MedWorm: Antiphospholipid Syndrome

Guidelines on the investigation and management of antiphospholipid syndrome

British Journal of Haematology — Wed, 08 Feb 2012 05:00:00 +0100

(Source: British Journal of Haematology)

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Azathioprine withdrawal: Antiphospholipid syndrome flare: case report

Reactions — Thu, 02 Feb 2012 18:34:27 +0100

(Source: Reactions)

Efficacy of the antiphospholipid score for the diagnosis of antiphospholipid syndrome and its predictive value for thrombotic events

Arthritis and Rheumatism — Mon, 30 Jan 2012 13:25:17 +0100

ConclusionThe aPL‐S is a useful quantitative index for diagnosing APS and may be a predictive marker for thrombosis in autoimmune diseases. (Source: Arthritis and Rheumatism)

Beyond the “syndrome”: Antiphospholipid antibodies as risk factors

Arthritis and Rheumatism — Mon, 30 Jan 2012 13:24:49 +0100

(Source: Arthritis and Rheumatism)

Thrombin inhibition profiles in healthy individuals and thrombophilic patients.

Thrombosis and Haemostasis — Wed, 25 Jan 2012 05:00:00 +0100

Authors: Rühl H, Müller J, Harbrecht U, Fimmers R, Oldenburg J, Mayer G, Pötzsch B Abstract Inhibition of thrombin by endogenous inhibitors plays a central role in the spatiotemporal control of clot formation. A failure to adequately inactivate thrombin such as in antithrombin deficiency generates a strong prothrombotic phenotype. To study if and to what extent delayed thrombin inactivation rates beyond antithrombin deficiency contribute to the prothrombotic phenotype we measured thrombin inhibition profiles in plasma samples obtained from 16 healthy individuals and 39 thrombophilic patients, including 17 patients diagnosed positive for anti-prothrombin/phospholipid antibodies. To test thrombin inhibition, thrombin was added to plasma, and endogenous thrombin inhibition stopped ...

Lupus anticoagulant, but not anticardiolipin antibody, predicts adverse pregnancy outcome in patients with antiphospholipid antibodies

Arthritis and Rheumatism — Tue, 24 Jan 2012 05:00:00 +0100

CONCLUSIONS:LAC is the primary predictor of APO after 12 weeks gestation in aPL‐associated pregnancies. ACL and anti‐β2‐GP‐I, if LAC is not also present, do not predict APO. (Source: Arthritis and Rheumatism)

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Predicting the risk of venous thromboembolism recurrence

American Journal of Hematology — Fri, 20 Jan 2012 05:00:00 +0100

AbstractVenous thromboembolism (VTE) is a chronic disease with a 30% ten‐year recurrence rate. The highest incidence of recurrence is in the first 6 months. Active cancer significantly increases the hazard of early recurrence, and the proportions of time on standard heparin with an APTT≥0.2 anti‐Xa U/mL, and on warfarin with an INR≥2.0, significantly reduce the hazard. The acute treatment duration does not affect recurrence risk after treatment is stopped. Independent predictors of late recurrence include increasing patient age and body mass index, leg paresis, active cancer and other persistent VTE risk factors, idiopathic VTE, antiphospholipid antibody syndrome, antithrombin, protein C or protein S deficiency, hyperhomocysteinemia and a persistently increased plasma fibrin D‐di...

A novel pathway for human endothelial cell activation by antiphospholipid/anti-{beta}2 glycoprotein I antibodies

Blood — Thu, 19 Jan 2012 05:00:00 +0100

Antiphospholipid Abs (APLAs) are associated with thrombosis and recurrent fetal loss. These Abs are primarily directed against phospholipid-binding proteins, particularly β2GPI, and activate endothelial cells (ECs) in a β2GPI-dependent manner after binding of β2GPI to EC annexin A2. Because annexin A2 is not a transmembrane protein, the mechanisms of APLA/anti-β2GPI Ab–mediated EC activation are uncertain, although a role for a TLR4/myeloid differentiation factor 88–dependent pathway leading to activation of NF-B has been proposed. In the present study, we confirm a critical role for TLR4 in anti-β2GPI Ab–mediated EC activation and demonstrate that signaling through TLR4 is mediated through the assembly of a multiprotein signaling complex on the E...

To cardiovascular disease and beyond: new therapeutic perspectives of statins in autoimmune diseases and cancer.

Current Drug Targets — Tue, 17 Jan 2012 05:00:00 +0100

Authors: Lopez-Pedrera C, Ruiz-Limón P, Valverde-Estepa A, Barbarroja N, Rodriguez-Ariza A Abstract Statins have been successfully used in patients with hypercholesterolemia and cardiovascular diseases, but there is increasing evidence that they exert effects by much exceeding the lowering of cholesterol levels. Statins have antiatherosclerotic, antiinflammatory, antioxidant, immunomodulatory and antithrombotic effects. These "pleiotropic" effects stem from their inhibition of prenylation of the small GTP-binding proteins Ras and Rho, and to the disruption, or depletion, of cholesterol rich membrane micro-domains (membrane rafts). Through these pathways statins modulate immune responses by altering cytokine levels and by affecting the function of cells involved in both innate and ...

Hormone replacement and contraceptive therapy in autoimmune diseases.

Journal of Autoimmunity — Tue, 17 Jan 2012 05:00:00 +0100

Authors: Lateef A, Petri M Abstract Sex hormones, including estrogens, influence the immune system in a complex manner, playing an important role in the pathophysiology of autoimmune diseases. Estrogen receptors can be found in almost all cells, including those of the adaptive and innate immune systems. Depending on the relative preponderance and stimulation of different receptors in various tissues, estrogens may demonstrate pro-inflammatory or anti-inflammatory properties. Traditionally, exogenous estrogens were considered to have the potential of worsening the autoimmune processes and hence were avoided in patients with rheumatic diseases. Recent studies have demonstrated the efficacy of exogenous hormone therapies, either in the form of oral contraceptives or post-menopausal ho...

Long-term membranous glomerulonephritis as the presenting manifestation of systemic lupus erythematosus in a patient with human immunodeficiency virus infection.

Lupus — Mon, 16 Jan 2012 05:00:00 +0100

We present the case of a 45-year-old Hispanic woman with long-standing HIV infection who developed membranous glomerulonephritis with histological features of lupus nephritis. Five years after onset of renal disease she developed clinically evident SLE. PMID: 22249649 [PubMed - as supplied by publisher] (Source: Lupus)

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B-cell directed therapies in antiphospholipid antibody syndrome - New directions based on murine and human data.

Autoimmunity Reviews — Mon, 16 Jan 2012 05:00:00 +0100

Authors: Khattri S, Zandman-Goddard G, Peeva E Abstract The increased awareness of the role of humoral immunophysiology in antiphospholipid syndrome (APS) has aroused interest in B cells as therapeutic targets in this disease. This paper reviews the literature on B cell directed therapies in human and experimental APS. The clinical data is limited to B cell depletion with rituximab and comprises case reports and case series. Murine studies include use of modulators of B cell function such as belimumab and abatacept. In both human and murine studies, B cell directed therapies appeared to have clinical and serologic beneficial effects including a decrease in the antiphospholipid antibody titers after treatment. Randomized controlled clinical trials are needed to determine whether B c...

Adjuvant immunization induces high levels of pathogenic antiphospholipid antibodies in genetically prone mice: another facet of the ASIA syndrome.

Lupus — Sun, 15 Jan 2012 14:30:03 +0100

Authors: Katzav A, Kivity S, Blank M, Shoenfeld Y, Chapman J Abstract Adjuvants may induce autoimmune diseases in susceptible individuals, a phenomenon recently defined as autoimmune/inflammatory syndrome induced by adjuvants (ASIA). Patients with both antiphospholipid antibodies (aPL) and the genetic coagulopathy factor V Leiden (FVL) are frequently found. We therefore evaluated whether adjuvant can induce aPL in heterozygous FVL mice. aPL were measured in naïve mice and at 1 and 5 months after immunization with either complete or incomplete Freund's adjuvant (CFA, IFA) in FVL and control C57/B6 background mice. We defined antibody levels 3 SD above the mean of C57/B6 mice immunized with adjuvant as positive (specificity of 99%). For β(2)GPI-dependent aPL, 28.6% (6/21) of FVL mi...

Vaccine model of antiphospholipid syndrome induced by tetanus vaccine.

Lupus — Sun, 15 Jan 2012 14:30:03 +0100

Authors: Dimitrijevic L, Zivkovic I, Stojanovic M, Petrusic V, Zivancevic-Simonovic S Abstract Successful induction of antiphospholipid syndrome (APS) in two different non-autoimmune prone mouse strains, BALB/c and C57BL/6, was achieved by tetanus toxoid (TTd) hyperimmunization using different adjuvants (glycerol or aluminium hydroxide), and different adjuvant pretreatments (glycerol or Complete Freund's Adjuvant (CFA)). APS had different manifestations of reproductive pathology in BALB/c and C57BL/6 mice: fetal resorption (as a consequence of extreme T-cell activation obtained in the course of pretreatment), and lowering of fecundity (as a consequence of polyclonal B-cell stimulation), respectively. In BALB/c mice fetal resorption coincided with glycerol and CFA pretreatments, whi...

Influenza vaccination can induce new-onset anticardiolipins but not β2-glycoprotein-I antibodies among patients with systemic lupus erythematosus.

Lupus — Sun, 15 Jan 2012 14:30:03 +0100

Conclusions: This study shows transient increases in aCL, but not anti-β2GPI responses, after influenza vaccination. PMID: 22235049 [PubMed - as supplied by publisher] (Source: Lupus)

Morbidity, Mortality, and Organ Damage in Patients with Antiphospholipid Syndrome.

J Rheumatol — Sun, 15 Jan 2012 05:00:00 +0100

CONCLUSION: APS is a disease of young individuals, who experience increased morbidity. Neurologic damage is the most common cause of morbidity. AT at presentation as well as coexistent SLE are associated with poor outcome. PMID: 22247356 [PubMed - as supplied by publisher] (Source: J Rheumatol)

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Mesothelial/monocytic incidental cardiac excrescence in a patient with antiphospholipid syndrome

Interactive CardioVascular and Thoracic Surgery — Thu, 12 Jan 2012 05:00:00 +0100

We describe an additional case of MICE in a 24-year-old female with antiphospholipid syndrome. A mobile hyperechogenic mass attached to the left ventricular surface of the aortic valve was documented by transthoracic echocardiography (TTE). The patient did have cardiac catheterization one month before the cardiac surgery. Histopathologic and immunohistochemical examination showed a lesion composed of histiocytes and mesothelial cells together with fibrin and scattered inflammatory cells. To our knowledge, this is the first case of MICE detected in a patient with antiphospholipid syndrome. (Source: Interactive CardioVascular and Thoracic Surgery)

eComment: Cardiac mesothelial/monocytic incidental excrescence and antiphospholipid syndrome

Interactive CardioVascular and Thoracic Surgery — Thu, 12 Jan 2012 05:00:00 +0100

(Source: Interactive CardioVascular and Thoracic Surgery)

[Kidney allograft: A target for systemic disease.]

Presse Medicale — Thu, 12 Jan 2012 05:00:00 +0100

Authors: Canaud G, Legendre C Abstract Recurrence of disease after transplantation is frequent and represents the third cause of allograft loss. Recurrence of lupus nephritis after transplantation is rare. Kidney transplantation in patients with antiphospholipid syndrome or lupus anticoagulant is challenging due to the high risk of immediate post-transplant thrombosis and bleeding risk associated to the subsequent anticoagulation. Moreover, vascular changes associated to the presence of antiphospholipid antibodies negatively impact allograft rate survival. Recurrence of pauci immune glomerulonephritis or Goodpasture syndrome is exceptional. PMID: 22244721 [PubMed - as supplied by publisher] (Source: Presse Medicale)

Complement activation in patients with isolated antiphospholipid antibodies or primary antiphospholipid syndrome.

Thrombosis and Haemostasis — Wed, 11 Jan 2012 05:00:00 +0100

Authors: Breen KA, Seed P, Parmar K, Moore GW, Stuart-Smith SE, Hunt BJ Abstract The antiphospholipid syndrome (APS) is the association of thrombosis and recurrent pregnancy loss and/or pregnancy morbidity with persistent antiphospholipid antibodies (aPL). Increased complement activation has been implicated in the pathogenesis of APS in animal models. It was our objective to evaluate complement activation in patients with aPL or primary antiphospholipid syndrome (PAPS). We measured complement activation products, fragments Bb and C3a-desArg by ELISA in 186 aPL/PAPS patients and 30 healthy controls. All patients with aPL had significantly increased levels of complement activation products. Fragment Bb levels (mean, 95% CI); (thrombotic APS 0.54 units/ml, 0.31-0.83, obstetric APS 0.6...

