Antiphospholipid syndrome

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 675-680 ◽  
Author(s):  
Wendy Lim
Keyword(s):  
Antiphospholipid Syndrome ◽  
Arterial Thrombosis ◽  
Rapid Development ◽  
Multiorgan Failure ◽  
Severe Form ◽  
Anticardiolipin Antibodies ◽  
Recurrent Thrombosis ◽  
Thrombotic Risk ◽  
Pregnancy Morbidity ◽  
Increased Risk

Abstract The antiphospholipid syndrome (APS) is defined by venous or arterial thrombosis and/or pregnancy morbidity in patients with persistent presence of antiphospholipid antibodies (aPLs). Catastrophic APS is the most severe form of APS, which is associated with rapid development of microvascular thrombosis resulting in multiorgan failure in patients with aPLs. Patients with APS and catastrophic APS are recognized to have a high risk of recurrent thrombosis that can occur despite anticoagulant therapy. Although antithrombotic therapy remains the mainstay of treatment, bleeding manifestations can complicate management and contribute to increased morbidity. Patients with persistently elevated aPL levels, particularly those who exhibit positive testing for lupus anticoagulant, anticardiolipin antibodies, and anti-β2GPI antibodies (triple positivity), appear to be at increased risk for thrombosis and pregnancy complications, whereas isolated positivity for aPLs appears to be associated with low risk. Recognizing that patients with APS have different thrombotic risk profiles may assist clinicians in assessing the risks and benefits of anticoagulation. The optimal type, intensity, and duration of anticoagulation in the treatment of APS remain controversial, particularly for arterial thrombosis and recurrent thrombosis. Future studies that delineate thrombotic risk in APS and evaluate current and novel anticoagulants as well as nonanticoagulant therapies are required.

Antiphospholipid Syndrome: A Review

2018 ◽  
Vol 2 (02) ◽  
pp. 51-58
Author(s):  
Md. Motahar Hossain ◽  
Md. Akhter Hossain ◽  
Yasmin Rahman ◽  
Md. Kamrul Hasan
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Anticardiolipin Antibodies ◽  
Vitamin K Antagonist ◽  
Classification Criteria ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Pregnancy Morbidity ◽  
Pregnancy And Postpartum

Antiphospholipid syndrome (APS) is an autoimmune disease characterized by venous thromboembolism, arterial thrombosis, and obstetric morbidities in the setting of persistently positive levels of antiphospholipid antibodies. It may be primary or secondary. The latest classification criteria (Sydney 2006) recognize just three tests to define this syndrome- lupus anticoagulant, anticardiolipin antibodies and anti-?2-glycoprotein-1 antibodies. Treatment of thrombotic events involves lifelong anticoagulation with vitamin K antagonist warfarin. Antiphospholipid antibody syndrome (APS) with only pregnancy morbidity is treated with thromboprophylaxis with heparin during pregnancy and postpartum for 6 weeks. In this review we discuss the pathogenesis, diagnosis, treatment and prognosis of the APS.

scholarly journals SAT0194 LACK OF EFFICACY OF RIVAROXABAN IN THE TREATMENT OF ANTIPHOSPHOLIPID SYNDROME AND CLINICAL SIGNIFICANCE OF ANTIPHOSPHOLIPID ANTIBODIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1039.2-1039
Author(s):  
S. Vardanyan ◽  
K. Ginosyan ◽  
V. Vardanyan ◽  
A. Sargsyan ◽  
A. Simonyan
Keyword(s):  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Pregnancy Loss ◽  
Positive Association ◽  
Recurrent Thrombosis ◽  
Double Positive ◽  
Increased Risk ◽  
Single Positive

