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Antiphospholipid syndrome

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https://www.readbyqxmd.com/read/29781817/intravenous-immunoglobulin-therapy-in-refractory-autoimmune-dysautonomias-a-retrospective-analysis-of-38-patients
#1
Jill R Schofield, Kamal R Chemali
BACKGROUND: Intravenous immunoglobulin (IVIG) has recognized efficacy in autoimmune peripheral nerve disorders, but there has been limited study of the use of IVIG in autoimmune dysautonomias. STUDY QUESTION: To determine the efficacy and safety of IVIG in patients with disabling, refractory autoimmune dysautonomias, including patients with postural tachycardia syndrome and gastrointestinal dysmotility. STUDY DESIGN: Patients with one or more autonomic disorder(s) and persistent serological evidence for autoimmunity who were unable to work or attend school despite usual treatments for dysautonomia were treated with IVIG for at least 3 months at a dose of at least 1 gm/kg monthly...
May 14, 2018: American Journal of Therapeutics
https://www.readbyqxmd.com/read/29779928/the-diagnosis-and-clinical-management-of-the-catastrophic-antiphospholipid-syndrome-a-comprehensive-review
#2
REVIEW
Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation...
May 17, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29768970/neurologic-manifestations-of-antiphospholipid-syndrome
#3
I F Ricarte, L A Dutra, F F Abrantes, F F Toso, O G P Barsottini, G S Silva, A W S de Souza, D Andrade
Neurological involvement in antiphospholipid antibody syndrome (APS) is common, and its occurrence increases morbidity and mortality. Patients may present variable neurological involvement, such as cerebrovascular disease, cognitive dysfunction, headache, seizures, movement disorders, multiple sclerosis-like syndrome, transverse myelitis and ocular symptoms. Most neurological manifestations are associated with thrombosis of the microcirculation or of large vessels; nonetheless, there is compelling evidence suggesting that, in some cases, symptoms are secondary to an immune-mediated pathogenesis, with direct binding of aPL on neurons and glia...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29764818/necrotising-scleritis-keratitis-and-uveitis-in-primary-antiphospholipid-syndrome
#4
Brijesh Takkar, Sudarshan Khokhar, Uma Kumar, Pradeep Venkatesh
Ocular manifestations of antiphospholipid syndrome typically include thromboembolic and neuro-ophthalmic complications. In this report we present a case of inflammation of the ocular coats in a patient diagnosed with antiphospholipid syndrome 16 years prior. We discuss management of the case and the possible aetiology of the rare association.
May 14, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29756580/subtypes-of-antiphospholipid-antibodies-in-neurologic-accidents-an-observational-study
#5
Maryam Sahebari, Maryam Rastin, Reza Boostani, Mohsen Forughipour, Kamila Hashemzadeh, Samira HaajSadeghi
OBJECTIVE: Until now, concomitant neurological manifestations and positive antiphospholipid antibodies (APA(s)) have been investigated in different manners. The most important conflicts in this field are whether those manifestations are directly related to APA(s) or not and treatment strategies. METHOD: In this study, adopting a different manner, we selected patients with a recently defined neurological problem (according to study protocol) and positive APA without any other risk factor, to compare types, amounts, and titer of APAs...
May 14, 2018: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/29756268/does-rotational-thromboelastometry-accurately-predict-coagulation-status-in-patients-with-lupus-anticoagulant
#6
L Hensch, V Kostousov, K Bruzdoski, M Losos, M Pereira, M de Guzman, S Hui, J Teruya
INTRODUCTION: Rotational thromboelastometry (ROTEM) is increasingly used as a tool for monitoring coagulation status. However, ROTEM is susceptible to misinterpretation due to particular coagulation abnormalities. Here, we report the effects of lupus anticoagulant (LA) on ROTEM. METHODS: A prospective observational analysis was performed on 16 children with prior studies indicating the presence of LA or antiphospholipid antibodies. ROTEM analysis was performed, and samples were further analyzed by adding phospholipids (PL) to repeat ROTEM analysis if clotting time (CT) abnormalities were discovered with comparison to ROTEM using an equal volume of isotonic saline...
May 13, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29755633/coronary-artery-thromboses-stent-thrombosis-and-antiphospholipid-antibody-syndrome-case-report
#7
Augusto Ferreira Correia, Dinaldo Cavalcanti Oliveira, Marcio Sanctos
The antiphospholipid antibody syndrome (APS) is defined by a state of hypercoagulability secondary to an autoimmune disorder. There are evidences that approximately 2.8-5.5% of cases of acute myocardial infarction (AMI) in young individuals are secondary to APS. In this case report, three coronary artery thromboses occurring within a short period are described. Initially, there was an ST-segment elevation (STEMI) in the presence of coronary artery disease (CAD), with the vessel being treated with stent implantation...
