexin A1, A3, and A5. Further studies will evaluate the diagnostic value of IgM isotype antibodies against annexin A1-A5, and A8 for seronegative APS and recurrent miscarriages.

  5. Antithyroglobulin antibody

    Science.gov (United States)

    Thyroglobulin antibody; Thyroiditis - thyroglobulin antibody; Hypothyroidism - thyroglobulin antibody; Thyroiditis - thyroglobulin antibody; Graves disease - thyroglobulin antibody; Underactive thyroid - thyroglobulin antibody

  6. Progress of Research with Antiphospholipid Syndrome%抗磷脂综合征的研究进展

    Institute of Scientific and Technical Information of China (English)

    李坦; 吴竞生

    2008-01-01

    抗磷脂综合征(antiphospholipid syndrome,APS)是一组以反复发生动脉、静脉血栓或和习惯性流产为临床表现,伴持续性抗磷脂抗体(antiphospholipid antibody APL)或抗132糖蛋白Ⅰ(β2GPI)抗体阳性,且多系统受累的非炎症性自身免疫性疾病,又称抗磷脂-血栓综合征 (antiphospholipid- thrombosis syndrome , APE-T)。 APS 分原发性和继发性两类,前者无任何诱因,后者多继发于系统性红斑狼疮(systemic lupus erythematosus,SLE)、类风湿性关节炎、干燥综合征等结缔组织疾病,也可继发于各种感染与肿瘤等。

  7. Heart Failure with Multi-organ Thrombosis: A Case of Antiphospholipid Syndrome Co-existing with Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Xueqi Li and Shipeng Wei

    2012-09-01

    Full Text Available Antiphospholipid Syndrome (APS is an autoimmune disease featured by venous or arterial thrombosis, fetal losses and thrombocytopenia in the presence of antiphospholipid antibodies. Here we reported one case of antiphospholipid syndrome together with dilated cardiomyopathy. A 46-year-old female patient complaining short of breath was found enlargement of atrial and ventricular compartments. The ecletrocardiogram and blood test revealed anteroseptal myocardial infarction, while no pulmonary thrombosis was present and therefore diagnosis of dilated cardiomyopathy was made. There were also thrombi formed in the cardial chambers and deep venous. During hospitalization, there was an onset of ischemic brain stroke and head MRI showed newly developed small infarctions. An elevation of anticardiolipin immunoglobulin A (ACAIGA was detected from the blood sample. The patient was discharged after being treated with anticoagulant, corticosteroid and other medicines for improving heart function. In our case, APS is the basic cause leading to multi-organ thrombosis and heart failure is mainly due to dilated cardiomyopathy, thus independent of APS. So this is the first time that cardiomyopathy co-existing with APS was reported.

  8. Polimorfismo Val247Leu do gene β2-glicoproteína 1 pode justificar a gênese de anticorpos antiβ2GP1 e síndrome do anticorpo antifosfolípide na hanseníase multibacilar Val247Leu polymorphism of β2 glycoprotein 1 gene may justify the genesis of anti β2GP1 antibodies and Antiphospholipid Syndrome in Multibacillary Leprosy

    Directory of Open Access Journals (Sweden)

    Maria José Franco Brochado

    2009-08-01

    Full Text Available FUNDAMENTOS - Anticorpos antifosfolípides (AAF, como antiβ2GP1 (β2-glicoproteína 1, são descritos na hanseníase multibacilar (MB sem, contudo, caracterizar a síndrome do anticorpo antifosfolípide (SAF, constituída por fenômenos tromboembólicos (FTE. A mutação Val247Leu no V domínio da β2GP1 - substituição da leucina por valina - expõe epítopos crípticos com consequente formação de anticorpos antiβ2GP1. OBJETIVO: Avaliar a associação do polimorfismo Val247Leu do gene β2GP1 com títulos de anticorpos antiβ2GP1 na hanseníase. MÉTODO: O polimorfismo Val247Leu foi detectado por PCR-RFLP, e os títulos de anticorpos antiβ2GP1, por Elisa. RESULTADOS: O genótipo Val/Val estatisticamente predominou no grupo de hansênicos, em relação ao controle. Embora maiores títulos de anticorpos antiβ2GP1 IgM estivessem alocados no grupo MB com genótipos Val/Val e Val/Leu, não houve diferença estatística em relação ao genótipo Leu/Leu. Dos sete pacientes MB com FTE, quatro apresentaram heterozigose, e três Val/Val homozigose. CONCLUSÃO: A prevalência do genótipo Val/Val no grupo de hansênicos pode justificar parcialmente a presença de anticorpos antiβ2GP1 na forma MB. A heterozigose ou homozigose Val/Val nos sete pacientes com hanseníase MB e FTE corroboram a implicação de expressão fenotípica anômala da β2GPl e formação de anticorpos antiβ2GPl, com consequente FTE e SAF.BACKGROUND - Multibacillary (MB leprosy may be manifested with antiphospholipid antibodies (aPL, among which anti-β2GP1 (β2-glycoprotein 1. High titers of aPL are associated with APS (Antiphospholipid Syndrome, characterized by thrombosis. The mutation Val247Leu in the domain V of β2GP1 exposes hidden epitopes with consequent development of anti-β2GP1 antibodies. OBJECTIVE: To evaluate the Val247Leu polymorphism of β2GP1 gene and its correlation with anti-β2GP1 antibodies in leprosy patients. METHODS: The Val247Leu polymorphism was

  9. Cardiac manifestations in antiphospholipid syndrome - a brief review of the literature

    Directory of Open Access Journals (Sweden)

    Đoković Aleksandra

    2015-01-01

    Full Text Available Antiphospholipid syndrome (APS or Hughes syndrome represents a systemic autoimmune disorder characterized by arterial and/or venous thrombosis, multiple and recurrent fetal losses, accompanied by persistently elevated levels of antiphospholipid antibodies (aPL. This syndrome is considered primary if unassociated with any other connective tissue disease, or secondary if it appears in association with other autoimmune disorders, mainly systemic lupus erythematosus. Cardiac manifestations in APS are integral part of the syndrome. aPL are involved in the pathogenesis of pseudoinfective endocarditis (Libman Sacks and other valvular manifestations presented as their thickening and dysfunction. Intracardiac thrombi and myxomas, pulmonary hypertension and left ventricular dysfunction are also distinguishing features of APS. On the other hand, accelerated atherosclerosis, proven in APS and also aPL mediated, is accountable for the development of coronary and peripheral artery disease. This leads to higher cardiovascular mortality rate in the population of patients with low incidence of the traditional atherosclerosis risk factors. Furthermore, recent studies implied that presence of certain aPL could be a risk factor for a specific cardiac manifestation. Bearing all this in mind, early diagnosis of cardiac manifestations, control and abolition of traditional risk factors, as well as close cardiac follow-up of APS patients, are crucial in reducing their cardiovascular mortality.

  10. The role of TLR4 in pathophysiology of antiphospholipid syndrome-associated thrombosis and pregnancy morbidity.

    Science.gov (United States)

    Xie, Hongxiang; Sheng, Liangju; Zhou, Hong; Yan, Jinchuan

    2014-01-01

    The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the clinical features of recurrent thrombosis in the venous or arterial circulation and fetal losses. Antiphospholipid antibodies (aPL), particularly against the phospholipid binding protein beta-2 glycoprotein I (β2GPI), play an important role in APS pathological mechanisms. aPL can activate intracellular signal transduction in a β2GPI-dependent manner to induce inflammatory responses, and promote hypercoagulable state and recurrent spontaneous abortion when β2GPI is associated with the cell surface receptor. In vivo and in vitro studies show that Annexin A2 (ANXA2) is the high affinity receptor that connects β2GPI to the target cells. However, ANXA2 is not a transmembrane protein and lacks an intracellular signal transduction pathway. Growing evidences suggest that the transmembrane protein toll-like receptor 4 (TLR4) might act as an 'adaptor' for intracellular signal transduction. This review focuses on the role of TLR4 and its signalling pathway in APS pathological mechanisms which will help us better understand the pathological processes of this syndrome.

  11. Treatment of Thrombotic Antiphospholipid Syndrome: The Rationale of Current Management-An Insight into Future Approaches.

    Science.gov (United States)

    Chighizola, Cecilia Beatrice; Ubiali, Tania; Meroni, Pier Luigi

    2015-01-01

    Vascular thrombosis and pregnancy morbidity represent the clinical manifestations of antiphospholipid syndrome (APS), which is serologically characterized by the persistent positivity of antiphospholipid antibodies (aPL). Antiplatelet and anticoagulant agents currently provide the mainstay of APS treatment. However, the debate is still open: controversies involve the intensity and the duration of anticoagulation and the treatment of stroke and refractory cases. Unfortunately, the literature cannot provide definite answers to these controversial issues as it is flawed by many limitations, mainly due to the recruitment of patients not fulfilling laboratory and clinical criteria for APS. The recommended therapeutic management of different aPL-related clinical manifestations is hereby presented, with a critical appraisal of the evidence supporting such approaches. Cutting edge therapeutic strategies are also discussed, presenting the pioneer reports about the efficacy of novel pharmacological agents in APS. Thanks to a better understanding of aPL pathogenic mechanisms, new therapeutic targets will soon be explored. Much work is still to be done to unravel the most controversial issues about APS management: future studies are warranted to define the optimal management according to aPL risk profile and to assess the impact of a strict control of cardiovascular risk factors on disease control.

  12. Treatment of Thrombotic Antiphospholipid Syndrome: The Rationale of Current Management—An Insight into Future Approaches

    Directory of Open Access Journals (Sweden)

    Cecilia Beatrice Chighizola

    2015-01-01

    Full Text Available Vascular thrombosis and pregnancy morbidity represent the clinical manifestations of antiphospholipid syndrome (APS, which is serologically characterized by the persistent positivity of antiphospholipid antibodies (aPL. Antiplatelet and anticoagulant agents currently provide the mainstay of APS treatment. However, the debate is still open: controversies involve the intensity and the duration of anticoagulation and the treatment of stroke and refractory cases. Unfortunately, the literature cannot provide definite answers to these controversial issues as it is flawed by many limitations, mainly due to the recruitment of patients not fulfilling laboratory and clinical criteria for APS. The recommended therapeutic management of different aPL-related clinical manifestations is hereby presented, with a critical appraisal of the evidence supporting such approaches. Cutting edge therapeutic strategies are also discussed, presenting the pioneer reports about the efficacy of novel pharmacological agents in APS. Thanks to a better understanding of aPL pathogenic mechanisms, new therapeutic targets will soon be explored. Much work is still to be done to unravel the most controversial issues about APS management: future studies are warranted to define the optimal management according to aPL risk profile and to assess the impact of a strict control of cardiovascular risk factors on disease control.

  13. Seizures in Primary Antiphospholipid Syndrome: The Relevance of Smoking to Stroke

    Directory of Open Access Journals (Sweden)

    Jozélio Freire de Carvalho

    2012-01-01

    Full Text Available Objectives. To evaluate the frequency of seizures in primary antiphospholipid syndrome (PAPS and their possible clinical and laboratory associations. Methods. 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. Results. 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%, =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 (>0.05. Comparison between patients with seizures onset after PAPS diagnosis (=7 and those without convulsions (=79 demonstrated a higher frequency of current smoking (42.9 versus 10%, =0.042 and stroke in the first group (71.4 versus 30.4%, =0.041. Regression analysis confirmed that smoking (=0.030 and stroke (=0.042 were independently associated to seizures. Conclusion. About 10.2% of PAPS patients had convulsions, predominantly after PAPS diagnosis, and seizures were associated to current smoking and stroke.

  14. TNF-alpha and annexin A2: inflammation in thrombotic primary antiphospholipid syndrome.

    Science.gov (United States)

    Bećarević, Mirjana

    2016-12-01

    Antiphospholipid syndrome (APS) is characterized by thromboses and/or pregnancy losses. Laboratory criterion for the diagnosis of APS is the presence of antiphospholipid antibodies (anticardiolipin, anti-beta2-glycoprotein I (aβ2gpI) and lupus anticoagulant). On the one hand, the latest classification criteria for the diagnosis of APS emphasized that thrombotic manifestations of the syndrome should be without any signs of an inflammatory process, while on the other hand, some recent reports have suggested that APS is a "pro-inflammatory state." This article is focused on the importance of TNF-alpha and annexin A2 (anxA2) for patients with vascular (thrombotic) manifestations of the primary APS. The classic antithrombotic and antiplatelet therapy does not protect APS patients from the development of recurrent thrombosis. Therefore, an urgent need for the introduction of new therapeutic approaches in the treatment of APS patients is obvious. This review provides a rationale for the necessity for the use of immunomodulatory medications that could interfere with β2gpI binding to its receptor(s), such as anxA2, and/or inhibit TNF-alpha activity.

  15. 'Non-Criteria' Neurologic Manifestations of Antiphospholipid Syndrome: A Hidden Kingdom to be Discovered.

    Science.gov (United States)

    Islam, Md Asiful; Alam, Fahmida; Kamal, Mohammad Amjad; Wong, Kah Keng; Sasongko, Teguh Haryo; Gan, Siew Hua

    2016-01-01

    Neurological manifestations or disorders associated with the central nervous system are among the most common and important clinical characteristics of antiphospholipid syndrome (APS). Although in the most recently updated (2006) APS classification criteria, the neurological manifestations encompass only transient ischemic attack and stroke, diverse 'non-criteria' neurological disorders or manifestations (i.e., headache, migraine, bipolar disorder, transverse myelitis, dementia, chorea, epileptic seizures, multiple sclerosis, psychosis, cognitive impairment, Tourette's syndrome, parkinsonism, dystonia, transient global amnesia, obsessive compulsive disorder and leukoencephalopathy) have been observed in APS patients. To date, the underlying mechanisms responsible for these abnormal neurological manifestations in APS remain unclear. In vivo experiments and human observational studies indicate the involvement of thrombotic events and/or high titers of antiphospholipid antibodies in the neuro-pathogenic cascade of APS. Although different types of neurologic manifestations in APS patients have successfully been treated with therapies involving anti-thrombotic regimens (i.e., anticoagulants and/or platelet antiaggregants), antineuralgic drugs (i.e., antidepressants, antipsychotics and antiepileptics) and immunosuppressive drugs alone or in combination, evidence-based guidelines for the management of the neurologic manifestations of APS remain unavailable. Therefore, further experimental, clinical and retrospective studies with larger patient cohorts are warranted to elucidate the pathogenic linkage between APS and the central nervous system in addition to randomized controlled trials to facilitate the discovery of appropriate medications for the 'non-criteria' neurologic manifestations of APS.

  16. Musculoskeletal manifestations of the antiphospholipid syndrome.

    Science.gov (United States)

    Noureldine, M H A; Khamashta, M A; Merashli, M; Sabbouh, T; Hughes, G R V; Uthman, I

    2016-04-01

    The scope of clinical and laboratory manifestations of the antiphospholipid syndrome (APS) has increased dramatically since its discovery in 1983, where any organ system can be involved. Musculoskeletal complications are consistently reported in APS patients, not only causing morbidity and mortality, but also affecting their quality of life. We reviewed all English papers on APS involvement in the musculoskeletal system using Google Scholar and Pubmed; all reports are summarized in a table in this review. The spectrum of manifestations includes arthralgia/arthritis, avascular necrosis of bone, bone marrow necrosis, complex regional pain syndrome type-1, muscle infarction, non-traumatic fractures, and osteoporosis. Some of these manifestations were reported in good quality studies, some of which showed an association between aPL-positivity and the occurrence of these manifestations, while others were merely described in case reports.

  17. Intravenous immunoglobulins and antiphospholipid syndrome: How, when and why? A review of the literature.

    Science.gov (United States)

    Tenti, Sara; Cheleschi, Sara; Guidelli, Giacomo Maria; Galeazzi, Mauro; Fioravanti, Antonella

    2016-03-01

    The antiphospholipid syndrome (APS) is defined by the occurrence of venous and arterial thromboses and recurrent fetal losses, frequently accompanied by a moderate thrombocytopenia, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or anti-β2 glycoprotein-I (β2GPI) antibodies. The current mainstay of treatment for thrombotic APS is heparin followed by long-term anticoagulation, while in obstetric APS, the accepted first-line treatment consists in low-dose aspirin (LDA) plus prophylactic unfractionated or low-molecular-weight heparin (LMWH). Recently, new emerging treatment modalities, including intravenous immunoglobulins (IVIG), have been implemented to manage APS refractory to conventional therapy. The objective of this review is to summarize the currently available information on the IVIG therapy in APS, focusing on the use of IVIG in the obstetric form, CAPS and on primary or secondary thromboprophylaxis. We analyzed 35 studies, reporting the effects of IVIG in APS patients, and we discussed their results. IVIG in obstetric APS seem to be very useful in selected situations (patients not responsive to the conventional treatment, concomitant autoimmune manifestations or infections or patients in whom anticoagulation is contraindicated). IVIG treatment represents an important component of the combination therapy of CAPS and they could be useful, in addition to the standard therapy, to prevent recurrent thrombosis in APS patients refractory to conventional anticoagulant treatment. Anyway, in some cases we also found controversial results that claim the need of further well-designed studies to definitely state the efficacy and tolerability of IVIG in CAPS, obstetric and non-APS.

  18. Acute adrenal failure as the presenting feature of primary antiphospholipid syndrome in a child

    Directory of Open Access Journals (Sweden)

    Improda Nicola

    2012-09-01

    Full Text Available Abstract Introduction Antiphospholipid syndrome (APS is characterized by recurrent arterial and venous thrombosis and detection of antiphospholipid antibodies (aPLs. This syndrome may be associated with connective tissue disorders, or with malignancies, but it may also appear in isolated form (primary APS. We report on a pediatric patient presenting with acute adrenal failure as the first manifestation of primary APS. Case report A previously healthy 11-year-old boy developed fever, abdominal pain, and vomiting. An abdominal computed tomography scan showed nodular lesions in the adrenal glands. He was referred to our Department and a diagnosis of APS and acute adrenal failure was considered, based on positive aPLs (IgG and IgM, elevated ACTH levels and low cortisol levels. Other features were anemia, thrombocytopenia, elevated inflammatory parameters, hypergammaglobulinemia, prolonged partial thromboplastin time, positive antinuclear, anticardiolipin, anti-platelet antibodies, with negative double-stranded DNA antibodies. Lupus anticoagulant and Coomb’s tests were positive. MRI revealed a bilateral adrenal hemorrhage. A treatment with intravenous metylprednisolone, followed by oral prednisone and anticoagulant, was started, resulting in a progressive improvement. After 2 months he also showed hyponatremia and elevated renine levels, indicating a mineralcocorticoid deficiency, requiring fludrocortisones therapy. Conclusion The development of acute adrenal failure from bilateral adrenal haemorrhage in the context of APS is a rare but life-threatening event that should be promptly recognized and treated. Moreover, this case emphasizes the importance of the assessment of aPLs in patients with acute adrenal failure in the context of an autoreaction.

  19. Possible Association of Etanercept, Venous Thrombosis, and Induction of Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Shanti Virupannavar

    2014-01-01

    Full Text Available Tumor necrosis factor α (TNF α inhibitors are commonly used for treatment of aggressive rheumatoid arthritis and other rheumatic diseases. Etanercept is one of the medications approved for treatment of rheumatoid arthritis. Though many studies have documented the safety and efficacy of these medications, evidence for adverse effects is emerging including cancer, infections, and cardiovascular disease. There have been studies showing that these medications induce autoantibody production, including antinuclear antibodies and anti-dsDNA antibodies. Limited data exists, however, regarding induction of antiphospholipid antibodies (APLs by TNF α inhibitors, including anticardiolipin antibodies (ACLs, lupus anticoagulant (LAC, and anti-β2-glycoprotein I (anti-β2 GPI, or an association between antibody development and clinical manifestations. In this case series, we describe five patients who developed venous thromboembolism (VTE and APLs while receiving etanercept therapy. All five of our patients met the criteria for diagnosis of APS after receiving etanercept. Our case series supports the association between etanercept, APLs, and VTE. We believe that testing for APLs prior to initiation of anti-TNF therapy is reasonable, given this relationship and the risks associated with VTE.

