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Catastrophic Antiphospholipid Antibody syndrome

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https://www.readbyqxmd.com/read/29027631/detrimental-roles-of-tnf-alpha-in-the-antiphospholipid-syndrome-and-de-novo-synthesis-of-antiphospholipid-antibodies-induced-by-biopharmaceuticals-against-tnf-alpha
#1
REVIEW
Mirjana Bećarević
Antiphospholipid syndrome (APS) is an autoimmune disease that is characterized by arterial and/or venous thrombosis and/or recurrent pregnancy losses. Obstetric APS (OAPS) is considered as a distinct entity from vascular APS (VAPS). In the absence of any additional disease, APS is designated as primary (PAPS), while the term secondary APS (SAPS) is used when other diseases are associated. Catastrophic APS (CAPS) is characterized by the rapid development of multiple thrombosis in various vital organs. The presence of antiphospholipid antibodies (aPL Abs) is considered as a laboratory criterion for APS diagnosis...
November 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28991291/catastrophic-antiphospholipid-syndrome-case-report-and-literature-review
#2
Luis del Carpio-Orantes, Chantall Citlally Martínez-Anaya, Elías Bonilla-Casas
The present document is the report of a case of a very rare clinical entity, which presents with acute multiorganic failure after a thrombotic storm related to antiphospholipid antibodies, the so-called catastrophic antiphospholipid syndrome, which began as a recurrent picture of mesenteric thrombosis, with a previous history of venous insufficiency and distal ulcers probably associated with an unidentified antiphospholipid; deserving management in intensive care and the consultation by the world expert, Dr...
2017: Gaceta Médica de México
https://www.readbyqxmd.com/read/28829758/catastrophic-antiphospholipid-syndrome-ronald-asherson-syndrome-and-obstetric-pathology
#3
Alexander D Makatsariya, Jamilya Khizroeva, Viktoriya O Bitsadze
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called "thrombotic storm" and multiple organ failure, that is, CAPS...
August 22, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28638691/catastrophic-antiphospholipid-syndrome-treated-with-rituximab-a-case-report
#4
Atalay Doğru, Yunus Ugan, Mehmet Şahin, Nermin Karahan, Şevket Ercan Tunç
Catastrophic antiphospholipid syndrome (CAPS) is a rare and fatal condition that is characterized by diffuse venous and/or arterial thromboembolism within a short period of time and histopathological confirmation of small-vessel occlusion in at least one organ or tissue in the presence of positive antiphospholipid antibodies. Here we report the case of a 19-year-old woman with CAPS. During the first week of her hospitalization, she was diagnosed with CAPS on the basis of skin necrosis, pulmonary artery thrombosis, cerebral venous sinus thrombosis, and positive lupus anticoagulant...
June 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28632503/the-catastrophic-antiphospholipid-syndrome-in-children
#5
Ellen J L Go, Kathleen M O'Neil
PURPOSE OF REVIEW: To review the difficult syndrome of catastrophic antiphospholipid syndrome, emphasizing new developments in the diagnosis, pathogenesis and treatment. RECENT FINDINGS: Few recent publications directly address pediatric catastrophic antiphospholipid syndrome (CAPS). Most articles are case reports or are data from adult and pediatric registries. The major factors contributing to most pediatric catastrophic antiphospholipid syndrome include infection and the presence of antiphospholipid antibodies, but complement activation also is important in creating diffuse thrombosis in the microcirculation...
September 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28620416/antiphospholipid-syndrome-multiple-manifestations-in-a-single-patient-a-high-suspicion-is-still-needed
#6
Uroosa Ibrahim, Shiksha Kedia, Gwenalyn Garcia, Jean Paul Atallah
Antiphospholipid Syndrome (APS) is an autoimmune disorder with clinical and laboratory features of vascular thrombosis, pregnancy loss, and persistent antiphospholipid antibodies (aPLs). The pathophysiology is thought to involve the activation of endothelial cells, monocytes, platelets, and complement by aPLs. Disease can range from asymptomatic to rapidly fatal catastrophic APS. We present a case of a 34-year-old male referred for pancytopenia and splenomegaly. On examination, he had decreased sensation and 4/5 power in the left upper extremity...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28516871/diagnosis-of-catastrophic-anti-phospholipid-syndrome-in-a-patient-tested-negative-for-conventional-tests
#7
Fabrizio Conti, Roberta Priori, Cristiano Alessandri, Roberta Misasi, Antonella Capozzi, Monica Pendolino, Simona Truglia, Silvia Frisenda, Maurizio Sorice, Guido Valesini
Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of aPL (lupus anticoagulant and/or anticardiolipin antibodies and/or anti-Beta2-glcyoprotein I antibodies). Here we report a case of a 39-year-old woman patient who developed a CAPS which was negative to the conventional aPL but positive for aPL in thin layer chromatography immunostaining and vimentin/cardiolipin antibodies by ELISA test...
