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Anti phospholipid syndrome

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https://www.readbyqxmd.com/read/29879927/arterial-venous-thrombosis-fetal-loss-and-stillbirth-in-pregnant-women-with-systemic-lupus-erythematosus-versus-primary-and-secondary-antiphospholipid-syndrome-a-systematic-review-and-meta-analysis
#1
Pravesh Kumar Bundhun, Mohammad Zafooruddin Sani Soogund, Feng Huang
BACKGROUND: We aimed to systematically compare arterial/venous thrombosis, fetal loss and stillbirth in pregnant women with systemic lupus erythematosus (SLE), primary anti-phospholipid syndrome (PAPS) and secondary anti-phospholipid syndrome (SAPS). METHODS: Online databases were carefully searched for relevant publications comparing SLE with PAPS and/or SAPS in pregnancy. Studies were included if: they compared SLE with APS [SLE versus PAPS or SLE versus SAPS or SLE versus PAPS and SAPS respectively] in pregnant women; and they reported specific adverse outcomes as their clinical endpoints including arterial/venous thrombosis, fetal loss and stillbirth...
June 7, 2018: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/29867966/autoantibodies-recognizing-secondary-necrotic-cells-promote-neutrophilic-phagocytosis-and-identify-patients-with-systemic-lupus-erythematosus
#2
Mona H C Biermann, Sebastian Boeltz, Elmar Pieterse, Jasmin Knopf, Jürgen Rech, Rostyslav Bilyy, Johan van der Vlag, Angela Tincani, Jörg H W Distler, Gerhard Krönke, Georg Andreas Schett, Martin Herrmann, Luis E Muñoz
Deficient clearance of apoptotic cells reportedly contributes to the etiopathogenesis of the autoimmune disease systemic lupus erythematosus (SLE). Based on this knowledge, we developed a highly specific and sensitive test for the detection of SLE autoantibodies (AAb) utilizing secondary NEcrotic cell (SNEC)-derived material as a substrate. The goal of the present study was to validate the use of SNEC as an appropriate antigen for the diagnosis of SLE in large cohort of patients. We confirmed the presence of apoptotically modified autoantigens on SNEC (dsDNA, high mobility group box 1 protein, apoptosis-associated chromatin modifications, e...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29867951/cellular-and-molecular-mechanisms-of-anti-phospholipid-syndrome
#3
REVIEW
Marko Radic, Debendra Pattanaik
The primary anti-phospholipid syndrome (APS) is characterized by the production of antibodies that bind the phospholipid-binding protein β2 glycoprotein I (β2GPI) or that directly recognize negatively charged membrane phospholipids in a manner that may contribute to arterial or venous thrombosis. Clinically, the binding of antibodies to β2GPI could contribute to pathogenesis by formation of immune complexes or modification of coagulation steps that operate along cell surfaces. However, additional events are likely to play a role in pathogenesis, including platelet and endothelial cell activation...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29850957/thrombosis-and-anti-phospholipid-syndrome-a-5-year-update-on-treatment
#4
REVIEW
Cecilia Beatrice Chighizola, Pier Luigi Meroni
PURPOSE OF REVIEW: The aim of this review is to provide an update of the therapeutic tools for thrombotic anti-phospholipid syndrome (APS), focusing on the last 5 years. RECENT FINDINGS: Early studies appointed anticoagulation at moderate intensity as the mainstay of treatment of thrombotic APS; in the last 5 years, the strategy has not much mutated. Some uncertainties regarding the role of direct oral anticoagulants and the optimal regimen for arterial thrombotic APS still persist: high-intensity anticoagulation, anticoagulation plus anti-platelet agent, and double anti-platelet agents being the possible alternatives...
May 31, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29846160/rheumatic-diseases-and-pregnancy-a-national-survey-about-practice-patterns-among-rheumatologists-and-obstetricians
#5
Fouad Fayad, Nelly Ziade, Ghada Abi Karam, Wadih Ghaname, Munther Khamashta
OBJECTIVES: Management of rheumatic diseases (RD) is often problematic in pregnant patients, hence the need for guideline implementation. This survey-based study aimed to assess beliefs among obstetricians and rheumatologists about managing RD in pregnant Lebanese patients. METHODS: Questionnaires were completed by a representative sample of rheumatologists and obstetricians practicing throughout Lebanon. Collected data included physicians' information, opinion on pregnancy in RD patients, compatible drugs with fertility, pregnancy and breastfeeding, references used in their clinical management, referral to specialists, and knowledge about guidelines...
