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https://www.readbyqxmd.com/read/28265683/-recurrent-thromboembolisms-despite-full-anticoagulation-in-a-patient-with-antiphospholipid-syndrome
#1
S Wernicke, K Selleng, S B Felix, A Greinacher, F Hammer
We report on a female patient with confirmed secondary antiphospholipid syndrome (APS) due to underlying systemic lupus erythematosus (SLE). Despite a thromboplastin time within the normal range (international normalized ratio, INR) under treatment with a vitamin K antagonist (VKA), a recurrent thrombotic event occurred, this time as pulmonary embolism due to bilateral deep vein thrombosis. Despite an INR value in the therapeutic range, clotting factors II, VII, IX and X were found to be insufficiently decreased suggesting inefficient anticoagulation...
March 6, 2017: Der Internist
https://www.readbyqxmd.com/read/28258475/expanding-the-therapeutic-options-for-renal-involvement-in-lupus-eculizumab-available-evidence
#2
REVIEW
Savino Sciascia, Massimo Radin, Jinoos Yazdany, Maria Tektonidou, Irene Cecchi, Dario Roccatello, Maria Dall'Era
In this study, we aimed to systematically review available literature on the efficacy of eculizumab for the treatment of renal involvement in patients with systemic lupus erythematosus (SLE). We conducted a literature search developed a priori, to identify articles reporting clinical experience with the use of eculizumab in SLE patients, focusing on renal involvement. The search strategy was applied to Ovid MEDLINE, EMBASE, In-Process and Other Non-Indexed Citation, Cochrane Central Register of Controlled Trials and Scopus from 2006 to present...
March 3, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28130684/clinical-characteristics-and-laboratory-findings-of-252-chinese-patients-with-anti-phospholipid-syndrome-comparison-with-euro-phospholipid-cohort
#3
Hui Shi, Jia-Lin Teng, Yue Sun, Xin-Yao Wu, Qiong-Yi Hu, Hong-Lei Liu, Xiao-Bing Cheng, Yu-Feng Yin, Jun-Na Ye, Pojen P Chen, Cheng-de Yang
This study aims to characterize the Chinese Han patients with anti-phospholipid syndrome (APS) and compare the data with those of the Euro-Phospholipid cohort. We conducted a single center study consisting of 252 patients with definite APS from 2000 to 2015. We analyzed the clinical and laboratory characteristics of our cohort and compared the data with those of the Euro-Phospholipid cohort. Our cohort consisted of 216 females and 36 males, with a mean age at entry into this study of 41 years (range 11-74 years)...
March 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/27989533/the-use-of-direct-oral-anticoagulants-in-56-patients-with-antiphospholipid-syndrome
#4
Katarzyna Malec, Tadeusz Góralczyk, Anetta Undas
INTRODUCTION: Antiphospholipid syndrome (APS) is a common acquired thrombophilia associated with a high thrombotic risk, in which vitamin K antagonists (VKA) represent the mainstay of therapy. Case series involving up to 35 patients with APS suggested limited efficacy and safety of direct oral anticoagulants (DOACs). MATERIAL AND METHODS: In the prospective case series we followed 56 consecutive patients with APS (44 women and 12 men, aged from 22 to 64years), including 33 (60%) associated with systemic lupus erythematosus (SLE) and 16 (28...
December 14, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27976568/an-unexpected-diagnostic-course-of-systemic-lupus-erythematosus
#5
Aysel Taktak, Adem Köksoy, Özge Başaran, Saba Kiremitçi, Banu Acar, Nermin Uncu, Nilgün Çakar
Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27927040/hydroxychloroquine-in-systemic-lupus-erythematosus-sle
#6
REVIEW
C Ponticelli, G Moroni
Hydroxychloroquine (HCQ) is an alkalinizing lysosomatropic drug that accumulates in lysosomes where it inhibits some important functions by increasing the pH. HCQ has proved to be effective in a number of autoimmune diseases including systemic lupus erythematosus (SLE). Areas covered: In this review the mechanisms of action, the efficacy, and the safety of HCQ in the management of patients with SLE have been reviewed. HCQ may reduce the risk of flares, allow the reduction of the dosage of steroids, reduce organ damage, and prevent the thrombotic effects of anti-phospholipid antibodies...
