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antiphospholipid antibody syndrome

Hee Kyung Yang, Ki Won Moon, Min Jung Ji, Sang Beom Han, Jeong-Min Hwang
Purpose: To report a case of primary antiphospholipid syndrome presenting with isolated homonymous superior quadrantanopsia. Observations: A 50-year-old Korean man presented with subjective visual disturbance for 1 month. Visual field testing showed a right homonymous superior quadrantanopsia. Brain magnetic resonance imaging (MRI) revealed an old infarct in his left occipital lobe and multiple lesions in other areas of the brain. Laboratory tests showed a marked increase in serum anti-β2 glycoprotein I antibody, which remained elevated after 12 weeks...
June 2018: American Journal of Ophthalmology Case Reports
Carlos Perez-Sánchez, Iván Arias-de la Rosa, María Ángeles Aguirre, María Luque-Tévar, Patricia Ruiz-Limón, Nuria Barbarroja, Yolanda Jiménez-Gómez, María Carmen Ábalos-Aguilera, Eduardo Collantes-Estévez, Pedro Segui, Francisco Velasco, María Teresa Herranz, Jesús Lozano-Herrero, María Julia Hernandez-Vidal, Constantino Martínez, Rocío González-Conejero, Massimo Radin, Savino Sciascia, Irene Cecchi, María José Cuadrado, Chary López-Pedrera
The present study aimed at identifying the plasma miRNA profile of antiphospholipid syndrome patients and to investigate the potential role of specific circulating miRNAs as non-invasive disease biomarkers. Ninety antiphospholipid syndrome patients and 42 healthy donors were recruited. Profiling of miRNAs by PCR-array in plasma of antiphospholipid syndrome patients allowed identifying a set of miRNAs differentially expressed and collectively involved in clinical features. Logistic Regression and ROC-curve analyses identified a signature of 10 miRNA ratios as biomarkers of disease...
March 15, 2018: Haematologica
Tatsuya Seki, Yasushige Shingu, Hiroshi Sugiki, Satoru Wakasa, Hiroki Katoh, Tomonori Ooka, Tsuyoshi Tachibana, Suguru Kubota, Yoshiro Matsui
Antiphospholipid syndrome (APS) is a complex autoimmune disease often related to systemic lupus erythematosus. Although adequate anticoagulation is important for APS patients during cardiopulmonary bypass, clotting tests can be potentially misleading due to antiphospholipid antibodies. We performed cardiac surgery safely in two APS patients under anticoagulation monitoring determined using preoperative heparin titration. We performed heparin titration for activated clotting time to determine the appropriate heparin concentration during cardiac surgery...
March 14, 2018: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
F Popescu, C R Jaslow, W H Kutteh
STUDY QUESTION: Will the addition of 24-chromosome microarray analysis on miscarriage tissue combined with the standard American Society for Reproductive Medicine (ASRM) evaluation for recurrent miscarriage explain most losses? SUMMARY ANSWER: Over 90% of patients with recurrent pregnancy loss (RPL) will have a probable or definitive cause identified when combining genetic testing on miscarriage tissue with the standard ASRM evaluation for recurrent miscarriage...
March 12, 2018: Human Reproduction
Dileep Unnikrishnan, Nasreen Shaikh, Ahmad Sharayah, Chandler Patton
A 47-year-old woman with a medical history of Raynaud's phenomenon presented with fever, cough and shortness of breath. She was found to have left lower lobe consolidation and pleural effusion and was treated as a case of pneumonia. During the hospital course, her respiratory status worsened, and she was intubated on the third hospital day. To investigate the high A-a gradient, a Computerized Tomographic Pulmonary Embolism (CTPE) study was done which identified a large left lower pulmonary artery embolism. She was also found to have a new murmur, and an echocardiogram demonstrated a large lesion on tricuspid valve...
March 13, 2018: BMJ Case Reports
K M J Devreese, T L Ortel, V Pengo, B de Laat
No abstract text is available yet for this article.