  20. Diagnosis and management of non-criteria obstetric antiphospholipid syndrome.

    Science.gov (United States)

    Arachchillage, Deepa R Jayakody; Machin, Samuel J; Mackie, Ian J; Cohen, Hannah

    2015-01-01

    Accurate diagnosis of obstetric antiphospholipid syndrome (APS) is a prerequisite for optimal clinical management. The international consensus (revised Sapporo) criteria for obstetric APS do not include low positive anticardiolipin (aCL) and anti β2 glycoprotein I (aβ2GPI) antibodies (< 99th centile) and/or certain clinical criteria such as two unexplained miscarriages, three non-consecutive miscarriages, late pre-eclampsia, placental abruption, late premature birth, or two or more unexplained in vitro fertilisation failures. In this review we examine the available evidence to address the question of whether patients who exhibit non-criteria clinical and/or laboratory manifestations should be included within the spectrum of obstetric APS. Prospective and retrospective cohort studies of women with pregnancy morbidity, particularly recurrent pregnancy loss, suggest that elimination of aCL and/or IgM aβ2GPI, or low positive positive aCL or aβ2GPI from APS laboratory diagnostic criteria may result in missing the diagnosis in a sizeable number of women who could be regarded to have obstetric APS. Such prospective and retrospective studies also suggest that women with non-criteria obstetric APS may benefit from standard treatment for obstetric APS with low-molecular-weight heparin plus low-dose aspirin, with good pregnancy outcomes. Thus, non-criteria manifestations of obstetric APS may be clinically relevant, and merit investigation of therapeutic approaches. Women with obstetric APS appear to be at a higher risk than other women of pre-eclampsia, placenta-mediated complications and neonatal mortality, and also at increased long-term risk of thrombotic events. The applicability of these observations to outcomes in women with non-criteria obstetric APS remains to be determined.

  1. A importância da intervenção nutricional na redução do peso corpóreo em pacientes com síndrome do anticorpo antifosfolípide The importance of nutritional intervention in the reduction of body weight in patients with the antiphospholipid antibody

    Directory of Open Access Journals (Sweden)

    Karin Klack

    2008-06-01

    Full Text Available OBJETIVO: Avaliar a eficácia da intervenção nutricional na redução do excesso de peso (EP, em pacientes com síndrome do anticorpo antifosfolípide (SAF. MÉTODO: Incluídos 40 pacientes, acima de 18 anos, com diagnóstico de SAF primária ou secundária, acompanhados no Serviço de Reumatologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP e recrutados no período de outubro de 2005 a fevereiro de 2006. Foram coletados dados demográficos e realizados a revisão de prontuários, a mensuração de peso e da altura, o cálculo do índice de massa corpórea (IMC atual e a adequação da dieta pelo IMC ideal. Foi realizada avaliação na primeira consulta e após intervalo mínimo de dois meses. RESULTADOS: A média de idade dos pacientes foi de 41 anos, sendo 93% de mulheres. Os pacientes com SAF primária perfaziam 25%, com média de dois anos da doença. A trombose venosa ocorreu em 63%, arterial em 48% e manifestações obstétricas em 27%. Na primeira consulta, 68% apresentavam EP, 27% eram eutróficos e 5% estavam com baixo peso (BP. Após três meses de intervenção, os eutróficos mantiveram o peso e os de BP tornaram-se eutróficos, segundo o IMC. Interessantemente, entre os pacientes com EP (n = 27, 82% emagreceram, 14% engordaram e 4% se mantiveram. Especificamente, 11 pacientes apresentaram 1% a 3% de perda ponderal de peso, oito perderam de 4% a 7%, dois reduziram 8% a 9% e um reduziu 13,6% com o acompanhamento nutricional. CONCLUSÃO: Foi demonstrado no presente estudo que a intervenção nutricional conseguiu atingir metas para redução de peso, possibilitando diminuição no risco trombótico num curto período, sendo, portanto, uma modalidade terapêutica inicial e de eleição para corrigir o EP em pacientes com SAF.OBJECTIVE: To evaluate the efficacy of the nutricional intervention in the loss of the weight excess (WE, in patients with the antiphospholipid antibody syndrome (APS

  2. Spontaneous coronary artery dissection in the context of positive anticardiolipin antibodies and clinically undiagnosed systemic lupus erythematosus.

    Science.gov (United States)

    Nisar, M K; Mya, T

    2011-11-01

    Spontaneous coronary artery dissection (SCAD) is an extremely uncommon condition that can lead to fatal acute myocardial infarction. There have been very few case reports of SCAD in patients with systemic lupus erythematosus (SLE) and even fewer in association with antiphospholipid antibodies - mainly postpartum. This is the first reported case of SCAD in a patient who was confirmed to have SLE and tested positive for anticardiolipin antibody and lupus anticoagulant. This case demonstrates the importance of carefully considering the differential diagnoses of SCAD at presentation. It also highlights the need for further research to explore the link between SLE, antiphospholipid antibodies and SCAD.

  3. [Sneddon Syndrome and antiphospholipid antibodyies: an etiology of later al homonymous hemianopia].

    Science.gov (United States)

    Kriet, M; Fiqhi, A; Bouya, Y; Louaya, S; Laktaoui, A

    2010-01-01

    Sneddon's syndrome is a particular and rare entity that mostly affects young women and whose diagnosis is based on the coexistence of a cuteaneous livedo and a cerebrovascular ischemic attack. It had be considered as being an expression of an occlusive vasculitis or of antiphospholipid antibody syndrome. We report the case of a 20-year-old female, who had developed a left homonymous hemianopia after ischemic encephalopathy. Visual field examination confirmed the presence of a complete left homonymous hemianopia. Cerebral Magnetic Resonance Imaging revealed right occipital cerebrovascular ischemic lesions. Sneddon's syndrome diagnosis was considered on the presence of cutaneous livedo reticularis and associated cerebral ischemic events. With medical treatment, a small functional improvement could be noticed but without net improvement in the visual field defect.

  4. The relationship between mean platelet volume and thrombosis recurrence in patients diagnosed with antiphospholipid syndrome.

    Science.gov (United States)

    Rupa-Matysek, Joanna; Gil, Lidia; Wojtasińska, Ewelina; Ciepłuch, Katarzyna; Lewandowska, Maria; Komarnicki, Mieczysław

    2014-11-01

    Increased mean platelet volume (MPV) is associated with platelet reactivity and is a predictor of cardiovascular risk and unprovoked venous thromboembolism. The aim of our study was to evaluate MPV in patients with confirmed antiphospholipid antibody syndrome (APS) and to identify the correlation between the value of MPV and the recurrence of thrombosis. The studied group consists of 247 patients with a history of thrombosis and/or pregnancy loss (median age 38, range 18-66 years) classified as APS group (n = 70) or APS negative patients (n = 177) according to the updated Sapporo criteria. The control group consisted of 98 healthy subjects. MPV was significantly higher in the group of patients with clinically and laboratory confirmed APS (median 7.85, range 4.73-12.2 fl) in comparison with the controls. It was also higher than in APS negative patients (7.61, range 5.21-12.3 fl). APS patients with triple positivity for antiphospholipid antibodies with respect to Miyakis classification categories had higher MPV values than other APS patients (9.69 ± 1.85 vs. 7.29 ± 1.3 fl, p = 0.001). Recurrent thrombotic episodes were observed in 83 patients, but among the triple positive high-risk patients with APS in 80 % cases (p = 0.0046). In receiver operating characteristic curve analysis, the value of MPV level for thrombosis recurrence prediction in the APS group with sensitivity of 86 % and specificity of 82 % was 7.4 fl. In the multivariate logistic regression model, MPV above 7.4 fl (OR 3.65; 95 % CI 1.38-9.64, p = 0.009) significantly predicts thrombosis recurrence. Our results identify the value of MPV as a prognostic factor of thrombosis recurrence in patients with APS.

  5. Diagnosis and treatment of antiphospholipid syndrome in pregnancy%妊娠合并抗磷脂综合征的诊治

    Institute of Scientific and Technical Information of China (English)

    邱丽华

    2010-01-01

    抗磷脂综合征(antiphospholipid syndrome,APS)是指抗磷脂抗体(antiphospholipid antibody,APA)阳性并伴有血栓形成或病理妊娠的一组临床征象的总称,可引起早产、流产、先兆子痫、胎儿生长受限等多种不良妊娠结局.2006年公布的提出了APS的最新诊断标准.妊娠合并APS的治疗包括单独或联合应用免疫抑制剂和抗凝剂、静脉注射免疫球蛋白、血浆交换等.

  6. 抗磷脂综合征与妊娠丢失的研究进展%Research progress in relationship of antiphospholipid syndrome and pregnancy loss

    Institute of Scientific and Technical Information of China (English)

    王艳梅; 凌国灿

    2012-01-01

    自身免疫性疾病或免疫功能异常影响妊娠,在自身免疫疾病中,抗磷脂综合征(APS)占了一定比例.本文就抗磷脂抗体(APA)的形成及APA导致妊娠丢失的作用机制进行阐述,旨为今后APS的临床治疗带来更多的启示.%Autoimmue disease or abnormal immunologic function can affect pregnancy. In autoimmune diseases, the antiphospholipid syndrome (APS) has accounted for a certain proportion. This paper illustrated the formation of antiphospholipid antibody (APA) and the mechanism of pregnancy loss caused by APA. It brings some enlightenment for the future APS treatment.

  7. ADAMTS-13 gene expression in antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Veysel Sabri Hançer

    2011-09-01

    Full Text Available Antiphospholipid syndrome (APS is an autoimmune disorder characterized by recurrent thrombosis and fetal mortality. Thrombotic microangiopathy (TMA is an important histological finding in catastrophic APS (CAPS and in APS patients with nephropathy. Analysis of familial thrombotic thrombocytopenic purpura patients showed that there are mutations in the ADAMTS-13 gene that lead to functional defects in the ADAMTS-13 enzyme. The aim of this study was to investigate the prevalence of the aforementioned mutations in APS, as well as to evaluate the level and activity of the ADAMTS-13 enzyme in patients with APS. C365del, Q449stop codon, P475S, and C508Y mutations were analyzed in APS patients. Transcriptions were analyzed using real-time PCR, and the level and activity of ADAMTS-13 were analyzed via fluorogenic assay. None of the mutations tested were present in the patient or control groups. The level of ADAMTS-13 mRNA in the patient group was 50% lower than that in the control group. Although a significant difference in ADAMTS-13 activity was not observed between the patient and control groups, a significant association was observed with the level of ADAMTS-13 (p<0.0001. The level and activity of ADAMTS-13 were not associated with thrombotic complications, thrombocytopenia, or pregnancy complications in the patients with APS.

  8. Antiphospholipid syndrome and rheumatic fever: a case spanning three decades of changing concepts and common immunological mechanisms.

    Science.gov (United States)

    Alcock, R; Elsik, M; Yiannikas, C; Yiannikas, J

    2011-10-01

    We present a case of primary antiphospholipid syndrome (APS), initially diagnosed as acute rheumatic fever, resulting in severe mitral valve incompetence. This case raises questions of the specificity of the Jones diagnostic criteria for rheumatic fever in a population where it is infrequently encountered. There are similarities in clinical, pathological and echocardiographic presentations between rheumatic fever and APS, in addition to common immunological mechanisms. Our case highlights the possibility that rather than rheumatic fever being primarily responsible for her recurrent attacks of chorea and arthritis, the streptococcal infections in our patient occurred either in the setting of underlying antiphospholipid antibodies ('second hit' phenomenon), or may have triggered the development of pathogenic antibodies (molecular mimicry), subsequently leading to the clinical evolution of APS. During the three decades of our patient and her recurrent problems, there has been an evolving knowledge of the mechanisms of APS and rheumatic fever, allowing us to extend our understanding beyond symptoms and syndromes, to a better realization of the underlying immunological relationship between the two.

  9. Paroxysmal non-kinesigenic dyskinesia in antiphospholipid syndrome

    NARCIS (Netherlands)

    Engelen, M; Tijssen, MAJ

    2005-01-01

    We report on a patient with a mixed movement disorder classifiable as a paroxysmal nonkinesigenic dyskinesia, occurring as the first manifestation of primary antiphospholipid syndrome (PAPS). Possible pathophysiology is discussed based on recent literature, and we stress that PAPS must be considered

  10. Low dose aspirin after ischemic stroke associated with antiphospholipid syndrome

    NARCIS (Netherlands)

    Derksen, RHWM; de Groot, PG; Kappelle, LJ

    2003-01-01

    The authors describe course and outcome of eight patients with ischemic stroke as the first thrombotic manifestation of antiphospholipid syndrome who received low-dose aspirin as prophylactic treatment. During 8.9 years of follow-up, two patients had a recurrent stroke. Recurrent stroke rate per 100

  11. Overlapping humoral autoimmunity links rheumatic fever and the antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Blank, M; Krause, I; Magrini, L

    2006-01-01

    Rheumatic fever (RF) and the antiphospholipid syndrome (APS) are autoimmune diseases that share similar cardiac and neurological pathologies. We assessed the presence of shared epitopes between M protein, N-acetyl-beta-D-glucosamine (GlcNAc) and beta2 glycoprotein-I (beta2GPI), the pathogenic...

  12. Chronic periaortitis and antiphospholipid syndrome: is there a link?

    Directory of Open Access Journals (Sweden)

    Liliana Carneiro

    2016-06-01

    Full Text Available Chronic periaortitis (CP is a rare fibro-inflammatory disease characterized by periaortic fibrosis and/or aortic aneurysms formation, mostly localized in retroperitoneum and occasionally in the mediastinum. Recent studies have shown its common association with autoimmune diseases, therefore autoimmunity has been proposed as a contributing factor. Herein, we describe the second case in the literature of CP associated with antiphospholipid syndrome. A 64-year-old man with history of open surgery for inflammatory thoracic aneurysm and recurrent deep vein thrombosis was referred for abdominal pain and weight loss in the last 6 months. Further investigation revealed elevated acute-phase reactant levels, positive antiphospholipid autoantibodies, radiological and histological evidence of periaortic fibrosis and inflammation causing abdominal aneurysm and ureteral obstruction. Diagnosis of CP and antiphospholipid syndrome were made and steroid therapy was implemented with clinical and radiological improvement. The present report further supports the potentially immune-mediated origin of CP, highlighting its possible linkage with antiphospholipid syndrome.

  13. Lipid peroxidation as risk factor for endothelial dysfunction in antiphospholipid syndrome patients.

    Science.gov (United States)

    Stanisavljevic, Natasa; Stojanovich, L; Marisavljevic, D; Djokovic, A; Dopsaj, V; Kotur-Stevuljevic, J; Martinovic, J; Memon, L; Radovanovic, S; Todic, B; Lisulov, D

    2016-10-01

    The aim of this study was to evaluate oxidative stress markers and it relations to endothelial damage as risk factor for thrombosis in patients with primary (PAPS) and secondary (SAPS) antiphospholipid syndrome (APS) in correlation to traditional risk factors. Flow-mediated (FMD) and nitroglycerine (NMD)-induced dilation of the brachial artery were studied in 140 APS patients (90 PAPS, 50 SAPS) and 40 controls matched by age, sex, and conventional risk factors for atherosclerosis. Markers of oxidative stress, lipid hydroperoxydes (LOOH), advanced oxidation protein products (AOPP), total sulfhydryl groups (tSHG), and paraoxonase 1 activity (PON1) were determined by spectrophotometric method. Oxidative stress dominates in APS patients. LOOH and AOPP correlate to lipid fractions (p antiphospholipid antibody positivity (p < 0.05). FMD was lower in APS patients comparing to controls (p < 0.001). Cholesterol is independent variable for FMD impairment in control group (p = 0.011); LOOH in PAPS (p = 0.004); LOOH, aCL, and triglycerides in SAPS patients (p = 0.009, p = 0.049, and p = 0.012, respectively). Combined predictive of aCL and LOOH is better for FMD impairment than LOOH alone in both PAPS and SAPS patients (AUC 0.727, p = 0.001, 95 % CI 0.616-0.837 and AUC 0.824, p˂0.001, 95 % CI 0.690-0.957, respectively). Lipid peroxidation is independent predictor for endothelial dysfunction in APS patients. We demonstrated synergistic effect of aCL and LOOH as risk for endothelial impairment in both PAPS and SAPS patients.

  14. Clinico-genealogical investigation of antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    N N Chapaeva

    2009-01-01

    Full Text Available Objective. Clinicogenealogical investigation of the families of patients with antiphospholipid syndrome (APS. Material and methods. Families of 82 pts with APS fulfilled diagnostic criteria of S. Miyakis et al., 2006 (mean age 47,2±12,1 years, men:women ratio 1:10,7 were studied. “Severe” course of APS (43 patients was characterized by presence of recurrent thrombotic events and/or thrombosis of several types and/or localizations. Clinicogenealogical investigation included pedigrees analysis (the number of relatives - 615. 46 individuals-relatives of 26 patients with APS (37 first-degree and 9 second-degree relatives, mean age 29,5±16,4 years were followed up for 4 years. Incidence of “minor” features (livedo reticularis, neurological disorders, heart valves disease, thrombocytopenia was studied, lupus anticoagulant (LA test was performed. Statistical analysis was performed with Student’s t test and logistic regression analysis (SPSS 11.5.0. Results. Definite APS was diagnosed in 7 individuals, more frequently - in first-degree relatives and women (the most frequent form of inheritance - mother/daughter. “Minor” features were precursors of APS in 76% patients with APS. These features were found in more than half relatives. LA was detected in 39% of relatives. LA was 5-fold more prevalent in relatives of LA-positive patients with APS as compared with relatives of LA-negative patients. Primary prevention of thrombosis has been successfully conducted in patients with “minor” features of APS and/or positive LA (“pre-APS”. “Severe” course of APS was associated with the presence of cardiovascular diseases in family history (OR 0,32, CI0,11-0,97, multivariate analysis. Conclusion. Girls and first-degree relatives are at higher risk of APS development than boysand second- or third-degree relatives if APS is present in family history. LA, presumably, 39has inherited character. The role of clinicogenealogical method in APS

  15. Catastrophic antiphospholipid syndrome in obstetric practice

    Directory of Open Access Journals (Sweden)

    Валерий Николаевич Запорожан

    2015-05-01

    Full Text Available Thus, the Catastrophic antiphospholipid syndrome (CAPS is much more common than has been assumed until now, in all patients the authors strongly recommend screening for AFA. Furthermore, eclampsia, HELLP-syndrome premature detachment of normally located placentae (PDNSP can develop in the presence of other defects of hemostasis, in particular in mutation FV Leiden, MTHFR C677T, deficiency of protein C (PC, protein S (PS. The combination of acquired thrombophilia due to APS, with genetic defects worsen hemostasis during the pathological process leading to the development of thrombotic complications. Perhaps a combination of hereditary thrombophilia and APS creates a favorable environment in which, under certain conditions, possible decompensation of the hemostatic system and the development of CAPS. Patients with APS constitute a group of very high risk of thromboembolic complications in the perioperative period. Even a minimally invasive intervention (biopsy, curettage, tooth extraction may trigger the development of CAPS. Thus, according to Erkan et al. (2003, 40% of patients develop CAPS was provoked by surgery. The main reasons for the development of thrombotic complications in connection with surgical intervention is the damage to the vessel wall, blood stasis and the abolition of indirect anticoagulants. In the study on the presence of genetic thrombophilia was found heterozygous form of FV Leiden mutation and homozygous mutation of MTHFR C677T. He was diagnosed with pregnancy 14 weeks, APS, mixed form of thrombophilia (a combination of acquisitions and multigenic thrombophilia, hyperhomocysteinemia, weighed down by obstetric and somatic history.It is very urgent and important problem remains diagnosis CAPS, which is inconceivable without the determination of AFA. The latter should be mandatory for all pregnant women with preeclampsia habitual miscarriage, Premature detachment of normally situated placenta (PDNSP, genital herpes history

  16. Antiphospholipid-associated thrombocytopenia or autoimmune hemolytic anemia in patients with or without definite primary antiphospholipid syndrome according to the Sapporo revised classification criteria: a 6-year follow-up study.

