Management Of Antiphospholipid Syndrome Research Articles

Obstetrical Considerations and Management of Antiphospholipid Syndrome

Antiphospholipid syndrome is a pro-thrombotic, pro-inflammatory condition defined by at least one clinical criterion and one laboratory finding. Clinical criteria are met by history of thrombosis or obstetric morbidity, including recurrent early pregnancy loss, fetal death, or delivery prior to 34 weeks gestation due to pre-eclampsia or placental insufficiency. Laboratory criteria are evidence of lupus anticoagulant or high titers of anticardiolipin or anti-β2-glycoprotein-I IgG or IgM. Treatment during pregnancy is primarily based on anticoagulant therapy, either at prophylactic or therapeutic doses depending on thrombosis history. This treatment certainly reduces thrombosis risk and may also improve obstetric outcome.

Le syndrome des antiphospholipides en néphrologie. Atteinte rénale et aspects pratiques de la prise en charge

The antiphospholipid syndrome is a thrombophilia characterized by the combination of arterial and/or venous thrombotic events or obstetric clinical events, associated with persistent presence of antiphospholipid antibodies. In this syndrome, thromboses may affect all of the vascular tree, renal damage is frequently associated with a specific antiphospholipid syndrome nephropathy. We propose in this review to provide updated recommendations on the management of antiphospholipid syndrome in nephrology. Treatment is based on long-term anticoagulant therapy with or without antiplatelet agents according to clinical events. The use of a conventional nephroprotection must not be forgotten (strict control of blood pressure with drugs blocking the renin–angiotensin–aldosterone system). Catastrophic antiphospholipid syndrome is an extremely severe complication which can threaten the vital prognosis of the patient. This justifies particular surveillance, as well as prevention in high-risk situations. We also illustrate the difficulties of long-term management in these patients, both in dialysis or kidney transplantation.

Antiphospholipid syndrome in northwest Italy (APS Piedmont Cohort): demographic features, risk factors, clinical and laboratory profile

We report the experience from the Antiphospholipid Antibodies (aPL) Regional Consortium in northwest Italy, meant to support clinical research and foster collaboration among health professionals regarding the diagnosis and management of antiphospholipid syndrome (APS) patients. This cohort-study (APS Piedmont Cohort) was designed to register the clinical characteristics at inception and associated immunological manifestations at diagnosis (if any) of patients who strictly fulfilled the current criteria for APS, all recruited at the Piedmont and Valle d'Aosta regions. Clinical and laboratory data from 217 APS patients (171 with vascular events, 33 with pregnancy morbidity and 13 with both), from 16 centres within the geographical area were collected. Venous thrombosis was recorded in 45.6% of patients, arterial thrombosis in 35%, small-vessel thrombosis in 1.12% and mixed arterial and venous thrombosis in the remaining 19.4% of the cases. Pregnancy morbidity included 19 patients with unexplained fetal death beyond the 10th week of pregnancy, 17 with premature birth before the 34th week and 10 with three or more unexplained spontaneous abortions before the 10th week of gestation. This consortium represents an instrument by which to audit clinical practice, to provide counselling to local centres and to sustain future basic and clinical APS research.

Guidelines on the investigation and management of antiphospholipid syndrome

This guidance updates and replaces the previous guideline on the investigation and management of antiphospholipid syndrome (APS) published in 2000 (Greaves et al, 2000), though where there have not been changes we refer back to them when appropriate. The guidance is updated with reference to relevant publications since 2000. Publications known to the writing group were supplemented with additional papers identified by searching PubMed for publications in the last 11 years using the key words: lupus anticoagulant, anticardiolipin, antiphospholipid, b2–glycoprotein I, antiprothrombin and limits (clinical trial, randomized control trial, meta-analysis, humans, core clinical journals, English language). The writing group produced the draft guideline, which was subsequently revised by consensus by members of the Haemostasis and Thrombosis Task Force of the British Committee for Standards in Haematology. The guideline was then reviewed by a sounding board of approximately 50 UK haematologists, the Royal College of Obstetricians and Gynaecologists (RCOG), and the British Committee for Standards in Haematology (BCSH) Committee and comments incorporated where appropriate. The ‘GRADE’ system was used to quote levels and grades of evidence, details of which can be found at http://www.bcshguidelines.com/BCSH_PROCESS/EVIDENCE_LEVELS_AND_GRADES_OF_RECOMMEN DATION/43_GRADE.html. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of patients with antiphospholipid syndrome though individual patient circumstances may dictate an alternative approach.

