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Anti-phospholipid syndrome

Lai Heng Lee
The group of new oral anticoagulants or NOACs, now termed direct oral anticoagulants or DOACs, with their favourable results from large scale phase III clinical trials, represent a major advancement and expanded armamentarium in antithrombotic therapy. Dabigatran, rivaroxaban, apixaban and edoxaban are now in clinical routine use for prevention and treatment of arterial and venous thrombotic diseases as addressed in their clinical trials. Usage of the DOACs is expected to increase as clinicians gain more experience and reassurance with data from the real world studies which are generally consistent with that from clinical trials...
2016: Thrombosis Journal
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
Carlos Wong-Baeza, Albany Reséndiz-Mora, Luis Donis-Maturano, Isabel Wong-Baeza, Luz Zárate-Neira, Juan Carlos Yam-Puc, Juana Calderón-Amador, Yolanda Medina, Carlos Wong, Isabel Baeza, Leopoldo Flores-Romo
Anti-lipid IgG antibodies are produced in some mycobacterial infections and in certain autoimmune diseases [such as anti-phospholipid syndrome, systemic lupus erythematosus (SLE)]. However, few studies have addressed the B cell responses underlying the production of these immunoglobulins. Anti-lipid IgG antibodies are consistently found in a murine model resembling human lupus induced by chlorpromazine-stabilized non-bilayer phospholipid arrangements (NPA). NPA are transitory lipid associations found in the membranes of most cells; when NPA are stabilized they can become immunogenic and induce specific IgG antibodies, which appear to be involved in the development of the mouse model of lupus...
2016: Frontiers in Immunology
X Qi, G Han, X Guo, V De Stefano, K Xu, Z Lu, H Xu, A Mancuso, W Zhang, X Han, D C Valla, D Fan
BACKGROUND: China may have the largest number of Budd-Chiari syndrome (BCS) cases in the world (at least 1914 original papers were published, and at least 20 191 BCS patients were reported). Considering the discrepancy in the clinical profiles and preferred treatment selection of primary BCS between the West and China, understanding its aetiology in these two different regions is very important. AIM: To review the data from large cohort studies and meta-analyses to illustrate the epidemiology of risk factors for BCS in the West and China...
October 13, 2016: Alimentary Pharmacology & Therapeutics
Tomoko Matsumoto, Keiji Nogami, Midori Shima
Patients with mild/moderate hemophilia (H)A, acquired HA (AHA) and lupus anticoagulants (LA), have prolonged aPTTs with low levels of factor (F)VIII activity, but the differentiation of these disorders is complex and time consuming. We established an approach to quickly differentiate these disorders using comprehensive coagulation tests. Patients' plasmas with mild/moderate HA, AHA, LA without anti-phospholipid syndrome [LA-APS(-)], and LA with APS [LA-APS(+)] were examined using clot waveform analysis (CWA) and thrombin generation test (TGT)...
October 11, 2016: International Journal of Hematology
Junzo Nojima, Yukari Motoki, Kazusa Hara, Toshiyuki Sakata, Kiyoshi Ichihara
Antiphospholipid syndrome, which often complicates systemic lupus erythematosus (SLE), features high occurrence of arterial and/or venous thrombosis and recurrent fetal loss. However, which antibody subclass contributes to which clinical event remains uncertain. We newly developed an up-to-date enzyme immunoassay system using the AcuStar automated analyzer (Instrumentation Laboratory, Bedford, Massachusetts, USA) for parallel detection of six subclasses of antiphospholipid antibodies (aPLs): anticardiolipin antibodies (aCL) of IgG, IgM, and IgA and anti-β2-glycoprotein I antibodies (aβ2GPI) of IgG, IgM, and IgA...
September 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Alban Deroux, Chantal Dumestre-Perard, Camille Dunand-Faure, Laurence Bouillet, Pascale Hoffmann
On average, 10 % of infertile couples have unexplained infertility. Auto-immune disease (systemic lupus erythematosus, anti-phospholipid syndrome) accounts for a part of these cases. In the last 20 years, aspecific auto-immunity, defined as positivity of auto-antibodies in blood sample without clinical or biological criteria for defined diseases, has been evoked in a subpopulation of infertile women. A systematic review was performed (PUBMED) using the MESH search terms "infertility" and "auto-immunity" or "reproductive technique" or "assisted reproduction" or "in vitro fertilization" and "auto-immunity...