    Science.gov (United States)

    Comellas-Kirkerup, Lucía; Hernández-Molina, Gabriela; Cabral, Antonio R

    2010-10-21

    The updated Sapporo classification criteria for antiphospholipid syndrome (APS) only include thrombosis or pregnancy morbidity as clinical criteria. To test this notion, we studied 55 patients (80% women) with hematologic manifestations. All fulfilled the laboratory criteria for primary APS. Thirty-five patients (64%) had thrombocytopenia, 14 (25%) had autoimmune hemolytic anemia, and 6 (11%) had both. Twenty-five patients (22 women, 88%) also fulfilled one clinical criterion for APS after a median follow-up of 13.2 years (range, 1.45-37 years), whereas the remaining 30 patients (22 women, 73%) have not had any thrombotic event nor pregnancy morbidity after a median follow-up of 5.4 years (range, 0.12-24 years). No patient developed systemic lupus erythematosus during follow-up. The hematologic manifestation was asynchronous with the APS onset in 84% of patients. The response to treatment was similar regardless of the APS status. Patients with definite APS were more frequently positive for the lupus anticoagulant (63%) than lupus anticoagulant-positive patients without APS (30%; odds ratio, 3.5; 95% confidence interval, 1.07-11.4; P < .02). Anticardiolipin or anti-β(2)-glycoprotein-I antibodies were highly prevalent among the study groups. Our study suggests that, depending upon their antiphospholipid profile, patients with hemocytopenias appear to comprise a peculiar subset of patients with APS; some develop thrombotic and/or obstetric APS whereas others continue with hematologic APS.

  17. Comparison between single antiplatelet therapy and combination of antiplatelet and anticoagulation therapy for secondary prevention in ischemic stroke patients with antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Hirohisa Okuma, Yasuhisa Kitagawa, Takashi Yasuda, Kentaro Tokuoka, Shigeharu Takagi

    2010-01-01

    Full Text Available Satisfactory results have not yet been obtained in therapy for secondary prevention in ischemic stroke patients with antiphospholipid syndrome (APS. We therefore compared single antiplatelet therapy and a combination of antiplatelet and anticoagulation therapy for secondary prevention in ischemic stroke patients with APS. The subjects were 20 ischemic stroke patients with antiphospholipid antibody, 13 with primary antiphospholipid syndrome and 7 with SLE-related antiphospholipid syndrome. Diagnosis of APS was based on the 2006 Sydney criteria. Eligible patients were randomly assigned to either single antiplatelet therapy (aspirin 100 mg or a combination of antiplatelet and anticoagulation therapy (target INR: 2.0-3.0; mean 2.4±0.3 for the secondary prevention of stroke according to a double-blind protocol. There was no significant difference between the two groups in age, gender, NIH Stroke Scale on admission, mRS at discharge, or rate of hypertension, diabetes mellitus, hyperlipidemia, or cardiac disease. We obtained Kaplan-Meier survival curves for each treatment. The primary outcome was the occurrence of stroke. The mean follow-up time was 3.9±2.0 years. The cumulative incidence of stroke in patients with single antiplatelet treatment was statistically significantly higher than that in patients receiving the combination of antiplatelet and anticoagulation therapy (log-rank test, p-value=0.026. The incidence of hemorrhagic complications was similar in the two groups. The recent APASS study did not show any difference in effectiveness for secondary prevention between single antiplatelet (aspirin and single anticoagulant (warfarin therapy. Our results indicate that combination therapy may be more effective in APS-related ischemic stroke.

  18. Cutaneous manifestations of antiphospholipid syndrome%抗磷脂综合征的皮肤表现

    Institute of Scientific and Technical Information of China (English)

    赵玉磊; 苏晓红

    2014-01-01

    Antiphospholipid syndrome (APS) is a systemic auto-immune disorder characterised by recurrent arterial and/or venous thrombosis, pregnancy losses, thrombocytopenia and the presence of elevated and persistent levels of antiphospholipid antibodies. It may potentially affect any organ including the skin. Dermatologic manifestations may be the ifrst indication of APS and may be noticeable as livedo reticularis, Sneddon's syndrome, atrophie blanche, skin ulcerations and necrosis, anetoderma, or Degos' disease. Knowledge of the cutaneous manifestations of APS is critical in the early identiifcation and fast treatment of APS patients.%抗磷脂综合征是一种以反复动静脉形成、流产、血小板减少及血抗磷脂抗体持续阳性为特征的系统性自身免疫性疾病。该综合征可以累及包括皮肤在内的全身任何器官。临床上皮肤表现可作为其首要症状,多见于网状青斑、Sneddon综合征、白色萎缩、皮肤溃疡坏死、斑状萎缩和Degos病等。掌握抗磷脂综合征的皮肤表现对其早期诊断、及时治疗极为关键。

  19. A role for Toll-like receptor mediated signals in neutrophils in the pathogenesis of the anti-phospholipid syndrome.

    Directory of Open Access Journals (Sweden)

    Gerd Gladigau

    Full Text Available The anti-phospholipid syndrome (APS is characterized by recurrent thrombosis and occurrence of anti-phospholipid antibodies (aPL. aPL are necessary, but not sufficient for the clinical manifestations of APS. Growing evidence suggests a role of innate immune cells, in particular polymorphonuclear neutrophils (PMN and Toll-like receptors (TLR to be additionally involved. aPL activate endothelial cells and monocytes through a TLR4-dependent signalling pathway. Whether this is also relevant for PMN in a similar way is currently not known. To address this issue, we used purified PMN from healthy donors and stimulated them in the presence or absence of human monoclonal aPL and the TLR4 agonist LPS monitoring neutrophil effector functions, namely the oxidative burst, phagocytosis, L-Selectin shedding and IL-8 production. aPL alone were only able to induce minor activation of PMN effector functions at high concentrations. However, in the additional presence of LPS the activation threshold was markedly lower indicating a synergistic activation pathway of aPL and TLR in PMN. In summary, our results indicate that PMN effector functions are directly activated by aPL and boosted by the additional presence of microbial products. This highlights a role for PMN as important innate immune effector cells that contribute to the pathophysiology of APS.

  20. 抗磷脂综合征的心血管系统表现%Cardiovascular manifestations in antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    姚鸿梅; 黄鹤

    2012-01-01

    Antiphospholipid syndrome (APS) is a systemic autoimmune disease involving multi-organs, characterized by recurrent arterial or venous thrombotic events, spontaneous abortion, thrombocytopenia and persistent positivity of antiphospholipid antibodies. The heart is one of the important target organs of APS. In this paper, we reviewed the cardiovascular manifestations of APS, such as valve disease, myocardial infarction, intracardiac thrombus, myocardial microthrombosis and therapeutic strategy.%抗磷脂综合征( APS)是一种累及多器官的系统性自身免疫性疾病,临床以反复发作的动静脉血栓形成、自发性流产、血小板减少以及持续的血清抗磷脂抗体阳性为主要特征.心脏是APS的重要靶器官之一.本文从APS的心血管系统表现如心瓣膜病变、心肌梗死、心腔内血栓形成、冠状动脉微血管血栓形成及治疗策略方面作一综述.

  1. Pre-eclampsia as a manifestation of antiphospholipid syndrome: assessing the current status.

    Science.gov (United States)

    Gibbins, K J; Ware Branch, D

    2014-10-01

    The presence of antiphospholipid antibodies is considered a risk factor for pre-eclampsia. Two meta-analyses and a number of case-control and cohort studies have found associations between pre-eclampsia and lupus anticoagulant, anticardiolipin, and/or anti-β2 glycoprotein I. However, existing literature is inconsistent, with varying severity of pre-eclampsia phenotype examined, differing aPL titer cutoffs used to define positive status, and an overwhelming lack of repeat confirmatory aPL testing. This calls into question the link between aPLs and pre-eclampsia, or at least makes it less well defined. There is evidence for a mechanistic pathway between aPLs and adverse pregnancy outcomes (APOs) including pre-eclampsia via the complement pathway. Complement appears to be overactive in pregnancies affected by APOs. A mouse model has show that the fetal wastage caused by treatment with human aPLs can be salvaged by either creating genetic knockouts along the complement, TNF-alpha, and tissue factor pathways or be treating mice with monoclonal antibodies blocking key complement factors. Thus, this is worth further investigation to clarify the likely association of aPLs and pre-eclampsia in humans, as well is to further evaluate the interaction with complement in human pregnancies.

  2. Adalimumab-associated antiphospholipid syndrome: a case report and review of the literature.

    Science.gov (United States)

    Hemmati, Iman; Kur, Jason

    2013-07-01

    This study aims for the presentation of the first reported case of adalimumab-associated antiphospholipid syndrome (APS) and review of the literature on adalimumab-induced vasculitis and APS. A case of APS associated with adalimumab use in a 67-year-old woman is reported. The English medical literature was reviewed for antitumor necrosis factor (TNF) agents and their association with APS and vasculitis. Adalimumab is a fully humanized monoclonal antibody targeted against TNF alpha that is widely used in the treatment of rheumatoid arthritis, juvenile idiopathic arthritis, ankylosing spondylitis, psoriatic arthritis, psoriasis, and Crohn's disease. Literature review reveals several cases of anti-TNF-induced vasculitis including cases associated with adalimumab. We report the first case of adalimumab-induced APS in a 67-year-old woman who developed APS and vasculitis associated with de novo positive anti-cardiolipin (aCL) antibody following the third dose of adalimumab therapy for the treatment of spondyloarthropathy. This is the first case demonstrating that a short course of adalimumab therapy may induce immunoglobulin M aCL autoantibodies leading to APS. With the growing use of anti-TNF medications in immune-mediated and inflammatory diseases, adalimumab and other anti-TNF medications should be considered as a possible explanation for APS.

  3. Type III mixed cryoglobulinemia and antiphospholipid syndrome in a patient with partial DiGeorge syndrome.

    Science.gov (United States)

    Chang, Alice D; Tachdjian, Raffi; Gallagher, Kerry; McCurdy, Deborah K; Lassman, Charles; Stiehm, E Richard; Yadin, Ora

    2006-01-01

    We studied a 14 year-old boy with partial DiGeorge syndrome (DGS), status post complete repair of Tetralogy of Fallot, who developed antiphospholipid syndrome (APS) and type III mixed cryoglobulinemia. He presented with recurrent fever and dyspnea upon exertion secondary to right pulmonary embolus on chest computed tomography (CT). Coagulation studies revealed homozygous methylene tetrahydrofolate reductase 677TT mutations, elevated cardiolipin IgM antibodies, and elevated beta(2)-glycoprotein I IgM antibodies. Infectious work-up revealed only positive anti-streptolysin O (ASO) and anti-DNAse B titers. Autoimmune studies showed strongly positive anti-platelet IgM, elevated rheumatoid factor (RF), and positive cryocrit. Renal biopsy for evaluation of proteinuria and hematuria showed diffuse proliferative glomerulonephritis (DPGN) with membranoproliferative features consistent with cryoglobulinemia. Immunofixation showed polyclonal bands. Our patient was treated successfully with antibiotics, prednisone, and mycophenolate mofetil (MMF). This is the first report of a patient with partial DGS presenting with APS and type III mixed cryoglobulinemia possibly due to Streptococcal infection.

  4. Diffusion tensor imaging in patients with obstetric antiphospholipid syndrome without neuropsychiatric symptoms

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, Fabricio R. [University Hospital Center of Nimes and Research Team EA 2415, Department of Radiology (France); Macri, Francesco; Beregi, Jean-Paul [University Hospital Center of Nimes and Research Team EA 2415, Department of Radiology (France); Montpellier University, Faculty of Medicine, Montpellier (France); Jackowski, Marcel P. [University of Sao Paulo, Department of Computer Science, Institute of Mathematics and Statistics, Sao Paulo (Brazil); Kostis, William J. [Harvard Medical School, Massachusetts General Hospital, Boston, MA (United States); Athinoula A. Martinos Center for Biomedical Imaging, Charlestown, MA (United States); Gris, Jean-Christophe [Montpellier University, Faculty of Medicine, Montpellier (France); University Hospital Center of Nimes, Department and Laboratory of Hematology (France); Mekkaoui, Choukri [University Hospital Center of Nimes and Research Team EA 2415, Department of Radiology (France); Montpellier University, Faculty of Medicine, Montpellier (France); Harvard Medical School, Massachusetts General Hospital, Boston, MA (United States); Athinoula A. Martinos Center for Biomedical Imaging, Charlestown, MA (United States)

    2016-04-15

    To evaluate white matter (WM) integrity in neurologically asymptomatic antiphospholipid syndrome (APS) using diffusion tensor imaging (DTI) in women with no thrombotic history but with pregnancy loss. Imaging was performed with a 3 T scanner using structural MRI (T1-weighted, fluid attenuation inversion recovery [FLAIR]) and DTI sequences in 66 women with APS and a control group of 17 women. Women with APS were further categorized as positive for lupus anticoagulant (LA) and/or aβ2GPI-G antibodies (LA/aβ2GPI-G-positive, N = 29) or negative (LA/aβ2GPI-G-negative, N = 37) for both. Tract-based spatial statistics of standard DTI-based indices were compared among groups. Women with APS had significantly lower fractional anisotropy (p < 0.05) associated with higher mean diffusivity and radial diffusivity compared to the control group. There was a stronger association of abnormal DTI features among women positive for LA and/or aβ2GPI-IgG antibodies than those who were negative. DTI appears sensitive to subtle WM changes in women with APS with no thrombotic history but with pregnancy loss, compatible with alterations in axonal structure and in the myelin sheath. The preferential association of abnormal DTI features with the two most pathogenic aPLAbs reinforces the pathophysiological relevance of our findings. (orig.)

  5. Type III Mixed Cryoglobulinemia and Antiphospholipid Syndrome in a Patient With Partial DiGeorge Syndrome

    Directory of Open Access Journals (Sweden)

    Alice D. Chang

    2006-01-01

    Full Text Available We studied a 14 year-old boy with partial DiGeorge syndrome (DGS, status post complete repair of Tetralogy of Fallot, who developed antiphospholipid syndrome (APS and type III mixed cryoglobulinemia. He presented with recurrent fever and dyspnea upon exertion secondary to right pulmonary embolus on chest computed tomography (CT. Coagulation studies revealed homozygous methylene tetrahydrofolate reductase 677TT mutations, elevated cardiolipin IgM antibodies, and elevated β2-glycoprotein I IgM antibodies. Infectious work-up revealed only positive anti-streptolysin O (ASO and anti-DNAse B titers. Autoimmune studies showed strongly positive anti-platelet IgM, elevated rheumatoid factor (RF, and positive cryocrit. Renal biopsy for evaluation of proteinuria and hematuria showed diffuse proliferative glomerulonephritis (DPGN with membranoproliferative features consistent with cryoglobulinemia. Immunofixation showed polyclonal bands. Our patient was treated successfully with antibiotics, prednisone, and mycophenolate mofetil (MMF. This is the first report of a patient with partial DGS presenting with APS and type III mixed cryoglobulinemia possibly due to Streptococcal infection.

  6. The presentation and evaluation of a case of systemic Lupus erythematosus and anthiphospholipid antibody syndrome with primary clinical manifestation of chorea

    Directory of Open Access Journals (Sweden)

    Asgary S

    1998-06-01

    Full Text Available Manifestation of chorea in patients with systemic lupus erythematosus (SLE and antiphospholipid antibody syndrome (APA synd. is not common. Moreover, primary presentation of the disease with chorea is rare and only few such cases are reported in literature in recent years. We report here the case of a 28 year old woman who was first seen at the age of 10 with clinical manifestations of chorea. Later she developed deep vein thrombosis, thrombocytpenia, stroke, cardiac valve involvement and recurrent abortions. Laboratory investigations confirmed the diagnosis of SLE and the presence of antiphospholipid antibodies. We present this patient as a case of SLE and antiphospholipid antibody syndrome with chorea being her primary clinical presentation

  7. ANTIPHOSPHOLIPID SYNDROME: DIAGNOSIS AND CLINICAL MANIFESTATIONS (A LECTURE)

    OpenAIRE

    Tat’yana M Reshetnyak

    2014-01-01

    The lecture provides information about the etiology and pathogenesis of antiphospholipid syndrome (APS) and genetic susceptibility to its development. The most recent international diagnostic criteria for APS and its variants have been reported. This syndrome can affect multiple organ systems depending on localization of thrombosis; therefore, nowadays the problem of APS is multidisciplinary. Clinical manifestations of APS are rather general (thrombosis of different localization); thus, the d...

  8. C-reactive protein in antiphospholipid syndrome: relationship with cardiovascular pathology

    Directory of Open Access Journals (Sweden)

    N V Seredavkina

    2009-01-01

    Full Text Available Objective. To assess relationship of high sensitivity C reactive protein (hsCRP level in pts with antiphospholipid syndrome (APS with clinico-laboratory features and cardiovascular pathology. Material and methods. 206 pts were included. 58 from them had primary APS (PAPS, 72 –systemic lupus erythematosus (SLE with APS and 76 – SLE. 29 from 76 pts of the latter group were positive on anticardiolipin antibodies (ACA – SLE with antiphospholipid antibodies (APhL and 47 – low positive or negative on ACA – SLE without APhL. 72 persons without autoimmune diseases were included into control group. CRP (with high sensitivity immuno-nephelometric assay, APhL (with solid phase immuno-enzyme assay, plasma lipids were evaluated, sonography with measurement of intima-media complex (IMC thickness of common carotid arteries, carotid artery bulbs and internal carotid arteries, electrocardiography (ECG, echocardiography (EchoCG, Holter ECG monitoring were performed. Results. HsCRP serum level in pts was significantly higher than in control: 2,55 [0,71; 7,04] mg/l (varied from 0,15 to 39,85 vs 0,68 [0,26; 1,97] mg/l (varied from 0,1 to 9,61, p<0,001. Most high hsCRP concentration was found in SLE with APS (p=0,02. HsCRP level in pts with PAPS with history of combined or isolated arterial thrombosis was significantly higher than in pts with SLE and APS having the same localization of thrombosis. HsCRP concentration less than 3 mg/l correlated with duration of postthrombotic period in pts with PAPS. HsCRP level also correlated with triglyceride concentration, body mass index, summated coronary risk and magistral arteries IMC thickness. Conclusion. HsCRP elevation in pts with APS was associated with development of combined and arterial thrombosis as well as with traditional risk factors of atherosclerosis.