May 24, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29845583/anti-phospholipid-igg-antibodies-detected-by-line-immunoassay-differentiate-patients-with-anti-phospholipid-syndrome-and-other-autoimmune-diseases
#6
Cecilia Nalli, Valentina Somma, Laura Andreoli, Thomas Büttner, Peter Schierack, Michael Mahler, Dirk Roggenbuck, Angela Tincani
PURPOSE: Anti-phospholipid antibodies (aPL) analyzed by line immunoassay (LIA) can recognize beta2 -glycoprotein I (β2 GPI) domain 1 (D1) epitopes depending on β2 GPI binding to distinct phospholipids. The aPL LIA was compared with consensus ELISA to investigate whether both techniques can discriminate anti-phospholipid syndrome (APS) patients from aPL-positive, systemic autoimmune rheumatic diseases (SARD) patients without clinical symptoms of APS and controls. METHODS: Thirty-four APS patients (14 arterial/venous thrombosis, 16 pregnancy morbidity, and 4 both), 41 patients with SARD lacking clinical APS criteria but demonstrating positivity for anti-β2 GPI (aβ2 GPI) IgG, and 20 healthy subjects (HS) were tested for aPL to cardiolipin (aCL), phosphatidic acid, phosphatidylcholine, phosphatidylethanolamine, phosphatidylglycerol (aPG), phosphatidylinositol, phosphatidylserine, β2 GPI, prothrombin, and annexin V by LIA...
May 29, 2018: Auto- Immunity Highlights
https://www.readbyqxmd.com/read/29757439/risk-of-thrombosis-with-anti-phospholipid-syndrome-in-systemic-lupus-erythematosus-treated-with-thrombopoietin-receptor-agonists
#7
Zelie Guitton, Louis Terriou, Jean-Christophe Lega, Raphaele Nove-Josserand, Miguel Hie, Zahir Amoura, James B Bussel, Mohamed Hamidou, Eric Rosenthal, Bertrand Lioger, Dominique Chauveau, Axel Chaminade, Nadine Magy-Bertrand, Marc Michel, Sylvain Audia, Bertrand Godeau, Matthieu Mahevas
Objectives: The use of thrombopoietin-receptor agonists (TPO-RAs) has increased as a second-line therapy in ITP, but the efficacy and safety of such drugs has not been evaluated in SLE-associated ITP. Methods: This was a multicentre retrospective cohort study from 2009 to 2016. Participating centres (n = 11) were secondary- or tertiary-care hospitals belonging to the French national network for adult ITP. Results: We included 18 patients with SLE-ITP treated with TPO-RAs; 10 (55%) had aPL, 5 (27%) showing definite APS...
May 10, 2018: Rheumatology
https://www.readbyqxmd.com/read/29751260/forkhead-box-c1-gene-variant-causing-glaucoma-and-small-vessel-angiopathy-can-mimic-multiple-sclerosis
#8
Jagannadha R Avasarala, Julie R Jones, Curtis R Rogers
A 34-year old Caucasian female was initially diagnosed with multiple small-vessel strokes at age 20 years which were etiologically classified as secondary to anti-phospholipid antibody syndrome (APS) although she had no history or laboratory data to suggest APS. Based on her MRI of brain findings, one of her neurologists was concerned she could have multiple sclerosis (MS) and hence the patient was referred to our clinic for further evaluation. The patient's MRI of brain showed confluent lesions in the periventricular and juxta-cortical lesions that fulfil 2017 McDonald criteria for dissemination in space...
April 21, 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29730433/the-tolerogenic-peptide-hcdr1-immunomodulates-cytokine-and-regulatory-molecule-gene-expression-in-blood-mononuclear-cells-of-primary-sjogren-s-syndrome-patients
#9
Zev Sthoeger, Amir Sharabi, Ilan Asher, Heidy Zinger, Rafael Segal, Gene Shearer, Ori Elkayam, Edna Mozes
Primary Sjogren's syndrome (pSS) is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands. We investigated whether the tolerogenic peptide, hCDR1, that ameliorates lupus manifestations would have beneficial effects on pSS as well. The in vitro effects of hCDR1 on gene expression of pro-inflammatory cytokines and regulatory molecules were tested in peripheral blood mononuclear cells (PBMC) of 16 pSS patients. hCDR1, but not a control peptide, significantly reduced gene expression of IL-1β, TNF-α, MX-1 and BlyS and up-regulated immunosuppressive (TGF-β, FOXP3) molecules in PBMC of pSS patients...
