The Antiphospholipid Syndrome

Hematology ◽  
2007 ◽  
Vol 2007 (1) ◽  
pp. 136-142 ◽  
Author(s):  
Jacob H. Rand
Keyword(s):  
Antiphospholipid Syndrome ◽  
Pregnancy Complications ◽  
Binding Proteins ◽  
Clinical Manifestations ◽  
Therapeutic Approaches ◽  
Vascular Thrombosis ◽  
Phospholipid Binding ◽  
Thrombophilic Condition

Abstract The antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition that is marked by the presence of antibodies that recognize phospholipid-binding proteins. The clinical manifestations of APS include vascular thrombosis and pregnancy complications, especially recurrent spontaneous miscarriages. This article provides an update on diagnostic and therapeutic approaches to this disorder.

scholarly journals Antiphospholipid Syndrome and Pregnancy-Diagnosis, Complications and Management: An Overview

2021 ◽  
Author(s):  
Panagiotis Tsikouras ◽  
Christina Tsiggalou ◽  
Anastasia Bothou ◽  
Aggeliki Gerede ◽  
Ifigenia Apostolou ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Pregnancy Complications ◽  
Binding Proteins ◽  
Phospholipid Binding ◽  
Blood Clots ◽  
Human Proteins ◽  
Specific Proteins ◽  
Normal Human

Antiphospholipid syndrome which is also known as APS is an autoimmune disease which represents an acquired form of thrombophilia. The etiology of APS remains unknown. This disorder occurs when the immune system mistakenly attacks some of the normal human proteins and manifests itself as recurrent arterial or venous thrombosis and it could emerge after abortions or in recurrent pregnancy loss. In APS, the body produces the wrong antibodies against phospholipid-binding proteins, that is present in the blood and plays an important role in coagulation. Antibodies are specific proteins that usually target and neutralize the body’s invaders, such as viruses and bacteria. When antibodies attack phospholipid-binding proteins, blood clots abnormally. Specifically, it could cause blood clots in veins or arteries leading to stroke and various pregnancy complications such as: endometrial death, miscarriage, preeclampsia, intrauterine growth restriction and prematurity. APS is divided into primary and secondary, which is associated with autoimmune diseases and more often with systemic lupus erythematosus (SLE), while antibodies against cardiolipin are detected in many other conditions (infections, malignancies, drugs, etc.). The symptoms of APS, in addition to arterial and/or venous thrombosis and pregnancy complications, are multisystemic and the differential diagnosis of the primary APS from the secondary, in the context of SLE, is of particular clinical interest and is subject of this literature review.

Cutaneous manifestations of antiphospholipid syndrome

Lupus ◽  
2021 ◽  
pp. 096120332199010
Author(s):  
Minerva Gomez-Flores ◽  
Genesis Herrera-Argaez ◽  
Osvaldo Vazquez-Martinez ◽  
Maira Herz-Ruelas ◽  
Jorge Ocampo-Candiani ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Binding Proteins ◽  
Cutaneous Manifestations ◽  
Phospholipid Binding

Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.

scholarly journals Antiphospholipid Syndrome

2019 ◽  
Vol 9 (4) ◽  
pp. 37-42
Author(s):  
Thaís da Silva Santos ◽  
Izabel Galhardo Demarchi ◽  
Tatiane França Perles Mello ◽  
Jorge Juarez Vieira Teixeira ◽  
Maria Valdrinez Campana Lonardoni
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Binding Proteins ◽  
Clinical Manifestations ◽  
Cellular Activation ◽  
Pathophysiological Mechanisms ◽  
Individual Character ◽  
History Of ◽  
Autoimmune Condition ◽  
In Pregnancy

Antiphospholipid syndrome (APS) was characterized as an autoimmune condition with the production of antiphospholipid antibodies (aPL) associated with thrombosis and morbidity in pregnancy. The prevalence of aPL in the population ranges from 1% to 5% in patients with APS. The hypotheses regarding pathophysiological mechanisms are strongly related to binding proteins and antiphospholipid antibodies. The exact mechanisms by which they lead to clinical manifestations appear to be heterogeneous, but it is believed which aPL contribute to the cellular activation/coagulation, and so cause the thrombotic events. The treatment of APS should be an individual character and several factors should be taken into accounts, such as a number of antibodies, the age of the patient and the history of thrombotic events.