  9. TLR4 is involved in the pathogenic effects observed in a murine model of antiphospholipid syndrome.

    Science.gov (United States)

    Xie, Hongxiang; Kong, Xiangmin; Zhou, Hong; Xie, Yachao; Sheng, Liangju; Wang, Ting; Xia, Longfei; Yan, Jinchuan

    2015-10-01

    Antiphospholipid (aPL)/anti-β2-glycoprotein I (β2GPI) antibodies are considered to play a pivotal pathogenic role in antiphospholipid syndrome (APS) by inducing an intracellular signaling and procoagulant/proinflammatory phenotype that leads to thrombosis. There is increasing evidence that Toll-like receptor 4 (TLR4) could serve as an important molecule for anti-β2GPI recognition on target cells. However, few studies have focused on the effects of TLR4 in in vivo models. Here, we investigated the role of TLR4 in the pathogenic effects of aPL/anti-β2GPI more precisely using TLR4-intact (C3H/HeN) and TLR4-defective (C3H/HeJ) mice. C3H/HeN and C3H/HeJ mice were injected with either IgG isolated from patient with APS (IgG-APS) or epitope-specific anti-β2GPI purified from β2GPI peptide-immunized rabbits. We found that, following anti-β2GPI injections and vascular injury, thrombus formation in both the carotid artery and femoral vein was markedly reduced in C3H/HeJ mice when compared with C3H/HeN mice. IgG-APS or anti-β2GPI-induced carotid artery and peritoneal macrophage tissue factor activity/expression was significantly lesser in C3H/HeJ than in C3H/HeN mice. Furthermore, the IgG-APS or anti-β2GPI induced expression of VCAM-1, ICAM-1, and E-selectin in the aorta and of IL-1β, IL-6, and TNF-α in peritoneal macrophages of C3H/HeJ mice was also significantly reduced compared to C3H/HeN mice. Together, these data suggest that TLR4 is involved in the pathogenic effects of aPL/anti-β2GPI antibodies in vivo.

  10. IgG accumulates in inhibitory hippocampal neurons of experimental antiphospholipid syndrome.

    Science.gov (United States)

    Katzav, Aviva; Menachem, Assaf; Maggio, Nicola; Pollak, Lea; Pick, Chaim G; Chapman, Joab

    2014-12-01

    Mice immunized with β2-glycoprotein I (β2GPI) are an experimental model of the antiphospholipid syndrome (eAPS) displaying elevated titers of antiphospholipid antibodies (aPL). We presently studied whether the behavioral hyperactivity in eAPS mice is associated with in vivo binding and accumulation of IgG in the brain. At 6 weeks post immunization eAPS mice had significantly higher levels of aPL (1.32 ± 0.28 and 0.02 ± 0.01 AU, p < 0.001 by t-test) compared to adjuvant immunized controls, as measured by ELISA. Significant hyperactivity in a staircase test in the eAPS mice compared to controls was found in stair-climbing (18.4 ± 0.9 and 12.0 ± 1.7, respectively) and rearing measures (23.5 ± 2.1 and 12.5 ± 1.9, p < 0.01 by t-test). Immunofluorescence staining in eAPS mice revealed significant in vivo accumulation of IgG in cortical and hippocampal neurons which was not seen in controls. Staining for IgG was markedly intense in inhibitory interneurons co-stained for GAD67 in the hippocampus of eAPS mice. The integrity of the blood brain barrier (BBB) evaluated by injection of Evans blue (EB) was impaired in eAPS and adjuvant immunized mice compared to naïve mice. Electrophysiological recordings in hippocampal brain slices showed altered response to paired pulse stimulation as well as dysregulation of carbachol-induced γ- oscillations in eAPS mice compared to control. Penetration into the brain and direct interaction of aPL with inhibitory interneurons in the hippocampus may explain the hyperactive behavior of the eAPS mice. A direct role of aPL in causing CNS dysfunction points to these antibodies as an important therapeutic target in APS.

  11. Severe Renal Hemorrhage in a Pregnant Woman Complicated with Antiphospholipid Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Shohei Kawaguchi

    2011-01-01

    Full Text Available Antiphospholipid syndrome is a systemic autoimmune disease with thrombotic tendency. Consensus guidelines for pregnancy with antiphospholipid syndrome recommend low-dose aspirin combined with unfractionated or low-molecular-weight heparin because antiphospholipid syndrome causes habitual abortion. We report a 36-year-old pregnant woman diagnosed with antiphospholipid syndrome receiving anticoagulation treatment. The patient developed left abdominal pain and gross hematuria at week 20 of pregnancy. An initial diagnosis of left ureteral calculus was made. Subsequently abdominal-pelvic computed tomography was required for diagnosis because of the appearance of severe contralateral pain. Computed tomography revealed serious renal hemorrhage, and ureteral stent placement and pain control by patient-controlled analgesia were required. After treatment, continuance of pregnancy was possible and vaginal delivery was performed safely. This is the first case report of serious renal hemorrhage in a pregnant woman with antiphospholipid syndrome receiving anticoagulation treatment and is an instructive case for urological and obstetrical practitioners.

  12. Association of beta2-glycoprotein I IgG and IgM antibodies with thrombosis and thrombocytopenia

    DEFF Research Database (Denmark)

    Voss, Anne-Sofie Boertmann; Jacobsen, Søren; Heegaard, Niels Henrik Helweg

    2001-01-01

    Antiphospholipid antibodies (APA) have been known for decades. Their relation to clinical manifestations, primarily thromboses and thrombocytopenia, was recognised in the 1980s. In this clinical study two cohorts of patients, a population-based (84 patients with systemic lupus erythematosus (SLE)...

  13. 脑梗死急性期并抗磷脂综合征1例治疗体会%The experience for treating 1 case of the acute cerebral infarction and antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    褚爱华

    2011-01-01

    抗磷脂综合征(Antiphospholipid syndrome,APS)是一组以反复动静脉血栓形成、习惯性流产和血小板减少等症状为表现的,伴有抗磷脂抗体阳性的临床综合征(Antiphospholipid antibody,APA),血管栓塞是临床表现的根源,常累及多个系统,本例患者反复发生心、脑血管缺血事件,治疗中不单纯应用西医治疗而且应用中医活血通络及补肾健脾的方法收到良好效果

  14. Didaktiske paradigmer og refleksion

    DEFF Research Database (Denmark)

    Christensen, Torben Spanget

    2014-01-01

    this article. A possible utilitarian didactical paradigm, already indicated by Krogh as a historical paradigm prominent in our time, is also discussed. It is suggested that reflection could be seen as a normative response to the utilitarian paradigm, and not as a paradigm in its own right. It is concluded...... that reflection must be understood as an overarching cultural phenomenon and a very important qualification of all Nielsen’s paradigms, and also a possible utilitarian paradigm, because it has the potential to add dynamic elements to the more or less static didactic paradigms. Thus the semiotic analysis may...

  15. Henoch-Sch(o)nlein purpura combined with antiphospholipid syndrome: one case report and literature review%过敏性紫癜合并抗磷脂综合征1例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    张宏文; 钟旭辉; 黄建萍; 肖慧捷

    2012-01-01

    目的 探讨过敏性紫癜与抗磷脂综合征的关系.方法 通过对1例以间断皮疹、血尿、蛋白尿及上肢肿痛为主诉的患儿进行肾组织病理、抗磷脂抗体检查,随访观察治疗反应,并结合文献复习进行综合分析.结果 患儿临床表现为过敏性紫癜混合型,肾脏病理为紫癜性肾炎(Ⅳb型);同时有右上肢静脉血栓形成,狼疮抗凝血因子、抗心磷脂抗体IgM及抗β2糖蛋白-I抗体IgM阳性,诊断为过敏性紫癜合并抗磷脂综合征.给予泼尼松口服、环磷酰胺冲击以及抗凝治疗,患儿临床症状好转,尿蛋白和抗磷脂抗体转阴.结论 过敏性紫癜可以合并抗磷脂综合征,临床上应引起高度重视.%Objective To explore the relationship of Henoch-Sch(o)nlein purpura (HSP) and antiphospholipid syndrome (APS). Methods One patient presenting with purpura, hematuria, proteinuria and right extremity pain was followed up. The information about kidney tissue pathology, antiphospholipid antibody test, and response to treatment was collected and analyzed. The literatures were reviewed. Results The patient clinically manifested Henoch-Sch(o)nlein purpura. The pathologic diagnosis was Henoch-Sch(o)nlein purpura nephritis (type Ivb). The vein thrombosis was formed in right upper extremity veins. The anticoagulant, anticardiolipin antibodies and anti β2 glycoprotein I antibodies were positive. The diagnosis was Henoch-Sch(o)nlein purpura combined with antiphospholipid syndrome. After given oral pred-nisone, cyclophosphamide, and anticoagulant therapy, clinical symptoms of the patient were improved. The urine protein and antiphospholipid antibody were negative. Conclusions Henoch-Sch(o)nlein purpura could be combined with antiphospholipid syndrome. More attention should be paid to it in clinic.

  16. Infliximab improves endothelial dysfunction in a mouse model of antiphospholipid syndrome: Role of reduced oxidative stress.

    Science.gov (United States)

    Benhamou, Ygal; Miranda, Sébastien; Armengol, Guillaume; Harouki, Najah; Drouot, Laurent; Zahr, Noel; Thuillez, Christian; Boyer, Olivier; Levesque, Hervé; Joannides, Robinson; Richard, Vincent

    2015-08-01

    Antiphospholipid syndrome (APS), induces endothelial dysfunction, oxidative stress and systemic inflammation that may be mediated by TNFα. Thus, we investigated the possible protective effect of the anti-TNFα antibody infliximab (5μg/g) on endothelial function in a mouse APS model (induced by injection of purified human anti-β2GP1-IgG). Seven days after anti-β2GPI-IgG injection, we observed an increase in plasma sVCAM-1 and sE-selectin levels and in aortic mRNA expression of VCAM-1 and E-selectin. This was associated with a decreased endothelium-dependent relaxation of isolated mesenteric arteries to acetylcholine, together with decreased mesenteric eNOS mRNA expression and increased eNOS uncoupling, accompanied by increased iNOS and gp91phox mRNA and increased left ventricular GSH/GSSH ratio. Infliximab significantly improved the NO-mediated relaxing responses to acetylcholine, and induced a decrease in iNOS and gp91phox mRNA expression. The õpro-adhesive and pro-coagulant phenotypes induced by the anti-β2GP1-IgG were also reversed. This study provides the first evidence that TNFα antagonism improves endothelial dysfunction in APS and suggests that endothelial dysfunction is mediated by TNFα and oxidative stress. Therefore, infliximab may be of special relevance in clinical practice.

  17. Primary antiphospholipid syndrome progressing to systemic lupus erythematosus: a case report

    Directory of Open Access Journals (Sweden)

    Rocco Manganelli

    2013-04-01

    Full Text Available Introduction: Primary antiphospholipid syndrome (APS is a thrombophilic disease that should be suspected in the presence of thrombotic events associated with hematologic abnormalities such as thrombocytopenia and prolongation of the activated partial thromboplastin time. The diagnosis must be confirmed by the demonstration of autoantibodies directed against anionic phospholipids and/or phospholipid-binding proteins. The disease can cause arterial thrombosis in any vascular district, including those of the kidney and central nervous system. Case report: In 2006 a 29-year-old male presented with kidney and brain involvement that was attributed to primary APS. The clinical diagnosis was confirmed by the results of a renal biopsy, which excluded the presence of systemic lupus erythematosus (SLE. The patient remained stable through 32 months of follow-up and then developed a malar rash with deteriorating renal function, decreasing platelet count, and reduced complement levels. Serological studies revealed positivity for ANA (homogeneous pattern, dsDNA, ACA, and beta-2-glycoprotein-1 antibodies. The diagnosis was revised to APS secondary to SLE. Conclusions: A diagnosis of primary APS should not be considered permanent: progression to SLE can occur, in some cases years after the original diagnosis. This case highlights the importance of ongoing follow-up of patients diagnosed with primary APS to detect changes that herald the emergence of SLE.

  18. Antiphospholipid Syndrome: primary or secondary to Systemic Lupus Erythematosus? Description of a clinical case of avitaminosis D in premenopausal woman with pseudo-Cushing syndrome

    Directory of Open Access Journals (Sweden)

    Mauro Turrin

    2014-06-01

    Full Text Available Low vitamin D levels have been described in obese individuals and in some autoimmune diseases, such as Systemic Lupus Erythematosus (SLE and primary antiphospholipid syndrome (pAPS. In particular, more than 50% of premenopausal women with pAPS have hypovitaminosis D. In this issue we report a case of an obese, premenopausal, and hypertensive woman with pseudo-Cushing syndrome, affected by deep venous thrombosis associated with pulmonary embolism after rib fracture who presented hypovitaminosis D. 7 years before, diagnosis of pAPS had been made after the detection of thrombocytopenia (present at a young age and arterial ischemia of a lower limb. For seven years she was treated with acetylsalicylic acid without complications. We found positive anti-dsDNA antibodies, a triple antiphospholipid antibodies (aPL positivity and levels of vitamin D < 4 µg/l. The case report arises some questions: is vitamin D deficiency due to obesity or APS? Is the positivity of anti-dsDNA indicative of progression to SLE? Is preventive therapy with hydroxychloroquine indicated? Does the high-risk aPL profile justify a high-intensity and life-long anticoagulation regimen?http://dx.doi.org/10.7175/cmi.v8i2.912

  19. The autoimmune tautology with a focus on antiphospholipid syndrome.

    Science.gov (United States)

    Franco, J-S; Anaya, J-M

    2014-10-01

    Autoimmune diseases (ADs) are often diagnosed according to classification criteria; however, they share similar subphenotypes including signs and symptoms, non-specific autoantibodies and other immune changes, which are prone to taxonomic problems. Polyautoimmunity is defined as the presence of more than one AD in a single patient. The close relationship between antiphospholipid syndrome (APS) and systemic lupus erythematosus has been studied throughout the years. However, APS may coexist with several other ADs confirming polyautoimmunity in this systemic disease. Herein, we summarized the common characteristics shared between APS and others ADs in light of the autoimmune tautology (that is, common mechanisms of autoimmune diseases).

  20. Antiphospholipid Syndrome and Libman-Sacks Endocarditis in a Bioprosthetic Mitral Valve.

    Science.gov (United States)

    Sladek, Eric H; Accola, Kevin D

    2016-02-01

    This report describes one the first cases of antiphospholipid syndrome and Libman-Sacks endocarditis in a bioprosthetic valve. A redo mitral valve replacement was carried out owing to early deterioration of the prior valve. Initially it was considered secondary to rheumatic heart disease; however, pathology analysis and autoimmune workup revealed antiphospholipid syndrome with Libman-Sacks endocarditis. We believe certain populations with mitral valve stenosis may have an underlying antiphospholipid syndrome. As a result, there needs to be a lower threshold for identifying this disease.

  1. Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Dimitri Titeca-Beauport

    2016-01-01

    Full Text Available Objective. Report of a case of catastrophic antiphospholipid syndrome (CAPS with multiple organ involvement leading to a life-threatening condition despite early combination corticosteroid and heparin therapy. Initiation of plasma exchange led to rapid improvement of the patient’s general condition. Design. Case report. Setting. University teaching hospital medical intensive care unit. Patient. Single case: 52-year-old man hospitalized for catastrophic antiphospholipid syndrome (CAPS with cardiac, renal, and cutaneous involvement. Despite early methylprednisolone and heparin therapy, the patient’s condition progressively deteriorated, resulting in acute renal failure, right adrenal hemorrhage, and pulmonary involvement, leading to acute respiratory distress on day 6, requiring high-flow nasal cannula oxygen therapy with FiO2 of 1.0. Interventions. Plasma exchange was started on day 6. Endpoints and Main Results. A marked improvement of the patient’s general condition was observed after initiation of plasma exchange, with successful weaning of oxygen therapy and normalization of platelet count, troponin, and serum creatinine within four days. Conclusions. This case illustrates the efficacy of plasma exchange in CAPS and the difficulty for physicians to determine the optimal timing of plasma exchange.

  2. Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome

    Science.gov (United States)

    Salle, Valery; Kontar, Loay; Maizel, Julien

    2016-01-01

    Objective. Report of a case of catastrophic antiphospholipid syndrome (CAPS) with multiple organ involvement leading to a life-threatening condition despite early combination corticosteroid and heparin therapy. Initiation of plasma exchange led to rapid improvement of the patient's general condition. Design. Case report. Setting. University teaching hospital medical intensive care unit. Patient. Single case: 52-year-old man hospitalized for catastrophic antiphospholipid syndrome (CAPS) with cardiac, renal, and cutaneous involvement. Despite early methylprednisolone and heparin therapy, the patient's condition progressively deteriorated, resulting in acute renal failure, right adrenal hemorrhage, and pulmonary involvement, leading to acute respiratory distress on day 6, requiring high-flow nasal cannula oxygen therapy with FiO2 of 1.0. Interventions. Plasma exchange was started on day 6. Endpoints and Main Results. A marked improvement of the patient's general condition was observed after initiation of plasma exchange, with successful weaning of oxygen therapy and normalization of platelet count, troponin, and serum creatinine within four days. Conclusions. This case illustrates the efficacy of plasma exchange in CAPS and the difficulty for physicians to determine the optimal timing of plasma exchange. PMID:27833765

  3. Pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Practical messages from the EULAR guidelines.

    Science.gov (United States)

    Vagelli, Roberta; Tani, Chiara; Mosca, Marta

    2017-01-25

    Over the last few decades, reproductive medicine has observed an improvement in the management and outcome of pregnancy in connective tissue diseases, such as systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). However, pregnancy and related issues remain a challenge in these patients. In routine clinical practice, health professionals dealing with SLE and APS need to consider the numerous aspects of the reproductive life of their patients, such as pregnancy, family planning, fertility, contraception, cancer surveillance, and menopause. The new European League Against Rheumatism recommendations for women's health and family planning reflect the need for a novel approach to communication in the patient-physician relationship. Preconception counseling is essential to ensure optimal pregnancy outcomes through a careful risk stratification involving disease activity, organ involvement, autoantibody profile, use of drugs, and previous pregnancy outcomes, as well as to ensure better preventive and therapeutic strategies to limit complications. In patients with stable/inactive disease and low risk of thrombosis, adequate hormonal contraception and menopausal replacement therapy should be recommended. Assisted reproductive techniques can be safely used in these patients, but anticoagulation or low-dose aspirin (or both) should be added in those with positive antiphospholipid antibody titers. All menstruating women should be counseled on the possibility to preserve fertility with gonadotropin- ‑releasing hormone analogues if receiving alkylating agents. Strict clinical, serological, laboratory, and multidisciplinary monitoring during pregnancy is mandatory to early recognize and effectively treat disease flares or obstetric complications. Doppler ultrasonography and fetal biometry should be regularly performed, especially in the second and third trimesters. Physicians should recommend screening for cervical dysplasia related to human papillomavirus

  4. Positioning Theory in Paradigms

    Institute of Scientific and Technical Information of China (English)

    FU Xiao-qiu

    2015-01-01

    This article discusses the importance of theory and paradigm to a researcher. It starts from introducing and analyzing the definition of the two terms, by using the theories in the field of intercultural communication as examples. To a good researcher, he needs not only clarifying the paradigm his research is positioned, but also integrating the theories in his paradigm.

  5. Paradigms of polyamory.

    Science.gov (United States)

    Zambrano, M

    1999-01-01

    SUMMARY The paradigm theory of Thomas Kuhn is used as a framework to discuss alternative ways of intimacy. The author discusses the implications of structuring actual lesbian relationships by a paradigm of monogamy among Latin-American women. The author proposes that creating alternative paradigms of multiple relationships would be useful for many lesbians as models for alternative life patterns.

  6. Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

    Directory of Open Access Journals (Sweden)

    Steven S. Saraf

    2015-01-01

    Full Text Available A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient’s vision improved and remained stable at 20/200 and 20/80.

  7. Organ Damage and Quality of Life in Antiphospholipid Syndrome.

    Science.gov (United States)

    Alba, P; Gómez-Puerta, J A; Goycochea-Robles, M V; Amigo, M C

    2016-02-01

    Antiphospholipid syndrome (APS) affects young patients in the most productive years of their life, and the consequences of organic or tissue damage involve a decrease in health-related quality of life (HRQoL). While acute disease manifestations of APS are well known, information on the long-term prognosis and damage in affected patients is still very limited. Systemic lupus erythematosus (SLE) patients would be expected to experience long-term complications and even die as a consequence of APS. Organ damage in APS has been evaluated using different methods and definitions, including the SLICC/ACR Damage Index (SDI), which tend to underestimate aPL-related damage. A new damage index in APS has been proposed (DIAPS), and it seems to be more accurate than SDI. Given the implications for morbidity and mortality, it is imperative to assess accurately aPL-related damage and HRQoL in patients with APS.