May 3, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29660074/the-effect-of-triple-therapy-on-the-mortality-of-catastrophic-anti-phospholipid-syndrome-patients
#10
Ignasi Rodríguez-Pintó, Gerard Espinosa, Doruk Erkan, Yehuda Shoenfeld, Ricard Cervera
Objectives: The objective of this study was to assess the effect that triple therapy (anticoagulation plus CS plus plasma exchange and/or IVIGs) has on the mortality risk of patients with catastrophic APS (CAPS) included in the CAPS Registry. Methods: Patients from the CAPS Registry were grouped based on their treatments: triple therapy; drugs included in the triple therapy but in different combinations; and none of the treatments included in the triple therapy...
April 11, 2018: Rheumatology
https://www.readbyqxmd.com/read/29608758/neutrophil-extracellular-trap-release-is-associated-with-antinuclear-antibodies-in-systemic-lupus-erythematosus-and-anti-phospholipid-syndrome
#11
Maarten van der Linden, Lucas L van den Hoogen, Geertje H A Westerlaken, Ruth D E Fritsch-Stork, Joël A G van Roon, Timothy R D J Radstake, Linde Meyaard
Objectives: Increased release of neutrophil extracellular traps (NETs) is implicated in the activation of plasmacytoid dendritic cells, vascular disease and thrombosis in SLE and APS. However, studies comparing NET release between patients with SLE and APS are lacking. Here we evaluated plasma-induced NET release in a large cohort of patients with SLE, SLE + APS and primary APS in relation to clinical and serological parameters. Methods: Neutrophils from healthy controls were exposed to plasma of heterologous healthy controls (n = 27) or SLE (n = 55), SLE + APS (n = 38) or primary APS (PAPS) (n = 28) patients and NET release was quantified by immunofluorescence...
March 28, 2018: Rheumatology
https://www.readbyqxmd.com/read/29604397/oxidative-post-translational-modification-of-%C3%AE-eta-2-glycoprotein-i-in-the-pathophysiology-of-the-anti-phospholipid-syndrome
#12
REVIEW
James C Weaver, Steven A Krilis, Bill Giannakopoulos
The anti-phospholipid syndrome (APS) is a prothrombotic autoimmune disorder characterized by either thrombosis or pregnancy complications in the setting of persistent anti-phospholipid antibodies (aPL). βeta 2-glycoprotein I (β2-GPI) is the major autoantigen in APS that binds anionic phospholipids as well as specific receptors on platelets and endothelial cells resulting in activation of prothrombotic pathways. β2-GPI consists of 5 Domains that exist in a circular or linear form, with the latter occurring after binding to anionic phospholipids...
March 29, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29557233/aetiology-of-recurrent-miscarriage-and-the-role-of-adjuvant-treatment-in-its-management-a-retrospective-cohort-review
#13
Samuel James Alexander Dobson, Kanna Mannadiar Jayaprakasan
We conducted a retrospective review into the role of commonly prescribed conventional adjuvant treatments in improving live birth rates after recurrent miscarriage (RM). Data from 301 couples attending the RM clinic in two Tertiary teaching hospitals were analysed with their live birth rate following a further pregnancy and a prevalence of conditions investigated in RM being the main outcomes measured. We found that 26% of women had explained RM and 74% had unexplained RM. Adjuvant versus conservative management did not improve the live birth rates in those with unexplained RM (68...
March 20, 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/29551574/unilateral-localized-conjunctival-amyloidosis-in-a-patient-with-a-history-of-contralateral-orbit-eyelid-lymphoma
#14
Joshua T Byers, Xin Qing, Christopher Lo, Samuel W French, Ping Ji
Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy...
April 2018: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29513853/anti-phospholipid-syndrome-in-seven-leprosy-patients-with-thrombotic-events-on-corticosteroid-and-or-thalidomide-regimen-insights-on-genetic-and-laboratory-profiles
#15
Sebastian Vernal, Maria Jose Franco Brochado, Roberto Bueno-Filho, Paulo Louzada-Junior, Ana Maria Roselino
INTRODUCTION: Corticosteroids and/or thalidomides have been associated with thromboembolism events (TBE) in multibacillary (MB) leprosy. This report aimed to determine genetic and laboratory profiles associated with leprosy and TBE. METHODS: Antiphospholipid antibodies (aPL), coagulation-related exams, prothrombin and Leiden's factor V mutations, and ß2-glycoprotein-I (ß2GPI) Val247Leu polymorphism were assessed. RESULTS: Six out of seven patients with leprosy were treated with prednisone and/or thalidomide during TBE and presented at least one positive aPL...
