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APLA Syndrome

Alina Dima, Simona Caraiola, C Jurcut, Eugenia Balanescu, P Balanescu, Doina Ramba, Camelia Badea, V Pompilian, R Ionescu, Anda Baicus, C Baicus, G A Dan
BACKGROUND: The antiphospholipid syndrome (APS) is one of the most encountered autoimmunity in systemic lupus erythematosus (SLE) patients and pathogenesis of these two seems to be intricate. AIM: To investigate the association of antiphospholipid antibodies (APLAs) titer with the presence of secondary APS diagnosis in SLE patients. METHODS: 65 patients fulfilling the 2012 Systemic Lupus Collaborating International Clinics (SLICC) SLE's criteria were included...
October 2015: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
Venkata Rama Rao Mikkiliineni, Nagarjuna Panidapu, Mrunalini Parasa, Mastan Saheb Shaik
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.
September 2015: Anesthesia, Essays and Researches
M Thakre, J Inshasi
UNLABELLED: Role of lumbar puncture and high dose steroids have been implicated previously in causing cerebral venous sinus thrombosis in multiple sclerosis. We report a case of clinically isolated syndrome who developed cerebral venous sinus thrombosis after Lumbar puncture and high dose steroids. Forty two years old gentleman presented with right hand numbness and weakness over 1 week. MRI brain and spine showed periventricular and cerebellar white matter lesions suggestive of multiple sclerosis...
November 2014: Multiple Sclerosis and related Disorders
Zhi-wei Lai, Ivan Marchena-Mendez, Andras Perl
Anti-phospholipid antibodies (APLA) represent a diagnostic criterion of systemic lupus erythematosus (SLE) and cause morbidity, termed anti-phospholipid syndrome (APS). Activation of the mechanistic target of rapamycin (mTOR) has been recently associated with APS. mTOR is a sensor of oxidative stress. Therefore, we examined mitochondrial mass, superoxide production, mTOR activity and FoxP3 expression in 72 SLE patients, twelve of whom also had APS, and 54 healthy controls by flow cytometry. Mitochondrial mass was increased in CD4(-)CD8(-) double-negative (DN) T cells of SLE patients with APS (2...
June 2015: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Amy E Lin, Nadine Shehata, Heather Reich, David Barth, Carl Laskin, Stephen Lapinsky, Shital Gandhi
OBJECTIVES: We outline a case of a woman with lupus (SLE) and antiphospholipid syndrome (APLA) at 26 weeks' gestation who develops a complex multiorgan condition with a broad differential. METHODS: Case report. RESULTS: A 34 year old woman, G1P0, presented at 26 weeks gestation with a 5 day history of epigastric pain and emesis. She has a history of lupus anticoagulant (LAC), anticardiolipin (ACL) and anti-dsDNA (ADD) positive SLE and APLA syndrome diagnosed at age 13 with multiple small cerebral infarcts...
January 2015: Pregnancy Hypertension
Shachaf Ofer-Shiber, Yair Molad
Antiphospholipid syndrome (APLS) is an autoimmune hypercoagulable syndrome characterized by thrombotic and obstetric manifestations. We sought to determine the rate of APLS feature in patients tested positive for antiphospholipid antibodies (APLA) regardless of the serum level of anticardiolipin (ACL) and/or anti-β2-glycoprotein I (β2GPI) antibodies. An inception cohort of individuals who were tested positive for ACL and/or β2GPI IgG/IgM antibody, and/or lupus anticoagulant (LAC) on two occasions of at least 12 weeks apart...
April 2015: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Kaushik Saha, Arnab Saha, Mrinmoy Mitra, Prabodh Panchadhyayee
Rhupus syndrome is a rare syndrome characterized by overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Our patient was a diagnosed case of RA and developed SLE 2 years after. She was a middle-aged woman, presented with bilateral pleural effusion with exacerbation of skin and joint symptoms of SLE. We diagnosed the case as tubercular pleural effusion by positive Mycobacterium tuberculosis in bactec 460 culture. She had also anti-phospholipid antibody positivity without any symptoms and signs of thrombosis...