Val247Leu beta2-glycoprotein-I allelic variant is associated with antiphospholipid syndrome: Systematic review and meta-analysis.

Autoimmunity Reviews — Sun, 08 Jan 2012 05:00:00 +0100

CONCLUSIONS: Val/Val genotype of β(2)-GPI gene is associated with a significant excess risk to suffer from APS and, among patients with APS, to have anti-β(2)-GPI antibodies. No definite conclusions can be made regarding the association of this polymorphism with thrombosis among APS patients. PMID: 22246055 [PubMed - as supplied by publisher] (Source: Autoimmunity Reviews)

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Resolutive pulmonary endarterectomy in a non-compliant patient with systemic lupus erythematosus and antiphospholipid syndrome

Rheumatology International — Thu, 05 Jan 2012 17:08:10 +0100

We report a case and review the literature of successful PEA for CTEPH due to antiphospholipid syndrome associated with systemic lupus erythematosus. The definitive and decisive approach needed to treat this high-risk patient with a history of comorbidity, long-term illness and poor compliance was found with a therapy of PEA. Content Type Journal ArticleCategory Short CommunicationPages 1-5DOI 10.1007/s00296-011-2346-4Authors Anna Kuzenko, Allergologia ed Immunologia Clinica, Ospedale Umberto I, Ordine Mauriziano, Turin, ItalySavino Sciascia, Allergologia ed Immunologia Clinica, Ospedale Umberto I, Ordine Mauriziano, Turin, ItalyErika Silvestro, Allergologia ed Immunologia Clinica, Ospedale Umberto I, Ordine Mauriziano, Turin, ItalyIuliana Badiu, Allergologia ed Immunologia Clini...

Evaluation of coagulation and fibrinolytic parameters in adult onset GH deficiency and the effects of GH replacement therapy: A placebo controlled study.

Growth Hormone and IGF Research — Thu, 05 Jan 2012 05:00:00 +0100

CONCLUSIONS: We observed protein S deficiency more frequent than seen in the general population and normalization of protein S activity and decreases, in other natural anticoagulants following GHRT. Further studies are required to understand the impact of these changes in cardiovascular morbidity and mortality in this patient population. PMID: 22226996 [PubMed - as supplied by publisher] (Source: Growth Hormone and IGF Research)

Cognitive impairment in antiphospholipid syndrome: evidence from animal models

Clinical Rheumatology — Wed, 04 Jan 2012 16:51:32 +0100

Abstract Although antiphospholipid syndrome (APS) is a multisystem prothrombotic condition, its inflammatory nature has been increasingly recognized in recent years. Stroke and transitory ischemic attacks are the neurological manifestations included in APS criteria, however many other neurological involvements have been attributed to antiphospholipid antibodies (aPL), such as seizures, transverse myelitis, and cognitive impairment. In this article we will review evidence from animal model that explain the role of aPL in cognition. Content Type Journal ArticleCategory Review ArticlePages 1-4DOI 10.1007/s10067-011-1922-zAuthors Simone Appenzeller, Rheumatology Unit, Department of Medicine, Faculty of Medical Science—State University of Campinas, Campinas, BrazilAline ...

International consensus guidelines on anticardiolipin and anti–β2‐glycoprotein I testing: Report from the 13th International Congress on Antiphospholipid Antibodies

Arthritis and Rheumatism — Sun, 01 Jan 2012 05:00:00 +0100

(Source: Arthritis and Rheumatism)

Low‐molecular‐weight heparin added to aspirin in the prevention of recurrent early‐onset pre‐eclampsia in women with inheritable thrombophilia: the FRUIT‐RCT

Journal of Thrombosis and Haemostasis — Sun, 01 Jan 2012 05:00:00 +0100

Conclusions: Adding LMWH to aspirin at < 12 weeks gestation reduces recurrent HD onset < 34 weeks gestation in women with inheritable thrombophilia and prior delivery for HD/SGA <34 weeks. However, close monitoring of the mother and fetus remains important throughout pregnancy. (Source: Journal of Thrombosis and Haemostasis)

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Catastrophic antiphospholipid syndrome presenting as fever of unknown origin.

Saudi Journal of Kidney Diseases and Transplantation — Sun, 01 Jan 2012 05:00:00 +0100

We report a case of a man with catastrophic antiphospholipid syndrome (CAPS), which occurs when three or more organ systems are affected by thrombosis in less than a week. Catastrophic antiphospholipid syndrome is uncommon but often fatal. The patient received a successful treatment that controlled this disease and included intravenous heparin, antiplatelet, intravenous corticosteroid, and plasmapheresis. PMID: 22237230 [PubMed - in process] (Source: Saudi Journal of Kidney Diseases and Transplantation)

Post-translational oxidative modification of β2-glycoprotein I and its role in the pathophysiology of the antiphospholipid syndrome.

Autoimmunity Reviews — Sat, 31 Dec 2011 05:00:00 +0100

Authors: Mirarabshahi P, Abdelatti M, Krilis S Abstract Vascular thrombosis and/or recurrent miscarriages are the main characteristics defining Antiphospholipid Syndrome (APS). Currently there is no well-defined clinical features and/or laboratory tests that predicts the risk of adverse prognostic outcomes in APS. In this short review, we report the importance of posttranslational modification of beta2 glycoprotein I, the major autoantigen in the APS beta2 glycoprotein I that may, in part, explain possible mechanisms for the generation of auto antibodies to beta2 glycoprotein I. A specific ELISA measuring the level of oxidised beta2 glycoprotein I could be used as a potential new laboratory test - along with other laboratory tests - to more accurately predict the risk of having a c...

Thrombotic microangiopathy due to multiple autoantibodies related to antiphospholipid syndrome

Pediatric Nephrology — Fri, 30 Dec 2011 16:42:08 +0100

Conclusions Childhood TMA due to APS has rarely been reported. To the best of our knowledge this is the first report of pediatric TMA due to APS with positive aPS/PT. Physicians need to be aware of aPS/PT in pediatric APS and/or SLE. Content Type Journal ArticleCategory Brief ReportPages 1-5DOI 10.1007/s00467-011-2085-5Authors Shunsuke Noda, Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535 JapanMasao Ogura, Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535 JapanAkiko Tsutsumi, Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 1...

Ovarian stimulation and embryo banking for fertility preservation in a woman with severe mixed connective tissue disease: is it safe?

Journal of Assisted Reproduction and Genetics — Thu, 29 Dec 2011 06:43:47 +0100

Conclusion(s) Controlled ovarian hyperstimulation for embryo banking in women with MCTD, PH and APS may pose a risk for potentially catastrophic complications. A multidisciplinary approach to these patients is necessary to optimize the outcomes of such procedures. More data are needed regarding the safety of fertility preservation technologies in patients with complex medical diseases. Content Type Journal ArticleCategory Fertility PreservationPages 1-5DOI 10.1007/s10815-011-9697-3Authors Vasileios D. Sioulas, Division of Reproductive Endocrinology and Infertility, University of Pennsylvania Medical Center, 3701 Market Street, Philadelphia, PA 19104, USAClarisa R. Gracia, Division of Reproductive Endocrinology and Infertility, University of Pennsylvania Medical Cent...

Clinical manifestations of antiphospholipid syndrome (APS) with and without antiphospholipid antibodies (the so-called 'seronegative APS')

Annals of the Rheumatic Diseases — Wed, 28 Dec 2011 05:00:00 +0100

Conclusions Classic and SN-APS patients show similar clinical profiles. The results suggest that clinical management in patients with APS should not be based only on the presence of conventional aPL. (Source: Annals of the Rheumatic Diseases)

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Heparin in Patients With Antiphospholipid Antibody SyndromeHeparin in Patients With Antiphospholipid Antibody Syndrome

Medscape Today Headlines — Tue, 27 Dec 2011 04:00:00 +0100

Can the aPTT be used effectively to monitor heparin in patients with antiphospholipid syndrome? Pharmacotherapy (Source: Medscape Today Headlines)

Pregnancy implications for systemic lupus erythematosus and the antiphospholipid syndrome.

Journal of Autoimmunity — Mon, 26 Dec 2011 05:00:00 +0100

Authors: Andreoli L, Fredi M, Nalli C, Reggia R, Lojacono A, Motta M, Tincani A Abstract Multidisciplinary approach and patient counselling have been the key points in the improvement of the management of pregnancy in women with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Most of these women can have successful pregnancy when thoroughly informed and instructed on several different issues. Disease activity should be in stable remission prior to pregnancy in order to reduce the chance for flare during pregnancy. To this purpose, medications must be modulated: "safe" drugs should be continued throughout pregnancy, embryotoxic/foetotoxic drugs should be withdrawn timely, and beneficial drugs such as low dose aspirin and heparin should be added for prophylaxi...

Thrombosis and Occlusion of Vascular Access in Hemodialyzed Patients

Seminars in Thrombosis and Hemostasis — Fri, 23 Dec 2011 05:00:00 +0100

Semin Thromb Hemost 2011; 37: 946-954DOI: 10.1055/s-0031-1297373ABSTRACTPatients undergoing chronic hemodialysis have a high risk of arterial thrombotic events as well as vascular access thrombosis (VAT). The latter complication has been consistently associated with inherited (i.e., the prothrombin 20210 polymorphism, and polymorphisms in the genes encoding for transforming growth factor-β1, nitric oxide synthase, plasminogen activator inhibitor-1, angiotensin converting enzyme, and methylene tetrahydrofolate reductase), and acquired thrombotic risk factors (i.e., diabetes, obesity, atrial fibrillation, hypertension, hyperhomocysteinemia, hyperlipoproteinemia(a), low serum albumin, antiphospholipid antibodies, autoantibodies against protein C and S, erythropoietin administration, malnutri...

Reevaluation of predictive value of ACL and anti-β2GP1 antibody for thrombosis in patients with systemic lupus erythematosus: from a perspective of a practical world

Rheumatology International — Thu, 22 Dec 2011 12:47:33 +0100

Abstract Detection of ACL (anticardiolipin, ACL) and anti-β2GP1 (beta2 glycoprotein1, β2GP1) antibody has been widely used, and the criteria of APS (Antiphospholipid syndrome, APS) have been used for the prediction of thrombosis in patients with SLE. What is the exact predictive value of these two antibodies? Is it really necessary to apply the criteria of APS to each patient just for the purpose of prediction of thrombosis? The aim of this retrospective study is to reevaluate the predictive value of different combination of ACL and anti-β2GP1 antibody for thrombosis formation in Chinese patients with SLE. Patients fulfilling the 1997 ACR classification criteria for SLE were enrolled and retrospectively analyzed. Thrombosis was confirmed by ultrasound, cerebral MRI, com...

Dietetic issues in antiphospholipid syndrome

Rheumatology International — Thu, 22 Dec 2011 12:47:28 +0100

This article focuses on main factors such as vitamin K ingestion and its relationship with anticoagulants and healthy diet for obesity, dyslipidemia, hypertension, diabetes mellitus that are important risk factors for hypercoagulability. Last, diet rich in calcium is also important since these patients are under anticoagulants and heparins that are related to osteoporosis/osteopenia. The medical follow-up in combination with nutrition counseling was carried out for guiding patients to acquire healthy habits in order to improve their quality of life and gain control of the comorbidities. Content Type Journal ArticleCategory Letter to the EditorsPages 1-2DOI 10.1007/s00296-011-2313-0Authors Karin Klack, Nutrition Division, Hospital das Clínicas da Faculdade de Medicina da Univer...

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Clinical and laboratory characteristics of children positive for antiphospholipid antibodies.