July 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28515823/lupus-negative-libman-sacks-endocarditis-complicated-by-catastrophic-antiphospholipid-syndrome
#8
Ghulam Murtaza, Joy Iskandar, Tara Humphrey, Sujeen Adhikari, Aneesh Kuruvilla
Libman-Sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves. In most cases, patients do not have significant valvular dysfunction. However, patients with significant valvular dysfunction may present with serious complications such as cardiac failure, arrhythmias, and thromboembolic events. Recently, association of Libman-Sacks endocarditis with antiphospholipid antibody syndrome (APS) has been made. APS is most commonly defined by venous and arterial thrombosis, recurrent pregnancy loss, and thrombocytopenia...
April 2017: Cardiology Research
https://www.readbyqxmd.com/read/28502938/successful-treatment-of-catastrophic-antiphospholipid-antibody-syndrome-associated-with-malt-lymphoma-by-autologous-hematopoietic-stem-cell-transplantation
#9
Satoko Oka, Kazuo Ono, Masaharu Nohgawa
A 37-year-old woman with extranodal marginal-zone lymphoma was admitted with a fever, hemiplegia, and severe dyspnea after chemotherapy. Catastrophic antiphospholipid antibody syndrome (CAPS) was suspected based on the histopathological confirmation of small-pulmonary vessel occlusion, evidence of the involvement of three organs, and elevated lupus anticoagulant assay results in a short time span. The patient responded to the initial treatment. One month later, the CAPS and lymphoma relapsed, and the patient underwent autologous hematopoietic stem cell transplantation...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28473426/european-evidence-based-recommendations-for-diagnosis-and-treatment-of-paediatric-antiphospholipid-syndrome-the-share-initiative
#10
REVIEW
Noortje Groot, Nienke de Graeff, Tadej Avcin, Brigitte Bader-Meunier, Pavla Dolezalova, Brian Feldman, Gili Kenet, Isabelle Koné-Paut, Pekka Lahdenne, Stephen D Marks, Liza McCann, Clarissa A Pilkington, Angelo Ravelli, Annet van Royen-Kerkhof, Yosef Uziel, Sebastiaan J Vastert, Nico M Wulffraat, Seza Ozen, Paul Brogan, Sylvia Kamphuis, Michael W Beresford
Antiphospholipid syndrome (APS) is rare in children, and evidence-based guidelines are sparse. Consequently, management is mostly based on observational studies and physician's experience, and treatment regimens differ widely. The Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative was launched to develop diagnostic and management regimens for children and young adults with rheumatic diseases. Here, we developed evidence-based recommendations for diagnosis and treatment of paediatric APS...
October 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28357636/probable-catastrophic-antiphospholipid-syndrome-with-intracerebral-hemorrhage-secondary-to-epstein-barr-viral-infection
#11
Mark P Plummer, Adam M H Young, Ronan O'Leary, Maxwell S Damian, Andrea Lavinio
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe variant of antiphospholipid syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein-Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management. METHODS: All data are from a single patient admitted to the Neurosciences Critical Care Unit in Addenbrooke's Hospital, Cambridge, in March 2016...
March 29, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28355988/direct-oral-anticoagulants-an-alternative-treatment-for-thrombotic-antiphospholipid-syndrome
#12
A S Resseguier, B Pereira, V Rieu, G Le Guenno, V Grobost, M Ruivard
Background Direct oral anticoagulants (DOACs) demonstrate a lower risk-benefit ratio than vitamin K antagonists (VKAs) for secondary thromboprophylaxis of thrombotic events. But there are no data on the efficacy of DOACs for the prevention of thrombotic recurrence in patients with antiphospholipid syndrome (APS). In this study, we evaluated the efficacy of DOACs to prevent recurrences of thrombotic events in patients with APS. Methods This was a single-center pilot, using a multi-step Fleming design. If seven or fewer patients presented treatment failure with rivaroxaban, the study could conclude efficacy...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28355985/cessation-of-oral-anticoagulants-in-antiphospholipid-syndrome
#13
C Comarmond, P Jego, C Veyssier-Belot, I Marie, A Mekinian, A Elmaleh-Sachs, G Leroux, D Saadoun, E Oziol, T Fraisse, H Hyvernat, M-F Thiercein-Legrand, F Sarrot-Reynauld, N Ferreira-Maldent, M de Menthon, C Goujard, D Khau, Y Nguen, S Monnier, A Michon, B Castel, O Decaux, J-C Piette, P Cacoub
Objective To study the outcome of patients with antiphospholipid syndrome (APS) after oral anticoagulant treatment cessation. Methods We performed a retrospective study of patients with APS experiencing cessation of oral anticoagulant and enrolled in a French multicentre observational cohort between January 2014 and January 2016. The main outcome was the occurrence of recurrent thrombotic event after oral anticoagulation cessation. Results Forty four APS patients interrupted oral anticoagulation. The median age was 43 (27-56) years...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28278524/management-of-thrombotic-antiphospholipid-syndrome
#14
Cecilia Beatrice Chighizola, Maria Gabriella Raimondo, Pier Luigi Meroni
Persistent serum positivity for antiphospholipid antibodies (aPL) is required to diagnose antiphospholipid syndrome (APS), an autoimmune disease characterized by recurrent vascular thrombosis and/or pregnancy morbidity. The current therapeutic management of patients with thrombotic APS aims at preventing recurrences and long-term complications by attenuating the procoagulant state. There is overall consensus to reserve moderate-intensity anticoagulation to aPL-positive patients with a previous venous thrombosis; the therapeutic options for those with a history of arterial event comprise antiplatelet agents and high-intensity anticoagulation...