Background:Chronic anticoagulation with vitamin K antagonists (VKA) is the standard treatment to prevent thrombotic events in antiphospholipid syndrome (APS). But in recent years treatment schemes began to include rivaroxaban. Use of direct oral anticoagulants (DOAC) is an attractive and often preferred alternative to VKAs in other medical settings owing to greater ease of use, fewer food and drug interactions, and lower bleeding risks [1].However, according to last guidelines, rivaroxaban should not be used in patients with triple aPL positivity due to the high risk of recurrent events [2].Objectives:1.To determine the risk of recurrent thrombosis in single or double positive APS patients treated with rivaroxaban.2.Find out possible association between presence of particular antiphospholipid antibodies or high level of lupus anticoagulant (LA) and type of vascular events.Methods:33 patients with confirmed APS (25 female (75.8%), 8 male (24.2%), mean age 43.2±11.6 years) were included in the study. 17 (51.5%) of investigated patients had primary APS, in remaining 16 (48.5%) APS was included in the framework of SLE. 18 (54.5%) patients were treated with warfarin 2.5-7.5 mg/daily, 15 (45.5%) patients - with rivaroxaban 20mg/daily for a follow-up period of 12 months. The data is introduced as odds ratios (OR) with 95% confidence interval (CI). The results were considered significant when p <0.05.Results:At baseline 21 (63.6%) patients had history of arterial thrombosis, 10 (30.3%) - venous thrombosis, 17 (51.5%) - pregnancy loss. According to results of serum immunology check, 29 (87.9%) patients were anticardiolipin antibody (ACA) positive, 9 (27.3%) - LA positive, 19 (57.6%) - anti-ß2-glycoprotein antibodies (anti-ß2-gp) positive; 20 (60.6%) patients were double positive (12 (36.4%) of them had positive ACA and anti-ß2-gp, 6 (18.2%) - ACA and LA, and 2 (6.1%) - anti-ß2-gp and LA), 4 (12.1%) patients were triple positive.No association between vascular event and/or pregnancy loss in patients with single positive ACA was found. We have found positive association between arterial thrombosis and single positive anti-ß2-gp (OR /CI 95%/ = 5.0 /2.08 – 23.06/, p< 0.05), positive association between positive LA and venous thrombosis (OR /CI 95%/ = 10 /1.7-57.7/, p< 0.05), strong positive association between positive LA and thromboembolism of pulmonary artery (OR /CI 95%/ = 46.0 /4.0-525.1/, p< 0.001), negative association between positive LA and pregnancy loss (OR /CI 95%/ =0.06 /0.03 – 0.1/, p< 0.01).Risk of thrombosis and/or pregnancy loss was not significantly increased in double positive patients, but triple positive patients had increased risk of venous thrombosis (OR /CI 95%/ =9.4 /3.2 – 105.8/, p<0.04).Recurrent thrombosis was detected in 16 patients: 2 patients (12.5%) were on warfarin, 14 (87.5%) - on rivaroxaban (10 (71.4%) arterial thrombosis, 4 (28.6%) venous thrombosis).No association between warfarin 2.5-7.5 mg/daily and occurrence of recurrent thrombosis was detected. An association between use of warfarin and increased risk of bleeding was found, but the risk was not significant (OR /CI 95%/ = 7.0 /0.7 – 66/, p= 0.09). Rivaroxaban 20 mg/daily was associated with recurrent thrombosis not only for triple positive patients (p< 0.02), but also in double positive patients (OR /CI 95%/ = 21.3 /1.8 – 251/, p< 0.04).Conclusion:Rivaroxaban does not prevent recurrent thrombosis not only in triple positive patients, but also in single and double positive APS patients. Type of antiphospholipid antibodies can be predictive for the type of further vascular event.References:[1]Vittorio Pengo, Gentian Denas, Giacomo Zoppellaro et al.Rivroxaban vs warfarin in high risk patients with antiphospholipid syndrome, Blood. 2018 Sep 27; 132(13); 1357-1358.[2]EULAR recommendations for the management of antiphospholipid syndrome in adults, Annals of the Rheumatic Diseases/ Volume 78: Issue 10:1296-1304.Disclosure of Interests:None declared

Thrombosis in IBD in the Era of JAK Inhibition

2020 ◽  
Vol 22 (1) ◽  
pp. 126-136
Author(s):  
Virginia Solitano ◽  
Gionata Fiorino ◽  
Ferdinando D’Amico ◽  
Laurent Peyrin-Biroulet ◽  
Silvio Danese
Keyword(s):  
Small Molecules ◽  
Arterial Thrombosis ◽  
Protective Role ◽  
Jak Inhibitors ◽  
Thrombotic Risk ◽  
Increased Risk ◽  
Bowel Diseases ◽  
Thrombotic Complications ◽  
Inflammatory Bowel ◽  
Inflammatory Effect

: Patients with inflammatory bowel diseases (IBD) have an increased risk of thrombosis. The interaction between inflammation and coagulation has been extensively studied. It is well-known that some drugs can influence the haemostatic system, but several concerns on the association between therapies and increased risk of thrombosis remain open. While biologics seem to have a protective role against thrombosis via their anti-inflammatory effect, some concerns about an increased risk of thrombosis with JAK inhibitors have been raised. We conducted a literature review to assess the association between biologics/small molecules and venous/arterial thrombotic complications. An increased risk of venous and arterial thrombosis was found in patients treated with corticosteroids, whereas anti-TNF were considered protective agents. No thromboembolic adverse event was reported with vedolizumab and ustekinumab. In addition, thromboembolic events rarely occurred in patients with ulcerative colitis (UC) after therapy with tofacitinib. The overall risk of both venous and arterial thrombosis was not increased based on the available evidence. Finally, in the era of JAK inhibitors, treatment should be individualized by evaluating the pre-existing potential thrombotic risk balanced with the intrinsic risk of the medication used.