Blood Transfusion — Thu, 22 Dec 2011 05:00:00 +0100

DISCUSSION: Our study shows that aPL-positive children have different features that should be taken into account in the classification of criteria for paediatric APS. PMID: 22244004 [PubMed - as supplied by publisher] (Source: Blood Transfusion)

Familial antiphospholipid syndrome presenting as bivessel arterial occlusion in a 17-year-old girl

Rheumatology International — Wed, 21 Dec 2011 20:05:38 +0100

This article presents a case of a 17-year-old girl with primary antiphospholipid syndrome developing subacute signs of hand and leg ischaemia caused by radiologically verified radial and popliteal artery occlusion. She is successfully treated with a thrombolytic agent (alteplase) and recovers completely. Her laboratory results came positive for all three subtypes of antiphospholipid antibodies. This kind of antiphospholipid syndrome presentation is a very rare entity in itself. Shortly afterwards her mother is diagnosed with primary antiphospholipid syndrome as well. A familial form of antiphospholipid syndrome is suspected. Combination of a familial antiphospholipid syndrome presenting as bivessel arterial thrombosis is a unique case, to the best of our knowledge, never described in...

Corrigendum to avidity of anti-β2-glycoprotein I antibodies in patients with or without antiphospholipid syndrome: a collaborative study in the frame of the European forum on antiphospholipid antibodies.

Lupus — Wed, 21 Dec 2011 11:48:06 +0100

Authors: PMID: 22127807 [PubMed - in process] (Source: Lupus)

The Michael Mason prize: Pathogenic antiphospholipid antibodies, stressed out antigens and the deployment of decoys

Rheumatology — Wed, 21 Dec 2011 05:00:00 +0100

The antiphospholipid syndrome is a common autoimmune cause of vascular thrombosis and recurrent miscarriages. aPLs that target the N-terminal domain [Domain I (DI)] of the phospholipid binding protein ß2-glycoprotein I (ß2GPI) represent the key sub-population of aPLs that promote thrombosis. This review describes two research arms relating to the study of this autoantigen. The first arm describes recent novel biochemical and functional insights into the molecular structure of ß2GPI in vivo and how this may be altered in APS. These findings support the emerging hypothesis that redox modification of ß2GPI may be relevant to the pathogenesis of APS and the development of pathogenic anti-ß2GPI antibodies. The second arm describes how a recombinant DI peptide engin...

Comments on “Essential Thrombocytosis and Antiphospholipid Antibody Syndrome Causing Chronic Budd-Chiari Syndrome”

Indian Journal of Pediatrics — Tue, 20 Dec 2011 06:47:33 +0100

Content Type Journal ArticleCategory CorrespondencePages 1-2DOI 10.1007/s12098-011-0627-2Authors Thein H. Oo, Department of Medicine, Section of Thrombosis and Benign Hematology, The University of Texas M. D. Anderson Cancer Center, 1400 Pressler Street, Unit 1464, Houston, TX 77030, USA Journal Indian Journal of PediatricsOnline ISSN 0973-7693Print ISSN 0019-5456 (Source: Indian Journal of Pediatrics)

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Impact of Persistent Antiphospholipid Antibodies on Risk of Incident Symptomatic Thromboembolism in Children: A Systematic Review and Meta-Analysis

Seminars in Thrombosis and Hemostasis — Tue, 20 Dec 2011 05:00:00 +0100

Semin Thromb Hemost 2011; 37: 802-809DOI: 10.1055/s-0031-1297171ABSTRACTThe aim of this study was to estimate the impact of antiphospholipid (aPL) antibodies on the risk of incident thromboembolism (TE; arterial and venous) in children via meta-analysis of published observational studies. A systematic search of electronic databases (Medline, EMBASE, OVID, Web of Science, The Cochrane Library) for studies published from 1966 to 2010 was conducted using keywords in combination both as MeSH terms and text words. Two authors independently screened citations and those meeting the a priori defined inclusion criteria were retained. Data on year of publication, study design, country of origin, number of patients/controls, ethnicity, TE type, and frequency of recurrence were abstracted. Heterogenei...

Mitral Valve Libman–Sacks Endocarditis Visualized by Real Time Three‐Dimensional Transesophageal Echocardiography

Echocardiography — Fri, 16 Dec 2011 05:00:00 +0100

In this report, mitral valve LSE was visualized by real time 3D transesophageal echocardiography (TEE). 3D TEE provides a unique en face view of the mitral valve akin to a surgical or autopsy view that allows for an accurate determination of the size, shape, and location of the vegetations. (Echocardiography ****;**:E1‐E2) (Source: Echocardiography)

[Fine specificity of anti-β2glycoprotein I antibodies in systemic autoimmune diseases is mostly directed against domain 1].

Reumatismo — Thu, 15 Dec 2011 06:36:02 +0100

CONCLUSIONS: IgG anti-β2 GPI against D1 seem to cluster in patients with systemic autoimmune conditions. Their pathogenic potential in determine APS manifestations may be mitigated by adequate prophylaxis. PMID: 21776445 [PubMed - indexed for MEDLINE] (Source: Reumatismo)

Prevalence of Annexin A5 Resistance in Children and Adolescents with Rheumatic Diseases.

J Rheumatol — Thu, 15 Dec 2011 05:00:00 +0100

CONCLUSION: Children and adolescents with rheumatic diseases and persistent aPL have reduced annexin A5 anticoagulant activity, whereas transient, nonpathogenic aPL have less effect on annexin A5 activity. PMID: 22174207 [PubMed - as supplied by publisher] (Source: J Rheumatol)

Prevalence and Risk Factors for Liver Biochemical Abnormalities in Canadian Patients with Systemic Lupus Erythematosus.

J Rheumatol — Thu, 15 Dec 2011 05:00:00 +0100

CONCLUSION: Metabolic abnormalities such as obesity and hypertension and hepatotoxic effects of medication used to treat SLE may contribute more than SLE-related factors to liver biochemical abnormalities in patients with SLE. PMID: 22174205 [PubMed - as supplied by publisher] (Source: J Rheumatol)

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Role of the Vitamin D in Leprosy.

The American Journal of the Medical Sciences — Tue, 13 Dec 2011 05:00:00 +0100

Authors: Luʼoʼng KV, Nguyê Combining Tilde N LT Abstract There is an evidence of abnormal metabolism in the vitamin D endocrine system of patients with leprosy. Bone deformities usually occur in patients with leprosy. Genetic factors, such as the vitamin D receptor, the major histocompatibility complex region, chromosome 20, human toll-like receptors, the natural resistance-associated macrophage protein 1, the nucleotide-binding oligomerization domain containing 2, phosphate-regulating gene with homologies to endopeptidase on the X chromosome and the tyrosine kinase growth factor receptor-ErbB-2, contribute to both vitamin D status and leprosy. The role of vitamin D in leprosy has been demonstrated by its effects on Bacillus Calmette-Guérin vaccination, vascular endothelial gro...

Propylthiouracil-Induced Lupus, Antiphospholipid Syndrome, and Stroke in a Patient With Graves Hyperthyroidism [Observation]

Archives of Neurology — Mon, 12 Dec 2011 05:00:00 +0100

Conclusion In this young man with Graves hyperthyroidism, treatment with propylthiouracil was associated with transient autoimmune reactions suggestive of drug-induced lupus, antiphospholipid syndrome, and acute ischemic stroke. (Source: Archives of Neurology)

Fetal outcome in autoimmune diseases.

Autoimmunity Reviews — Sun, 11 Dec 2011 05:00:00 +0100

Authors: Carvalheiras G, Faria R, Braga J, Vasconcelos C Abstract The impact on fetal outcome in women with autoimmune diseases is a result of a several conditions. Fetal success depends on early immunological changes in the mother, which rely in modifications of the innate and adaptative immune system, inducing tolerance to the semi-allogenic fetus. Others crucial factors are maternal disease activity, severity of organ damage, circulating antibodies, and drug treatment. Although fetal outcome is becoming better still it has a worse prognosis in comparison with healthy women. Diseases like antiphospholipid syndrome, systemic lupus erythematosus and vasculitis have the higher risk while rheumatoid arthritis and spondiloarthopaties the least. In the majority of the diseases the risk...

Drug-Induced Lupus Anticoagulants and Antiphospholipid Antibodies

Current Rheumatology Reports — Fri, 09 Dec 2011 17:07:06 +0100

Abstract Lupus anticoagulants (LA) are immunoglobulins (IgG, IgM, and/or IgA) which interfere with one or more of phospholipid-dependent in vitro coagulation tests, eg, activated partial thromboplastin time (aPTT), kaolin clotting time (KCT), dilute Russell viper venom time (dRVVT), and dilute prothrombin time (dPT). LAs may be seen in a variety of clinical settings including the primary antiphospholipid syndrome (APS), systemic lupus erythematosus (SLE), other autoimmune diseases, secondary to infections, malignancies, and in association with certain drugs. LAs associated with the antiphospholipid syndrome and other autoimmune disease recognize certain phospholipid-binding proteins (β2-glycoprotein I [β2GPI] or prothrombin). Many drugs have been implicated as possibly ca...

Obstetric Antiphospholipid Syndrome: An Update on Pathophysiology and Management

Seminars in Reproductive Medicine — Thu, 08 Dec 2011 05:00:00 +0100

Semin Reprod Med 2011; 29: 522-539DOI: 10.1055/s-0031-1293206ABSTRACTAntiphospholipid antibodies (aPLs) are acquired antibodies directed against negatively charged phospholipids, a group of inner and outer cell membrane antigens found in mammals. Obstetric antiphospholipid antibody syndrome (APS) is diagnosed in the presence of certain clinical features in conjunction with positive laboratory findings. Although obstetric APS was originally reported in association with slow progressive thrombosis and infarction in the placenta, it is most often associated with a poor obstetric outcome. In fact, obstetric APS is one of the most commonly identified causes of recurrent pregnancy loss (RPL). Thus obstetric APS is distinguished from APS in other organ systems where the most common manifestation ...

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The association of systemic lupus erythematosus and myasthenia gravis: a series of 17 cases, with a special focus on hydroxychloroquine use and a review of the literature

Journal of Neurology — Wed, 07 Dec 2011 17:33:23 +0100

In conclusion, the clinical pattern of patients with SLE and MG seems to be characterized by a less severe course of SLE and higher frequency of antiphospholipid antibodies. Hydroxychloroquine treatment appears to be safe in this setting. Content Type Journal ArticleCategory Original CommunicationPages 1-8DOI 10.1007/s00415-011-6335-zAuthors M. Jallouli, Service de Médecine Interne, Centre de référence national pour le Lupus Systémique et le syndrome des Antiphospholipides, Centre Hospitalier Universitaire Pitié-Salpêtrière, Université Paris 6, AP-HP, 47-83 Boulevard de l’Hôpital, 75651 Paris Cedex 13, FranceD. Saadoun, Service de Médecine Interne, Centre de référence national pour le Lupus Systémique et le syndrome des Antiphospholipides, Centre Hospitalier Univers...

Mesothelial/monocytic incidental cardiac excrescence in a patient with antiphospholipid syndrome [Case report - Cardiac general]

Interactive CardioVascular and Thoracic Surgery — Mon, 05 Dec 2011 05:00:00 +0100

eComment: Cardiac mesothelial/monocytic incidental excrescence and antiphospholipid syndrome [eComment]

Interactive CardioVascular and Thoracic Surgery — Mon, 05 Dec 2011 05:00:00 +0100

(Source: Interactive CardioVascular and Thoracic Surgery)

Cognitive Dysfunction and Antiphospholipid Antibodies

Current Rheumatology Reports — Thu, 01 Dec 2011 19:29:36 +0100

Abstract Antiphospholipid syndrome (APS) is a multisystem prothrombotic condition, however, in recent years, its inflammatory nature has been studied extensively. Cerebral involvement is commonly observed in APS and results in different clinical manifestations. However, most of the studies include secondary APS. In this article, we review the prevalence, clinical characteristics, and physiopathology of cognitive impairment in patients with primary APS. Content Type Journal ArticleCategory ANTIPHOSPHOLIPID SYNDROME (RAS ROUBEY, SECTION EDITOR)Pages 1-4DOI 10.1007/s11926-011-0224-4Authors Simone Appenzeller, Department of Medicine, Faculty of Medical Science, State University of Campinas, Campinas, BrazilAline Tamires Lapa, Postgraduate Program in Child and Adolescent He...