April 2018: Cardiology Research
https://www.readbyqxmd.com/read/29753382/may-thurner-syndrome
#8
Yub Raj Sedhai, Reshma Golamari, Aliaksei Salei, Joseph Alukal, Soney Basnyat, Sunil Pathak, Santosh Timalsina, Subtain Zia, Suraj Malhan, Priyanka T Bhatacharya
This single-center, retrospective review identified 6 patients (n = 6, 100% female) treated by endovascular therapy for May-Thurner syndrome from June 2013 to September 2015. Patients consisted of 3 African American, 2 Caucasian and 1 Asian; mean age was 53.50 ± 8.31 years, range: 39-63 years. Clinical presentations consisted of left lower extremity deep vein thrombosis in 4, left lower extremity deep vein thrombosis with pulmonary embolism in 1 and pulmonary embolism with left common iliac vein thrombosis in 1 patient...
May 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29748168/the-antiphospholipid-syndrome-finally-fathomed
#9
Philip G de Groot
No abstract text is available yet for this article.
May 10, 2018: Blood
https://www.readbyqxmd.com/read/29747993/long-term-effect-of-b-cells-depletion-alone-as-rescue-therapy-for-severe-thrombocytopenia-in-primary-antiphospholipid-syndrome
#10
Savino Sciascia, Massimo Radin, Irene Cecchi, Elena Rubini, Mario Bazzan, Dario Roccatello
OBJECTIVES: To investigate the long-term effect of B-cell depletion therapy with Rituximab (RTX) alone as rescue therapy in primary antiphospholipid syndrome (PAPS) patients with severe thrombocytopenia. METHODS: We retrospectively retrieved data from patients who met the following inclusion criteria: (a) persistent antiphospholipid antibodies (aPL) positivity and fulfilled the Sydney criteria for PAPS (b) presented with severe thrombocytopenia (platelets <50,000/mm3 ) (c) were treated with RTX as a rescue therapy (d) had at least 1 year of follow-up after B-cells depletion therapy...
April 11, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29737455/current-and-future-use-of-chloroquine-and-hydroxychloroquine-in-infectious-immune-neoplastic-and-neurological-diseases-a-mini-review
#11
REVIEW
Domenico Plantone, Tatiana Koudriavtseva
The process of finding new therapeutic indications for currently used drugs, defined as 'repurposing', is receiving growing attention. Chloroquine and hydroxychloroquine, with an original indication to prevent or cure malaria, have been successfully used to treat several infectious (HIV, Q fever, Whipple's disease, fungal infections), rheumatological (systemic lupus erythematosus, antiphospholipid antibody syndrome, rheumatoid arthritis, Sjögren's syndrome), and other immunological diseases. Indeed, they have anti-inflammatory, immunomodulating, anti-infective, antithrombotic, and metabolic effects...
May 8, 2018: Clinical Drug Investigation
https://www.readbyqxmd.com/read/29735498/diffuse-alveolar-haemorrhage-and-libman-sacks-endocarditis-a-rare-presentation-of-antiphospholipid-syndrome
#12
Brianna Bielski, Nikhil H Shah, Donevan Westerveld, Carolyn Stalvey
A 26-year-old woman with a history of idiopathic thrombocytopaenic purpura and a 1-year history of blood-streaked sputum presented after a severe episode of haemoptysis with dyspnoea. Chest imaging revealed diffuse ground glass and bronchovascular nodules. Bronchoscopy revealed bilateral diffuse alveolar haemorrhage (DAH). Sputum and bronchoalveolar lavage studies were negative for infectious aetiologies. A transthoracic echocardiogram revealed Libman-Sacks endocarditis with severe mitral regurgitation and physical examination revealed retinal artery occlusion and Osler's nodes...
May 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29730854/renal-involvement-in-antiphospholipid-syndrome
#13
REVIEW
Francisco Vileimar Andrade de Azevedo, Diego Germano Maia, Jozelio Freire de Carvalho, Carlos Ewerton Maia Rodrigues
This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries...
May 5, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29723256/renal-protective-effect-of-antiplatelet-therapy-in-antiphospholipid-antibody-positive-lupus-nephritis-patients-without-antiphospholipid-syndrome
#14
Hironari Hanaoka, Harunobu Iida, Tomofumi Kiyokawa, Yukiko Takakuwa, Takahiro Okazaki, Hidehiro Yamada, Shoichi Ozaki, Kimito Kawahata
OBJECTIVE: We sought to evaluate the effect of antiplatelet therapy in addition to conventional immunosuppressive therapy for lupus nephritis (LN) patients positive for antiphospholipid antibodies (aPL) without definite antiphospholipid syndrome (APS). METHODS: Patients with biopsy-proven LN class III or IV were retrospectively evaluated. We selected patients positive for anticardiolipin antibody (aCL) or lupus anticoagulant (LA) who did not meet the criteria for a diagnosis of APS...