February 14, 2018: Journal of Thrombosis and Haemostasis: JTH
Luis J Jara, Gabriela Medina, Miguel A Saavedra
PURPOSE OF REVIEW: To provide an update about the interactions between infections and autoimmune diseases (AIDs), from the molecular perspective to the clinical spectrum and the differentiation between infection and disease activity. RECENT FINDINGS: Any kind of infection may modify the innate and adaptive immune response through the following mechanisms: molecular mimicry, superantigens, epitope spreading and B-cell activation. The consequence is the overproduction of antibodies shared with those found in AIDs...
March 9, 2018: Current Opinion in Rheumatology
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
Chrisanna Dobrowolski, Doruk Erkan
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder marked by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). At the present time, treatment is primarily focused on anticoagulation. However, there is increasing awareness of the mechanisms involved in APS pathogenesis, which has led to the trial of novel therapies targeting those mechanisms. Following a brief review of the etiopathogenesis of and current management strategies in APS, this paper focuses on the evidence for these potential, targeted APS treatments, e...
March 3, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Ami Schattner, Shilo Voichanski, Livnat Uliel
A healthy 38-year-old woman developed sudden unilateral vision loss due to retrobulbar optic neuritis in the wake of varicella-zoster virus infection. She had no further central nervous system (CNS) lesions. Antinuclear antibodies (ANA) and anti-aquaporin 4 antibodies were found, consistent with neuromyelitis optica (NMO). Later, serial MRIs showed dynamic short-segment and long-segment myelitis lesions, ANA titre increased and additional autoantibodies were found including anti-dsDNA, anti-chromatin/nucleosome and antiphospholipid antibodies...
March 5, 2018: BMJ Case Reports
Anastasia Sacharidou, Ken L Chambliss, Victoria Ulrich, Jane E Salmon, Yu-Min Shen, Joachim Herz, David Y Hui, Lance S Terada, Philip W Shaul, Chieko Mineo
In the antiphospholipid syndrome (APS), antiphospholipid antibody (aPL) recognition of β2 glycoprotein (β2GPI) promotes thrombosis, and preclinical studies indicate that this is due to endothelial NO synthase (eNOS) antagonism via apoER2-dependent processes. How apoER2 molecularly links these events is unknown. Here we show that in endothelial cells the apoER2 cytoplasmic tail serves as a scaffold for aPL-induced assembly and activation of the heterotrimeric protein phosphatase PP2A. Dab2 recruitment to the apoER2 NPXY motif promotes the activating L309 methylation of the PP2A catalytic subunit by leucine methyl transferase-1...
March 2, 2018: Blood
Amelia Ruffatti, Marta Tonello, Ariela Hoxha, Savino Sciascia, Maria J Cuadrado, José O Latino, Sebastian Udry, Tatiana Reshetnyak, Nathalie Costedoat-Chalumeau, Nathalie Morel, Luca Marozio, Angela Tincani, Laura Andreoli, Ewa Haladyj, Pier L Meroni, Maria Gerosa, Jaume Alijotas-Reig, Sara Tenti, Karoline Mayer-Pickel, Michal J Simchen, Maria T Bertero, Sara De Carolis, Véronique Ramoni, Arsène Mekinian, Elvira Grandone, Aldo Maina, Fátima Serrano, Vittorio Pengo, Munther A Khamashta
The effect of additional treatments combined with conventional therapy on pregnancy outcomes was examined in high-risk primary antiphospholipid syndrome (PAPS) patients to identify the most effective treatment strategy. The study's inclusion criteria were (1) positivity to lupus anticoagulant alone or associated with anticardiolipin and/or anti-β2 glycoprotein I antibodies; (2) a history of severe maternal-foetal complications (Group I) or a history of one or more pregnancies refractory to conventional therapy leading to unexplained foetal deaths not associated with severe maternal-foetal complications (Group II)...
February 28, 2018: Thrombosis and Haemostasis
Ayumi Koga, Ken Okamoto, Hirokazu Tazume, Toshihiro Fukui
Antiphospholipid syndrome is a rare autoimmune disease with a hypercoagulable state causing vascular thrombosis. We present the case of a 42-year old female who underwent mitral valve replacement with a mechanical valve 15 months ago. The postoperative course was uneventful, and echocardiography performed 14 months postoperatively showed good valve function. The patient developed sudden dyspnea 15 months postoperatively and was referred to our hospital. Echocardiography revealed mitral stenosis with stuck leaflets...