  8. NEW ORAL ANTICOAGULANTS IN THE THERAPY OF ANTIPHOSPHOLIPID SYNDROME

    Directory of Open Access Journals (Sweden)

    M. A. Satybaldyeva

    2016-01-01

    Full Text Available The vitamin K antagonist warfarin is an essential medicine from a group of anticoagulants, which is used to treat antiphospholipid syndrome (APS. However, it has a number of disadvantages especially in patients who need longterm and frequently lifetime prevention of thromboses. New oral anticoagulants, such as dabigatran etexilate (Pradaxa®, rivaroxaban (Xarelto®, apixaban (Eliquis and others, have been recently synthesized. Unlike warfarin, they are administered at fixed doses, require neither routine monitoring nor diet, and interact with drugs only in small amounts. The new oral anticoagulants have been approved for certain indications, but the data of performed trials are inapplicable to patients with APS. These medicines are expected to improve quality of life in patients with this condition. 

  9. Primary antiphospholipid syndrome and panhypopituitarism: a unique presentation.

    Science.gov (United States)

    da Silva, Bárbara Santos Pires; Bonin, Camila; Bueno, Cristina Bellotti Formiga; Glezer, Andrea; Bronstein, Marcello D; Carvalho, Jozélio Freire

    2012-01-01

    Lymphocytic hypophysitis (LH) has been described previously in systemic lupus erythematosus (1.3%), Sjögren's syndrome (0.8%). Lymphocytic hypophysitis (LH) is rarely associated with rheumatic diseases, although three cases of pituitary disease associated with antiphospholipid syndrome (APS) have been described. Here, we report a possible association between APS and LH for the first time. A 34-yr-old woman with primary APS presented with polyuria, polydipsia, hypernatremia and impaired vision. Her hormone profile was compatible with panhypopituitarism, and sellar magnetic resonance imaging (MRI) depicted a normal pituitary gland with a thickened and displaced stalk and infundibulum portion. Hormone replacement was started, and the patient experienced a good clinical evolution.

  10. Atherosclerotic vessel damage in systemic lupus erythematosus and antiphospholipid syndrome in men

    Directory of Open Access Journals (Sweden)

    A. I. Iljina

    2005-01-01

    Full Text Available Objective. To study prevalence of clinical and subclinical atherosclerosis signs in men with systemic lupus erythematosus (SLE and antiphospholipid syndrome, to assess relationship between atherosclerotic vessel damage, risk factors, CRP and anti-cardiolipin antibodies (АСА Material and methods. 62 pts were included. Mean age was 35,7+11,6 years, mean disease duration - 129,3± 102 months. Traditional and related to the disease risk factors were analyzed. To reveal atherosclerotic vessel damage carotid sonographic examination was performed. Serum CRP concentration was evaluated by high sensitivity nephelometric immunoassay. IgG and IgM АСА were assessed by solid-phase immuno-enzyme assay. Results. Sonographic signs of carotid damage was revealed in 58% of pts, clinical signs of atherosclerosis - in 42%. Pts were divided into two groups according to intima-media complex thickness (IMCT. Group I included 36 pts with atherosclerotic vessel damage signs (IMCT?0,9 mm. Group 2-26 pts with IMCT<0,9 mm. Mean age at the examination, age of disease onset, disease duration, smoking frequency damage index in group I pts were higher than in group 2 pts. Mean CRP concentration in atherosclerosis group was significantly higher than in group 2 (p=0,007. 19 pts had APS signs. 43 pts did not. CRP level significantly correlated with IMCT in SLE pts with and without APS (p<0,05. Pts with atherosclerosis had higher IgG АСА level though the differences were not statistically significant. Conclusion. Men with SLE with or without APS have high risk of atherosclerosis development. CRP elevation is associated with IMCT increase.

  11. Altered fibrin clot structure/function in patients with antiphospholipid syndrome: association with thrombotic manifestation.

    Science.gov (United States)

    Celińska-Lowenhoff, M; Iwaniec, T; Padjas, A; Musiał, J; Undas, A

    2014-08-01

    We tested the hypothesis that plasma fibrin clot structure/function is unfavourably altered in patients with antiphospholipid syndrome (APS). Ex vivo plasma clot permeability, turbidity and susceptibility to lysis were determined in 126 consecutive patients with APS enrolled five months or more since thrombotic event vs 105 controls. Patients with both primary and secondary APS were characterised by 11% lower clot permeability (p<0.001), 4.8% shorter lag phase (p<0.001), 10% longer clot lysis time (p<0.001), and 4.7% higher maximum level of D-dimer released from clots (p=0.02) as compared to the controls. Scanning electron microscopy images confirmed denser fibrin networks composed of thinner fibres in APS. Clots from patients with "triple-antibody positivity" were formed after shorter lag phase (p=0.019) and were lysed at a slower rate (p=0.004) than in the remainder. Clots from APS patients who experienced stroke and/or myocardial infarction were 8% less permeable (p=0.01) and susceptible to lysis (10.4% longer clot lysis time [p=0.006] and 4.5% slower release of D-dimer from clots [p=0.01]) compared with those following venous thromboembolism alone. Multivariate analysis adjusted for potential confounders showed that in APS patients, lupus anticoagulant and "triple-positivity" were the independent predictors of clot permeability, while "triple-positivity" predicted lysis time. We conclude that APS is associated with prothrombotic plasma fibrin clot phenotype, with more pronounced abnormalities in arterial thrombosis. Molecular background for this novel prothrombotic mechanism in APS remains to be established.

  12. Glycopeptide profiling of beta-2-glycoprotein I by mass spectrometry reveals attenuated sialylation in patients with antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Kondo, Akira; Miyamoto, Toshiaki; Yonekawa, Osamu;

    2009-01-01

    beta2-glycoprotein I (beta2GPI) is a five-domain protein associated with the antiphospholipid syndrome (APS), however, its normal biological function is yet to be defined. beta2GPI is N-glycosylated at several asparagine residues and the glycan moiety conjugated to residue 143 has been proposed...... and the pathology of antiphospholipid syndrome....

  13. Catastrophic Antiphospholipid Syndrome Presenting as Ischemic Pancreatitis in Systemic Lupus Erythematosus

    Directory of Open Access Journals (Sweden)

    Ajit Vyas

    2009-09-01

    Full Text Available Context Antiphospholipid syndrome is often associated with systemic lupus erythematosus. Both syndromes have different clinical manifestations based on organ involvement. Antiphospholipid syndrome commonly causes spontaneous abortions, cerebral vascular occlusion, and deep venous thrombosis. Catastrophic antiphospholipid syndrome occurs when three or more organ systems are affected by thromboses in less than a week. Case report We report a unique case of a young woman with a history of systemic lupus erythematosus and antiphospholipid syndrome who presented with recurrent ischemic pancreatitis. Pancreatitis was refractory to anticoagulation and low dose steroids. Secondary to recurrence of pancreatitis and other organ involvement, she was treated as a presumed case of catastrophic antiphospholipid syndrome. Aggressive treatment with plasmapheresis, corticosteroids, cyclophosphamide, and anticoagulation eventually led to her recovery. Conclusion Awareness of this rare, rapidly fatal medical condition prompts vital, early intervention to improve patients’ survival. This case report aims to add to the limited therapeutic data available as well as suggest a possible approach to treating this rare syndrome with very high morbidity and mortality.

  14. The Investment Paradigm

    Science.gov (United States)

    Perna, Mark C.

    2005-01-01

    Is marketing an expense or an investment? Most accountants will claim that marketing is an expense, and clearly that seems true when cutting the checks to fund these efforts. When it is done properly, marketing is the best investment. A key principle to Smart Marketing is the Investment Paradigm. The Investment Paradigm is understanding that every…

  15. Antimitochondrial antibody

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/003529.htm Antimitochondrial antibody To use the sharing features on this page, please enable JavaScript. Antimitochondrial antibodies (AMA) are substances ( antibodies ) that form against mitochondria. ...

  16. Eculizumab Induces Sustained Remission in a Patient With Refractory Primary Catastrophic Antiphospholipid Syndrome.

    Science.gov (United States)

    Zikos, Thomas A; Sokolove, Jeremy; Ahuja, Neera; Berube, Caroline

    2015-09-01

    Catastrophic antiphospholipid syndrome (CAPS) is fatal in approximately 44% of patients in whom the diagnosis is made, thus demonstrating the inadequacy of current medical therapy. In this report, we discuss a 47-year-old man with a known history of primary antiphospholipid syndrome, who presented with CAPS after undergoing cholecystectomy and a treatment-refractory early relapse after development of colitis. Given the potential therapeutic efficacy of complement inhibition in antiphospholipid syndrome, the patient was administered eculizumab, a terminal complement inhibitor. Progressive clinical improvement and laboratory improvement were observed upon initiation of eculizumab. He has remained in remission for over 16 months of follow-up while on eculizumab. In conclusion, this case represents successful use of eculizumab for the treatment of primary CAPS.

  17. Monoclonal antibody "gold rush".

    Science.gov (United States)

    Maggon, Krishan

    2007-01-01

    The market, sales and regulatory approval of new human medicines, during the past few years, indicates increasing number and share of new biologics and emergence of new multibillion dollar molecules. The global sale of monoclonal antibodies in 2006 were $20.6 billion. Remicade had annual sales gain of $1 billion during the past 3 years and five brands had similar increase in 2006. Rituxan with 2006 sales of $4.7 billion was the best selling monoclonal antibody and biological product and the 6th among the top selling medicinal brand. It may be the first biologic and monoclonal antibody to reach $10 billion annual sales in the near future. The strong demand from cancer and arthritis patients has surpassed almost all commercial market research reports and sales forecast. Seven monoclonal antibody brands in 2006 had sales exceeding $1 billion. Humanized or fully human monoclonal antibodies with low immunogenicity, enhanced antigen binding and reduced cellular toxicity provide better clinical efficacy. The higher technical and clinical success rate, overcoming of technical hurdles in large scale manufacturing, low cost of market entry and IND filing, use of fully human and humanized monoclonal antibodies has attracted funds and resources towards R&D. Review of industry research pipeline and sales data during the past 3 years indicate a real paradigm shift in industrial R&D from pharmaceutical to biologics and monoclonal antibodies. The antibody bandwagon has been joined by 200 companies with hundreds of new projects and targets and has attracted billions of dollars in R&D investment, acquisitions and licensing deals leading to the current Monoclonal Antibody Gold Rush.

  18. Parallel Programming Paradigms

    Science.gov (United States)

    1987-07-01

    GOVT ACCESSION NO. 3. RECIPIENT’S CATALOG NUMBER 4, TITL.: td Subtitle) S. TYPE OF REPORT & PERIOD COVERED Parallel Programming Paradigms...studied. 0A ITI is Jt, t’i- StCUI-eASSIICATION OFvrHIS PAGFrm".n Def. £ntered, Parallel Programming Paradigms Philip Arne Nelson Department of Computer...8416878 and by the Office of Naval Research Contracts No. N00014-86-K-0264 and No. N00014-85- K-0328. 8 ?~~ O .G 1 49 II Parallel Programming Paradigms

  19. A Case of Microangiopathic Antiphospholipid-Associated Syndromes during Pregnancy: Review of the Literature

    Directory of Open Access Journals (Sweden)

    Nobuhiro Suzumori

    2012-01-01

    Full Text Available Microangiopathic antiphospholipid-associated syndromes (MAPSs are reported as encompassing several conditions mainly affecting the microvasculature of selected organs: the liver in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet; kidney, brain, and skin in TTP (thrombotic thrombocytopenic purpura. It is predominant in patients with catastrophic antiphospholipid syndrome (APS. A recent report suggests that APS is not only a thrombotic disease but also associated with microangiopathic features, and it can explain the greater prevalence of HELLP syndrome in these patients. We here report a case of MAPS during pregnancy associated with systemic lupus erythematosus (SLE in early second trimester.

  20. Haemorrhage during cesarean section for parturient with antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Shruti Shah

    2015-01-01

    Full Text Available This case describes a 39-year-old G3P2 parturient with a history of the antiphospholipid syndrome (APS, who experienced severe hemorrhage during her cesarean section (CS delivery of twins. At 36 weeks gestation, the patient was being treated prophylactically with Lovenox and acetylsalicyclic acid. In preparation for delivery, her medications were discontinued 24 h prior to admission. Due to breech presentation, the patient required delivery by CS. The patient received epidural anesthesia and successfully delivered two healthy babies. Following delivery, the patient became hypotensive and unresponsive and experienced uterine atony with profuse bleeding. Based on the patient′s clinical symptoms and history of APS, hemorrhage was suspected. Airway patency was immediately established using rapid sequence intubation, and the patient was placed under general anesthesia for removal of her atonic uterus. Following massive fluid resuscitation and correction of her coagulopathy, the patient stabilized and was transferred to the surgical intensive care unit. Four days later, she was discharged from the hospital without further complications.

  1. Update in management of antiphospholipid syndrome%抗磷脂综合征诊治进展

    Institute of Scientific and Technical Information of China (English)

    鲍春德; 陈晓翔

    2004-01-01

    @@ 抗磷脂综合征(anti-phospholipid syndrome,APS)是一组以反复动静脉血栓形成、习惯性流产和血小板减少等症状为表现的一组临床综合征,该综合征与抗磷脂抗体(antiphospholipid antibody,aPL)密切相关.抗磷脂抗体综合征是涉及包括风湿科、血液科、神经科、皮肤科、眼科、妇产科和血管外科在内的众多学科的一种综合征,临床表现多变复杂,累及多个系统,但血管栓塞是临床表现的根源,近年又发现APS的远期表现还有动脉粥样硬化,颈动脉的内膜增厚的发生率也明显升高[1].

  2. Dilazep and dipyridamole inhibit tissue factor expression on monocytes induced by IgG from patients with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    Hong ZHON

    2004-01-01

    AIM: To investigate whether antiplatelet agents, dilazep and dipyridamole, inhibit tissue factor (TF) expression on monocytes induced by IgG from patients with antiphospholipid syndrome (APS). METHODS: Freshly isolated peripheral blood monocytes were allowed to adhere on plastic and then cultured in media containing patient or control antibodies and/or other agonists with or without dilazep or dipyridamole. The TF activity on monocytes was investigated by measuring factor VIIa-dependent generation of factor Xa, using a chromogenic substrate and the TF mRNA expression was examined by real-time PCR (TaqMan PCR). RESULTS: The TF activity on monocytes induced by APS IgG (250 mg/L) was inhibited by dilazep (0.15-150 μmol/L) and dipyridamole (0.2-200 μrmol/L) in a dose-dependent fashion. But, the TF mRNA expression induced by APS IgG was not inhibited. Theophylline (500 μmol/L), an adenosine receptor antagonist, could counteract the inhibitory effect of dilazep and dipyridamole on TF activity. CONCLUSION: Antiplatelet agents, dilazep and dipyridamole, block APS IgG-induced monocytes TF expression at a post-transcriptional level, partly by adenosine receptor pathway. Pharmacological agents that block monocytes TF activity, such as dilazep and dipyridamole, are a novel therapeutic approach in APS.

  3. Anti-Jo-1 myositis and the antiphospholipid syndrome showing right ventricular thrombus: a novel overlap syndrome with atypical presentation.

    Science.gov (United States)

    Wang, Ching-Hsun; Wang, Ning-Chi; Lin, Te-Yu; Chen, Chen-Hung

    2014-09-01

    It has long been recognized that patients with myositis and positive anti-Jo1 antibody tend to be associated with interstitial lung disease. Recent studies revealed that such patients may also have fever, Raynaud's phenomenon, mechanic's hand, polyarthralgia, or usually mild, self-limiting, non-erosive or erosive polyarthritis known as antisynthetase syndrome. The hallmark of this disorder is the presence of the autoantibodies that recognize the aminoacyl-tRNA synthetases, which play a critical role in protein synthesis. The most well recognized of the autoantibodies is anti-histidyl (Jo-1). Antisynthetase syndrome cases associated with other autoimmune diseases are rarely reported. We here present a case of antisynthetase syndrome presented with right ventricle thrombus and deep vein thrombosis in the lower limbs. Secondary antiphospholipid syndrome was then diagnosed after a series of examinations. The patient was successfully treated with anticoagulant alone without surgical thrombectomy. Our case revealed that clinical physicians should watch for thrombotic complications when facing patients with antisynthetase syndrome. Medical therapy with anticoagulants alone may be an alternative treatment option in patients with right ventricle thrombus who cannot tolerate surgical thrombectomy.

  4. Rituximab therapy for factor II inhibitor in a patient with antiphospholipid antibody syndrome.

    Science.gov (United States)

    Guddati, Achuta K; Kuter, David J

    2014-04-01

    Factor II inhibitors have been associated with an increased risk of bleeding. The management of patients with factor II inhibitors has not been adequately described. We describe a patient with an increased bleeding tendency due to factor II inhibitor who was unable to undergo surgery due to her bleeding tendency. The patient was successfully treated with a course of rituximab, which markedly reduced her factor II inhibitor: the factor II level rose from 12 to 61%; prothrombin time decreased from 20 to 14.7 s; and partial thromboplastin time (PTT) decreased from 148 to 38.8 s. She was able to undergo abdominal surgery without any hemorrhagic complications. This case exemplifies the possibility of treating patients with factor II inhibitors with rituximab therapy.

  5. Development of auto-antibodies towards ß2-glycoprotein I in the antiphospholipid syndrome

    NARCIS (Netherlands)

    van Os, G.M.A.

    2011-01-01

    Het plasma-eiwit ß2GPI kan binden aan oppervlakte-eiwitten van de bacterie Streptococcus pyogenes. Vier eiwitten van deze bacterie (M1-eiwit, eiwit H, SclA en SclB), kunnen binden aan ß2GPI. Alleen de binding aan eiwit H induceert een conformationele verandering in ß2GPI. Gwen van Os onderzocht de w

  6. Female Infertility and Serum Auto-antibodies: a Systematic Review.

    Science.gov (United States)

    Deroux, Alban; Dumestre-Perard, Chantal; Dunand-Faure, Camille; Bouillet, Laurence; Hoffmann, Pascale

    2016-09-14

    On average, 10 % of infertile couples have unexplained infertility. Auto-immune disease (systemic lupus erythematosus, anti-phospholipid syndrome) accounts for a part of these cases. In the last 20 years, aspecific auto-immunity, defined as positivity of auto-antibodies in blood sample without clinical or biological criteria for defined diseases, has been evoked in a subpopulation of infertile women. A systematic review was performed (PUBMED) using the MESH search terms "infertility" and "auto-immunity" or "reproductive technique" or "assisted reproduction" or "in vitro fertilization" and "auto-immunity." We retained clinical and physiopathological studies that were applicable to the clinician in assuming joint management of both infertility associated with serum auto-antibodies in women. Thyroid auto-immunity which affects thyroid function could be a cause of infertility; even in euthyroidia, the presence of anti-thyroperoxydase antibodies and/or thyroglobulin are related to infertility. The presence of anti-phospholipid (APL) and/or anti-nuclear (ANA) antibodies seems to be more frequent in the population of infertile women; serum auto-antibodies are associated with early ovarian failure, itself responsible for fertility disorders. However, there exist few publications on this topic. The methods of dosage, as well as the clinical criteria of unexplained infertility deserve to be standardized to allow a precise response to the question of the role of serum auto-antibodies in these women. The direct pathogenesis of this auto-immunity is unknown, but therapeutic immunomodulators, prescribed on a case-by-case basis, could favor pregnancy even in cases of unexplained primary or secondary infertility.