‘Immunomodulatory’ approaches to the management of antiphospholipid syndrome

1 ISSN 1758-4272 10.2217/IJR.11.77 © 2012 Future Medicine Ltd Int. J. Clin. Rheumatol. (2012) 7(1), 1–4 “The use of immunomodulatory agents has revolutionized the management of many autoimmune diseases including rheumatoid arthritis and SLE. Despite the comparatively slow pace of implementing these therapeutic agents in the management of APS patients, the last few years have produced several excellent clinical studies highlighting the efficacy of these drugs as well as international collaborative initiatives dedicated to furthering APS research.”

Diagnosis and management of antiphospholipid syndrome in pregnancy

Diagnosis and management of antiphospholipid syndrome in pregnancy

Management of antiphospholipid syndrome

The antiphospholipid syndrome (APS) is an autoimmune disorder presenting with tissue injury in various organs related to large- or small-vessel thrombosis associated with antiphospholipid and antiprotein/phospholipid complex antibodies. Although the pathophysiology, diagnosis, and clinical scenario may seem clear and straightforward, a more detailed examination reveals a more complex and uncertain picture related to the management of APS. This article reviews the current situation relating to APS therapy by evaluating the different clinical features of the syndrome ranging from thrombosis to pregnancy complications together with new strategies and pharmacological approaches.

Recent trends in the management of antiphospholipid syndrome (Hughes syndrome)

Antiphospholipid syndrome (Hughes syndrome) is characterized by the presence of arterial or venous thrombosis or recurrent miscarriages in a patient with positive laboratory tests for antiphospholipid antibodies (anticardiolipin antibodies and/or lupus anticoagulant and/or anti-beta(2) glycoprotein I). Despite the strong association between antiphospholipid antibodies, and thrombosis and obstetric morbidity, their pathogenic role in the development of these clinical features has not been fully elucidated. At present, patients with thrombosis are treated with long-term oral anticoagulation as standard. The approach for women with obstetric manifestations is based on the use of aspirin plus heparin. However, the knowledge of new pathogenic mechanisms might identify novel therapeutic targets and, therefore, may improve the management of these patients.

Pathogenesis and management of antiphospholipid syndrome

Antiphospholipid syndrome [APS] is an acquired thrombophilia which is associated with morbidity and mortality from thrombosis and pregnancy failure. It is important to recognise the condition, as thrombosis may be preventable through administration of anticoagulant therapy, and management of pregnancy in APS demands close monitoring and a multidisciplinary approach. APS may be a discrete disorder, or may occur against a background of another autoimmune disease, typically but not restricted to, systemic lupus erythematosus.

Management of antiphospholipid syndrome

Management of antiphospholipid syndrome

7B.3 Management of antiphospholipid syndrome in pregnancy

7B.3 Management of antiphospholipid syndrome in pregnancy

A Case of Primary Antiphospholipid Syndrome with Pulmonary Thromboembolism

Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.

Therapeutic potential of toleragens in the management of antiphospholipid syndrome.