Laboratory Diagnosis of the Lupus Anticoagulant

Current Rheumatology Reports — Thu, 01 Dec 2011 19:29:34 +0100

This article reviews the guidelines developed by the Lupus Anticoagulant/Phospholipid Dependent Antibodies Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis and addresses several common issues encountered during testing for these clinically relevant autoantibodies. Content Type Journal ArticleCategory ANTIPHOSPHOLIPID SYNDROME (RAS ROUBEY, SECTION EDITOR)Pages 1-7DOI 10.1007/s11926-011-0225-3Authors Thomas L. Ortel, Division of Hematology, Duke University Medical Center, Room 0563, Stead Building, Box 3422 DUMC, Durham, NC 27710, USA Journal Current Rheumatology ReportsOnline ISSN 1534-6307Print ISSN 1523-3774 (Source: Current Rheumatology Reports)

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Immune responses against domain I of β2‐glycoprotein I are driven by conformational changes: Domain I of β2‐glycoprotein I harbors a cryptic immunogenic epitope

Arthritis and Rheumatism — Thu, 01 Dec 2011 05:00:00 +0100

ConclusionThe results of our studies indicate that the exposure of cryptic epitopes due to conformational changes in β2GPI can induce autoantibody formation. (Source: Arthritis and Rheumatism)

Purpura, cutaneous necrosis, and antineutrophil cytoplasmic antibodies associated with levamisole‐adulterated cocaine

Arthritis and Rheumatism — Thu, 01 Dec 2011 05:00:00 +0100

ConclusionRheumatologists should be aware of this distinctive form of necrotic purpura, its associated autoantibodies, and its link to levamisole‐adulterated cocaine. (Source: Arthritis and Rheumatism)

Induction of anti‐β2‐glycoprotein I autoantibodies in mice by protein H of Streptococcus pyogenes

Journal of Thrombosis and Haemostasis — Thu, 01 Dec 2011 05:00:00 +0100

Conclusions: Our study has demonstrated that a bacterial protein can induce a conformational change in β2‐GPI, resulting in the formation of antiβ2‐GPI autoantibodies. This constitutes a novel mechanism for the formation of anti‐β2‐GPI autoantibodies. (Source: Journal of Thrombosis and Haemostasis)

The use of sex hormones in women with rheumatological diseases.

Hong Kong Med J — Thu, 01 Dec 2011 05:00:00 +0100

This article reviews the up-to-date evidence regarding the safety of using these agents in women with various rheumatological diseases, with emphasis on systemic lupus erythematosus and rheumatoid arthritis. In the absence of antiphospholipid antibody or other prothrombotic risk factors, combined hormonal contraception is not contra-indicated in most rheumatological conditions including inactive systemic lupus erythematosus. Moreover, hormone therapy is generally not contra-indicated except for women with active systemic lupus erythematosus disease where its effect on disease flare is less clear and individual judgement is required. PMID: 22147320 [PubMed - in process] (Source: Hong Kong Med J)

Clinical implications of elevated antiphospholipid antibodies in adult patients with primary immune thrombocytopenia.

The Korean Journal of Internal Medicine — Thu, 01 Dec 2011 05:00:00 +0100

CONCLUSIONS: Our data suggest that aPL levels should be determined at the initial presentation of ITP and that patients found to be aPL-positive should receive closer follow-up for thrombotic events. PMID: 22205846 [PubMed - in process] (Source: The Korean Journal of Internal Medicine)

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Risk of thromboembolic events after recurrent spontaneous abortion in antiphospholipid syndrome: a case-control study

Annals of the Rheumatic Diseases — Wed, 30 Nov 2011 05:00:00 +0100

Conclusions Our data indicate that a history of RSA associated with aPL is a risk factor for subsequent thrombosis in the long term. (Source: Annals of the Rheumatic Diseases)

Tolerogenic dendritic cells inhibit antiphospholipid syndrome derived effector/memory CD4+ T cell response to {beta}2GPI

Annals of the Rheumatic Diseases — Wed, 30 Nov 2011 05:00:00 +0100

Conclusions The inherent tolerance induction resistance of activated T cells present during the development of autoimmune diseases has delayed the application of tDC as an alternative therapy. This study highlights the 10/TGF-DC feasibility to induce antigen-specific unresponsiveness in autoreactive T cells generated in patients with APS by inducing apoptosis or T cells with regulatory abilities. (Source: Annals of the Rheumatic Diseases)

Renal transplantation in lupus nephritis: a Brazilian cohort.

Lupus — Wed, 30 Nov 2011 05:00:00 +0100

Conclusion: LN is the major cause of morbidity in SLE, with progression to ESRD in 10-22% of cases. Despite concerns about LN recurrence after renal transplantation, the data obtained in our sample indicate this procedure as a safe alternative therapy for ESRD in this population. PMID: 22129601 [PubMed - as supplied by publisher] (Source: Lupus)

Autoantibodies in patients with chronic hepatitis C virus infection: pitfalls for the diagnosis of rheumatic diseases.

Autoimmunity Reviews — Wed, 30 Nov 2011 05:00:00 +0100

Authors: Palazzi C, Buskila D, D'Angelo S, D'Amico E, Olivieri I Abstract Hepatitis C virus infection (HCV) is one of the best mimes in medicine. About 40-70% of patients suffering from this disorder develop at least one extra-hepatic disorder that can have a rheumatic nature (arthralgias, arthritis, vasculitis and sicca syndrome) and must be differentiated from the primitive rheumatic diseases. In addition, HCV infection can also alter the laboratory tests. Several alterations of first line laboratory tests can be usually found in both chronic HCV infection and chronic inflammatory rheumatic disorders. In the present review we analyze the interference of HCV in tests more specifically used in rheumatology: rheumatoid factor and other autoantibodies (ANA, anti-ENA, ANCA, anti-DNA, ...

A case of catastrophic antiphospholipid antibody syndrome complicated with systemic lupus erythematosus, double positive for anti-cardiolipin/β2 glycoprotein I and anti-phosphatidylserine/prothrombin autoantibodies

Modern Rheumatology — Mon, 28 Nov 2011 16:58:21 +0100

Abstract A 16-year-old male with severe thrombocytopenia and progressive multiple organ infarctions was diagnosed as having catastrophic antiphospholipid syndrome (CAPS) complicated with systemic lupus erythematosus, and was successfully treated with combination of anticoagulants, corticosteroids, plasma exchange, and intravenous cyclophosphamide. Antibodies to phosphatidylserine/prothrombin (PS/PT) complex and cardiolipin (CL)/β2-glycoprotein I (β2GPI) were simultaneously detected, indicating that the different pathways of both PS/PT and CL/β2GPI might be associated with the radical manifestation of CAPS. Content Type Journal ArticleCategory Case ReportPages 1-5DOI 10.1007/s10165-011-0563-zAuthors Eri Hirakawa, The First Department of Internal Medicine, School...

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Gangrene of the auricle as the first sign of antiphospholipid antibody syndrome

Revista Brasileira de Reumatologia — Fri, 25 Nov 2011 22:38:28 +0100

A síndrome do anticorpo antifosfolípide (SAF), mais comum em mulheres, manifesta-se clinicamente como trombose e/ou abortamentos de repetição. Anemia hemolítica autoimune e manifestações neurológicas, cardíacas e cutâneas são comuns. Relata-se o caso de um paciente do gênero masculino cuja manifestação inicial da doença foi gangrena em pavilhão auricular, e o diagnóstico de SAF se deu por meio de biópsia de pele do membro inferior, que mostrava vasculopatia trombótica, sem evidência de vasculite. Esse resultado é um dos dois critérios maiores que, associados a um critério menor, fecham o diagnóstico dessa doença. Discutem-se neste caso os possíveis diagnósticos diferenciais e como eles se diferenciam da doença em foco, além da importância que a biópsia teve n...

Opinion of some brazilian rheumatologists about biosimilars

Revista Brasileira de Reumatologia — Fri, 25 Nov 2011 22:38:28 +0100

Cholesterol and chloroquine

Revista Brasileira de Reumatologia — Fri, 25 Nov 2011 22:38:28 +0100

Intensive short-term treatment with rituximab, cyclophosphamide and methylprednisolone pulses induces remission in severe cases of SLE with nephritis and avoids further immunosuppressive maintenance therapy

Nephrology Dialysis Transplantation — Fri, 25 Nov 2011 05:00:00 +0100

Conclusions. Long-lasting remissions were obtained in patients with severe SLE and major organ involvement by this intensive administration of rituximab combined with low doses of intravenous cyclophosphamide and methylprednisolone pulses followed by a rapid tapering of prednisone to 5 mg/day as a sole maintenance therapy. (Source: Nephrology Dialysis Transplantation)

Thin layer chromatography immunostaining in detecting antiphospholipid antibodies in seronegative antiphospholipid syndrome

Clinical and Experimental Immunology — Thu, 24 Nov 2011 23:51:27 +0100

ABSTRACTIn clinical practice it is possible to find patients with clinical signs suggestive of antiphospholipid syndrome (APS) who are persistently negative for the routinely used antiphospholipid antibodies (aPL). Therefore, it was proposed for these cases the term of seronegative APS (SN‐APS).We investigated the clinical usefulness of thin layer chromatography (TLC) immunostaining in detecting serum aPL in patients presenting clinical features of SN‐APS.Sera from 36 patients with SN‐APS, 19 patients with APS, 18 patients with systemic lupus erythematosus (SLE), 20 anti‐HCV positive subjects and 32 healthy controls were examined for aPL using TLC immunostaining. Anti‐β2‐glycoprotein‐I, anti‐annexin II, anti‐annexin V and anti‐prothrombin antibodies were tested by enzy...

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Thirteen‐year‐old female with an unusual presentation of deep venous thrombosis

Journal of Paediatrics and Child Health — Wed, 23 Nov 2011 05:00:00 +0100

We report a case of a 13‐year‐old female presenting with deep venous thrombosis in her left lower limb. Investigations led by her symptoms and history revealed persistently positive lupus anticoagulant and anticardiolipin antibodies, indicating the diagnosis of antiphospholipid syndrome. Double‐stranded DNA antibodies were detected positive pointing to the coexistence of systemic lupus erythematosus (SLE). Antiphospholipid syndrome‐related vascular occlusive events are causing high morbidity and mortality. Therefore, a high index of suspicion is mandatory for the recognition of children being at risk for primary or recurrent thrombotic complications. (Source: Journal of Paediatrics and Child Health)

The Management of Stroke in Antiphospholipid Syndrome

Current Rheumatology Reports — Tue, 22 Nov 2011 17:58:30 +0100

Abstract Ischemic stroke is one of the most common complications of the antiphospholipid syndrome (APS). Because of the relative lack of definitive prospective studies, there is still some debate as to whether the persistent presence of antiphospholipid antibodies (aPLs) increases the risk of recurrent stroke. There is more evidence for aPLs as a risk factor for first stroke. The mechanisms of ischemic stroke are considered to be thrombotic and embolic. APS patients with thrombotic stroke frequently have other, often conventional vascular risk factors. Transesophageal echocardiogram is strongly recommended in APS patients with ischemic stroke because of the high yield of valvular abnormalities. The appropriate management of thrombosis in patients with APS is still controve...

Hughes Syndrome: The Antiphospholipid Syndrome(Hughes et al.)

Springer Medicine titles — Tue, 22 Nov 2011 01:13:27 +0100

A Guide for StudentsHughes Syndrome: The Antiphospholipid Syndrome, A Guide for Students provides an in-depth analysis into the main effects of Hughes Syndrome. In 1983, Dr Graham Hughes, and his team in London, described a syndrome and subsequently developed simple blood tests to diagnose the condition. This syndrome is characterised by thrombosis (both in limbs and internal organs), headaches, memory loss, ... (Source: Springer Medicine titles)

Management of Obstetric Antiphospholipid Syndrome

Current Rheumatology Reports — Mon, 21 Nov 2011 18:07:56 +0100

Abstract Recurrent early miscarriages (excluding chromosomal anomalies), late fetal loss, and maternal thrombosis are characteristic of obstetric antiphospholipid syndrome (APS). Obstetric complications such as preeclampsia, fetal growth restriction, premature delivery, and fetal death also occur in higher frequency in APS patients than in the general population. A high-risk obstetric center is needed for proper evaluation of and intervention with pregnant women with APS. Association with lupus carries additional risk of thrombosis when antiphospholipid antibodies (aPLs) are present. Gestational results with live births are improved to about 80% when antithrombotic therapy is used, but failure in 20% to 30% of the cases despite correct treatment with low-dose aspirin with ...

