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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
Hannah Cohen, Maria Efthymiou, David Isenberg
The direct oral anticoagulants (DOACs) are therapeutic alternatives to warfarin and other vitamin K antagonists (VKAs), and the standard of care for many indications. VKAs are conventional therapy for the treatment and secondary thromboprophylaxis of thrombotic antiphospholipid syndrome (APS), but are often problematic due to the variable sensitivity of thromboplastins to lupus anticoagulant. Thus, the International Normalised Ratio may not accurately reflect anticoagulation intensity, or be clinically effective...
April 6, 2018: Journal of Thrombosis and Haemostasis: JTH
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
S Sciascia, E Coloma-Bazán, M Radin, M L Bertolaccini, C López-Pedrera, Gerard Espinosa, P L Meroni, R Cervera, M J Cuadrado
The current mainstay of treatment in patients with thrombotic antiphospholipid syndrome (APS) is long-term anticoagulation, mainly with Vitamin K antagonist agents. Some recently available studies have created new ground for discussion about the possible discontinuation of anticoagulation therapy in patients with a history of thrombotic APS in whom antiphospholipid antibodies (aPL) are not detected any longer (i.e. aPL seroconversion). We report the main points discussed at the last CORA Meeting regarding the issue whether or not anticoagulation can be stopped after aPL seroconversion...
November 2017: Autoimmunity Reviews
Laurent Arnaud, Fabrizio Conti, Laura Massaro, Gentian Denas, François Chasset, Vittorio Pengo
Whether primary prophylaxis should be prescribed in individuals with antiphospholipid antibodies (aPL) remains controversial due to the lack of relevant evidence-based data. Indeed, it is unclear whether the benefit of LDA outweighs the risk of major bleeding associated with LDA in a low-risk population. On the contrary, stratification of aPL-positive subjects according to their aPL profile (combination, isotype and titer), presence of other concomitant risk factors for thrombosis and coexistence of an underling autoimmune disease is essential to decide whether primary prophylactic therapy should be prescribed...
November 2017: Autoimmunity Reviews
Shruti Chaturvedi, Keith R McCrae
Antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy complications in the presence of persistent antiphospholipid antibodies (APLA). Laboratory diagnosis of APLA depends upon the detection of a lupus anticoagulant, which prolongs phospholipid-dependent anticoagulation tests, and/or anticardiolipin (aCL) and anti-β2-glycoprotein-1 (β2GPI) antibodies. APLA are primarily directed toward phospholipid binding proteins. Pathophysiologic mechanisms underlying thrombosis and pregnancy loss in APS include APLA induced cellular activation, inhibition of natural anticoagulant and fibrinolytic systems, and complement activation, among others...
November 2017: Blood Reviews
D Erkan, O Unlu, S Sciascia, H M Belmont, D Ware Branch, M J Cuadrado, E Gonzalez, J S Knight, I Uthman, R Willis, Z Zhang, D Wahl, S Zuily, M G Tektonidou
Objective The objective of this study was to determine the efficacy of hydroxychloroquine (HCQ) in the primary thrombosis prevention of antiphospholipid antibody (aPL)-positive patients with no other systemic autoimmune diseases. Methods Under the auspices of Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking, a multicenter, international, randomized controlled trial (RCT) was initiated, in which persistently aPL-positive but thrombosis-free patients without systemic autoimmune diseases were randomized to receive HCQ or no treatment in addition to their standard regimen...
March 2018: Lupus
Jorge Rojas-Serrano, Denisse Herrera-Bringas, Diana I Pérez-Román, Renzo Pérez-Dorame, Heidegger Mateos-Toledo, Mayra Mejía
Interstitial lung disease (ILD) is a severe rheumatoid arthritis (RA) manifestation. The worst survival has been associated with usual interstitial pneumonia (UIP) definitive pattern in high-resolution chest tomography (HRCT) scans. Moreover, the use of methotrexate in RA-ILD is controversial. Our aim was to evaluate prognostic factors including methotrexate in an RA-ILD cohort and their association with survival. RA-ILD patients referred for medical evaluation and treatment at a single center were included...
July 2017: Clinical Rheumatology
Vikki M Abrahams, Lawrence W Chamley, Jane E Salmon
No abstract text is available yet for this article.
September 2017: Arthritis & Rheumatology
Deepa R J Arachchillage, Mike Laffan
Antiphospholipid antibodies are a heterogeneous group of autoantibodies that have clear associations with thrombosis and pregnancy morbidity, and which together constitute the 'antiphospholipid syndrome' (APS). However, the pathophysiology of these complications is not well understood and their heterogeneity suggests that more than one pathogenic process may be involved. Diagnosis remains a combination of laboratory analysis and clinical observation but there have been significant advances in identifying specific pathogenic features, such as domain I-specific anti-β2-glycoprotein-I antibodies...
July 2017: British Journal of Haematology
X-J Zhou, M Chen, S-X Wang, F-D Zhou, M-H Zhao
Background Microvascular manifestations of antiphospholipid antibody syndrome in the kidneys include acute renal failure, thrombotic microangiopathy and hypertension. Therapy has been largely empiric. Case report A 49-year-old Chinese man presented with anuric acute renal failure without abundant proteinuria and heavy haematuria, but markedly low levels of urinary sodium, potassium and chlorine upon admission. On day 1 of hospitalization, his thrombocytopenia, anaemia and renal failure showed rapid progression...