scholarly journals Pathophysiology of the Antiphospholipid Antibody Syndrome

2021 ◽  
Author(s):  
David Green
Keyword(s):  
Antiphospholipid Syndrome ◽  
Binding Proteins ◽  
Membrane Receptors ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Clotting Factors ◽  
Anionic Phospholipid ◽  
Autoantibody Production ◽  
Phospholipid Binding ◽  
Glycoprotein I

The antiphospholipid syndrome is characterized by antibodies directed against phospholipid-binding proteins and phospholipids attached to cell membrane receptors, mitochondria, oxidized lipoproteins, and activated complement components. When antibodies bind to these complex antigens, cells are activated and the coagulation and complement cascades are triggered, culminating in thrombotic events and pregnancy morbidity that further define the syndrome. The phospholipid-binding proteins most often involved are annexins II and V, β2-glycoprotein I, prothrombin, and cardiolipin. A distinguishing feature of the antiphospholipid syndrome is the “lupus anticoagulant”. This is not a single entity but rather a family of antibodies directed against complex antigens consisting of β2-glycoprotein I and/or prothrombin bound to an anionic phospholipid. Although these antibodies prolong in vitro clotting times by competing with clotting factors for phospholipid binding sites, they are not associated with clinical bleeding. Rather, they are thrombogenic because they augment thrombin production in vivo by concentrating prothrombin on phospholipid surfaces. Other antiphospholipid antibodies decrease the clot-inhibitory properties of the endothelium and enhance platelet adherence and aggregation. Some are atherogenic because they increase lipid peroxidation by reducing paraoxonase activity, and others impair fetal nutrition by diminishing placental antithrombotic and fibrinolytic activity. This plethora of destructive autoantibodies is currently managed with immunomodulatory agents, but new approaches to treatment might include vaccines against specific autoantigens, blocking the antibodies generated by exposure to cytoplasmic DNA, and selective targeting of aberrant B-cells to reduce or eliminate autoantibody production.

scholarly journals Dos and don'ts in diagnosing antiphospholipid syndrome

Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 455-459 ◽  
Author(s):  
Jacob H. Rand ◽  
Lucia R. Wolgast
Keyword(s):  
Antiphospholipid Syndrome ◽  
Pregnancy Complications ◽  
Laboratory Tests ◽  
Diagnostic Testing ◽  
Placental Insufficiency ◽  
Vascular Thrombosis ◽  
History Of ◽  
Practical Information ◽  
Thrombotic Tendency

Abstract Antiphospholipid syndrome (APS) is an acquired autoimmune thrombotic tendency that is identified by the presence of abnormal antiphospholipid laboratory tests in patients who have a history of vascular thrombosis and/or pregnancy complications including recurrent spontaneous miscarriages and a group of other complications due to placental insufficiency. Diagnostic testing for APS is often problematic because of many misconceptions regarding these empirically derived assays. This chapter is intended to provide hematology-oncology consultants with practical information about the uses and limitations of assays used to diagnose APS.

scholarly journals Primary antiphospholipid syndrome in children: experience from two tertiary centres in South India

2019 ◽  
Vol 6 (2) ◽  
pp. 243
Author(s):  
Mahesh Janarthanan ◽  
Dhaarani Jayaraman ◽  
Julius Scott ◽  
M. S. Latha ◽  
Saravanan Margabandhu ◽  
...  
Keyword(s):  
Clinical Features ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Fetal Loss ◽  
Primary Antiphospholipid Syndrome ◽  
Retrospective Case ◽  
Case Record ◽  
Vascular Thrombosis ◽  
Immunologic Profile

Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by the presence of episodes of vascular thrombosis, recurrent fetal loss and other clinical features in the presence of antiphospholipid antibodies. The aim of the study was to analyze the clinical manifestations and immunologic profile of children presenting with APS.Methods: Authors did a retrospective case record study of patients admitted with thrombotic events between September 2013 and August 2018 and identified patients with positive antiphospholipid antibodies. Children who had clinical features of active lupus were not included.Results: The clinical and immunologic profile of 7 pediatric patients presenting with APS over 5 years from 2013 to 2018 were analysed. Symptoms secondary to vascular thrombosis were limb swelling, stroke, gangrene of toes and Budd Chiari syndrome.Conclusions:APS though rare should be considered in the differential diagnosis of children presenting with thrombotic events. They need long term anticoagulants to prevent further episodes. 

scholarly journals Hypoxia and Mitochondrial Dysfunction in Pregnancy Complications