  7. Spontaneous Thrombosis of a Bicuspid Aortic valve due to Primary Antiphospholipid Syndrome

    Directory of Open Access Journals (Sweden)

    Sarah Farrell

    2010-08-01

    Full Text Available We present the case of a 51-year-old man who was admitted as an emergency with spontaneous thrombosis of the aortic valve and ascending aorta. At operation he was found to have a congenitally bicuspid aortic valve and subsequent investigation revealed primary antiphospholipid syndrome. He underwent successful removal of the thrombus combined with mechanical replacement of the aortic valve.

  8. Myocardial ischaemia with a normal coronary angiogram due to the primary antiphospholipid syndrome

    NARCIS (Netherlands)

    de Vries, PAM; van der Sluis, A; van der Horst, JCC; van Veldhuisen, DJ

    2002-01-01

    In this case report, we describe a 33-year-old woman with a history of two unprovoked thrombo-embolic events presenting with acute myocardial ischaemia. She had a normal coronary angiogram (CAG). The diagnosis primary antiphospholipid syndrome (APS), an acquired hypercoagulability disorder, was esta

  9. Clinical significance of C4d in SLE and antiphospholipid syndrome

    NARCIS (Netherlands)

    Cohen, Danielle

    2012-01-01

    This thesis describes the clinical significance of thebiomarker C4d, a split product of the complement system, in several manifestations of systemic autoimmunediseases such as SLE and antiphospholipid syndrome. The findings in this thesis suggest that this biomarker might be of use in unraveling dis

  10. Cardiac involvement in antiphospholipid syndrome associated with Sneddon syndrome: a challenging diagnosis.

    Science.gov (United States)

    Faustino, Ana; Paiva, Luís; Morgadinho, Ana; Trigo, Emília; Botelho, Ana; Costa, Marco; Leitão-Marques, António

    2014-02-01

    Sneddon syndrome is a rare clinical entity characterized by the association of ischemic cerebrovascular disease and livedo reticularis. The authors report a case of stroke and myocardial infarction in a 39-year-old man with Sneddon syndrome and antiphospholipid syndrome who subsequently met some criteria for systemic lupus erythematosus, highlighting the complexity of cardiovascular involvement in systemic diseases.

  11. Post-partum bilateral renal cortical necrosis in antiphospholipid syndrome and systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Venkat Sainaresh Vellanki

    2013-01-01

    Full Text Available In the presence of systemic lupus erythematosus or related autoimmune disorders, antiphospholipid syndrome (APS is termed secondary APS. Pregnancy-related renal failure due to SAPS is rarely reported in the literature. We present the case of a young primgravida woman with bilateral renal cortical necrosis due to secondary APS in late pregnancy.

  12. [Embolic stroke by thrombotic non bacterial endocarditis in an Antiphospholipid Syndrome patient].

    Science.gov (United States)

    Graña, D; Ponce, C; Goñi, M; Danza, A

    2016-01-01

    The antiphospholipid syndrome (APS) is an acquired thrombophilia, considered a systemic autoimmune disorder. We report a patient with APS who presented multiple cerebral infarcts (stroke) as a complication of a thrombotic non bacterial endocarditis. We review the literature focused on the physiological mechanism that produce this disease and its complications. Clinical features and their prognostic value and the different therapeutic options were also studied.

  13. Varfarin in the complex treatment of antiphospholipid syndrome: preliminary results

    Directory of Open Access Journals (Sweden)

    T M Reshetnyak

    2003-01-01

    Full Text Available Objective. To assess efficacy and tolerance of varfarin in prophylaxis and therapy of thrombotic complications in patients with antiphospholipid syndrome (APS. Methods. 20 pts with APS (5 male and 15 female received varfarin during a year. 8 of them had primary APS (PAPS and 12 -systemic lupus erythematosus with APS (SLE+APS. 2 other pts (I with SLE+APS and I with PAPS received varfarin during the last 4 years. Nobody from 9 pts with PAPS received corticosteroids (CS. In SLE+APS pts CS dose varied from 4 to 20 mg/day and was not increased during follow up. During the study prothrombine time (PT was examined with thromboplastin ( manufactured by Renam having international sensitivity index 1,2 and international normalization relation (INR. Depending on treatment scheme APS pts were divided into 3 groups. Group 1 included 8 pts with INR<2,0, Group 2-7 with INR >3,0, group 3 - 7 pts with INR<2,0 receiving as additional treatment thrombo ASS 100 mg/day and vasonit from 600 to 1200 mg/day. Results. Two pts with INR = 1,8 had thrombosis recurrence (due to leg thrombophlebitis. There were no recurrences in other groups. 2 from 22 pts had "large" bleedings. "Small" bleedings episodes were noted in 7 from 22 pts. Largely that were subcutaneous bleedings (in 4 pts no more than 5 cm of size. Two pts receiving varfarin with INR 1,8 and 2,4 had renal colic. Conclusion. Our preliminary results prove the necessity of inclusion of varfarin in the treatment of pts with APS and thrombosis but intensive anticoagulant effect is not always desired.

  14. Antiphospholipid syndrome presenting as progressive neuropsychiatric disorders: two case reports

    Directory of Open Access Journals (Sweden)

    Li CH

    2013-05-01

    Full Text Available Chien-Hsun Li,1–3 Mei-Chuan Chou,2,4 Ching-Kuan Liu,2,3 Chiou-Lian Lai2,31Department of Neurology, Fooyin University Hospital, Pingtung, Taiwan; 2Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan; 3Department of and Master’s Program in Neurology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; 4Department of Neurology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, TaiwanAbstract: The antiphospholipid syndrome (APS is a rare form of autoimmune coagulopathy. In this syndrome, the most common neurologic abnormality is transient ischemic attack. This can be easily overlooked if a patient presents with progressive neuropsychiatric disorders, such as depression or dementia. We report two cases of young women, aged 35 and 22 years, presenting with progressive depression and mental decline over a certain period. The neuropsychological diagnoses of the two patients were, respectively, dementia with disinhibition and borderline dementia with depression. Brain magnetic resonance imaging showed multiple old infarcts with encephalomalacia in the former case, and only one cortical hemorrhagic infarction, over the right temporoparietal lobe, observed in the latter case. The outcomes of the two cases were also very different. Progressive neuropsychiatric disorders are increasingly observed in the young; therefore, APS and other autoimmune diseases should be considered during the differential diagnosis. Brain imaging examinations may prevent a delay in the detection of a structural lesion and facilitate the early intervention with good prognosis. Careful investigations by experts from different disciplines are always encouraged in complicated cases.Keywords: autoimmune disease, brain imaging, dementia, depression, cerebrovascular disease

  15. Winnicott's paradigm outlined

    Directory of Open Access Journals (Sweden)

    Zeljko Loparic

    Full Text Available The main objective of this paper is to present a unified view of Winnicott’s contribution to psychoanalysis. Part I (Sections 1-4 starts off by recalling that, according to some important commentators, Winnicott introduced a change in paradigms in psychoanalysis. In order to show that this change can be viewed as an overall “switch in paradigms”, in the sense given by T. S. Kuhn, this paper presents an account of the Kuhn’s view of science and offers a reconstruction of Freud’s Oedipal, Triangular or “Toddler-in-the-Mother’s-Bed” Paradigm. Part II (Sections 5-13 shows that as early as the 1920’s Winnicott encountered insurmountable anomalies in the Oedipal paradigm and, for that reason, started what can be called revolutionary research for a new framework of psychoanalysis. This research led Winnicott, especially during the last period of his life, to produce an alternative dual or “Baby-on-the-Mother’s-Lap” Paradigm. This new paradigm is described in some detail, especially the paradigmatic dual mother-baby relation and Winnicott’s dominant theory of maturation. Final remarks are made regarding Winnicott’s heritage and the future of psychoanalysis.

  16. Antiphospholipid Syndrome - A Case Report of Pulmonary Thromboembolism, Followed with Acute Myocardial Infarction in Patient with Systemic Sclerosis

    Directory of Open Access Journals (Sweden)

    Marija Vavlukis

    2015-11-01

    CONCLUSION: The acquired antiphospholipid syndrome is common condition in patients with systemic autoimmune diseases, but relatively rare in patients with systemic sclerosis. Never the less, we have to be aware of it when treating the patients with systemic sclerosis.

  17. Three paradigms of horror

    Directory of Open Access Journals (Sweden)

    Dejan Ognjanović

    2016-07-01

    Full Text Available Starting with the definition of horror as a literary genre the core story of which is based on a meeting with threatening Otherness whose influx into consensual reality and it’s tacit normality creates unrest and awakens fear in the protagonists and the audience, this paper defines the three key paradigms of the horror genre, based on the causes of fear, or rather the “monstrous” Otherness in them. Paradigm 1 concerns the “fear of one’s own self”: the root of the fear is inside, in the individual psyche, in the split, deceived, or in some other way unreliable self which is, consciously or unconsciously, harmful to others, and ultimately to itself. Paradigm 2 deals with the “Fear of others”: the root of fear is outside and is concerned with other people and other creatures which have an urge to occupy a certain human microcosm. Paradigm 3 is concerned with the “Fear of the numinous”: the root of the fear is mostly situated on the outside; however its shape is amorphous, ambivalent and unknowable. The “monster” is faceless; it touches on primary forces of the divine/demonic, and as such is situated on the very border between inside/outside. All three paradigms, with their main approaches and constitutive elements, are modulated through two basic possible treatments: the conservative and the progressive (liberal, which affords a total of six basic variations of horror. Starting from definitions given by John Carpenter, Robin Wood and his own, the author analyzes representative examples from horror literature and film for each paradigm and its variation, with a special accent on the image of Otherness and its connection to the norm, its intrusion into the status quo, anthropocentrism and the presence or absence of a happy ending. The paper demonstrates the richness of connotative potential within the horror genre and provides a basis for its taxonomy.

  18. Alternative Evaluation Research Paradigm.

    Science.gov (United States)

    Patton, Michael Quinn

    This monograph is one of a continuing series initiated to provide materials for teachers, parents, school administrators, and governmental decision-makers that might encourage reexamination of a range of evaluation issues and perspectives about schools and schooling. This monograph is a description and analysis of two contrasting paradigms: one…

  19. Anti-β2 Glycoprotein-I Antibody in Acute Myocardial Infarction

    Directory of Open Access Journals (Sweden)

    Mohammad Shojaei

    2011-01-01

    Full Text Available Problem statement: Ischemic cardiac manifestations have been reported in a various percentage of patients with anti-phospholipid antibodies. Data concerning the relation between anti- Phospholipid (aPL antibodies and myocardial infarction in subjects without evidence of overt autoimmune disease are conflicting. Anti-beta2 glycoprotein-I (anti-beta2-GPI antibody is detected in various diseases like rheumatoid arthritis, systemic lupus erythematosus and anti-phospholipid antibody syndrome. The study of anti-beta2-GPI antibody in Acute Myocardial Infarction (AMI might shed light on etiologic mechanisms in the pathogenesis of acute coronary syndromes. The purpose of the present study was to determine association of plasma aPL antibodies, namely, antibeta2- GPI antibodies, with AMI. This study was designed to investigate whether prevalence of antibeta2- GPI antibodies, in patients who had acute myocardial infarction and to analyze their relationship with traditional cardiovascular risk factors. Approach: We investigated the prevalence of anti-beta2- GPI IgG in a well characterized group of patients with AMI as a case group. Sera from 74 patients with AMI and from 76 healthy subjects, matched for age and sex as a control group. Using ELISA to evaluate the presence of IgG isotype of anti-beta2-GPI autoantibodies in their sera. Results: The prevalence of anti-beta2-GPI IgG in the control group (10.50% resulted significantly lower than in patients with AMI (37.80% (pConclusion: Our findings suggest that anti-beta2-GPI IgG antibodies seemed to behave as independent risk factors for myocardial infarction, which may represent a link between autoimmunity and atherosclerosis in patients with acute myocardial infarction. Further studies with bigger patients are needed to explore association of anti-β2-GPI IgG with STEMI and NSTEMI.

  20. 膜联蛋白A2在抗磷脂综合征致病机制中的研究进展%Progress in study on role of annexin A2 in the pathogenesis of antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    周婷; 赵福涛

    2011-01-01

    @@ 抗磷脂抗体(antiphospholipid antibodies,APL)综合征(antiphospholipid syndrome,APS)是一种自身免疫性疾病,以血清中持续存在阳性APL、动静脉血栓、习惯性流产和死胎为主要临床表现.APL是一组针对磷脂或磷脂复合物的自身抗体总称,主要包括狼疮抗凝物(LA)、抗心磷脂抗体(ACL)、抗β2糖蛋白1(β2GP1).一直以来,很多学者都致力于研究APS的发病机制,先后提出过几种观点,如凝血机制异常和纤溶系统紊乱等假说[1].最近的研究表明,在APS血栓形成中,膜联蛋白家族,尤其是膜联蛋白A2起着十分重要的作用.本文就膜联蛋白A2在APS发病机制中的作用作一综述.

  1. 表现为皮肤大片瘀斑和坏死的抗磷脂抗体综合征%A case of antiphospholipid syndrome (APS) presenting with large areas of ecchymosisand necrosis on skin

    Institute of Scientific and Technical Information of China (English)

    夏济平; 许阳; 侯麦花; 张美华

    2012-01-01

    报告1例表现为皮肤大片瘀斑和坏死的抗磷脂抗体综合征.患者女性,42岁.双侧臀部及下肢大片瘀斑、坏死,血小板减少,抗核抗体、抗心磷脂抗体异常升高,补体降低.皮损组织病理示真皮血管有血栓存在.给予抗凝、血管扩张剂、糖皮质激素、免疫抑制剂和静脉注射用人免疫球蛋白等控制病情的同时,外科手术修复大片坏死皮肤.%A case of antiphospholipid syndrome (APS) presenting with large areas of ecchymosis and necrosis on skin is reported. A 42-year-old female suffered from skin ecchymosis and necrosis in buttocks and lower limbs with thrombocytope-nia, abnormal elevation of ANA and antiphospholipid antibodies (APL), and low complements. Histopathology revealed thrombosis of dermal and subcutaneous vessels. As treatment with anticoagulation, vasodilation, immunosuppressant, intravenous im-munoglobulin, the condition of the patient was changed successfully. And then the effective surgical treatment restored the large area of the necrotic skin.

  2. 抗磷脂综合征患者血栓事件的危险因素分析%Analysis of risk factors in development of thrombosis in patients with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    李茹; 周云杉; 贾园; 栗占国

    2012-01-01

    目的:探讨影响抗磷脂综合征( antiphospholipid syndrome,APS)患者血栓事件发生的危险因素.方法:回顾性分析北京大学人民医院收治的61例APS患者血栓事件的发生特点,评价血栓事件与患者临床及免疫学指标的相关性,包括性别、年龄、有无血小板减少、吸烟、高血压、糖尿病、高脂血症和抗磷脂抗体等.结果:70.49% APS患者发生血栓事件,其中动脉血栓发生率36.67%,静脉血栓发生率39.34%,下肢静脉血栓和脑梗塞最常见,分别占37.7%和24.59%,其次为肺栓塞、血栓性微血管病、肾梗死和脾梗死.抗心磷脂抗体阳性和高血压分别是静脉和动脉血栓事件的独立危险因素.结论:抗心磷脂抗体阳性者易发生静脉血栓,而合并高血压的APS患者易发生动脉血栓.%Objective: To analyze the risk factors in the development of thrombosis in antiphospholipid syndrome (APS) patients. Methods: We retrospectively analyzed the characteristics of thrombosis in 61 APS patients. Clinical and laboratory parameters such as sex, age, thrombocytopenia, smoking, hypertension, diabetes mellitus, hyperlipidemia and anti-phospholipid antibodies were investigated to find out the risk factors of thrombosis. Results: Thrombosis was found in 70.49% APS patients, of whom, the prevalence of arterial and venous thrombosis were 36. 67% and 39. 34% , respectively. Deep vein thrombosis of lower extremity (37. 7% ) and cerebral infarction (24. 59% ) were the most common thrombosis events, and then pulmonary embolism, thrombotic microangiopathy and renal artery thrombosis were also common in APS patients. The positive anti-cardiolipin antibody and hypertension were the independent risk factors for venous and arterial thrombosis, respectively. Conclusion: Anti-cardiolipin antibody is associated with a higher risk of venous thrombosis, and hypertension is associated with arterial thrombosis.

  3. Pregnancy outcome in women with antiphospholipid syndrome and alloimmunity: a case report

    Directory of Open Access Journals (Sweden)

    Serguei Abel Castañeda Ospina

    Full Text Available CONTEXT: Patients with antiphospholipid syndrome and alloimmunity have poor pregnancy outcomes. Several diagnostic and therapeutic options exist for these disorders, although there is no consensus as to the best treatment. CASE REPORT: We present here the clinical course and treatment of a woman with a history of two miscarriages who joined our program 10 years ago and has been followed up ever since. After antiphospholipid syndrome and alloimmune failure were diagnosed, she was given preconceptional treatment using unfractionated heparin, aspirin, prednisone and lymphocyte immunizations. She delivered two premature babies in the following two pregnancies. At present both children are healthy and are attending school. The fifth pregnancy was unsuccessful, in spite of having undergone a similar but postconceptional therapeutic scheme. We discuss this case focusing on the pathogenic mechanisms and the therapeutic aspects of these disorders.

  4. [Maternal adrenal necrosis in the third trimester of pregnancy: a rare complication of antiphospholipid syndrome].

    Science.gov (United States)

    Legendre, G; Vauthier-Brouzes, D; Cornet, A; Al Hawari, M; Renard-Penna, R; Piette, J-C; Dommergues, M

    2008-04-01

    Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy.

  5. Prolactin May Not Play a Role in Primary Antiphospholipid (Hughes') Syndrome

    OpenAIRE

    Manoel Tavares Neves Junior; Carlos Ewerton Maia Rodrigues; Jozelio Freire de Carvalho

    2011-01-01

    The relationship between prolactin (PRL) and the immune system has been demonstrated in the last two decades and has opened new windows in the field of immunoendocrinology. However, there are scarce reports about PRL in primary antiphospholipid syndrome (pAPS). The objective of this study was to evaluate PRL levels in patients with pAPS compared to healthy controls and to investigate their possible clinical associations. Fifty-five pAPS patients according to Sapporo criteria were age- and sex...

  6. Pancreaticoportal Fistula in Association with Antiphospholipid Syndrome Presenting as Ascites and Portal System Thrombosis

    Directory of Open Access Journals (Sweden)

    Li-Hsin Chang

    2002-01-01

    Full Text Available Fistulous communication between the pancreas and the portal venous system is extremely rare and is usually a complication of chronic pancreatitis or pancreatic pseudocysts. A patient who presented with abdominal pain and ascites secondary to a pancreaticoportal fistula and portal system thrombosis is described. The diagnosis was made by endoscopic retrograde cholangiopancreatography and confirmed by immediate postprocedure computed tomographic scanning. Laboratory studies identified concomitant antiphospholipid syndrome. The patient responded favourably to supportive medical therapy.

  7. Calcified right intraventricular thrombus in a patient with systemic lupus erythematous and antiphospholipid syndrome.

    Science.gov (United States)

    Bittencourt, Márcio Sommer; Seltmann, Martin; Muschiol, Gerd; Achenbach, Stephan

    2010-01-01

    A 37-year-old patient with known systemic lupus erythematous, antiphospholipid syndrome and previous pulmonary embolism presented with non-ST elevation myocardial infarction while on adequate anticoagulation therapy. The patient was further evaluated with cardiac computed tomography. A small diagonal branch occlusion was the only coronary lesion present. A partially calcified right ventricular thrombus was incidentally found. Because of the small vessel size, cardiac catheterization was deemed unnecessary, and the patient was discharged with adjustment of immunosuppressive therapy and anticoagulation.