March 9, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28277850/diagnosis-and-management-of-catastrophic-antiphospholipid-syndrome
#15
REVIEW
Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair Levy
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia...
April 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28122463/inflammatory-bowel-disease-a-potential-risk-factor-for-coronary-artery-disease
#16
Grigorios Tsigkas, Periklis Davlouros, Stefanos Despotopoulos, Stelios F Assimakopoulos, Georgios Theocharis, George Hahalis
Patients with inflammatory bowel disease (IBD) have a higher incidence of coronary artery disease (CAD) compared with the general population. Left main coronary artery (LMCA) thrombosis constitutes a very rare but catastrophic manifestation of acute coronary syndrome. Case reports describing young patients with IBD and LMCA thrombosis are scarce. Most importantly, patients with a positive family history of thrombotic events and those with significant genetic or acquired risk factors such as the antiphospholipid antibody syndrome, advanced age, immobilization, pregnancy, oral contraceptive use, obesity, diabetes, and cigarette smoking may have a higher risk of thrombosis among those with active IBD...
November 2017: Angiology
https://www.readbyqxmd.com/read/28072724/fatal-antiphospholipid-syndrome-following-endoscopic-transnasal-transsphenoidal-surgery-for-a-pituitary-tumor-a-case-report
#17
Chiao-Zhu Li, Chiao-Ching Li, Chih-Chuan Hsieh, Meng-Chi Lin, Dueng-Yuan Hueng, Feng-Chen Liu, Yuan-Hao Chen
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...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28028298/microrna-expression-in-antiphospholipid-syndrome-a-systematic-review-and-microrna-target-genes-analysis
#18
REVIEW
S Muhammad Shazwan, M Muhammad Aliff, A A Asral Wirda, A R Hayati, M Maizatul Azma, A R Nur Syahrina, A H Nazefah, S Jameela, M M Nur Fariha
INTRODUCTION: Antiphospholipid antibodies (aPL) are autoantibodies that attack phospholipid through anti-beta 2-glycoprotein 1. The actions of aPL are associated with events leading to thrombosis and morbidity in pregnancy. Antiphospholipid syndrome (APS) is diagnosed when a patient is persistently positive for aPL and also has recognised clinical manifestations such as recurrent pregnancy losses, arterial or venous thrombosis and in a catastrophic case, can result in death. Unfortunately, the pathogenesis of APS is still not well established...
December 2016: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/27939948/catastrophic-antiphospholipid-antibody-syndrome-presenting-as-severe-ischaemic-colitis
#19
Cristina González Puga, Inmaculada Lendínez Romero, Antonio Palomeque Jiménez
No abstract text is available yet for this article.
December 8, 2016: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/27886797/catastrophic-antiphospholipid-syndrome-the-current-management-approach
#20
REVIEW
Ignasi Rodriguez-Pintó, Gerard Espinosa, Ricard Cervera
The current recommendation for catastrophic antiphospholipid syndrome (CAPS) management is the standard triple therapy with anticoagulation (AC), glucocorticoids (GCs), plasma exchange (PE), and/or intravenous immunoglobulins (IVIGs). Of note, only AC has a significant effect on the prognosis of these patients. However, from the experimental or basic point of view, there is only indirect evidence to advocate the use of these immunomodulatory therapies (GC, PE, and IVIG) in CAPS. Recently, there have been reports of severe or refractory CAPS patients treated with the monoclonal antibodies rituximab and eculizumab...
April 2016: Best Practice & Research. Clinical Rheumatology
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