March 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/27914592/hyperuricemia-in-systemic-lupus-erythematosus-is-it-associated-with-the-neuropsychiatric-manifestations-of-the-disease
#7
Mahdi Sheikh, Shafieh Movassaghi, Mohammad Khaledi, Maryam Moghaddassi
OBJECTIVES: To assess the association between hyperuricemia and different neuropsychiatric manifestations and stroke risk factors in systematic lupus erythematosus (SLE) patients. METHODS: This study was conducted on 204 SLE patients who were admitted to a tertiary referral center. A standardized questionnaire was completed for all the participants and the medical records were reviewed regarding the occurrence of arterial or venous thrombotic events, stroke, seizure, depression, headache, psychosis, and peripheral neuropathy...
November 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27777394/systemic-lupus-erythematosus-presenting-as-refractory-thrombotic-thrombocytopenic-purpura-a-diagnostic-and-management-challenge-a-case-report-and-concise-review-of-the-literature
#8
REVIEW
Mohammad Abu-Hishmeh, Alamgir Sattar, Fnu Zarlasht, Mohamed Ramadan, Aisha Abdel-Rahman, Shante Hinson, Caroline Hwang
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE...
October 25, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27753626/a-unique-antiphospholipid-assay-recognizing-phospholipid-mixture-compared-with-criteria-antiphospholipid-immunoassays-in-lupus-patients
#9
Y Zuo, R Willis, E Papalardo, M Petri, E N Harris, A Schleh, K DeCeulaer, M Smikle, L M Vilá, J D Reveille, G S Alarcón, E B Gonzalez
BACKGROUND: While essential for the classification of antiphospholipid syndrome (APS), anticardiolipin (aCL) assays lack specificity and anti-β2glycoproteinI (anti-β2GPI) assays lack sensitivity in this regard. Our aim was to perform a comparative analysis of the APhL ELISA assay (IgG/IgM) and criteria antiphospholipid (aPL) immunoassays in identifying APS-related clinical manifestations in a large group of patients with systemic lupus erythematosus (SLE). METHODS: Serum samples from 1178 patients from the Hopkins (n = 543), LUMINA (n = 588) and Jamaican SLE cohorts (n = 47) were examined for IgG/IgM positivity in aCL (in-house), anti-β2GPI (two commercial kits) and APhL (Louisville APL) ELISA assays...
October 16, 2016: Lupus
https://www.readbyqxmd.com/read/27699076/heparin-related-thrombocytopenia-triggered-by-severe-status-of-systemic-lupus-erythematosus-and-bacterial-infection
#10
Satoshi Suzuki, Shihoko Nakajima, Taiki Ando, Keisuke Oda, Manabu Sugita, Kunimi Maeda, Yutaka Nakiri, Yoshinari Takasaki
A patient with severe lupus nephritis developed thrombocytopenia during treatment with high-dose steroids. In addition to viral- or disease-induced cytopenia, the pathology was believed to arise from diverse contributing factors, such as thrombotic microangiopathy and heparin-related thrombocytopenia (HIT). By combining plasma exchange therapy and intravenous cyclophosphamide, we successfully controlled the SLE activity and improved the thrombocytopenia. An antecedent bacterial infection or SLE activity is believed to have contributed to the concurrent HIT...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27698012/von-willebrand-factor-interacts-with-surface-bound-c1q-and-induces-platelet-rolling
#11
Robert Kölm, Monica Schaller, Lubka T Roumenina, Iga Niemiec, Johanna A Kremer Hovinga, Elham Khanicheh, Beat A Kaufmann, Helmut Hopfer, Marten Trendelenburg
Premature atherosclerosis and thrombotic complications are major causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). However, the high incidence of these complications cannot be explained by traditional risk factors alone, suggesting direct effects of an activated immune system on hemostasis. The unexpected nucleotide sequence homology between SLE patient -: derived autoantibodies against complement C1q (Fab anti-C1q) and von Willebrand factor (VWF) led us to investigate a potential interaction between the complement and hemostatic systems on the level of initiating molecules...