  8. New onset neuromyelitis optica in a young Nigerian woman with possible antiphospholipid syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Komolafe Morenikeji A

    2008-11-01

    Full Text Available Abstract Introduction Devic's neuromyelitis optica is an inflammatory demyelinating disease that targets the optic nerves and spinal cord. It has a worldwide distribution and distinctive features that distinguish it from multiple sclerosis. There has been no previous report of neuromyelitis optica from our practice environment, and we are not aware of any case associated with antiphospholipid syndrome in an African person. Case presentation We report the case of a 28-year-old Nigerian woman who presented with neck pain, paroxysmal tonic spasms, a positive Lhermitte's sign and spastic quadriplegia. She later developed bilateral optic neuritis and had clinical and biochemical features of antiphospholipid syndrome. Her initial magnetic resonance imaging showed a central linear hyperintense focus in the intramedullary portion of C2 to C4. Repeat magnetic resonance imaging after treatment revealed resolution of the signal intensity noticed earlier. Conclusion Neuromyelitis optica should be considered in the differential diagnoses of acute myelopathy in Africans. We also highlight the unusual association with antiphospholipid syndrome. Physicians should screen such patients for autoimmune disorders.

  9. Lepromatous leprosy patients produce antibodies that recognise non-bilayer lipid arrangements containing mycolic acids

    Directory of Open Access Journals (Sweden)

    Isabel Baeza

    2012-12-01

    Full Text Available Non-bilayer phospholipid arrangements are three-dimensional structures that form when anionic phospholipids with an intermediate structure of the tubular hexagonal phase II are present in a bilayer of lipids. Antibodies that recognise these arrangements have been described in patients with antiphospholipid syndrome and/or systemic lupus erythematosus and in those with preeclampsia; these antibodies have also been documented in an experimental murine model of lupus, in which they are associated with immunopathology. Here, we demonstrate the presence of antibodies against non-bilayer phospholipid arrangements containing mycolic acids in the sera of lepromatous leprosy (LL patients, but not those of healthy volunteers. The presence of antibodies that recognise these non-bilayer lipid arrangements may contribute to the hypergammaglobulinaemia observed in LL patients. We also found IgM and IgG anti-cardiolipin antibodies in 77% of the patients. This positive correlation between the anti-mycolic-non-bilayer arrangements and anti-cardiolipin antibodies suggests that both types of antibodies are produced by a common mechanism, as was demonstrated in the experimental murine model of lupus, in which there was a correlation between the anti-non-bilayer phospholipid arrangements and anti-cardiolipin antibodies. Antibodies to non-bilayer lipid arrangements may represent a previously unrecognised pathogenic mechanism in LL and the detection of these antibodies may be a tool for the early diagnosis of LL patients.

  10. Challenging the Innovation Paradigm

    CERN Document Server

    Sveiby, Karl Erik; Segercrantz, Beata

    2012-01-01

    Innovation is almost always seen as a "good thing". Challenging the Innovation Paradigm is a critical analysis of the innovation frenzy and contemporary innovation research. The one-sided focus on desirable effects of innovation misses many opportunities to reduce the undesirable consequences. Authors in this book show how systemic effects outside the innovating firms reduce the net benefits of innovation for individual employees, customers, as well as for society as a whole - also the innovators' own organizations. This book analyzes the dominant discourses that construct and recons

  11. SUSTAINABLE DEVELOPMENT PARADIGM - SYNOPSIS

    Directory of Open Access Journals (Sweden)

    Constantinescu Andreea

    2014-07-01

    Full Text Available Even if sustainable development is a concept that gained quite recently its scientific prestige, through contribution of researchers its content has upgraded to a high degree of conceptual luggage and, through contribution from governance representatives, has gained an impressive good-practice background. Allowing the use of different methodological premises and conceptual tools, sustainable development paradigm is equipped with all the elements that would allow the opening of new horizons of knowledge. Based on the facility which can operate the concept of sustainable development, the European Union aims to develop both a more competitive economy based on environmental protection as well as a new governance of economic policy. This on one hand demonstrates the sustainable development ability to irradiate creativity towards the establishment of interdisciplinary bridges and on the other hand explains the growing interest of researchers interested in the problem of analyzing in detail this fruitful concept. Launched first as a theoretical framework to serve justify actions responsible for weighting economic growth, the concept of Sustainable Development has quickly become a topic of ethical debate circumscribed to the area of perfectibility of human nature to the necessity registry. In this regard, the philosophical content of this paradigm could not remain outside researchers concerns, who want to provide both policy makers and the general public a wide range of evidence to demonstrate the viability of this paradigm. Academia waits until maximization of the contribution of governance to achieve sustainable economic development, which consists in conjunction of this upward path with the momentum given by public policy sync, perfectly adapted for globalization era and all crises to come. However, because this concept based its structure and composition on three pillars, equally important economy, society and environment any attempt to strengthen

  12. Marketing! Where is Paradigm?

    Directory of Open Access Journals (Sweden)

    Deosir Flávio Lobo de Castro Júnior

    2015-09-01

    Full Text Available The quantitative- qualitative debate is not a new discussion. The aim of this study therefore is to check through the concept of paradigm, new perspectives to understand the academic research in marketing, developments of marketing thinking and methodologies used in the studies of quality of service. Without pretending to exhaust the subject and present a final conclusion, studies that point to the need and importance of qualitative research, as it helps the researcher to better understand the complex nature of the social world in which we live are presented. According to Santana and Gomes (2007, after examining the discussion of Hegel and Kant, reason and conclude that epistemology itself are historical buildings and evolve from contradictions. This article is divided into five moments. The first part presents besides introducing the constitution of the goals of this theoretical essay. The second part presents a brief discussion of the concept of paradigm and marketing. The third part presents a historical retrospective of marketing and its evolution from its schools from studies of Miranda and Arruda (2004. The fourth part presents the methodology of the studies on quality of services and finally the fifth part presents the final considerations.

  13. Lipoproteína(a na síndrome antifosfolípide primária Lipoprotein(a in primary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Jozélio Freire de Carvalho

    2009-06-01

    Full Text Available OBJETIVO: Avaliar níveis de lipoproteína(a em pacientes com síndrome antifosfolípide primária (SAFP e suas possíveis associações clínicas e laboratoriais. MÉTODOS: Estudo transversal de 46 pacientes (93,5% do sexo feminino com SAFP (critérios de Sapporo. Foram avaliados os dados demográficos e clínicos, medicações, anticorpos antifosfolípides, além da medida dos níveis séricos em jejum da lipoproteína(a. RESULTADOS: Os níveis de lipoproteína(a ( > 30 mg/dL foram vistos em 43,5% dos pacientes com SAFP, com média de 42 ± 43,5 mg/dL. Comparando-se o grupo com níveis maiores que 30 mg/dL com o grupo de pacientes com níveis menores ou iguais a este valor, não foram observadas diferenças significativas em relação a dados demográficos (idade, sexo, cor branca, peso, altura e índice de massa corporal, manifestações da doença (eventos arteriais, venosos, obstétricos, plaquetopenia, eventos cardiovasculares (infarto agudo do miocárdio, angina, acidente vascular cerebral, comorbidades, estilo de vida (atividade física, tabagismo atual e pregresso, uso de medicações (corticoide atual e pregresso, estatina, cloroquina, bem como à frequência de positividade de anticorpos antifosfolípides. CONCLUSÃO: Pacientes com SAFP apresentam uma frequência elevada de níveis aumentados de lipoproteína(a. Entretanto, nenhuma associação dessa anormalidade com as variáveis clínicas e laboratoriais estudadas foi encontrada.OBJECTIVE: To evaluate levels of lipoprotein(a in patients with primary antiphospholipid syndrome (PAPS and its possible associations with clinical and laboratory features. METHODS: Transversal study with 46 (93.5% female PAPS patients (Sapporo criteria. Demographic, clinical, drugs use, and antiphospholipid antibodies data were evaluated, as well as measurements of lipoprotein(a serum fasting levels. RESULTS: Elevated levels of lipoprotein(a ( > 30 mg/dL were observed in 43.5% of PAPS patients, with a mean

  14. Investigation on relation between recurrent spontaneous abortion and antiphospholipid thrombosis syndrome%复发性自然流产与抗磷脂血栓综合征相关性探讨

    Institute of Scientific and Technical Information of China (English)

    雷蕾; 周志中

    2001-01-01

    目的探讨复发性自然流产(RSA)的原因,了解其与抗磷脂血栓综合征(APL-T)的关系。方法采用ELISA法和PTT-LA法对32例复发性流产及20例正常对照进行抗心磷脂抗体(ACA)和狼疮抗凝物(LA)检测。结果 32例RSA患者中17例APA阳性,阳性率(53.1%)明显高于对照,其中LA阳性率为28.1%,亦明显高于对照。8例诊断为抗磷脂血栓综合征。结论 APA中之LA可能是RSA发生的重要原因,对不明原因的复发性自然流产可考虑为抗磷脂血栓综合征。%Objective To investigate the causes of recurrent spontaneous abortion(RSA) and to know the relation between RSA and antiphospholipid thrombosis syndrome(APL-T). Methods ELISA and PTT-LA were used to detect anticardiolipin antibody(ACA) and lupus anticoagulant(LA) in 32 patients with RSA and 20 normal controls(NC). Results There were 17 positive of antiphospholipid antibody(APA) in RSA group. The incidence was significantly higher than that in NC group. The incidence of LA was also significantly higher than that in NC group. 8 patients were diagnosed as APL-T. Conclusion LA may be the more important reason of RSA. It should be considered as APL-T when RSA is unexplained.

  15. Thyroid Antibodies

    Science.gov (United States)

    ... e.g., at regular intervals after thyroid cancer treatment) Thyroid stimulating hormone receptor antibody, Thyroid Stimulating Immunoglobulin TRAb, TSHR Ab, TSI Graves disease When a person has symptoms of hyperthyroidism If a pregnant woman has a known autoimmune ...

  16. The Peter Pan paradigm

    Directory of Open Access Journals (Sweden)

    Larson Janet E

    2008-01-01

    Full Text Available Abstract Genetic and environmental agents that disrupt organogenesis are numerous and well described. Less well established, however, is the role of delay in the developmental processes that yield functionally immature tissues at birth. Evidence is mounting that organs do not continue to develop postnatally in the context of these organogenesis insults, condemning the patient to utilize under-developed tissues for adult processes. These poorly differentiated organs may appear histologically normal at birth but with age may deteriorate revealing progressive or adult-onset pathology. The genetic and molecular underpinning of the proposed paradigm reveals the need for a comprehensive systems biology approach to evaluate the role of maternal-fetal environment on organogenesis. You may delay, but time will not Benjamin Franklin USA Founding Father

  17. The Peter Pan paradigm.

    Science.gov (United States)

    Cohen, J Craig; Larson, Janet E

    2008-01-08

    Genetic and environmental agents that disrupt organogenesis are numerous and well described. Less well established, however, is the role of delay in the developmental processes that yield functionally immature tissues at birth. Evidence is mounting that organs do not continue to develop postnatally in the context of these organogenesis insults, condemning the patient to utilize under-developed tissues for adult processes. These poorly differentiated organs may appear histologically normal at birth but with age may deteriorate revealing progressive or adult-onset pathology. The genetic and molecular underpinning of the proposed paradigm reveals the need for a comprehensive systems biology approach to evaluate the role of maternal-fetal environment on organogenesis."You may delay, but time will not" Benjamin Franklin, USA Founding Father.

  18. PARADIGM OF ACCOUNTING CHANGE

    Directory of Open Access Journals (Sweden)

    Constanta Iacob

    2016-12-01

    Full Text Available The words and phrases swop with each other and the apparent stability of a word’s meaning sometimes change in time. This explains why the generic term of accounting is used when referring to the qualities attributed to accounting,but also when it comes to organizing financial accounting function within the entity, and when referring concretely to keeping a double record with its specific means, methods and tools specific, respectively seen as a technical accounting.Speaking about the qualities of accounting, but also about the organizational form it takes, we note that there is a manifold meaning of the word accounting, which is why the purpose of this article is to demonstrate that the paradigm shift aimed at a new set of rules and if the rules changes, then we can change the very purpose of accounting.

  19. Paradigms for parasite conservation.

    Science.gov (United States)

    Dougherty, Eric R; Carlson, Colin J; Bueno, Veronica M; Burgio, Kevin R; Cizauskas, Carrie A; Clements, Christopher F; Seidel, Dana P; Harris, Nyeema C

    2016-08-01

    Parasitic species, which depend directly on host species for their survival, represent a major regulatory force in ecosystems and a significant component of Earth's biodiversity. Yet the negative impacts of parasites observed at the host level have motivated a conservation paradigm of eradication, moving us farther from attainment of taxonomically unbiased conservation goals. Despite a growing body of literature highlighting the importance of parasite-inclusive conservation, most parasite species remain understudied, underfunded, and underappreciated. We argue the protection of parasitic biodiversity requires a paradigm shift in the perception and valuation of their role as consumer species, similar to that of apex predators in the mid-20th century. Beyond recognizing parasites as vital trophic regulators, existing tools available to conservation practitioners should explicitly account for the unique threats facing dependent species. We built upon concepts from epidemiology and economics (e.g., host-density threshold and cost-benefit analysis) to devise novel metrics of margin of error and minimum investment for parasite conservation. We define margin of error as the risk of accidental host extinction from misestimating equilibrium population sizes and predicted oscillations, while minimum investment represents the cost associated with conserving the additional hosts required to maintain viable parasite populations. This framework will aid in the identification of readily conserved parasites that present minimal health risks. To establish parasite conservation, we propose an extension of population viability analysis for host-parasite assemblages to assess extinction risk. In the direst cases, ex situ breeding programs for parasites should be evaluated to maximize success without undermining host protection. Though parasitic species pose a considerable conservation challenge, adaptations to conservation tools will help protect parasite biodiversity in the face of

  20. Anticardiolipin antibodies in pathogenesis of infertility

    Directory of Open Access Journals (Sweden)

    Lončar Dragan

    2010-01-01

    Full Text Available Background/Aim. Antiphospholipid syndrome (APS is an autoimmune disorder clinically characterized by arterial or venous thrombosis and/or specific obstetric complications and presence of antiphospholipid antibodies (aPL in the serum. It occurs in 0.3% of pregnant women, while 1% of them have two spontaneous abortions. The aim of this study was to analyze the frequency of biphospholipid antibodies in pregnant women with recurrent spontaneous abortions. Methods. We analyzed 60 pregnant women who had two or more recurrent miscarriages. The control group included 60 healthy pregnant women. We analyzed titres of anticardiolipin (aCL IgG and/or IgM with high titres (> 20 U/mL, lupus anticoagulant (LAC antibodies and anti-beta-2 glycoprotein (b2-GP1 IgG as well as parameters of coagulation status of pregnant women. Results. Analyzing Spearman's rank correlation coefficient in a group of affected patients, we noticed a slightly positive correlation of lupus anticoagulants (LAC with aCL antibodies of both classes, while the correlation with b2GP1 IgG was negative. Both classes of aCL antibodies and antib2GP1 IgG were in a discrete positive correlation with the given variables. In the control group, there was a lack of consistency in correlation of the study variables with LAC-aCl IgG, compared to the affected patients, and there was a standard negative coefficient of correlation with anti-b2GP1 IgG. The correlation ratio of anti-b2GP1 IgG was negative for all studied test parameters. Analysis of hemostatic parameters showed a statistically significant difference in the concentration of fibrinogen (p < 0.01 and thrombocyte count (p < 0.05 between the study and the control group of pregnant women. Lower mean values of fibrinogen (2.90 ± 0.45 g/L and lower thrombocyte count [(179.20 ± 6.00 × 109] were found in the study group of pregnant women with secondary infertility compared to the mean values of fibrinogen (3.60 ± 0.55 g/L and thrombocyte count

  1. The Consumption Paradigm in Marketing

    OpenAIRE

    Ardianto, Eka

    2003-01-01

    This article elaborates consumption paradigm in marketing. In background, this paper reviews different perspectives of consumption: economic perspective and marketing perspective. In ontology, this work describes various issues regarding consumption view. In epistemology, this article demonstrates how marketers especially researches explore the consumption phenomena. In methodology, the article describes experiential marketing –one of applied consumption paradigm in marketing, which could be ...

  2. Beta2-glycoprotein I dependent anticardiolipin antibodies and lupus anticoagulant in patients with recurrent pregnancy loss.

    Directory of Open Access Journals (Sweden)

    Kumar K

    2002-01-01

    Full Text Available AIM: The present study was aimed to define the incidence of antiphospholipid antibodies of different types lupus anticoagulant (LAC, venereal disease research laboratory test (VDRL and Beta2-glycoprotein I dependent anticardiolipin antibodies Beta2 I aCL in our cohort of population experiencing recurrent pregnancy loss (RPL from Andhra Pradesh, South India. SETTING AND DESIGN: A referral case-control study at a tertiary centre over a period of 5 years. PARTICIPANTS: 150 couples experiencing 3 or more recurrent pregnancy losses with similar number of matched controls. MATERIAL AND METHODS: LAC activity was measured by the activated partial thromboplastin time (aPTT according to the method of Proctor and Rapaport with relevant modifications. VDRL analysis was performed by the kit method supplied by Ranbaxy Diagnostics Limited and Beta2 Glycoprotein I dependent anticardiolipin antibodies were estimated by ELISA kit (ORGen Tech, GmbH, Germany with human Beta2 Glycoprotein I as co-factor. STATISTICAL ANALYSIS: Statistical analysis was performed using Student′s t test. RESULTS: LAC activity was found positive in 11 women (10.28%. The mean +/- SE Beta2 I aCL concentration in the study group was 14.53 (micro/ml +/- 1.79 (range 0 to 90.4 micro/ml which was higher than the control group with a mean +/- SE of 7.26 (micro/ml +/- 0.40 (range 0 to 18 u/ml. The binding of the antibodies to the antigen was observed in 40.24% (n=33 of the cases compared to 6.09% (n=5 in controls. VDRL test was positive in 7(2.34% individuals (3 couples and 1 male partner and none among controls. CONCLUSIONS: The present study indicates the importance of antiphospholipid antibodies in women experiencing RPL and suggests the usefulness of screening for these antibodies as a mandatory routine for instituting efficient therapeutic regimens for a successful outcome of pregnancy.

  3. Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab.

    Science.gov (United States)

    Berman, Horacio; Rodríguez-Pintó, Ignasi; Cervera, Ricard; Morel, Nathalie; Costedoat-Chalumeau, Nathalie; Erkan, Doruk; Shoenfeld, Yehuda; Espinosa, Gerard

    2013-09-01

    The catastrophic variant of the antiphospholipid syndrome (APS) is characterized by thrombosis in multiple organs developing over a short period of time. First-line treatment for the catastrophic APS is the combination of anticoagulation plus corticosteroids plus plasma exchange and/or intravenous immunoglobulin. Despite this regimen, the mortality remains high and new treatment options are needed. By a systematic review of the Catastrophic APS Registry (CAPS Registry), we identified 20 patients treated with rituximab. The purpose of this study is to describe the clinical manifestations, laboratory features, and outcomes of rituximab-treated CAPS patients. In addition, the rationale for using rituximab in catastrophic APS is discussed.

