Search results for "Antiphospholipid syndrome"

Lipid presentation by the protein C receptor links coagulation with autoimmunity.

2021

A lipid-protein autoimmunity target Several autoimmune diseases, including systemic lupus erythematosus and primary antiphospholipid syndrome, are characterized by the presence of antiphospholipid antibodies (aPLs). These molecules can activate the complement and coagulation cascades, which contributes to pathologies such as thrombosis, stroke, and pregnancy complications. Müller-Calleja et al. found that endothelial protein C receptor (EPCR) in complex with lysobisphosphatidic acid (LBPA) is the cell-surface target for aPL and mediates its internalization (see the Perspective by Kaplan). aPL binding to EPCR-LBPA resulted in the activation of tissue factor–mediated coagulation and interfero…

Pathogenic lipid‐binding antiphospholipid antibodies are associated with severity of COVID‐19

2021

Abstract Background Coronavirus disease 19 (COVID‐19)–associated coagulopathy is a hallmark of disease severity and poor prognosis. The key manifestations of this prothrombotic syndrome—microvascular thrombosis, stroke, and venous and pulmonary clots—are also observed in severe and catastrophic antiphospholipid syndrome. Antiphospholipid antibodies (aPL) are detectable in COVID‐19 patients, but their association with the clinical course of COVID‐19 remains unproven. Objectives To analyze the presence and relevance of lipid‐binding aPL in hospitalized COVID‐19 patients. Methods Two cohorts of 53 and 121 patients from a single center hospitalized for PCR‐proven severe acute respiratory syndro…

Chapter 13 Antiphospholipid Antibodies and Vasculitis

2009

Abstract Besides the large group of primary vasculitis diseases, vasculitis in autoimmune diseases is most commonly associated with systemic lupus erythematosus (SLE), scleroderma, rheumatoid arthritis, insulin-dependent diabetes mellitus, and dermatomyositis. The antiphospholipid syndrome (APS), as a relatively recently recognized autoimmune disorder, may also present with a variety of vasculitis features. These non-typical vasculitis syndromes have to be considered very carefully in the APS, and vasculitis manifestations presenting as systemic involvement in APS patients have to be identified very early to prevent patients from rapidly developing organ damage. The aim of this review artic…

Catastrophic antiphospholipid syndrome in a patient with V Leiden variant

2012

In Vitro Effects of Antiphospholipid Syndrome-IgG Fractions and Human Monoclonal Antiphospholipid IgG Antibody on Human Umbilical Vein Endothelial Ce…

2009

It has been shown that stimulation of endothelial cells and monocytes by antiphospholipid antibodies leads to a prothrombotic state involving upregulation of tissue factor (TF). We examined the in vitro effects of IgG fractions from patients with antiphospholipid syndrome (APS) and of a β-2-glycoprotein 1-independent human monoclonal antiphospholipid antibody (HL-5B) on human umbilical vein endothelial cells (HUVEC) in comparison to untreated cell controls and to exposure to monoclonal IgG control antibody. We also examined the effect of recombinant monocyte chemoattractant protein-1 (MCP-1) on peripheral blood monocytes. Stimulation of endothelial cells with APS IgG fractions or HL-5B resu…

Toll-like receptors play a crucial part in the pathophysiological activity of antiphospholipid antibodies

2011

The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombosis, recurrent fetal loss and the presence of a variety of antiphospholipid antibodies (aPL), directed to phospholipids like Cardiolipin and phospholipid binding proteins like β2-glycoprotein I. Till date, the pathophysiological processes underlying these thrombotic events were still not fully understood. Recent data support the idea that the aPL might act via enhanced cytokine release due to activation of certain Toll-like receptors. The investigation of some of those mechanisms in more detail enlightens the involvement of the intracellular receptors TLR7 and TLR8 in a central point. Using patients…

COMPLEMENT INVOLVEMENT IN ANTIPHOSPHOLIPID ANTIBODY-MEDIATED PLACENTAL DAMAGE: PROSPECTIVE STUDY IN APS PREGNANT WOMEN

2009

Pathogenic Role of Complement in Antiphospholipid Syndrome and Therapeutic Implications

2018

Antiphospholipid syndrome (APS) is an acquired autoimmune disease characterized by thromboembolic events, pregnancy morbidity, and the presence of antiphospholipid (aPL) antibodies. There is sound evidence that aPL act as pathogenic autoantibodies being responsible for vascular clots and miscarriages. However, the exact mechanisms involved in the clinical manifestations of the syndrome are still a matter of investigation. In particular, while vascular thrombosis is apparently not associated with inflammation, the pathogenesis of miscarriages can be explained only in part by the aPL-mediated hypercoagulable state and additional non-thrombotic effects, including placental inflammation, have b…

Antithrombotics and new interventions for venous thromboembolism: Exploring possibilities beyond factor IIa and factor Xa inhibition.

2021

Abstract Direct oral anti–activated factor X and antithrombin agents have largely replaced vitamin K antagonists as the standard of care in treatment of venous thromboembolism. However, gaps in efficacy and safety persist, notably in end‐stage renal disease, implantable heart valves or assist devices, extracorporeal support of the circulation, and antiphospholipid syndrome. Inhibition of coagulation factor XI (FXI) emerges as a promising new therapeutic target. Antisense oligonucleotides offer potential advantages as a prophylactic or therapeutic modality, with one dose‐finding trial in orthopedic surgery already published. In addition, monoclonal antibodies blocking activation and/or activ…

IVIG in APS pregnancy

2004

For more than two decades, the intravenous administration of high doses of IgG pooled from the plasma of healthy donors (immune globulin therapy, also known as ‘IVIG’) has benefited patients with a variety of autoimmune disorders. A potential therapeutic role of IVIG in the prevention of thrombosis and of miscarriages in antiphospholipid syndrome (APS) has been postulated. Multicenter randomized controlled trials attempted to define the role of IVIG in preventing pregnancy complications in APS indicate that simple anticoagulation could not be completely satisfactory, and certain patient subgroups might take advantage of IVIG therapy alone or in combination with heparin.