2018: PloS One
https://www.readbyqxmd.com/read/29716893/complement-c5-but-not-c3-is-expendable-for-tissue-factor-activation-by-cofactor-independent-antiphospholipid-antibodies
#15
Nadine Müller-Calleja, Svenja Ritter, Anne Hollerbach, Tanja Falter, Karl J Lackner, Wolfram Ruf
The complement and coagulation cascades interact at multiple levels in thrombosis and inflammatory diseases. In venous thrombosis, complement factor 3 (C3) is crucial for platelet and tissue factor (TF) procoagulant activation dependent on protein disulfide isomerase (PDI). Furthermore, C5 selectively contributes to the exposure of leukocyte procoagulant phosphatidylserine (PS), which is a prerequisite for rapid activation of monocyte TF and fibrin formation in thrombosis. Here, we show that monoclonal cofactor-independent antiphospholipid antibodies (aPLs) rapidly activate TF on myelomonocytic cells...
May 8, 2018: Blood Advances
https://www.readbyqxmd.com/read/29715499/new-and-upcoming-treatments-in-antiphospholipid-syndrome-a-comprehensive-review
#16
REVIEW
Flavio Signorelli, Gustavo Guimarães Moreira Balbi, Vinicius Domingues, Roger Abramino Levy
Current therapeutic recommendations for thrombosis prevention in patients with antiphospholipid syndrome (APS) are limited to anticoagulation with vitamin K antagonists (VKA) or heparins and to anti-platelet aggregating agents. Maintaining optimized anticoagulation to prevent recurrent thrombosis or bleeding remains a therapeutic challenge. Although there are important ongoing trials with direct oral anticoagulants, they still aim the same target. New insights about pathophysiology in APS have revealed a myriad of potential pathways to be investigated as treatment targets...
April 28, 2018: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29713925/antiphospholipid-antibody-profile-based-outcome-of-purely-vascular-and-purely-obstetric-antiphospholipid-syndrome
#17
Amihai Rottenstreich, Ariela Arad, Hadas Terespolsky, Uriel Elchalal, Hagai Amsalm, Batia Roth, Yosef Kalish
Antiphospholipid syndrome (APLS) is caused by antiphospholipid autoantibodies, and manifests with vascular and/or obstetric complications. The factors associated with initial disease presentation and course are unknown. We assessed the antibody profile associated with disease presentation and with the development of vascular and obstetric complications in women with initially vascular or initially obstetric APLS. A review of records of APLS women at childbearing age followed at one center during 2006-2015. Of 126 women, median age at diagnosis 29 [23-37] years, 62 were initially diagnosed with purely obstetric APLS and 64 with purely vascular APLS...
April 30, 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29709100/a-comparison-of-effectiveness-among-frequent-treatments-of-recurrent-spontaneous-abortion-a-bayesian-network-meta-analysis
#18
Sha Lv, Jing Yu, Xiaoxiao Xu
OBJECTIVE: A comprehensive network meta-analysis was designed to clarify contradictions and offer valuable clinical guidance in the treatment of recurrent spontaneous abortion (RSA). METHOD: The included clinical trials were selected from the relevant medical journal databases and screened. Treatments were ranked by the surface under the cumulative ranking curve. Heat plots were constructed to analyze the inconsistency between direct data and network results, and adjusted funnel plots were constructed to assess publication bias...
April 30, 2018: American Journal of Reproductive Immunology: AJRI
https://www.readbyqxmd.com/read/29701436/-abnormal-movements-and-internal-medicine-pathologies
#19
Joseph Ghika
Hyperkinesias can be revelaed by an internal medicine pathology. An acute chorea can be found in association with non ketotic hypergycemia, lupus, antiphospholipid syndrome, endocrinopathies, pregnancy or oral contraceptive initiation, or psychostimulant medication. Acute dystonic syndromes are found in association with the initiation of an old generation neuroleptic, metoclopramide, oral contraceptive and pregnancy. It should be differentiated from hyper- or hypo-calcémie tetany. Tremors are found in association with many drugs and hormones...
April 25, 2018: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29701415/antiphospholipid-syndrome-and-acute-postpartum-limb-ischemia
#20
Inês Antunes, Carlos Pereira, Rui Machado, Duarte Rego, Vitor Ferreira, João Gonçalves, Gabriela Teixeira, Carlos Veiga, Daniel Mendes, Rui Almeida
INTRODUCTION: The diagnosis of Antiphospholipid syndrome (APS) implies the identification of antiphospoholipid antibodies and arterial/venous thrombosis or pregnancy loss. During pregnancy, there is an increased risk of thrombotic complications. METHODS: Present a case of acute lower limb ischemia in a patient with APS during postpartum period Materials/ Methods: review of a clinical case and available literature Results: Patient diagnosed with APS (triple antibody positive and antecedent of 3 previous abortions) underwent cesarean at 29 weeks of gestation...
July 2017: Revista Portuguesa de Cirurgia Cardio-torácica e Vascular
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