  4. Catastrophic antiphospholipid syndrome and pulmonary embolism in a 3-year-old child

    Energy Technology Data Exchange (ETDEWEB)

    Olivier, Carine; Blondiaux, Eleonore; Dacher, Jean-Nicolas [University Hospital of Rouen, Department of Radiology, Rouen (France); Blanc, Thierry [University Hospital of Rouen, Department of Neonatal Medicine, Rouen (France); Borg, Jeanne-Yvonne [University Hospital of Rouen, Haematology Laboratory, Rouen (France)

    2006-08-15

    We report a rare example of catastrophic antiphospholipid syndrome (CAPS) in a young child. A 3-year-old girl with no previous medical history presented with extensive and recurrent thromboses. The diagnosis of CAPS was based on the occurrence of cardiopulmonary embolism in the child with a high titre of autoantibodies directed against phospholipids and beta-2-glycoprotein 1. In spite of a relatively rapid diagnosis and multiple treatments, the outcome was unfavourable. Multimodality imaging, including both ultrasonography and spiral CT, allowed close follow-up of the thromboses. (orig.)

  5. Antiphospholipid syndrome leading to venous brain thrombosis in an elderly patient

    Directory of Open Access Journals (Sweden)

    Joseph Bruno Bidin Brooks, MD, MSc

    2014-09-01

    Full Text Available Antiphospholipid syndrome (APS is a systemic autoimmune condition characterized by hypercoagulability, venous and/or arterial thromboses, and miscarriages. APS can be diagnosed according to specific criteria and is usually observed in young adults. We report a case of an elderly woman with past history of thrombosis and miscarriages who developed severe brain parenchymal hemorrhage and extensive thrombosis of the superior sagittal sinus due to APS. This case emphasizes that, although rare, APS may be diagnosed in elderly individuals and require effective anticoagulation.

  6. Imaging findings in the rare catastrophic variant of the primary antiphospholipid syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Thuerl, Christina; Altehoefer, Carsten; Laubenberger, Joerg [Freiburg Univ. (Germany). Abt. Radiologie; Spyridonidis, Alexandros [Freiburg Univ. (DE). Abt. Innere Medizin 1 (Haematologie und Onkologie)

    2002-03-01

    We report imaging findings in a case of the rare catastrophic variant of antiphospholipid syndrome (CAPS) characterized by widespread microvascular occlusions, which may lead to multiple organ failure. We present a case of a 66-year-old woman with bone marrow necrosis, acute acalculous cholecystitis (AAC), focal liver necrosis, subtle patchy splenic infarctions, and bilateral adrenal infarction. The demonstration of multiple microvascular organ involvement (three or more) is crucial for the diagnosis of the catastrophic variant of APS. This can be performed radiologically intra-vitam. Imaging can even reveal subclinical microinfarctions, which are often only diagnosed at autopsy. (orig.)

  7. Myocardial infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    ZHANG Bo; JIANG Da-ming; ZHOU Xu-chen; QI Guo-xian

    2011-01-01

    This case report we presented aims to report a-31-year-old man with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) who developed myocardial infarction (Ml) and also aims to discuss the possible mechanisms. The results showed that traditional risk factors alone do not cause coronary heart disease with SLE, and SLE-related factors influence the atherogenic process. We found that although SLE patients with acute Ml benefit from percutaneous coronary intervention (PCI) therapy, it is very important to choose the reasonable antithrombotic strategies in patients with SLE and APS undergoing PCI who require oral anticoagulant therapy.

  8. Antiphospholipid and antiprotein syndromes in non-thrombotic, non-autoimmune women with unexplained recurrent primary early foetal loss. The Nîmes Obstetricians and Haematologists Study--NOHA.

    Science.gov (United States)

    Gris, J C; Quéré, I; Sanmarco, M; Boutiere, B; Mercier, E; Amiral, J; Hubert, A M; Ripart-Neveu, S; Hoffet, M; Tailland, M L; Rousseau, O; Monpeyroux, F; Dauzat, M; Sampol, J; Daures, J P; Berlan, J; Marès, P

    2000-08-01

    Various antiphospholipid and/or antiprotein antibodies have been suspected to be associated with recurrent early foetal loss in absence of any habitual aetiology. We conducted a hospital-based case control study on women with no antecedent of thromboembolic or autoimmune disease. We studied 3 groups of 518 women: patients with unexplained primary recurrent early foetal loss, patients with explained episodes and mothers with no previous obstetrical accident. Matching the 3 groups was carried out on the basis of age, number or pregnancies and time elapsed since the end of the last pregnancy. Significant biological markers were then prospectively tested. The various antibodies were shown to be dependent on parity and on the presence of previous foetal loss: cut-off values were thus calculated using data obtained from the group of explained accidents, and adjusted for parity. Only anti-phosphatidylethanolamine IgM [odds ratio: 6.0, 95% confidence interval (2.3-15.7), p = 0.0003], anti-beta2-glycoprotein I IgG [4.4, (1.6-11.7), p = 0.0035] anti-annexin V IgG antibodies [3.2 (1.2-8.1), p = 0.015] and lupus anticoagulant [3.0, (1.3-6.8), p = 0.009], were found to be independent retrospective risk factors for unexplained early foetal loss. These four markers were subsequently found to be, during the following pregnancy, associated with a significant risk of foetal loss despite a low-dose aspirin treatment. In non-thrombotic, non-auto-immune women with unexplained primary recurrent early foetal loss, subgroups of patients with positive anti-phosphatidylethanolamine IgM antibodies, or positive anti-beta2-glycoprotein-I IgG antibodies, or positive anti-annexin V IgG antibodies or lupus anticoagulant must be particularised. This should allow therapeutic trials to be carried in well-defined patients.

  9. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period : A multicentre prospective study of 1000 patients

    NARCIS (Netherlands)

    Cervera, R.; Serrano, R.; Pons-Estel, G. J.; Ceberio-Hualde, L.; Shoenfeld, Y.; De Ramón, E.; Buonaiuto, V.; Jacobsen, S.; Zeher, M. M.; Tarr, T.; Tincani, A.; Taglietti, M.; Theodossiades, G.; Nomikou, E.; Galeazzi, M.; Bellisai, F.; Meroni, P. L.; Derksen, R. H W M; De Groot, P. G D; Baleva, M.; Mosca, S.; Bombardieri, M.; Houssiau, F.; Gris, J. C.; Quéré, I.; Hachulla, E.; Vasconcelos, C.; Fernández-Nebro, A.; Haro, M.; Amoura, Z.; Miyara, M.; Tektonidou, M.; Espinosa, G.; Bertolaccini, M. L.; Khamashta, M. A.

    2015-01-01

    Objectives: To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later. Methods: In 1999, we started an observational study of 1000 APS pat

  10. Morbidity and mortality in the antiphospholipid syndrome during a 5-year period : a multicentre prospective study of 1000 patients

    NARCIS (Netherlands)

    Cervera, R.; Khamashta, M. A.; Shoenfeld, Y.; Camps, M. T.; Jacobsen, S.; Kiss, E.; Zeher, M. M.; Tincani, A.; Kontopoulou-Griva, I.; Galeazzi, M.; Bellisai, F.; Meroni, P. L.; Derksen, R. H. W. M.; de Groot, P. G.; Gromnica-Ihle, E.; Baleva, M.; Mosca, M.; Bombardieri, S.; Houssiau, F.; Gris, J-C; Quere, I.; Hachulla, E.; Vasconcelos, C.; Roch, B.; Fernandez-Nebro, A.; Piette, J-C; Espinosa, G.; Bucciarelli, S.; Pisoni, C. N.; Bertolaccini, M. L.; Boffa, M-C; Hughes, G. R. V.

    2009-01-01

    Objectives: To identify the main causes of morbidity and mortality in patients with antiphospholipid syndrome (APS) during a 5-year period and to determine clinical and immunological parameters with prognostic significance. Methods: The clinical and immunological features of a cohort of 1000 patient

  11. Tolerogenic dendritic cells specific for β2-glycoprotein-I Domain-I, attenuate experimental antiphospholipid syndrome.

    Science.gov (United States)

    Zandman-Goddard, Gisele; Pierangeli, Silvia S; Gertel, Smadar; Blank, Miri

    2014-11-01

    Tolerogenic dendritic cells (tDCs) have the potential to control the outcome of autoimmunity by modulating the immune response. The aim of this study was to uncover the tolerance efficacy attributed to beta-2-glycoprotein-I (β2GPI) tDCs or β2GPI domain-I (D-I) and domain-V (D-V)-tDCs in mice with antiphospholipid syndrome (APS). tDCs were pulsed with β2GPI or D-I or D-V derivatives. Our results revealed that β2GPI related tDCs phenotype includes CD80(high), CD86(high) CD40(high) MHC class II(high). The miRNA profiling encompass miRNA 23b(high), miRNA 142-3p(low) and miRNA 221(low). In addition the β2GPI related tDCs showed reduced secretion of IL-1β, IL-12 and IL-23. D-I tDCs treatment was more efficient than β2GPI tDCs in inducing of tolerance in APS mice, manifested by lowered titers of anti- β2GPI antibodies (Abs) and reduced percentage of fetal loss. Tolerance induction was accompanied by poor T cell response to β2GPI, high numbers of CD4 + CD25 + FOXP3 + T-regulatory cells (Treg), reduced levels of IFNγ, IL-17 and increased expression of IL-10 and TGFβ. Tolerance was successfully transferred by Treg cells from the tolerized mice to β2GPI immunized mice. We conclude that predominantly D-I-tDCs and β2GPI tDCs have the potential to attenuate experimental APS by induction of Treg cells, reduction of anti- β2GPI Abs titers and increased expression of anti-inflammatory cytokines. We suggest that β2-GPI-D-I-tDCs may offer a novel approach for developing therapy for APS patients.

  12. 系统性红斑狼疮继发抗磷脂抗体综合征临床分析%Clinical analysis of systemic lupus erythematosus complicated with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    金莉; 马艳; 李向培; 厉小梅; 吴竞生; 汪国生; 陶金辉; 钱龙

    2014-01-01

    目的:探讨系统性红斑狼疮(system ic lupus erythematosus,SLE)继发抗磷脂抗体综合征(antiphospholipid syndrome,APS)的临床特点、实验室指标及相关因素,以早期发现继发性APS.方法:回顾性分析2005年1月至2014年1月收集的129例SLE和APS患者的临床资料,包括临床表现、妊娠情况、抗核抗体、抗心磷脂抗体等.结果:129例患者中,原发性APS (primary antiphospholipid syndrome,PAPS)8例;SLE者121例,其中SLE合并APS患者(SLE-APS)41例,仅诊断为SLE患者(SLE-APA-)40例及出现抗磷脂抗体(antiphospholipid antibody,APA)异常但APS诊断依据不足(SLE-APA+)的SLE患者40例.8例原发性APS患者中,3例男性均表现为下肢深静脉血栓形成及手微动脉血栓形成,有婚育史的女性患者中5例均有病态妊娠表现,自发性流产和(或)死胎,其中2例有肺栓塞、门(脾)静脉栓塞.41例SLE-APS患者中,表现为下肢深静脉血栓有15例,肺栓塞患者3例,脑梗死10例;SLE-APS组抗β2-糖蛋白1(抗β2-GP-1)浓度明显高于SLE-APA+组及SLE-APA-组,而血小板计数明显减低.结论:SLE合并APS患者的血清中存在高浓度抗β2-GP-1,提示高浓度抗β2-GP-1是SLE继发APS的独立危险因素.

  13. [In vitro fertilization and systemic lupus erythematosus or antiphospholipid syndrome: An update].

    Science.gov (United States)

    Orquevaux, P; Masseau, A; Le Guern, V; Gayet, V; Vauthier, D; Boutin, D; Wechsler, B; Morel, N; Guettrot-Imbert, G; Pennaforte, J-L; Piette, J-C; Costedoat-Chalumeau, N

    2015-03-01

    Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

  14. In vitro effect of anti-β2 glycoprotein I antibodies on P-selectin expression, a marker of platelet activation

    Directory of Open Access Journals (Sweden)

    A. Hoxha

    2012-03-01

    Full Text Available Antiphospholipid antibodies (aPL associated with thromboembolic events and/or pregnancy morbidity characterize the so-called antiphospholipid syndrome (APS. Beta2glycoprotein I (β2GPI is the main target antigen for aPL, but the pathogenic role of anti-β2GPI antibodies (aβ2GPI is still unclear. Some authors assume they play a role in activating platelets. We evaluated the effects of aβ2GPI antibodies on platelet P-selectin expression. Aβ2GPI antibodies in the plasma of a pregnant APS patient were isolated by affinity chromatography at two different stages (catastrophic and quiescent of the disease. Gel filtered platelets (100 x 109/L from healthy volunteers were incubated with β2-GPI (20 µg/mL and with different concentrations (5. 25 and 50 µg/mL of aβ2GPI antibodies. P-selectin surface expression on platelets was assessed by flow cytometry using a specific fluorescent antibody directed against P-selectin. Aβ2GPI antibodies induced platelet activation only in the presence of thrombin receptor activator for peptide 6 (TRAP-6, a platelet agonist, at a subthreshold concentration. Aβ2GPI antibody enhancement on platelet surface P-selectin expression was stronger in the catastrophic than in the quiescent phase of the disease (47 vs 15%. TRAP-6 dependent platelet activation by aβ2GPI antibodies is consistent with the “two hit” pathogenetic hypothesis for thrombosis. Aβ2GPI antibodies induce higher platelet P-selectin expression during the active rather than the acute phases.

  15. Towards reduction of Paradigm coordination models

    CERN Document Server

    Andova, Suzana; de Vink, Erik; 10.4204/EPTCS.60.1

    2011-01-01

    The coordination modelling language Paradigm addresses collaboration between components in terms of dynamic constraints. Within a Paradigm model, component dynamics are consistently specified at a detailed and a global level of abstraction. To enable automated verification of Paradigm models, a translation of Paradigm into process algebra has been defined in previous work. In this paper we investigate, guided by a client-server example, reduction of Paradigm models based on a notion of global inertness. Representation of Paradigm models as process algebraic specifications helps to establish a property-preserving equivalence relation between the original and the reduced Paradigm model. Experiments indicate that in this way larger Paradigm models can be analyzed.

  16. Anti-phosphatidylserine-prothrombin antibodies are associated with outcome in a TIA cohort

    Directory of Open Access Journals (Sweden)

    Michael T Mullen

    2012-09-01

    Full Text Available Background: Antiphospholipid antibodies (aPLs have been associated with thrombosis in the antiphospholipid antibody syndrome (APS and with atherosclerotic vascular events in patients without APS. We examined the significance of aPLs in transient ischemic attack (TIA.Patients/Methods: Patients with TIA <48 hours from symptom onset were prospectively enrolled. Traditional aPLs, including anti-cardiolipin (aCL and β2-glycoprotein-I (β2GPI, and newer aPLs, including anti-phosphatidylserine/prothrombin (aPS/PT, β2GPI Domain 4/5 and β2GPI Domain 1 were measured. Primary outcome was a composite of stroke or death within 90 days or identification of a high-risk stroke mechanism. Secondary outcomes were stroke or death and the presence of clinical/sub-clinical atherosclerosis. Results: Over 4.5 years, 167 patients were enrolled. 41 patients (25% had the composite endpoint. Antibodies were measured in 158 subjects. aPS/PT IgG antibodies were significantly associated with stroke/death (OR 16.3 95% CI 2.3-116.7 p=0.005 and were non-significantly associated with the composite endpoint (OR 4.7 95% CI 0.8-29.2 p=0.10. In multivariate analysis adjusting for ABCD2 risk score, aPS/PT IgG remained associated with stroke/death (OR 15.7 95% CI 2.0-125.6 p=0.009. Other aPLs were not associated with clinical outcome and no association between APLs and atherosclerosis was identifed. Conclusion: In contrast to other aPLs, anti-phosphatidylserine/prothrombin IgG antibodies are independently associated with stroke or death in patients with TIA.

  17. Lupus anticoagulants and anticardiolipin antibodies in Indian women with spontaneous, recurrent fetal loss.

    Science.gov (United States)

    Rawat, Akanksha; Sikka, Meera; Rusia, Usha; Guleria, Kiran

    2015-06-01

    Spontaneous and recurrent pregnancy loss are common complications of pregnancy resulting from varied causes including antiphospholipid syndrome (APS). Treatment of women with APS increases the chance of a subsequent successful pregnancy. The study aimed to find the prevalence of lupus anticoagulants (LA) and anticardiolipin antibodies (ACAs) in women with spontaneous/recurrent fetal loss and compare with women with normal obstetric history. Hundred women with spontaneous/recurrent fetal loss and 50 healthy pregnant controls were tested for LA by complete blood counts, Prothrombin time, Activated partial thromboplastin time (APTT), LA sensitive APTT and dilute Russell viper venom time (dRVVT) (screening and confirmatory) and ACAs (ELISA). LA was detected in 15 % patients using dRVVT confirmatory test and ACA in 5 %, all controls being negative. Twenty one % patients were detected by LA sensitive APTT (sensitivity 92.9 %, specificity 100 %) and 100 % with dRVVT screening test (sensitivity 98.8 %, specificity 100 %). We recommend that screening for antiphospholipid antibodies must be done in women with spontaneous/recurrent foetal loss even in the absence of other clinical manifestations using a combination of tests.

  18. PARADIGM SHIFT IN ISLAMIC STUDIES

    Directory of Open Access Journals (Sweden)

    Editor Al-Jami'ah: Journal of Islamic Studies

    2008-08-01

    Full Text Available In the early 1990s, there was a heated debate among students ofIAIN (the State Institute for Islamic Studies Sunan KalijagaYogyakarta about the future of Islamic studies, focusing on the possibilityof incorporating Thomas Kuhn’s paradigm to the discourse ofIslamic studies. Kuhn explains in detail the rise and decline of scientificparadigm in his classic work, The Structure of Scientific Revolutions,firstly published in 1970. Paradigm is defined as a set of beliefs thatguides the researchers to address some important problems or issuesunder a certain theoretical framework and provides procedures how tosolve those problems. A paradigm shift is a process whereby a newway of perceiving the world comes into existence and is accepted byscholars in a given time. Kuhn proposed two conditions for paradigmshift; first, the presence of anomalies in ‘normal science’, and secondly,the presence of alternative paradigm.

  19. The Prevalence of Anticardiolipin Antibody in Patients with Systemic Lupus Erythematosus and Its Association with Clinical Manifestations

    Directory of Open Access Journals (Sweden)

    Zahra Basiri

    2013-01-01

    Full Text Available The central immunological disturbance in systemic lupus erythematosus (SLE is autoantibody production. Some of these antibodies affecting components of the cell nucleus are the major characteristics of SLE. The present study was aimed to assess importance of anticardiolipin (ACL antibody and its association with clinical state in SLE patients. A cross sectional study was performed on 100 patients with SLE referred to rheumatology outpatient clinic in Ekbatan hospital in Hamadan (Iran between 2007 and 2008. Serum samples were extracted and screened for IgG and IgM using an ACL enzyme-linked immunosorbent assay. Up to 36% of patients were positive for ACL antibody that was more frequent in women than men (39.8% versus 8.3%. No association was revealed between ACL antibody and age. Clinical manifestations of antiphospholipid antibody syndrome were observed in 23.0% of patients that was more prevalent in ACL positive group compared with ACL negative group (41.7% versus 125%. The prevalence of other manifestations including pregnancy-related disorders (recurrent abortion, central nervous system defects, and deep vein thrombosis was 33.3%, 25.0%, and 30.6% in ACL positive group and was 9.4%, 7.8%, and 7.8% in ACL negative group that all were more frequent in the former group. The prevalence of thrombocytopenia was also higher in ACL positive group than another group (22.2% versus 15.6%. Among ACL positive patients with clinical manifestations of antiphospholipid antibody syndrome, 86.6% had medium to high titer of ACL. Our study emphasized value of (ACL antibody to assess clinical status in SLE patients

  20. Antiparietal cell antibody test

    Science.gov (United States)

    APCA; Anti-gastric parietal cell antibody; Atrophic gastritis - anti-gastric parietal cell antibody; Gastric ulcer - anti-gastric parietal cell antibody; Pernicious anemia - anti-gastric parietal cell antibody; ...