September 14, 2016: Clinical Reviews in Allergy & Immunology
Saartje Bloemen, Xiao Xuan Wu, Katrien M Devreese, Bas de Laat, Jacob H Rand, Ljiljana V Vasovic
INTRODUCTION: The antiphospholipid syndrome (APS) is an acquired autoimmune disorder predisposing patients to thrombosis or pregnancy complications. Since inverted erythrocyte membranes (iEMs) might provide a physiologically relevant source of anionic phospholipids, we studied the interactions of phospholipid-binding proteins and APS antibodies using iEMs. MATERIALS & METHODS: iEMs were prepared from packed erythrocytes by hypotonic lysis. Phosphatidylserine (PS) exposure was confirmed by annexin A5 (A5) binding using fluorescence microscopy and flow cytometry...
October 2016: Thrombosis Research
Marion Delcroix, Kim Kerr, Peter Fedullo
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism. As for most rare diseases, epidemiologic data are scarce, but recent registries suggest an incidence of at least 5 per million inhabitants per year. A history of massive or recurrent acute pulmonary embolism is observed in most patients with CTEPH, but the proportion of patients who develop CTEPH after acute pulmonary embolism is a matter of debate, further complicated by the possible misdiagnosis of CTEPH as acute pulmonary embolism...
July 2016: Annals of the American Thoracic Society
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...
August 21, 2016: Rheumatology
Pier Luigi Meroni
Pregnancy morbidity is part of the clinical spectrum of the anti-phospholipid syndrome (APS), with an important social and economical cost. Antiplatelet and anticoagulant agents are effective in preventing the clinical manifestations in the majority of the patients. However, a consistent proportion of the pregnant women present recurrences in spite of the standard therapy. Observational studies and anecdotal reports raised the issue of additional therapeutic strategies in these refractory cases. Among these, anti-malarials (AMs) and in particular hydroxychloroquine (HCQ) are becoming more and more popular in APS as well as in other systemic autoimmune rheumatic conditions...
August 2, 2016: Journal of Autoimmunity
Kenji Oku, Hiroyuki Nakamura, Michihiro Kono, Kazumasa Ohmura, Masaru Kato, Toshiyuki Bohgaki, Tetsuya Horita, Shinsuke Yasuda, Olga Amengual, Tatsuya Atsumi
The involvement of complement activation in the pathophysiology of antiphospholipid syndrome (APS) was first reported in murine models of antiphospholipid antibody (aPL)-related pregnancy morbidities. We previously reported that complement activation is prevalent and may function as a source of procoagulant cell activation in the sera of APS patients. Recently, autoantibodies against C1q, a component of complement 1, were reported to be correlated with complement activation in systemic lupus erythematosus. These antibodies target neoepitopes of deformed C1q bound to various molecules (i...
October 2016: Autoimmunity Reviews
Milica I Kojadinovic, Aleksandra C Arsic, Jasmina D Debeljak-Martacic, Aleksandra I Konic-Ristic, Nevena Dj Kardum, Tamara B Popovic, Marija D Glibetic
BACKGROUND: Pomegranate juice is a rich source of polyphenols and is thus a promising dietary antioxidant with numerous health-promoting effects. These include a beneficial impact on cardiovascular health that could be partly attributed to the effects of polyphenols on lipid metabolism. The aim of this study was to investigate whether consumption of pomegranate juice for 6 weeks could modify lipid peroxidation and phospholipid fatty acid composition of plasma and erythrocytes in subjects with metabolic syndrome...
August 1, 2016: Journal of the Science of Food and Agriculture
R Weiss, A Bitton, L Nahary, M T Arango, I Benhar, M Blank, Y Shoenfeld, J Chapman
Antiphospholipid syndrome (APS) affects coagulation and the brain by autoimmune mechanisms. The major antigen in APS is beta-2-glycoprotein I (β2-GPI) is known to complex with annexin A2 (ANXA2), and antibodies to ANXA2 have been described in APS. We measured these antibodies in mice with experimental APS (eAPS) induced by immunization with β2-GPI. Sera of these mice reacted significantly with recombinant ANXA2 by enzyme-linked immunosorbent assay (ELISA) and the eAPS mice had significantly high levels of immunoglobulin G (IgG) in the brain by immunoblot assays compared to adjuvant immunized controls...