October 2014: Lung India: Official Organ of Indian Chest Society
Gideon Nesher
Giant cell arteritis (GCA) is considered to be a T cell-dependent disease. Autoantibodies have not consistently been found in GCA. The exception is antiphospholipid antibodies (APLA), which were found in 30-80% of GCA cases. Recently, efforts have been made to seek autoantibodies in GCA using newer methods of detection: serological identification of antigens by recombinant cDNA expression cloning, and a proteomic approach. In these studies, lamin C (a nuclear envelope antigen) was recognized by antibodies in 32% of GCA sera and none of the controls...
July 2014: Israel Medical Association Journal: IMAJ
Ayman Alboudi, Pournamy Sarathchandran, Suhail Alrukn, Abubaker Al Madani
We report the case of a 30-year-old woman, without any previous comorbidities presenting with acute onset headache, altered sensorium and unsteadiness of gait. Neurological evaluation revealed a drowsy patient with papilloedema, bilateral lateral rectus palsy, generalised hyper-reflexia and up going plantar responses. Urgent imaging performed showed extensive cortical venous sinus thrombosis. Workup for secondary causes of cortical venous sinus thrombosis revealed very high titres of antinuclear antibody and anti-dsDNA, but negative antiphospholipid antibodies (APLA)...
2014: BMJ Case Reports
Luci Maria Santana Dusse, Fernanda Dias e Silva, Letícia Gonçalves Freitas, Danyelle Romana Alves Rios, Sandra Cristina Armond, Milena Soriano Marcolino
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...
March 2014: Revista da Associação Médica Brasileira
Maria Laura Bertolaccini, Olga Amengual, Laura Andreoli, Tatsuya Atsumi, Cecilia B Chighizola, Ricardo Forastiero, Philip de Groot, Gabriella Lakos, Marc Lambert, Pierluigi Meroni, Thomas L Ortel, Michelle Petri, Anisur Rahman, Robert Roubey, Savino Sciascia, Melissa Snyder, Anne E Tebo, Angela Tincani, Rohan Willis
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...
September 2014: Autoimmunity Reviews
K J Brandt, C Fickentscher, F Boehlen, E K O Kruithof, P de Moerloose
BACKGROUND: The antiphospholipid antibody syndrome (APS) is an autoimmune disease associated with arterial or venous thrombosis and/or recurrent fetal loss and is caused by pathogenic antiphospholipid antibodies (aPLA). We recently demonstrated that Toll-like receptor 2 (TLR2) and CD14 contribute to monocyte activation of aPLA. OBJECTIVE: To study the mechanisms of cell activation by aPLA, leading to pro-coagulant and pro-inflammatory responses. METHODS AND RESULTS: For this study, we used purified antibodies from the plasmas of 10 different patients with APS and healthy donors...
May 2014: Journal of Thrombosis and Haemostasis: JTH
Rohan Willis, Gabriella Lakos, E Nigel Harris
The measurement of antiphospholipid antibodies (aPL) has been an important aspect of antiphospholipid syndrome (APS) characterization since the disease was first described in the 1980s. Despite significant efforts geared toward the standardization of immunoassays that measure anticardiolipin antibodies and anti-β2-glycoprotein I spanning three decades, there are still reports of significant interassay and interlaboratory variation in the results of these assays. At the recent 13th International Congress on Antiphospholipid Antibodies (APLA 2010, April 13-16, 2010, Galveston, TX), a task force composed of internationally recognized experts in the field of APS was formed to address these issues...
March 2014: Seminars in Thrombosis and Hemostasis
Rose Willemze, Robert L Bradford, Micah J Mooberry, Robert A S Roubey, Nigel S Key
Antiphospholipid syndrome (APS) is defined by the association of autoantibodies to certain phospholipid-binding proteins with arterial or venous thrombosis ('AT' or 'VT', respectively), and/or pregnancy-related morbidity (PM). Antiphospholipid antibodies (aPLA) promote activation of several cell types including monocytes, resulting in procoagulant tissue factor (TF) expression that may contribute to the vascular complications. Since TF synthesis by monocytes is frequently accompanied by release of TF-bearing microparticles, we hypothesized that plasma microparticle TF activity (MP-TF) may be elevated in APS patients and contribute to thrombosis and/or PM...