June 2017: Lupus
Frank Bienaimé, Christophe Legendre, Fabiola Terzi, Guillaume Canaud
The antiphospholipid syndrome is a common autoimmune disease caused by pathogenic antiphospholipid antibodies, leading to recurrent thrombosis and/or obstetrical complications. Importantly for nephrologists, antiphospholipid antibodies are associated with various renal manifestations including large renal vessel thrombosis, renal artery stenosis, and a constellation of intrarenal lesions that has been termed antiphospholipid nephropathy. This last condition associates various degrees of acute thrombotic microangiopathy, proliferative and fibrotic lesions of the intrarenal vessels, and ischemic modifications of the renal parenchyma...
January 2017: Kidney International
V Pengo
No abstract text is available yet for this article.
December 2016: Journal of Thrombosis and Haemostasis: JTH
Ignasi Rodríguez-Pintó, Marta Moitinho, Irene Santacreu, Yehuda Shoenfeld, Doruk Erkan, Gerard Espinosa, Ricard Cervera
OBJECTIVE: To analyze the clinical and immunologic manifestations of patients with catastrophic antiphospholipid syndrome (CAPS) from the "CAPS Registry". METHODS: The demographic, clinical and serological features of 500 patients included in the website-based "CAPS Registry" were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test while T-test for independent variables was used to compare groups regarding continuous variables...
December 2016: Autoimmunity Reviews
Borja Gracia-Tello, David Isenberg
APS is an autoimmune disease defined by the presence of arterial or venous thrombotic events and/or pregnancy morbidity in patients who test positive for aPL. APS can be isolated (primary APS) or associated with other autoimmune diseases. The kidney is a major target organ in APS, and renal thrombosis can occur at any level within the vasculature of the kidney (renal arteries, intrarenal vasculature and renal veins). Histological findings vary widely, including ischaemic glomeruli and thrombotic lesions without glomerular or arterial immune deposits on immunofluorescence...
July 1, 2017: Rheumatology
O Amengual, R Forastiero, M Sugiura-Ogasawara, K Otomo, K Oku, C Favas, J Delgado Alves, P Žigon, A Ambrožič, M Tomšič, I Ruiz-Arruza, G Ruiz-Irastorza, M L Bertolaccini, G L Norman, Z Shums, J Arai, A Murashima, A E Tebo, M Gerosa, P L Meroni, I Rodriguez-Pintó, R Cervera, J Swadzba, J Musial, T Atsumi
Objective A task force of scientists at the International Congress on Antiphospholipid Antibodies recognized that phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT) might contribute to a better identification of antiphospholipid syndrome (APS). Accordingly, initial and replication retrospective, cross-sectional multicentre studies were conducted to ascertain the value of aPS/PT for APS diagnosis. Methods In the initial study (eight centres, seven countries), clinical/laboratory data were retrospectively collected...
March 2017: Lupus
Kimberly Janet Legault, Amaia Ugarte, Mark Andrew Crowther, Guillermo Ruiz-Irastorza
Antiphospholipid syndrome (APS) is characterized by arterial and/or venous thrombosis with or without pregnancy morbidity in the presence of autoantibodies targeting proteins that associate with membrane phospholipids, termed "antiphospholipid antibodies" (aPL). Management of arterial and venous thromboses shares some similarities with management of arterial and venous thromboses in the general population; however, there are key differences. The majority of studies addressing management of thrombotic APS focus on secondary prevention...
May 2016: Current Rheumatology Reports
Cecilia B Chighizola, Guilherme R de Jesus, D Ware Branch
Even though the association of anti-phospholipid antibodies (aPL) with infertility is debated, infertile women are commonly screened for aPL. To review evidence, a systematic PubMed search was conducted to retrieve papers addressing (i) the association between aPL and infertility, (ii) the positivity rate of criteria and non-criteria aPL in women with infertility, (iii) the association between aPL and assisted reproduction technologies (ART) outcome, (iv) the efficacy of medical treatments on ART outcome, and (v) the effects of ART on thrombotic risk...
June 2016: Autoimmunity Reviews
Ozan Unlu, Sarah B Lieber, Mary J Roman, Doruk Erkan, Michael D Lockshin
No abstract text is available yet for this article.
May 2016: Arthritis & Rheumatology
Mirhelen M Abreu, Adriana Danowski, Denis G Wahl, Mary-Carmen Amigo, Maria Tektonidou, Marcelo S Pacheco, Norma Fleming, Vinicius Domingues, Savino Sciascia, Julia O Lyra, Michelle Petri, Munther Khamashta, Roger A Levy
The purpose of this task force was to critically analyze nine non-criteria manifestations of APS to support their inclusion as APS classification criteria. The Task Force Members selected the non-criteria clinical manifestations according to their clinical relevance, that is, the patient-important outcome from clinician perspective. They included superficial vein thrombosis, thrombocytopenia, renal microangiopathy, heart valve disease, livedo reticularis, migraine, chorea, seizures and myelitis, which were reviewed by this International Task Force collaboration, in addition to the seronegative APS (SN-APS)...
May 2015: Autoimmunity Reviews
2016-09-24 15:53:06
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