Autoantibodies to beta2-glycoprotein I (beta2GPI) are believed to be the primary cause of coagulation abnormalities in patients with antiphospholipid syndrome (APS). Clinical features include a range of life-threatening thrombotic events and microangiopathies affecting multiple organ systems. Current standard of care relies on long-term, high-intensity anticoagulation and is associated with a high risk for serious bleeding events. The relation between autoantibodies and the pathophysiology of APS is not clearly understood, but numerous in vitro studies have characterized the effects of antiphospholipid autoantibodies on various components of the coagulation cascade, including tissue factor and the protein C pathway. The fine specificity of autoantibodies to beta2GPI is a subject of considerable debate; however, a body of evidence may offer resolution by integrating concepts of antibody affinity and assay sensitivity with carefully designed molecular studies. An investigational new therapy for APS is based on the approach that pathogenic antibodies may be reduced via depletion of circulating autoantibodies and induction of immune tolerance at the B-cell level. Preliminary results from a phase I/II clinical trial with LJP 1082, a B-cell toleragen, indicate the drug was well tolerated and may warrant further development for reduction of thrombotic events in patients with APS.

Inherited and acquired thrombophilias and poor pregnancy outcome: should we be treating with heparin?

The most important acquired thrombophilia related to poor pregnancy outcome is probably antiphospholipid syndrome. Inherited thrombophilias that have been implicated in venous thromboembolism and poor pregnancy outcome and for which standard tests are generally available are antithrombin III deficiency, the factor V Leiden mutation, prothrombin G20210A mutation and the C677T polymorphism in the methylenetetrahydrofolate reductase system implicated in mild hyperhomocysteinaemia. The management of antiphospholipid syndrome with previous fetal losses is well documented and substantiated by small clinical trials. It is the purpose of this review to investigate new contributions to this field since June 2002. Only one randomized trial was published during the review period, but a Cochrane review and several excellent review articles appeared detailing management. There is a dire lack of randomized trials in the literature on the efficacy of heparin or other coagulation modulators on pregnancy outcome in patients with inherited thrombophilias. There is consensus on thrombo-prophylaxis for antiphospholipid syndrome. Protocols for the management of venous thromboembolism and pulmonary emboli related to pregnancy are well established.

Antiphospholipid Syndrome

In Brief Antiphospholipid syndrome, a condition characterized by one or more thrombotic or pregnancy-related clinical features in association with medium to high levels of antiphospholipid antibodies, has emerged as an important diagnostic consideration in several medical fields. Antiphospholipid syndrome is one of the few treatable causes of pregnancy loss, and successful pregnancy rates of 70% or more can be achieved with appropriate treatment. Heparin, usually combined with low-dose aspirin, is used in patients at risk for thrombosis. Pregnancy in these women is associated with increased rates of preeclampsia, placental insufficiency, and preterm delivery, so that attentive clinical care is required for best outcomes. Recent studies indicate that women at low risk for thrombosis may be treated with low-dose aspirin. However, remaining controversies and unanswered questions in the field of antiphospholipid syndrome are a source of clinical confusion. This review highlights the most important controversies, taking into account the results of recent obstetric treatment trials and our own clinical experience. Diagnosis and management of antiphospholipid syndrome in pregnancyrequire a full understanding of the maternal and fetal risks, as well as controversies raised by recent studies.

Management of antiphospholipid syndrome during pregnancy.

Management of antiphospholipid syndrome during pregnancy.

Antiphospholipid antibodies and the endothelium.

This article reviews current understanding of the relationship between antiphospholipid antibodies and the endothelium. In vitro antiphospholipid antibodies produce endothelial cell activation. Clinical data in this area are scanty and worthy of future research, which could lead to new therapies in the management of antiphospholipid syndrome.

Clinical development in the management of lupus in the neonate, child, and adolescent.

The recent literature in pediatric aspects of systemic lupus erythematosus has focused primarily on the spectrum and management of antiphospholipid syndrome in children; this review summarizes developments in this area. In addition, the neonatal lupus syndrome is discussed in detail, and newer approaches to the management of children and adolescents with systemic lupus erythematosus will be summarized.