Potential Use of Statins in the Treatment of Antiphospholipid Syndrome

Current Rheumatology Reports — Mon, 21 Nov 2011 18:07:56 +0100

Abstract Antiphospholipid syndrome (APS) is a disorder characterized by the association of arterial or venous thrombosis and/or pregnancy morbidity with the presence of antiphospholipid antibodies (anticardiolipin antibodies, lupus anticoagulant antibodies, and/or anti–β2-glycoprotein I antibodies). Several studies have contributed to uncovering the basis of antiphospholipid antibody pathogenicity, including the targeted cellular components, affected systems, involved receptors, intracellular pathways used, and the effector molecules that are altered in the process. Therapy for thrombosis traditionally has been based on long-term oral anticoagulation; however, bleeding complications and recurrence despite high-intensity anticoagulation can occur. Based on all the data o...

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Case report: Cytomegalovirus‐induced thrombosis in an immunocompetent patient

Journal of Medical Virology — Fri, 18 Nov 2011 07:58:48 +0100

AbstractThrombotic manifestations of cytomegalovirus infection in immunocompetent individuals are rare. However, it has been postulated that cytomegalovirus infection can be both directly cytopathic and capable of inducing antiphospholipid antibodies due to shared “molecular mimicry” between cytomegalovirus virus antigens and antiphospholipid antibodies. The case of a previously well 30‐year‐old woman with primary cytomegalovirus infection complicated by splenic infarction and massive pulmonary embolus is described. The patient is unusual given the development of thromboses affecting both the arterial and venous circulation, associated with both transient anticardiolipin antibodies and persistently positive anti‐β2glycoprotein I antibodies. The temporal relationship between the ...

Capillaroscopy as a screening test for clinical antiphospholipid syndrome.

European Journal of Internal Medicine — Wed, 16 Nov 2011 17:56:01 +0100

Authors: Pyrpasopoulou A, Triantafyllou A, Anyfanti P, Douma S, Aslanidis S PMID: 22075311 [PubMed - in process] (Source: European Journal of Internal Medicine)

Striatal hypermetabolism in limbic encephalitis

Journal of Neurology — Sat, 12 Nov 2011 06:47:56 +0100

Abstract Striatal hypermetabolism on 18FDG-PET scan is a neuroradiological finding that has been described in association with autoimmune disorders such as Sydenham chorea, lupus or antiphospholipid syndrome. Here, we report three patients with non-paraneoplastic limbic encephalitis characterized by 18FDG-PET hypermetabolism of both striata, in contrast with diffuse hypometabolism in the rest of the brain. All patients developed subacute dementia, and antibodies to voltage-gated potassium channels were found in all cases. Brain metabolism and neurological status improved within a few months of immunosuppressive therapy. The finding of striatal hypermetabolism on 18FDG-PET images could thus be highly indicative of limbic encephalitis associated with anti-neuropil antibodies...

Antinuclear and antiphospholipid antibodies in patients with multiple sclerosis.

Lupus — Thu, 10 Nov 2011 05:00:00 +0100

This study investigated antiphospholipid and antinuclear antibodies in 85 patients with multiple sclerosis (MS) and clinically isolated syndrome (CIS) with regard to their association with demographic features, MS specific clinical features and symptoms of connective tissue diseases. Autoantibodies tested included antinuclear antibodies (ANA) with their specificities and anticardiolipin (aCL) and anti-beta-2-glycoprotein I (anti-β2GPI) antibodies.Antinuclear antibodies were more prevalent in MS patients than in controls (63.5% vs. 3.3%; p < 0.01) and in 19% of patients specific antinuclear antibodies were detected. Anti-β2GPI IgM antibodies were more frequent in MS patients than in the control group (20% vs. 3.3%; p < 0.05). The frequency of anticardiolipin antibodies did not diffe...

Moderate/severe erectile dysfunction in patients with antiphospholipid syndrome.

Lupus — Thu, 10 Nov 2011 05:00:00 +0100

Conclusion: To our knowledge, this was the first study that demonstrated moderate/severe ED in almost 50% of cases of a rare autoimmune disease. This alteration was linked to arterial events and longer disease duration. PMID: 22074844 [PubMed - as supplied by publisher] (Source: Lupus)

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Current state of evidence on "off label" therapeutic options for systemic lupus erythematosus, including biological immunosuppressive agents, in Germany, Austria, and Switzerland - a consensus report.

Lupus — Wed, 09 Nov 2011 05:00:00 +0100

Current state of evidence on "off label" therapeutic options for systemic lupus erythematosus, including biological immunosuppressive agents, in Germany, Austria, and Switzerland - a consensus report. Lupus. 2011 Nov 9; Authors: Aringer M, Burkhardt H, Burmester GR, Fischer-Betz R, Fleck M, Graninger W, Hiepe F, Jacobi AM, Kötter I, Lakomek HJ, Lorenz HM, Manger B, Schett G, Schmidt RE, Schneider M, Schulze-Koops H, Smolen JS, Specker C, Stoll T, Strangfeld A, Tony HP, Villiger PM, Voll R, Witte T, Dörner T Abstract Systemic lupus erythematosus (SLE) can be a severe and potentially life-threatening disease that often represents a therapeutic challenge because of its heterogeneous organ manifestations. Only glucocorticoids, chloroquine and hydroxychloroquine, azathioprine,...

[Aspirin versus anticoagulation in young patients with cerebral infarction secondary to primary antiphospholipid syndrome].

Revista de Neurologia — Tue, 08 Nov 2011 19:31:45 +0100

CONCLUSION. With the limitations of a nonrandomized study, our data suggest that the risk of recurrent arterial cerebral infarction in young patients with cerebral infarction secondary to PAS is low, probably non-uniform and independent of the type of antithrombotic. PMID: 22052173 [PubMed - in process] (Source: Revista de Neurologia)

Prevalence and clinical usefulness of the antiphospholipid and anti-cofactor antibodies in different Spanish preeclampsia subsets.

Lupus — Mon, 07 Nov 2011 05:00:00 +0100

Conclusions: Prevalence of antiphospholipid antibodies in preeclampsia patients is twice that in healthy pregnant women. Multipositive aPL test, IgM-anticardiolipin and IgM-anti-ß(2)glycoprotein-I isotypes showed an association with severe and early-onset preeclampsia. Larger studies are needed to establish the usefulness of antiphospholipid tests as risk markers for severe and early onset preeclampsia. PMID: 22065091 [PubMed - as supplied by publisher] (Source: Lupus)

Antiphospohlipid syndrome in obstetrics

Best Practice and Research. Clinical Obstetrics and Gynaecology — Fri, 04 Nov 2011 04:00:00 +0100

Antiphospholipid syndrome is characterised by a variety of clinical and immunological manifestations. The clinical hallmarks of this syndrome are thrombosis and poor obstetric outcomes, including miscarriages, fetal loss and severe pre-eclampsia. The main antiphospholipid antibodies include lupus anticoagulant, anticardiolipin and anti-β2-glycoprotein I. The combination of aspirin and heparin is considered the standard of care for women with antiphospholipid syndrome and embryo–fetal losses; however, aspirin in monotherapy may have a place in women with recurrent early miscarriage. A good benefit–risk ratio of low-molecular-weight heparin in pregnancy thrombosis treatment has been reported. Warfarin must be avoided if possible throughout the first trimester of pregnancy. Adequate preg...

Thrombophilia and early pregnancy loss

Best Practice and Research. Clinical Obstetrics and Gynaecology — Thu, 03 Nov 2011 04:00:00 +0100

Early pregnancy loss is the most common pregnancy complication. About 15% of pregnancies result in pregnancy loss and 1% of women experience recurrent miscarriage (more than three consecutive miscarriages). The influence of thrombophilia in pregnancy is a popular research topic in recurrent miscarriage. Both acquired and inherited thrombophilia are associated with a risk of pregnancy failure. Antiphospholipid syndrome is the only thrombophilia known to have a direct adverse effect on pregnancy. Historically, clinical research studying thrombophilia treatment in recurrent miscarriage has been of limited value owing to small participant numbers, poor study design and heterogeneity. The debate on the efficacy of aspirin and heparin has advanced with recently published randomised-controlled tr...

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Aspirin and heparin effect on Basal and antiphospholipid antibody modulation of trophoblast function.

Obstetrics and Gynecology — Tue, 01 Nov 2011 04:00:00 +0100

CONCLUSION: : In the absence of antiphospholipid antibodies, LMW heparin induces potentially detrimental proinflammatory and antiangiogenic profile in the trophoblast. In the presence of antiphospholipid antibodies, single-agent LMW heparin may be the optimal therapy to counter trophoblast inflammation, but also induces an antiangiogenic response. These findings may explain the inability of current therapies to consistently prevent adverse outcomes. LEVEL OF EVIDENCE: : II. PMID: 22015869 [PubMed - in process] (Source: Obstetrics and Gynecology)

Interactions of human monoclonal and polyclonal antiphospholipid antibodies with serine proteases involved in hemostasis

Arthritis and Rheumatism — Tue, 01 Nov 2011 04:00:00 +0100

ConclusionHigh‐avidity antithrombin antibodies, which prevent antithrombin inactivation of thrombin, distinguish patients with APS from SLE/aPL+ patients, and thus may contribute to the pathogenesis of vascular thrombosis in APS. (Source: Arthritis and Rheumatism)

Management of Acquired Thrombophilic Disorders in 2011: Focus on Heparin-induced Thrombocytopenia, Antiphospholipid Syndrome, Myeloproliferative Neoplasms and Paroxysmal Nocturnal Hemoglobinuria.

Archives of Iranian Medicine — Tue, 01 Nov 2011 04:00:00 +0100

This article will review the evidence-based management of heparin induced thrombocytopenia with thrombosis, anti-phospholipid antibody syndrome and catastrophic anti-phospholipid antibody syndrome, thrombohemorrhagic manifestations of Philadelphia chromosome-negative chronic myeloproliferative neoplasms including polycythemia vera, essential thrombocythemia and primary myelofibrosis, as well as paroxysmal nocturnal hemoglobinuria. PMID: 22039845 [PubMed - in process] (Source: Archives of Iranian Medicine)

The Presence of Antiphospholipid Antibodies in Healthy Bernese Mountain Dogs

Journal of Veterinary Internal Medicine — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions and Clinical ImportanceLupus anticoagulants and aCL antibodies could be the cause of prolonged aPTT in healthy Bernese Mountain Dogs. The importance of the antiphospholipid antibodies in the dogs remains unknown. (Source: Journal of Veterinary Internal Medicine)

Low‐Molecular‐Weight Heparin Added to Aspirin in the Prevention of Recurrent Early‐Onset Preeclampsia in women with Inheritable Thrombophilia: the FRUIT‐RCT

Journal of Thrombosis and Haemostasis — Tue, 01 Nov 2011 04:00:00 +0100

Conclusions: Adding LMWH to aspirin at <12 weeks gestation reduces recurrent HD onset <34 weeks gestation in women with inheritable thrombophilia and prior delivery for HD/SGA <34 weeks. Close monitoring of mother and fetus remains important throughout pregnancy. (Source: Journal of Thrombosis and Haemostasis)

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Acute Q Fever Presenting as Antiphospholipid Syndrome, Pneumonia, and Acalculous Cholecystitis and Masquerading as Mycoplasma pneumoniae and Hepatitis C Viral Infections.

Japanese Journal of Infectious Diseases — Tue, 01 Nov 2011 04:00:00 +0100

We report a case of Q fever-related antiphospholipid syndrome in a patient presenting with acalculous cholecystitis and pneumonia. Serial laboratory tests indicated that the previous serological tests suggesting hepatitis C virus and Mycoplasma pneumoniae infections were false-positives. The patient's fever persisted despite treatment with doxycycline, but disappeared 1 day after initiation of steroid treatment. To avoid incorrect diagnosis and subsequent delays in appropriate treatment of Q fever patients, the possibility that Q fever can masquerade as other infections or as an autoimmune disease should be kept in mind. PMID: 22116335 [PubMed - in process] (Source: Japanese Journal of Infectious Diseases)

Symmetrical peripheral gangrene: a rare presentation of antiphospholipid syndrome

Internal and Emergency Medicine — Mon, 31 Oct 2011 17:02:04 +0100

Content Type Journal ArticleCategory CE - MEDICAL ILLUSTRATIONPages 1-3DOI 10.1007/s11739-011-0714-4Authors Abdul Hai, Department of Surgery, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard University, New Delhi, IndiaMohammed Aslam, Department of Medicine, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard University, New Delhi, IndiaTarique H. Ashraf, Department of Surgery, Chattarpati Sahuji Maharaj Medical University, Lucknow, India Journal Internal and Emergency MedicineOnline ISSN 1970-9366Print ISSN 1828-0447 (Source: Internal and Emergency Medicine)

Antiphospholipid antibodies in patients with persistent lyme disease symptoms.