Antioxidants ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 405 ◽  
Author(s):  
Xiang-Qun Hu ◽  
Lubo Zhang
Keyword(s):  
Pregnancy Complications ◽  
Intrauterine Growth Restriction ◽  
Animal Studies ◽  
Major Effect ◽  
Causative Role ◽  
Therapeutic Approaches ◽  
Maternal Complications ◽  
Mitochondrial Ros ◽  
Subsequent Risk

Hypoxia is a common and severe stress to an organism’s homeostatic mechanisms, and hypoxia during gestation is associated with significantly increased incidence of maternal complications of preeclampsia, adversely impacting on the fetal development and subsequent risk for cardiovascular and metabolic disease. Human and animal studies have revealed a causative role of increased uterine vascular resistance and placental hypoxia in preeclampsia and fetal/intrauterine growth restriction (FGR/IUGR) associated with gestational hypoxia. Gestational hypoxia has a major effect on mitochondria of uteroplacental cells to overproduce reactive oxygen species (ROS), leading to oxidative stress. Excess mitochondrial ROS in turn cause uteroplacental dysfunction by damaging cellular macromolecules, which underlies the pathogenesis of preeclampsia and FGR. In this article, we review the current understanding of hypoxia-induced mitochondrial ROS and their role in placental dysfunction and the pathogenesis of pregnancy complications. In addition, therapeutic approaches selectively targeting mitochondrial ROS in the placental cells are discussed.

scholarly journals A calcyclin-associated protein is a newly identified member of the Ca2+/phospholipid-binding proteins, annexin family.

1992 ◽  
Vol 267 (13) ◽  
pp. 8919-8924
Author(s):  
H Tokumitsu ◽  
A Mizutani ◽  
H Minami ◽  
R Kobayashi ◽  
H Hidaka
Keyword(s):  
Binding Proteins ◽  
Phospholipid Binding

scholarly journals Case of an unusual diagnosis of primary antiphospholipid syndrome with multiple clinical complications

2020 ◽  
Vol 2020 (12) ◽  
Author(s):  
Stathis Tsiakas ◽  
Chrysanthi Skalioti ◽  
Paraskevi Kotsi ◽  
Ioannis Boletis ◽  
Smaragdi Marinaki
Keyword(s):  
Antiphospholipid Syndrome ◽  
Thrombotic Event ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Young Male ◽  
Multiple Organ ◽  
Systemic Autoimmune Disease ◽  
Antiphospholipid Antibody ◽  
Primary Antiphospholipid Syndrome ◽  
Wide Range

ABSTRACT Antiphospholipid syndrome (APS) is a systemic autoimmune disease defined by the presence of antiphospholipid antibodies in association with thrombotic events and/or obstetric complications. Renal involvement is not infrequent in both primary and secondary APS. Kidney manifestations comprise a wide range of clinical features, including hypertension, major renal vessel thrombosis or microvascular endothelial injury, also described as APS nephropathy. In the absence of a thrombotic event, clinical manifestations of APS are often non-specific. We recently encountered a case of primary APS in a young male with newly diagnosed hypertension and renal impairment. The diagnosis of APS was initially suspected by his kidney biopsy findings, when electron microscopy examination showed the features of chronic microangiopathy, and was later confirmed by a triple positive antiphospholipid antibody profile and multiple organ involvement.

scholarly journals Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Gunay Uludag ◽  
Neil Onghanseng ◽  
Anh N. T. Tran ◽  
Muhammad Hassan ◽  
Muhammad Sohail Halim ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Autoimmune Disorder ◽  
Ocular Involvement ◽  
Ocular Manifestations ◽  
Different Types ◽  
Thrombotic Complications ◽  
Specific Factors ◽  
Arteries And Veins

AbstractAntiphospholipid syndrome (APS) is an autoimmune disorder associated with obstetrical complications, thrombotic complications involving both arteries and veins, and non-thrombotic manifestations affecting multiple other systems presenting in various clinical forms. Diagnosis requires the presence of antiphospholipid antibodies. The exact pathogenesis of APS is not fully known. However, it has recently been shown that activation of different types of cells by antiphospholipid antibodies plays an important role in thrombosis formation. Ocular involvement is one of the important clinical manifestations of APS and can vary in presentations. Therefore, as an ophthalmologist, it is crucial to be familiar with the ocular findings of APS to prevent further complications that can develop. Furthermore, the ongoing identification of new and specific factors contributing to the pathogenesis of APS may provide new therapeutic options in the management of the disease in the future.

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