January 31, 2018: Heart Surgery Forum
Yu Zuo, Jennifer Fan, Ravi Sarode, Song Zhang, Una E Makris, David R Karp, Yu-Min Shen
The evaluation of thrombotic and pregnancy risks associated with antiphospholipid antibodies (aPL)in individual patients without antiphospholipid syndrome (APS) clinical manifestation is challenging. Our aim is to identify additional risk factors or potential candidate "second hits" for APS clinical events in a large cohort of ethnically diverse aPL-positive patients. We included 219 consecutive aPL-positive patients who attended clinic at our institution. All patients had at least 1 persistent high titer (≥99 percentiles) aPL...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
Michelle Remião Ugolini Lopes, Adriana Danowski, Andreas Funke, Jozelia Rêgo, Roger Levy, Danieli Castro Oliveira de Andrade
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging...
November 2017: Revista da Associação Médica Brasileira
K T El Hadidi, B M Medhat, N M Abdel Baki, H Abdel Kafy, W Abdelrahaman, A Y Yousri, D H Attia, M Eissa, D El Dessouki, I Elgazzar, F T Elgengehy, N El Ghobashy, H El Hadary, G El Mardenly, H El Naggar, A M El Nahas, R M El Refai, H Allah El Rwiny, R M Elsman, M Galal, S Ghoniem, L A Maged, S M Sally, H Naji, S Saad, M Shaaban, M Sharaf, N Sobhy, R M Soliman, T S El Hadidi
Introduction Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can vary among different ethnic and racial groups. Objective The objective of this paper is to study the prevalence of various manifestations of SLE in a sample of the Egyptian population. Patients and methods Information in this study was derived from the medical records of SLE patients who sought medical advice at a private clinic in Cairo from January 1980 to June 2016. Results This study included 1109 SLE patients, of whom 114 (10...
January 1, 2018: Lupus
José Fidel Baizabal-Carvallo, Joseph Jankovic
Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
M Cheloufi, C Wackenheim, C Dumestre-Pérard, A Gueniffey, V Equy, C Thong-Vanh, C Dunand-Faure, P Hoffmann, A Deroux
OBJECTIVE: Presence of non-specific autoimmunity (antinuclear antibodies without antigenic specificities and/or antiphospholipid antibodies without criteria of antiphospholipid syndrome) seems to be associated with unexplained female infertility. The objective is to study the characteristics of patients who undergone treatment for non-specific antibodies in Medically Assisted Procreation (MAP). METHODS: Ten patients were prospectively followed at MAP center of Grenoble University Hospital...
February 2, 2018: Gynecologie, Obstetrique, Fertilite & Senologie
Massimo Radin, Michelle Remião Ugolini-Lopes, Savino Sciascia, Danieli Andrade
OBJECTIVES: Extra-criteria manifestations of antiphospholipid syndrome (APS) might impact on prognosis and morbidity of the disease. In this study, we aimed to evaluate a population of patients with primary APS (PAPS) whether the extra-criteria manifestations were more frequently found in subjects with higher adjusted Global APS Score (aGAPSS) values when compared to patients with thrombotic and/or obstetric APS ("criteria" manifestations) only. METHODS: Clinical records were analyzed to retrieve extra-criteria manifestation of APS, cardiovascular risk factors and antiphospholipid antibodies profile...
January 5, 2018: Seminars in Arthritis and Rheumatism
Olga Amengual, Tatsuya Atsumi
The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the occurrence of venous and arterial thromboses, often multiple, and obstetric-related adverse events in the presence of antiphospholipid antibodies (aPL). APS, first described in 1983, as thrombosis, abortion and cerebral disease, is nowadays recognised as a systemic disease with a wide constellation of clinical manifestations related to acute and chronic vascular lesions. The presence of aPL is the serological hallmark of APS representing a heterogeneous population of autoantibodies with many antigenic specificities directed to phospholipid-binding proteins, either alone or in combination with phospholipids...
February 21, 2018: Modern Rheumatology
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