Lupus — Mon, 31 Oct 2011 04:00:00 +0100

Authors: Stricker RB, Johnson L PMID: 22040690 [PubMed - as supplied by publisher] (Source: Lupus)

Double-trouble: An unusual case of two simultaneous arterial thromboses in thrombophilia

Central European Journal of Medicine — Fri, 28 Oct 2011 17:22:22 +0100

We present herein a patient with the double simultaneous arterial thromboses as the manifestation of thrombophilia (heterozygous for methylenetetrahydrofolate-reductase (MTHFR) C677T gene mutation). There was no patent foramen ovale suspected upon the patient’s admittance to hospital. To the best of our knowledge there have been no similar cases presented to date. Content Type Journal ArticleCategory Case reportPages 1-4DOI 10.2478/s11536-011-0110-5Authors Mirjana Krotin, Department of Cardiology, University Clinical Hospital Center Bezanijska Kosa, 11000 Belgrade, SerbiaMarija Zdravkovic, Department of Cardiology, University Clinical Hospital Center Bezanijska Kosa, 11000 Belgrade, SerbiaDanica Popovic-Lisulov, Department of Cardiology, University Clinical Hospital Center Beza...

Incidence of a first thromboembolic event in asymptomatic carriers of high-risk antiphospholipid antibody profile: a multicenter prospective study

Blood — Thu, 27 Oct 2011 04:00:00 +0100

In conclusion, the occurrence of a first TE in carriers of high-risk aPL profile is considerable; it is more frequent among male subjects and in the presence of additional risk factors for venous TE. These data can help in the decision to initiate primary thromboprophylaxis in these subjects. (Source: Blood)

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Nonhealing Ulcer: Acroangiodermatitis of Mali

Clinical and Developmental Immunology — Wed, 26 Oct 2011 00:06:55 +0100

An 18-year-old male presented with a nonhealing wound on left lower limb, pain and swelling over multiple joints, weight loss, and yellowish discoloration of eyes and urine for the past 4 years. On examination, the patient had pallor, icterus, and generalized lymphadenopathy with a nonhealing unhealthy ulcer over left medial malleolus. He had deformed joints with hepatomegaly and splenomegaly. His laboratory investigations were positive for antinuclear antibody (ANA) and anticardiolipin antibody (ACLA). Synovial fluid analysis showed inflammatory findings. Biopsy of margin of the ulcer showed findings consistent with Acroangiodermatitis of Mali. The patient was treated with disease-modifying antirheumatic drugs (DMARDs) and aspirin for juvenile idiopathic arthritis and secondary antiphosph...

[Systemic autoimmune disorders and pregnancy].

Orvosi Hetilap — Sun, 23 Oct 2011 04:00:00 +0100

Authors: Kiss E, Kiss CG, Poór G Abstract The coincidence of systemic autoimmune diseases and pregnancy may modify the outcome of the disease and the pregnancy due to the background immunologic and hormonal processes. The great majority of patients with autoimmune diseases are young females in their reproductive ages willing to have babies. Consequently we have to prepare for this special situation. Our concept on childbearing in autoimmune women has changed within the last 30 years. Earlier, systemic lupus erythematosus flared in about 50% of patients during pregnancy, but the flare rate has significantly decreased recently. This improvement can be attributed to increased attention to low diseases activity at the time of conception, which might reduce to the half of the risk for ...

Does seronegative antiphospholipid syndrome really exist?

Autoimmunity Reviews — Sat, 22 Oct 2011 04:00:00 +0100

Authors: Cervera R, Conti F, Doria A, Iaccarino L, Valesini G Abstract The diagnosis of seronegative (SN-) antiphospholipid syndrome (APS) has been suggested for patients with clinical manifestations indicative of APS but with persistently negative results in the commonly used assays to detect anti-cardiolipin (aCL) antibodies, anti-β2 Glycoprotein I antibodies (aβ2GPI), and lupus anticoagulant (LA). To date the best management of these patients is still unclear. New emerging anti-phospholipid (aPL) assays could improve our ability in diagnosing APS. However, the availability of aPL assays in routine laboratory practice is limited. In fact, even aβ2GPI is routinely tested in only a small number of laboratories, and other aPL, such as anti-prothrombin or anti-annexin antibodies, ...

High intensity anticoagulation in the prevention of the recurrence of arterial thrombosis in antiphospholipid syndrome: 'PROS' and 'CONS'

Autoimmunity Reviews — Sat, 22 Oct 2011 04:00:00 +0100

In conclusion, there is currently a lack of consensus on the use of high intensity anticoagulation for the secondary prophylaxis of arterial thrombosis. However, such a treatment may be particularly recommended in those APS patients who have a high risk aPL profile and other concomitant cardiovascular risk factors, provided that the potential benefit outweighs the risk of bleeding. PMID: 22036827 [PubMed - as supplied by publisher] (Source: Autoimmunity Reviews)

Thromboprophylaxis in carriers of antiphospholipid antibodies (APL) without previous thrombosis: "Pros" and "Cons"

Autoimmunity Reviews — Sat, 22 Oct 2011 04:00:00 +0100

Thromboprophylaxis in carriers of antiphospholipid antibodies (APL) without previous thrombosis: "Pros" and "Cons" Autoimmun Rev. 2011 Oct 22; Authors: Ceccarelli F, Chighizola C, Finazzi G, Meroni PL, Valesini G Abstract The presence of anti-phospholipid (aPL) is necessary but not sufficient to induce a thrombotic event. The "second hit" hypothesis suggested that an additional trigger may be needed to develop a vascular event in aPL carriers. In this article, pro and con of primary thromboprophylaxis in aPL carriers is deeply discussed, concluding that univocal data are not available, due to conflicting results of available clinical trials. However, in clinical practice the primary thromboprophylaxis is not indicated in all unselected asymptomatic aPL carriers, and the bes...

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Sulfasalazine-Induced Hypersensitivity Syndrome Associated With Human Herpesvirus 6 Reactivation and Induction of Antiphospholipid Syndrome.

Actas Dermo-Sifiliograficas — Thu, 20 Oct 2011 04:00:00 +0100

We describe a new case of sulfasalazine-induced hypersensitivity syndrome associated with HHV-6 reactivation and the induction of anticardiolipin and anti-thyroid peroxidase antibodies. PMID: 22018653 [PubMed - as supplied by publisher] (Source: Actas Dermo-Sifiliograficas)

Oxidized Human Beta2-Glycoprotein I: Its Impact on Innate Immune Cells.

Current Molecular Medicine — Tue, 18 Oct 2011 13:00:22 +0100

Authors: Buttari B, Profumo E, Capozzi A, Sorice M, Riganò R Abstract Beta2-glycoprotein I (β 2-GPI), an abundant 50 kDa plasma glycoprotein, is the most common target for antiphospholipid antibodies (aPLs). These autoantibodies are associated with thrombotic events in patients with anti-phospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE) and are proatherogenic. β2-GPI can also stimulate a vigorous adaptive cellular immune response in these patients. Although much is known about β2-GPI as a cofactor in autoimmune diseases, crucial information is still lacking to clarify why this abundant self plasma protein is the target of autoimmune responses. Throughout the years, a remarkable number of theories have been proposed to explain how the immune system reco...

Primary antiphospholipid syndrome: morphofunctional penile abnormalities with normal sperm analysis.

Lupus — Mon, 17 Oct 2011 04:00:00 +0100

Conclusions: Normal testicular function has been identified in PAPS patients, in spite of morphofunctional penile abnormalities. Previous arterial thrombosis may underlie penile anthropometry alteration. PMID: 22004973 [PubMed - as supplied by publisher] (Source: Lupus)

Catastrophic antiphospholipid syndrome in the obstetric period

European Journal of Obstetrics, Gynecology, and Reproductive Biology — Mon, 17 Oct 2011 04:00:00 +0100

We report a case to highlight the importance of considering CAPS in pregnant patients with thrombotic microangiopathy in the obstetric period. (Source: European Journal of Obstetrics, Gynecology, and Reproductive Biology)

Thalidomide in the treatment of refractory cutaneous lupus: prognostic factors of clinical outcome

British Journal of Dermatology — Sun, 16 Oct 2011 04:00:00 +0100

Conclusions: Low‐dose thalidomide is an effective treatment for refractory CLE, but its benefits need to be balanced with the potential adverse effects. Whereas DLE forms tended to relapse and required a long‐term maintenance dose, SCLE forms showed a sustained remission after its withdrawal. (Source: British Journal of Dermatology)

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Thalidomide in the treatment of refractory cutaneous lupus: prognostic factors of clinical outcome.

The British Journal of Dermatology — Sun, 16 Oct 2011 04:00:00 +0100

Conclusions: Low-dose thalidomide is an effective treatment for refractory CLE, but its benefits need to be balanced with the potential adverse effects. Whereas DLE forms tended to relapse and required a long-term maintenance dose, SCLE forms showed a sustained remission after its withdrawal. PMID: 21999437 [PubMed - as supplied by publisher] (Source: The British Journal of Dermatology)

Thalidomide in the treatment of refractory cutaneous lupus erythematosus: prognostic factors of clinical outcome

British Journal of Dermatology — Sun, 16 Oct 2011 04:00:00 +0100

Conclusions Low‐dose thalidomide is an effective treatment for refractory CLE, but its benefits need to be balanced against the potential adverse effects. Whereas DLE forms tended to relapse and required a long‐term maintenance dose of thalidomide, SCLE forms showed a sustained remission after withdrawal. (Source: British Journal of Dermatology)

Standards and reference materials for the anticardiolipin and anti-β(2)glycoprotein I assays: A report of recommendations from the APL Task Force at the 13th International Congress on Antiphospholipid Antibodies.

International Journal of Clinical Chemistry — Sat, 15 Oct 2011 04:00:00 +0100

Authors: Pierangeli SS, Favaloro EJ, Lakos G, Meroni PL, Tincani A, Wong RC, Harris EN Abstract BACKGROUND: The confirmation of diagnosis of the Antiphospholipid Syndrome (APS) relies on laboratory tests. Current classification criteria for definite APS mandate the use of three "standardized" laboratory assays to detect antiphospholipid antibodies (aPL) [viz: anticardiolipin (aCL) IgG and IgM, anti-β(2)glycoprotein I (anti-β(2)GPI) antibodies IgG and IgM and/or a lupus anticoagulant (LAC)], when at least one of the two major clinical manifestations (thrombosis or pregnancy losses) are present. Several attempts have been made to standardize the aCL and anti-β(2)GPI tests, though, a considerable degree of inconsistencies still exist, limiting the clinical and diagnostic value of a...

Circulating Cell‐Derived Microparticles in Severe Preeclampsia and in Fetal Growth Restriction

American Journal of Reproductive Immunology — Thu, 13 Oct 2011 04:00:00 +0100

Conclusion Normal pregnancy is accompanied by endothelial and platelet cell activation. Endothelial cell activation has been shown in PE but not in isolated FGR. In PE, aPL may contribute to endothelial and possibly to leukocyte cell activation. (Source: American Journal of Reproductive Immunology)

In vivo distribution of {beta}2 glycoprotein I under various pathophysiologic conditions

Blood — Thu, 13 Oct 2011 04:00:00 +0100

In vitro studies have documented β2 glycoprotein I (β2GPI) binding to endothelial cells (ECs) and trophoblast using antiphospholipid antibodies. The in vivo binding of β2GPI to these cells and the conditions that favor their interaction have not been investigated. We analyzed the in vivo distribution of cyanine 5.5-labeled β2GPI in mice and evaluated the effect of pregnancy and circulating antibodies on its tissue localization. The signal was detected in the liver by whole body scan and ex vivo analysis. The β2GPI failed to bind to the vascular endothelium and reacted only with the ECs of uterine vessels. In pregnant mice the protein was localized on ECs and trophoblast at the embryo implantation sites. Immunized mice showed a similar β2GPI biodistribution to ...

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Calciphylaxis in a patient with systemic lupus erythematosus without renal insufficiency or hyperparathyroidism.