January 2018: Revista da Sociedade Brasileira de Medicina Tropical
https://www.readbyqxmd.com/read/29454510/beyond-thrombosis-anti-%C3%AE-2gpi-domain-1-antibodies-identify-late-pregnancy-morbidity-in-anti-phospholipid-syndrome
#16
Cecilia Beatrice Chighizola, Francesca Pregnolato, Laura Andreoli, Caterina Bodio, Laura Cesana, Chiara Comerio, Maria Gerosa, Claudia Grossi, Rajesh Kumar, Maria Grazia Lazzaroni, Michael Mahler, Elena Mattia, Cecilia Nalli, Gary L Norman, Maria Gabriella Raimondo, Amelia Ruffatti, Marta Tonello, Laura Trespidi, Angela Tincani, Maria Orietta Borghi, Pier Luigi Meroni
Antibodies against β2 glycoprotein I (anti-β2GPI) have been identified as the main pathogenic autoantibody subset in anti-phospholipid syndrome (APS); the most relevant epitope is a cryptic and conformation-dependent structure on β2GPI domain (D) 1. Anti-β2GPI domain profiling has been investigated in thrombotic APS, leading to the identification of antibodies targeting D1 as the main subpopulation. In contrast, scarce attention has been paid to obstetric APS, hence this study aimed at characterizing the domain reactivity with regards to pregnancy morbidity (PM)...
February 14, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29449131/the-treatment-of-anti-phospholipid-syndrome-a-comprehensive-clinical-approach
#17
REVIEW
Cecilia Beatrice Chighizola, Laura Andreoli, Maria Gerosa, Angela Tincani, Amelia Ruffatti, Pier Luigi Meroni
Anti-phospholipid syndrome (APS) is an acquired pro-thrombotic autoimmune disease that predisposes to thrombotic events and/or obstetric complications, in the persistent presence of anti-phospholipid antibodies (aPL). Life long moderate-intensity anticoagulation is the option of choice for aPL-positive patients with a previous thrombosis; critical issues concern the management of those with a history of arterial event due to the high rate of recurrence. Alternatives comprise anti-platelet agents and high-intensity anticoagulation...
February 12, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29373704/comment-on-failure-of-rivaroxaban-to-prevent-thrombosis-in-four-patients-with-anti-phospholipid-syndrome
#18
COMMENT
Quentin Scanvion, Sandrine Morell-Dubois, Cécile M Yelnik, Johana Bene, Sophie Gautier, Marc Lambert
No abstract text is available yet for this article.
May 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/29373692/comment-on-failure-of-rivaroxaban-to-prevent-thrombosis-in-four-patients-with-anti-phospholipid-syndrome-reply
#19
COMMENT
Virginie Dufrost, Jessie Risse, Denis Wahl, Stéphane Zuily
No abstract text is available yet for this article.
May 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/29343759/incorporation-of-iloprost-in-phospholipase-resistant-phospholipid-scaffold-enhances-its-barrier-protective-effects-on-pulmonary-endothelium
#20
Olga Oskolkova, Nicolene Sarich, Yufeng Tian, Grzegorz Gawlak, Fanyong Meng, Valery N Bochkov, Evgeny Berdyshev, Anna A Birukova, Konstantin G Birukov
Correction of barrier dysfunction and inflammation in acute lung injury (ALI) represents an important problem. Previous studies demonstrate barrier-protective and anti-inflammatory effects of bioactive lipid prostacyclin and its stable analog iloprost (ILO). We generated a phospholipase resistant synthetic phospholipid with iloprost attached at the sn-2 position (ILO-PC) and investigated its biological effects. In comparison to free ILO, ILO-PC caused sustained endothelial cell (EC) barrier enhancement, linked to more prolonged activation of Rap1 and Rac1 GTPases and their cytoskeletal and cell junction effectors: cortactin, PAK1, p120-catenin and VE-cadherin...
January 17, 2018: Scientific Reports
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