October 3, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27651919/increased-von-willebrand-factor-levels-in-patients-with-systemic-lupus-erythematosus-reflect-inflammation-rather-than-increased-propensity-for-platelet-activation
#12
Johannes C Nossent, Warren D Raymond, Gro Østli Eilertsen
BACKGROUND: von Willebrand factor (VWF) is involved in platelet plug formation and protein transport. Increased VWF levels in systemic lupus erythematous (SLE) are considered risk factors for vascular events. VWF protein levels, however, do not accurately reflect its platelet-aggregating function, which has not been examined in SLE. METHODS: Cross-sectional study with clinical and laboratory data obtained in patients with SLE (n=92) from a regional lupus registry...
2016: Lupus Science & Medicine
https://www.readbyqxmd.com/read/27464767/renal-thrombotic-microangiopathy-in-proliferative-lupus-nephritis-risk-factors-and-clinical-outcomes-a-case-control-study
#13
Ana Barrera-Vargas, Rodrigo Rosado-Canto, Javier Merayo-Chalico, José M Arreola-Guerra, Juan M Mejía-Vilet, Ricardo Correa-Rotter, Diana Gómez-Martín, Jorge Alcocer-Varela
BACKGROUND: Renal thrombotic microangiopathy (TMA) may be associated with lupus nephritis. Its relationship to other disease factors and its specific effect on prognosis are not precisely known. Evidence regarding these aspects is controversial, and information focusing on kidney-limited TMA in systemic lupus erythematosus (SLE) patients is scarce. OBJECTIVES: The aims of this study were to identify risk factors for renal TMA in patients with lupus nephritis and to determine its impact on clinical outcomes...
August 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/27403332/assessment-of-the-independent-associations-of-igg-igm-and-iga-isotypes-of-anticardiolipin-with-thrombosis-in-sle
#14
Vinicius Domingues, Laurence S Magder, Michelle Petri
OBJECTIVE: The Sydney classification criteria for antiphospholipid syndrome include lupus anticoagulant or moderate-to-high titre anticardiolipin IgG or IgM. We explored the association of all anticardiolipin isotypes, lupus anticoagulant and the combination with venous and arterial thrombosis. METHODS: Patients with systemic lupus erythematosus (SLE) in a large clinical cohort seen quarterly were repeatedly tested by protocol for anticardiolipin antibodies and lupus anticoagulant...
2016: Lupus Science & Medicine
https://www.readbyqxmd.com/read/27349351/lupus-nephritis-when-and-how-often-to-biopsy-and-what-does-it-mean
#15
REVIEW
Gabriella Moroni, Federica Depetri, Claudio Ponticelli
Renal disease is a frequent complication of SLE which can lead to significant illness and even death. Today, a baseline renal biopsy is highly recommended for all subjects with evidence of lupus nephritis. Biopsy allows the clinician to recognize and classify different forms of autoimmune lupus glomerulonephritis, and to detect other glomerular diseases with variable pathogenesis which are not directly related to autoimmune reactivity, such as lupus podocytopathy. Moreover, not only glomerular diseases, but other severe forms of renal involvement, such as tubulo-interstitial nephritis or thrombotic microangiopathy may be detected by biopsy in lupus patients...