Lupus — Thu, 13 Oct 2011 04:00:00 +0100

We report a patient with SLE and no renal failure or hyperparathyroidism who developed severe calciphylaxis. PMID: 21997968 [PubMed - as supplied by publisher] (Source: Lupus)

Impaired control of the tissue factor pathway of blood coagulation in systemic lupus erythematosus.

Lupus — Wed, 12 Oct 2011 04:00:00 +0100

This study investigated whether the major physiological trigger of blood coagulation, the tissue factor (TF) pathway, was altered in SLE patients. Furthermore, we investigated potential associations between the TF pathway, the presence of antiphospholipid (APL) antibodies and other abnormalities present in SLE. A total of 101 participants (40 SLE patients and 61 age- and sex-matched controls) were recruited from Tasmania, Australia. Markers of the TF pathway, hypercoagulability, inflammation and endothelial cell damage were measured in plasma. Serum levels of APL antibodies (anti-cardiolipin antibodies [ACL], lupus anticoagulants [LAC], anti-beta2-glycoprotein-1 [anti-β2GP1] and anti-prothrombin antibodies) were also determined. Despite similar TF and TF pathway inhibitor (TFPI) total ant...

Rituximab induces resolution of recurrent diffuse alveolar haemorrhage in a patient with primary antiphospholipid antibody syndrome.

Lupus — Wed, 12 Oct 2011 04:00:00 +0100

We describe a patient with primary APS and refractory recurrent episodes of DAH. The patient was admitted 15 times due to recurrent episodes of DAH in a period of 18 months. Multiple immunosuppressive drugs did not improve his condition. Two years after his presentation, he was treated with rituximab (two doses of 1 g, 2 weeks apart). Six months later, the attacks of DAH have gradually disappeared. In a follow-up of more than 2 years after he received rituximab, the patient has had no further admissions due to DAH. Levels of antiphospholipid antibodies were measured during follow-up of 4 years. Anti-β2 glycoprotein IgG titer decreased to normal 6 months after therapy but anticardiolipin (aCL) antibody titer increased. We conclude that rituximab caused a dramatic clinical response in this ...

Influence of antiphospholipid antibody levels and type on thrombotic manifestations: results from the serbian national cohort study.

Lupus — Wed, 12 Oct 2011 04:00:00 +0100

The objective of this study was to observe the prevalence and localization of thrombosis, and to investigate the importance of aPL type and level for thrombosis-related events in patients diagnosed with APS. These are the first results of patients enrolled in Serbian National Cohort Study which comprises 256 patients: 162 with primary antiphospholipid syndrome (PAPS) and 94 with APS associated with systemic lupus erythematosus (SLE). aPL analysis included detection of aCL (IgG/IgM), β(2)GPI, and lupus anticoagulant. Thrombosis was diagnosed in 119 (46.5%) patients, with higher prevalence in PAPS compared with SLE patients (51.2% and 38.3%, respectively, p = 0.045). There was similar prevalence of arterial thrombosis in PAPS and SLE groups (34.6% and 34%, respectively, p = 0.932) although ...

Induction of Auto‐Antibodies Against β2‐Glycoprotein I in Mice by Protein H of Streptococcus Pyogenes

Journal of Thrombosis and Haemostasis — Tue, 11 Oct 2011 04:00:00 +0100

Conclusion: Our study demonstrated that a bacterial protein can induce a conformational change in β2‐GPI resulting in the formation of auto‐antibodies against β2‐GPI. This constitutes a novel mechanism for the formation of auto‐antibodies against β2‐GPI. (Source: Journal of Thrombosis and Haemostasis)

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Obstetric antiphospholipid syndrome.

Autoimmunity Reviews — Fri, 07 Oct 2011 04:00:00 +0100

Authors: Galarza-Maldonado C, Kourilovitch MR, Pérez-Fernández OM, Gaybor M, Cordero C, Cabrera S, Soroka NF Abstract Antiphospholipid syndrome (APS) in pregnancy has a serious impact on maternal and fetal morbidity. It causes recurrent pregnancy miscarriage and it is associated with other adverse obstetric findings like preterm delivery, intrauterine growth restriction, preeclampsia, HELLP syndrome and others. The 2006 revised criteria, which is still valid, is used for APS classification. Epidemiology of obstetric APS varies from one population group to another largely due to different inclusion criteria and lack of standardization of antibody detection methods. Treatment is still controversial. This topic should include a multidisciplinary team and should be individualized. Su...

Aggravated capillary non-perfusion after intravitreal bevacizumab for macular edema secondary to systemic lupus erythematosus and anti-phospholipid syndrome.

Lupus — Wed, 05 Oct 2011 04:00:00 +0100

Authors: Jeon S, Lee WK Abstract A 22-year-old female with history of systemic lupus erythematosus (SLE) was referred for evaluation of decreased visual acuity in her right eye. Her best-corrected visual acuity (BCVA) at the time of presentation was 20/160. Widespread cotton wool spots and macular edema were seen on biomicroscopy. Fluorescein angiography (FA) revealed retinal arterial and venous obstruction with capillary nonperfusion at the superotemporal retina. Antiphospholipid syndrome (APS) was diagnosed based on positive lupus anti-coagulant and ocular manifestations. Scattered laser photocoagulation was applied at the nonperfusion area but the visual acuity continued to deteriorate due to macular edema. Intravitreal bevacizumab (IVB) was administered for macular edema. One d...

Antiphospholipid Antibodies in Black South Africans with HIV and Acute Coronary Syndromes: Prevalence and Clinical Correlates

BMC Research Notes — Tue, 04 Oct 2011 04:00:00 +0100

Background: HIV infection is associated with a high prevalence of antiphospholipid antibodies (aPL) and increased thrombotic events but the aetiopathogenic link between the two is unclear.FindingsProspective single centre study from Soweto, South Africa, comparing the prevalence of aPL in highly active anti-retroviral therapy (HAART) naive HIV positive and negative patients presenting with Acute Coronary Syndromes (ACS). Between March 2004 and February 2008, 30 consecutive black South African HIV patients with ACS were compared to 30 black HIV negative patients with ACS .The HIV patients were younger (43 +/- 7 vs. 54 +/- 13, p=0.004) and besides smoking (73% vs. 33%, p=0.002) and lower HDL levels (0.8 +/- 0.3 vs. 1.1 +/- 0.4, p=0.001) had fewer risk factors than the control group. HIV pati...

Evidence for heterogeneity of the obstetric antiphospholipid syndrome: thrombosis can be critical for antiphospholipid‐induced pregnancy loss

Journal of Thrombosis and Haemostasis — Sat, 01 Oct 2011 04:00:00 +0100

Conclusions: This human monoclonal antibody is responsible for pregnancy loss through a new mechanism involving thrombosis. This mechanism adds to the heterogeneity of the obstetric antiphospholipid syndrome. (Source: Journal of Thrombosis and Haemostasis)

Catastrophic antiphospholipid antibody syndrome with bone marrow necrosis: a rare complication.

J Rheumatol — Sat, 01 Oct 2011 04:00:00 +0100

Authors: Khalil YK, Pistoria MJ, Wright RE PMID: 21965704 [PubMed - in process] (Source: J Rheumatol)

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Coagulation inhibitors and activated protein C resistance in recurrent pregnancy losses in Indian women.

Indian Journal of Pathology and Microbiology — Sat, 01 Oct 2011 04:00:00 +0100

Conclusion: There is a significant risk of RPL in pregnant women with thrombophilias. Therefore, screening for thrombophilias may be justified in pregnant women with unexplained recurrent fetal wastage, especially in second and third trimester. PMID: 22234103 [PubMed - in process] (Source: Indian Journal of Pathology and Microbiology)

A Warfarin Alternative in Antiphospholipid SyndromeA Warfarin Alternative in Antiphospholipid Syndrome

Medscape Today Headlines — Fri, 30 Sep 2011 15:00:00 +0100

Is low-molecular-weight heparin a safe and effective warfarin alternative in antiphospholipid antibody syndrome? Medscape Rheumatology (Source: Medscape Today Headlines)

Primary Antiphospholipid Antibody Syndrome and Libmann-Sachs Endocarditis

Mayo Clinic Proceedings — Fri, 30 Sep 2011 04:00:00 +0100

(Source: Mayo Clinic Proceedings)

Prolactin, estradiol and anticardiolipin antibodies in premenopausal women with systemic lupus erythematosus: a pilot study

Revista Brasileira de Reumatologia — Fri, 30 Sep 2011 00:24:58 +0100

CONCLUSION: These results suggest, for the first time, an inverse association between ACA and estradiol levels in premenopausal SLE patients. Considering that both lower endogenous estradiol levels and ACA positivity are related to atherosclerosis, our finding may be clinically relevant in predicting cardiovascular risk and/or APLS development in SLE. (Source: Revista Brasileira de Reumatologia)

Phlegmasia cerulea dolens in patient with systemic lupus erythematosus in the remote postpartum period

Revista Brasileira de Reumatologia — Fri, 30 Sep 2011 00:24:58 +0100

We report one case of phlegmasia cerulea dolens with rapid evolution to death in a patient with systemic lupus erythematosus in the remote postpartum period. (Source: Revista Brasileira de Reumatologia)

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Beyond the ‘syndrome’: Antiphospholipid antibodies as risk factors

Arthritis and Rheumatism — Tue, 27 Sep 2011 04:00:00 +0100

(Source: Arthritis and Rheumatism)

The efficacy of antiphospholipid score for the diagnosis of antiphospholipid syndrome and its predictive value for thrombotic events

Arthritis and Rheumatism — Tue, 27 Sep 2011 04:00:00 +0100

Conclusion:The aPL‐Score is a useful quantitative index for diagnosing APS, and may be a predictive marker of thrombosis in autoimmune diseases. © 2011 American College of Rheumatology. (Source: Arthritis and Rheumatism)

International consensus guidelines on anticardiolipin and anti‐β2glycoprotein I testing: A report from the APL task force at the 13th international congress on antiphospholipid antibodies

Arthritis and Rheumatism — Tue, 27 Sep 2011 04:00:00 +0100

AbstractThe confirmation of diagnosis of the Antiphospholipid Syndrome (APS) relies on laboratory tests. Current classification criteria for definite APS mandate the use of three “standardized” laboratory assays to detect antiphospholipid antibodies (aPL) [viz: anticardiolipin (aCL) IgG and IgM, anti‐β2glycoprotein I (anti‐β2GPI) antibodies IgG and IgM and/or a lupus anticoagulant (LAC)], when at least one of the two major clinical manifestations (thrombosis or pregnancy losses) are present. Although International Consensus Guidelines for the determination of LAC have been published and revised, the existence of “standardized” tests for detection of aCL and anti‐β2GPI has remained elusive. In spite of the publication of several proposals, consensus documents and expert opi...

Evolutionary conservation of the lipopolysaccharide binding site of β2-glycoprotein I.

Thrombosis and Haemostasis — Thu, 22 Sep 2011 04:00:00 +0100

Authors: Ağar C, de Groot PG, Marquart JA, Meijers JC Abstract β2-Glycoprotein I (β2GPI) is a highly abundant plasma protein and the major antigen for autoantibodies in the antiphospholipid syndrome. Recently, we have described a novel function of β2GPI as scavenger of lipopolysaccharide (LPS). With this in mind we investigated the conservation of β2GPI in vertebrates and set out to identify the binding site of LPS within β2GPI. The genome sequences of 42 species were surveyed. Surface plasmon resonance (SPR) was performed with peptides to characterise the binding site of β2GPI for LPS. β2GPI could be identified in most tested vertebrates with a high overall amino acid homology of 80% or more in mammals. SPR revealed that a synthesised peptide (LAFWKTDA) from domain V of β...

Addition of enoxaparin to aspirin for the secondary prevention of placental vascular complications in women with severe pre-eclampsia. The pilot randomised controlled NOH-PE trial.

Thrombosis and Haemostasis — Thu, 22 Sep 2011 04:00:00 +0100

Authors: Gris JC, Chauleur C, Molinari N, Marès P, Fabbro-Peray P, Quere I, Lefrant JY, Haddad B, Dauzat M Abstract Administration of heparin in the secondary prevention of placental vascular complications is still experimental. In women with a previous severe pre-eclampsia, we investigated the effectiveness of enoxaparin, a low-molecular-weight heparin, in preventing these complications. Between January 2000 and January 2010, 224 women from the NOHA First cohort, with previous severe pre-eclampsia but no foetal loss during their first pregnancy and negative for antiphospholipid antibodies, were randomised to either a prophylactic daily dose of enoxaparin starting from the positive pregnancy test (n=112), or no enoxaparin (n=112). The primary outcome was a composite of at least on...