July 23, 2016: Immunologic Research
N Ruhrman-Shahar, J Torres-Ruiz, P Rotman-Pikielny, Y Levy
Autoimmune reaction after vaccination is sporadically reported in the medical literature. Vaccinations are generally safe and have an important role in eradicating endemic diseases worldwide. Nevertheless, the question arises as to whether there is a possibility of post-vaccination autoimmune phenomena. The anti-tetanus vaccine is being used since 1924, and it is part of the recommended immunization schedules for children. There are few reports of autoimmune diseases, such as rheumatoid arthritis and anti-phospholipid syndrome after anti-tetanus vaccination...
July 19, 2016: Immunologic Research
Valery Bochkov, Andreas W Schoenenberger, Olga Oskolkova, Ursula Toth, Johannes Stöckl, Otto Majdic, Armond Daci, Thérèse J Resink, Paul Erne, Maria Philippova
AIM: Oxidized phospholipids (OxPL) are the major pathogenic component of oxidized low-density lipoproteins (OxLDL). Endogenous anti-OxPL activity, defined as the ability to neutralize adverse effects of oxidized lipids, may have biomarker potential. METHODS & RESULTS: Using two anti-OxPL monoclonal antibodies (commercial mAB-E06 and custom mAB-509) we developed a novel ELISA that measures the global capacity of plasma to inactivate OxPL. Preincubation of OxLDL with plasma inhibits its binding of anti-OxPL mABs...
August 2016: Biomarkers in Medicine
Steven R Binder, Christine M Litwin
Antiphospholipid syndrome is characterized by the presence of antiphospholipid antibodies, specifically lupus anticoagulant, anticardiolipin antibodies, and anti-β2 glycoprotein-I antibodies. Antiphospholipid syndrome can occur on its own or in association with other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). A connection between cigarette smoking and anti-phospholipid antibodies (aPL) was first reported in the late1980s. Systemic lupus erythematosus patients with aPL are more likely to be smokers than those without aPL...
July 5, 2016: Clinical Reviews in Allergy & Immunology
Philipp Scholz, Markus Auler, Bent Brachvogel, Thomas Benzing, Peter Mallman, Thomas Streichert, Andreas R Klatt
Anti-phospholipid syndrome (APS) is one of the main causes for recurrent miscarriages. The diagnosis of APS is based on the occurrence of clinical symptoms such as thrombotic events or obstetric complications as well as the detection of antiphospholipid antibodies directed against β2-glycoprotein I and cardiolipin, or a positive lupus anticoagulant assay. However, there is a subpopulation of patients with clinical symptoms of APS, but the lack of serological markers (seronegative APS). In addition, a large proportion of patients with unexplained recurrent miscarriages exist...
2016: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
Gakuro Abe, Hirotoshi Kikuchi, Yoshiyuki Arinuma, Shunsei Hirohata
OBJECTIVE: The objective of this study is to explore the characteristics of brain MRI abnormalities in acute confusional state (ACS) in neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: Thirty-six patients with ACS admitted to our institutions from 1992 to 2015 were exhaustively enrolled. Their medical charts and brain MRI scans were reviewed. RESULTS: Eighteen of 36 ACS patients had MRI abnormalities, mostly high-intensity lesions of various sizes in the cerebral white matter...
June 20, 2016: Modern Rheumatology
Maha A Al-Mohaissen, Kwan-Leung Chan
Cardiovascular disease is an important extra-articular manifestation of rheumatologic diseases leading to considerable mortality and morbidity. Echocardiography emerges as a useful non-invasive technique for the screening and evaluation of cardiac involvement in these patients. With the technological advancement in echocardiographic techniques, we have gained a greater appreciation of the prevalence and nature of the cardiac involvement in these patients, as detection of subclinical disease is increasingly feasible...
August 2016: Current Cardiology Reports
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