November 2016: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27253369/measuring-iga-anti-%C3%AE-2-glycoprotein-i-and-igg-iga-anti-domain-i-antibodies-adds-value-to-current-serological-assays-for-the-antiphospholipid-syndrome
#16
Charis Pericleous, Isabel Ferreira, Orietta Borghi, Francesca Pregnolato, Thomas McDonnell, Acely Garza-Garcia, Paul Driscoll, Silvia Pierangeli, David Isenberg, Yiannis Ioannou, Ian Giles, Pier Luigi Meroni, Anisur Rahman
INTRODUCTION: Currently available clinical assays to detect antiphospholipid antibodies (aPL) test for IgG and IgM antibodies to cardiolipin (aCL) and β2-glycoprotein I (aβ2GPI). It has been suggested that testing for IgA aPL and for antibodies to Domain I (DI), which carries the key antigenic epitopes of β2GPI, could add value to these current tests. We performed an observational, multicenter cohort study to evaluate the utility of IgG, IgM and IgA assays to each of CL, β2GPI and DI in APS...
2016: PloS One
https://www.readbyqxmd.com/read/27225212/hughes-syndrome-and-epilepsy-when-to-test-for-antiphospholipid-antibodies
#17
M H A Noureldine, G Harifi, A Berjawi, A A Haydar, M Nader, R Elnawar, A Sweid, J Al Saleh, M A Khamashta, I Uthman
Epilepsy and seizures are reported among the neurological manifestations of antiphospholipid syndrome (APS) at a prevalence rate of approximately 8%, which is nearly 10 times the prevalence of epilepsy in the general population. The association of seizures with antiphospholipid antibodies (aPL) is even more significant in the presence of systemic lupus erythematosus (SLE). In this review, we discuss the epidemiological, pathophysiological, laboratory, clinical, and radiological aspects of this association, and derive suggestions on when to consider testing for aPL in epileptic patients and how to manage seizures secondary to APS based on literature data...
November 2016: Lupus
https://www.readbyqxmd.com/read/27215259/anti-neutrophil-cytoplasmic-antibody-negative-crescentic-paucimmune-glomerulonephritis-in-a-case-of-scleroderma-with-systemic-lupus-erythematosus-overlap
#18
Rohit Tewari, Sonia Badwal, Arun Kumar, Shankar Subramaniam, V S Nijhawan, V Srinivas
Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE) is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative...
May 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27158525/placental-histology-and-neutrophil-extracellular-traps-in-lupus-and-pre-eclampsia-pregnancies
#19
Wendy Marder, Jason S Knight, Mariana J Kaplan, Emily C Somers, Xu Zhang, Alexander A O'Dell, Vasantha Padmanabhan, Richard W Lieberman
OBJECTIVE: Systemic lupus erythematosus (SLE) is associated with increased risk of adverse pregnancy outcomes, including pre-eclampsia, particularly in association with antiphospholipid antibody syndrome (APS). While significant placental abnormalities are expected in pre-eclampsia, less is known about how lupus activity and APS in pregnancy affect the placenta. We describe placental pathology from a population of lupus pregnancies, several of which were complicated by APS-related thromboses, in which pre-eclampsia and other complications developed...
2016: Lupus Science & Medicine
https://www.readbyqxmd.com/read/27149490/plasmapheresis-is-associated-with-better-renal-outcomes-in-lupus-nephritis-patients-with-thrombotic-microangiopathy-a-case-series-study
#20
Qiu-Yu Li, Feng Yu, Fu-De Zhou, Ming-Hui Zhao
The aim of this study was to evaluate the efficacy of plasmapheresis in patients with lupus nephritis-combined thrombotic microangiopathy (TMA) in a Chinese cohort.Clinical and therapeutic data of patients with lupus nephritis-combined TMA were collected retrospectively. A comparison between those with and without plasmapheresis was performed.Seventy patients with renal biopsy-proven TMA in lupus nephritis were treated with conventional combined corticosteroid and immunosuppressive agents as induction therapy, 9 of the 70 patients received additional plasmapheresis...
May 2016: Medicine (Baltimore)
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