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Successful use of ECT in post-stroke depression

General Hospital Psychiatry — Wed, 21 Sep 2011 04:00:00 +0100

We report the case of a 30-year-old woman with a history of stroke and antiphospholipid syndrome, who became depressed and suicidal. Neurologic and Internal Medicine consults did not reveal any contraindications to ECT, but stroke risk factor management was identified as an important measure for patient safety. The patient tolerated ECT well, reporting improvement of mood and abatement of hopelessness. This case suggests that ECT may be a safe and well-tolerated treatment for post-stroke depression. (Source: General Hospital Psychiatry)

The PTPN22*R620W polymorphism does not confer genetic susceptibility to antiphospholipid syndrome in the Spanish population

International Journal of Immunogenetics — Mon, 19 Sep 2011 16:38:47 +0100

SummaryIn this work, we proposed to determine the association of the PTPN22*R620W SNP with primary antiphospholipid syndrome (PAPS) in a case–control association study of Spanish Caucasian individuals. A total of 81 PAPS patients were compared with 81 blood‐donor healthy control subjects. PTPN22 SNP (R620W) genotyping was performed by using a polymerase chain reaction–restricted fragment length polymorphism assay. No statistically significant differences were found between control subjects and PAPS patients for the PTPN22*R620W genotypes (P = 0.214). No statistically significant differences were found according to either the presence or absence of antiphospholipid antibodies or the clinical manifestations associated to PAPS. Our results indicate that this functional PTPN22*R620W ...

The United States to Africa lupus prevalence gradient revisited.

Lupus — Sat, 17 Sep 2011 10:04:06 +0100

Authors: Gilkeson G, James J, Kamen D, Knackstedt T, Maggi D, Meyer A, Ruth N Abstract Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that has a significantly higher prevalence, morbidity and mortality in African Americans compared with Americans of European descent. The pathogenesis of lupus is unclear but appears to be a result of environmental factors interacting with a genetically susceptible host. Despite the high disease load of SLE in African Americans, there is the perception that lupus is relatively rare in Africa. This prevalence gradient suggests that comparative studies of related cohorts from the two continents may provide insight into the genetic/environmental interactions that result in the development of lupus. To define if a lupus gradient exis...

Induction of decreased fecundity by tetanus toxoid hyper-immunization in C57BL/6 mice depends on the applied adjuvant.

Innate Immunity — Fri, 16 Sep 2011 04:00:00 +0100

In this study we have investigated reproductive pathology induced in C57BL/6 mice by TTd hyper-immunization using a combination of different pretreatments (complete Freund's adjuvant or glycerol) and adjuvants (alhydrogel or glycerol). A decrease in fecundity was recorded in only C57BL/6 mice immunized with alhydrogel adjuvant, irrespective of the kind of applied pretreatment; it was associated with an increase in abundance of low affinity anti-β(2)GPI IgG antibodies and Th1 prevalence. PMID: 21926163 [PubMed - as supplied by publisher] (Source: Innate Immunity)

Modified phosphatidylserine-dependent antiprothrombin ELISA enables identification of patients negative for other antiphospholipid antibodies and also detects low avidity antibodies

Clinical Chemistry and Laboratory Medicine — Thu, 15 Sep 2011 15:14:55 +0100

Clinical Chemistry and Laboratory Medicine 49 (9): 1573-1573 (Source: Clinical Chemistry and Laboratory Medicine)

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Analysis of polymorphisms Leiden Factor V G1691A and prothrombin G20210A as risk factors for acute myocardial infarction.

Biogerontology — Thu, 15 Sep 2011 04:00:00 +0100

In conclusion, our data suggest that FII G20210A and/or Leiden FV might be involved as risk factor for arterial disorders in about 5% of old subjects, justifying the opportunity of a genetic screening and an eventual preventive treatment, in particular in old subjects in which other and major risk factors, as hypertension and atherosclerosis, are detected. PMID: 21918818 [PubMed - as supplied by publisher] (Source: Biogerontology)

Nephrotic and anti‐phospholipid syndromes: multisystem conditions associated with acute myocardial infarction in young patients

Journal of Renal Care — Wed, 14 Sep 2011 04:00:00 +0100

We describe a case of myocardial infarction in a young man with both nephrotic and antiphospho‐lipid syndromes and present a brief literature review. (Source: Journal of Renal Care)

Arterial Thrombosis in Patients with Human Immunodeficiency Virus: Two-Case Reports and Review of the Literature

Journal of Oncology — Mon, 12 Sep 2011 18:10:52 +0100

Conclusion. Arterial thrombosis might occur in HIV infection. Several etiological factors could be involved in the pathogeny of these arterial thromboses. (Source: Journal of Oncology)

Scleroderma pattern of nailfold capillary changes as predictive value for the development of a connective tissue disease: a follow-up study of 3,029 patients with primary Raynaud’s phenomenon

Rheumatology International — Thu, 08 Sep 2011 06:09:48 +0100

Abstract To assess the prognostic value of scleroderma pattern of nailfold capillary changes for the development of connective tissue diseases (CTD) in subjects with primary Raynaud’s phenomenon (RP). The study included 3,029 consecutive patients with primary RP who had been followed at 6-month intervals during the mean of 4.8 years. The pathological features of nailfold capillaroscopy were recorded in all patients who had neither clinical nor serological signs of a CTD. In patients who developed CTD, capillary changes obtained 6 months prior to diagnosis were analyzed. A possible relationship between capillary changes and the presence of associated CTD was assessed. At the end of follow-up, 1,660 (54,8%) patients have still the primary RP, 246 (8,1%) had suspe...

Antiphospholipid Antibodies and Antiphospholipid Syndrome: Role in Portal Vein Thrombosis in Patients With and Without Liver Cirrhosis

Clinical and Applied Thrombosis/Hemostasis — Thu, 08 Sep 2011 04:00:00 +0100

Conclusions: aPL play no role in PVT associated with liver cirrhosis but can be tested in idiopathic PVT. (Source: Clinical and Applied Thrombosis/Hemostasis)

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{beta}2-glycoprotein I, the major target in antiphospholipid syndrome, is a special human complement regulator

Blood — Thu, 08 Sep 2011 04:00:00 +0100

The human plasma protein β2-glycoprotein I (β2-GPI) is the major target of autoantibodies associated with antiphospholipid syndrome. However, the biologic function of this abundant protein is still unclear. Here we identify β2-GPI as a complement regulator. β2-GPI circulates in the plasma in an inactive circular form. On surface binding, such as to apoptotic cells, β2-GPI changes conformation to an elongated form that acquires C3/C3b binding activities. β2-GPI apparently changes conformation of C3, so that the regulator factor H attaches and induces subsequent degradation by the protease factor I. β2-GPI also mediates further cleavage of C3/C3b compared with factor H alone. Our data provide important insights into innate immune regulation by plasma protei...

Variceal Hemorrhage of Ascending Colon

Clinical Gastroenterology and Hepatology — Mon, 05 Sep 2011 04:00:00 +0100

A 33-year-old woman with a history of systemic lupus erythematosus was admitted to the hospital with melena, hematochezia, and syncope. Upper endoscopy did not demonstrate any bleeding source. Colonoscopy revealed varices between mid and distal ascending colon and blood clots at colonic mucosa ( A). Therefore, variceal hemorrhage in ascending colon was suspected. To find a cause of ascending colonic varices, abdominal computed tomography (CT) angiography ( B and C) was performed. CT angiogram demonstrated development of collateral vessels at retroperitoneum (black arrow, B and C), abdominal wall (white arrow, B), and drainage of both renal veins to the mentioned collateral vessels (4 white lightning arrows, C), but liver cirrhosis, portal vein obstruction, and splenomegaly were not found. ...

Chorea in primary antiphospholipid syndrome is associated with rheumatic fever

Rheumatology International — Thu, 01 Sep 2011 06:11:24 +0100

This study demonstrated that 4.5% of PAPS patients had chorea, predominately before PAPS diagnosis, and this neurological abnormality was associated with rheumatic fever and thrombocytopenia. These data reinforce the need for RF diagnosis in those PAPS patients with chorea. Content Type Journal ArticleCategory Original ArticlePages 1-5DOI 10.1007/s00296-011-2120-7Authors Simone Appenzeller, Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de Campinas, Campinas, SP, BrazilSteeven Yeh, Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3o andar, Reumatologia, sala 3190, São Paulo, SP 01246-903, BrazilMarcelo Maruyama, Rheumatology Division, Hospital das Clínicas da Faculd...

Food anaphylaxis, antiphospholipid syndrome and thrombosis.

Allergologia et Immunopathologia — Thu, 01 Sep 2011 04:00:00 +0100

Authors: Wiwanitkit V PMID: 21889829 [PubMed - as supplied by publisher] (Source: Allergologia et Immunopathologia)

Livedo reticularis from amantadine.

Skinmed — Thu, 01 Sep 2011 04:00:00 +0100

Authors: Xu LY, Liu A, Kerr HA Abstract A 70-year-old Caucasian man with a medical history of Parkinson's disease presented with a 3-month history of violaceous reticulated patches on his upper and lower extremities. The lesions were asymptomatic. The patient did not have a history of cardioembolic events or autoimmune disorders. No new medications were started before the onset of the lesions. Review of systems was unremarkable. On examination, large erythematous to violaceous patches were present in a reticulated net-like pattern on the patient's upper and lower extremities (Figure 1 and Figure 2). No edema, erosions, or ulcerations were noted. An extensive workup for autoimmune, infectious, and hematologic causes of livedo reticularis was performed. Complete blood cell count, ant...

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Lupus anticoagulant: performance of the tests as recommended by the latest ISTH guidelines

Journal of Thrombosis and Haemostasis — Wed, 31 Aug 2011 23:00:00 +0100

Conclusions: LA tested by aPTT and/or dRVVT (at least one out of two tests positive), as recommended by the guidelines, was associated less strongly with a history of thrombosis (OR = 4.1) than either of these tests separately (OR = 5.0 and 4.3, respectively). With both tests positive (‘double LA positivity’) the association with thrombosis was stronger (OR = 6.5) compared with only one positive test. In fact, ‘double LA positivity’, detected by combinations of any of the tests studied, was markedly associated with a history of thrombosis. (Source: Journal of Thrombosis and Haemostasis)

Engraftment of peripheral blood mononuclear cells from systemic lupus erythematosus and antiphospholipid syndrome patient donors into BALB‐RAG‐2−/−IL‐2Rγ−/− mice: A promising model for studying human disease

Arthritis and Rheumatism — Wed, 31 Aug 2011 23:00:00 +0100

ConclusionWe established a novel humanized SLE‐DKO mouse exhibiting many of the immunologic and clinical features of human SLE. (Source: Arthritis and Rheumatism)

Novel assays of thrombogenic pathogenicity in the antiphospholipid syndrome based on the detection of molecular oxidative modification of the major autoantigen β2‐glycoprotein I

Arthritis and Rheumatism — Wed, 31 Aug 2011 23:00:00 +0100

ConclusionThis large retrospective multicenter study shows that posttranslational modification of β2GPI via thiol‐exchange reactions is a highly specific phenomenon in the setting of APS thrombosis. Quantification of posttranslational modifications of β2GPI in conjunction with standard laboratory tests for APS may offer the potential to more accurately predict the risk of occurrence of a thrombotic event in the setting of APS. (Source: Arthritis and Rheumatism)

Antiphospholipid antibody syndrome.

Archives of Pathology and Laboratory Medicine — Wed, 31 Aug 2011 23:00:00 +0100

Authors: Sangle NA, Smock KJ Abstract Antiphospholipid antibodies are directed against phospholipid-protein complexes and include lupus anticoagulant, anticardiolipin antibodies, and anti-beta-2 glycoprotein I antibodies. Antiphospholipid antibody syndrome is a common cause of acquired thrombophilia and is characterized by venous or arterial thromboembolism or pregnancy morbidity and the presence of antiphospholipid antibodies. Antibodies should be demonstrable on at least 2 occasions separated by 12 weeks. Heterogeneity of the autoantibodies and absence of gold standard assays makes interpretation of laboratory results a challenge for both laboratorians and clinicians. This review discusses the key laboratory and clinical aspects of antiphospholipid antibody syndrome. Particular ...