February 2014: Thrombosis Research
Paul Froom, Enas Saffuri-Elias, Orit Rozenberg, Mira Barak
AIMS: We hypothesised that there is a threshold value for the association of dilute Russell's viper venom times (dRVVT) with positive immunoglobin G antiphospholipid antibody (IgG-APLA) test results. METHODS: We tested 120 controls and a cohort of 2412 outpatients who had concomitant test results for dRVVT and IgG-APLA (IgG antibodies to cardiolipins and β2-glycoprotein I). We also selected a subgroup who had repeated IgG-APLA tests at least 12 weeks apart (1398 patients with multiple β2-glycoprotein I tests and 672 with multiple aCL tests)...
May 2014: Journal of Clinical Pathology
Sarthak Gupta, Robert Zivadinov, Deepa Ramasamy, Julian L Ambrus
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...
December 2014: Clinical Rheumatology
R Forastiero, E Papalardo, M Watkins, H Nguyen, C Quirbach, K Jaskal, M Kast, M Teodorescu, G Lakos, W Binder, Z Shums, V Nelson, G Norman, J Puig, A Cox, W Vandam, J Hardy, S Pierangeli
BACKGROUND: The performance and standardization of anticardiolipin (aCL) and anti-β₂ glycoprotein I antibodies (aβ₂GPI) tests for the confirmation of diagnosis of antiphospholipid syndrome (APS) remain a matter of debate and concern. We evaluated the performance of different ELISAs and other new immunoassays for the detection of aCL and aβ₂GPI in a wet workshop at the 13th International Congress on Antiphospholipid Antibodies in Galveston, TX (April 13th, 2010, APLA 2010). METHODS: Aliquots of 26 un-identified APS or persistently aPL positive serum samples and 21 controls (9 from healthy individuals and 5 from patients with infectious diseases and 7 with various autoimmune diseases) were distributed to all participants/groups...
January 20, 2014: Clinica Chimica Acta; International Journal of Clinical Chemistry
Oshrat E Tayer-Shifman, Eldad Ben-Chetrit
A patient with antiphospholipid syndrome, SLE and refractory fever is described. The cause for the fever was macrophage activation syndrome (MAS). The diagnosis of MAS was made with the help of PET-CT. Since the syndrome was refractory to conventional therapy with high-dose steroids and cyclosporin, anakinra was administered with complete recovery of the patient. The present case illustrates the difficulties in diagnosing MAS when multiple bone marrow biopsies fail to show hemophagocytosis. It emphasizes the significance of PET-CT in the diagnosis and evaluation of treatment of MAS...
2015: Modern Rheumatology
Karim J Brandt, Egbert K O Kruithof, Philippe de Moerloose
The antiphospholipid syndrome (APS) is an autoimmune disease associated with arterial or venous thrombosis and/or recurrent fetal loss and is caused by pathogenic antiphospholipid antibodies (aPLA). The plasma protein β2-glycoprotein 1 (β2GP1) has been identified as a major target of aPLA associated with APS. Cell activation by aPLA appears to be a major pathogenic cause in the pathogenesis of APS. Receptors, co-receptors and accessory molecules are known to assist the pathogenic effects of aPLA. Members of the TLR family and the platelet receptor apolipoprotein E receptor 2' (apoER2'), a receptor belonging to the low-density lipoprotein receptor (LDL-R) family, as well as GPIbα, were identified as putative candidates for aPLA recognition...
October 2013: Thrombosis Research
Venkaiah Betapudi, George Lominadze, Linda Hsi, Belinda Willard, Meifang Wu, Keith R McCrae
The antiphospholipid syndrome is characterized by thrombosis and recurrent fetal loss in patients with antiphospholipid antibodies (APLAs). Most pathogenic APLAs are directed against β2-glycoprotein I (β2GPI), a plasma phospholipid binding protein. One mechanism by which circulating antiphospholipid/anti-β2GPI antibodies may promote thrombosis is by inducing the release of procoagulant microparticles from endothelial cells. However, there is no information available concerning the mechanisms by which anti-β2GPI antibodies induce microparticle release...
November 28, 2013: Blood
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