scholarly journals Prevention of thrombosis in antiphospholipid syndrome

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 707-713 ◽  
Author(s):  
Wendy Lim
Keyword(s):  
Risk Factors ◽  
Primary Prevention ◽  
Secondary Prevention ◽  
Antiphospholipid Syndrome ◽  
Antithrombotic Therapy ◽  
Thrombotic Risk ◽  
Pregnancy Morbidity ◽  
Asymptomatic Patients ◽  
Risk Patients ◽  
Thrombotic Complications

Abstract Antiphospholipid syndrome (APS) is an acquired autoimmune condition characterized by thrombotic events, pregnancy morbidity, and laboratory evidence of antiphospholipid antibodies (aPL). Management of these patients includes the prevention of a first thrombotic episode in at-risk patients (primary prevention) and preventing recurrent thrombotic complications in patients with a history of thrombosis (secondary prevention). Assessment of thrombotic risk in these patients, balanced against estimated bleeding risks associated with antithrombotic therapy could assist clinicians in determining whether antithrombotic therapy is warranted. Thrombotic risk can be assessed by evaluating a patient’s aPL profile and additional thrombotic risk factors. Although antithrombotic options for secondary prevention of venous thromboembolism (VTE) have been evaluated in clinical trials, studies in primary prevention of asymptomatic aPL-positive patients are needed. Primary prevention with aspirin may be considered in asymptomatic patients who have a high-risk aPL profile, particularly if additional risk factors are present. Secondary prevention with long-term anticoagulation is recommended based on estimated risks of VTE recurrence, although routine evaluation of thrombotic risk can assist in determining whether ongoing anticoagulation is warranted. Studies that stratify thrombotic risk in aPL-positive patients, and patients with APS evaluating antithrombotic and non-antithrombotic therapies will be useful in optimizing the management of these patients.

The Lupus Anticoagulant Paradox

2017 ◽  
Vol 44 (05) ◽  
pp. 445-452 ◽  
Author(s):  
Jessica Molhoek ◽  
Philip de Groot ◽  
Rolf Urbanus
Keyword(s):  
Antiphospholipid Syndrome ◽  
Clinical Studies ◽  
Lupus Anticoagulant ◽  
Antiphospholipid Antibody ◽  
Bleeding Tendency ◽  
Clotting Time ◽  
Pregnancy Morbidity ◽  
Increased Risk ◽  
Antibody Population

AbstractLupus anticoagulant (LA) represents the most enigmatic antibody population in patients with antiphospholipid syndrome and represents a paradox that is still unsolved. This class of antiphospholipid antibody causes a phospholipid-dependent prolongation of the clotting time but is associated with an increased risk of thrombosis and pregnancy morbidity. In this review, we will provide an overview of the different antibodies that have been associated with LA activity, their importance based on clinical studies, and address the question why this prolongation of the clotting time is associated with thrombosis rather than a bleeding tendency.

scholarly journals Acute myocardial infarction,arterial thrombosis and thrombophilia in young patients

2020 ◽  
Vol 26 (4) ◽  
pp. 26-39
Author(s):  
Ivo Petrov ◽  
Naydenka Zlatareva-Gronkova ◽  
Todor Kundurdjiev ◽  
Viktoria Dimitrova
Keyword(s):  
Myocardial Infarction ◽  
Acute Myocardial Infarction ◽  
Arterial Thrombosis ◽  
Young Patients ◽  
Specific Treatment ◽  
Premature Coronary Artery Disease ◽  
Thrombotic Risk ◽  
Coronary Syndrome ◽  
Increased Risk ◽  
Blood Coagulation Abnormality

Acute coronary syndrome (ACS) represent emergency state in an intensive cardiovascular care unit, which implies immediate and specific treatment. Of peculiar interest for cardiologists are young patients with acute myocardial infarction (AMI). The family history taking for premature coronary artery disease (CAD) and establishment of genetic factors, responsible for coagulation, both are on target for this group of patients. Gold standard for AMI diagnosis is coronary angiography (CA), which usually implies endovascular treatment (EVT). When coronary thrombus formation is found in young patients, different diagnostic opportunities are possible. Thrombophilia (TF) represents blood coagulation abnormality resulting in an increased risk of thrombosis. It could affect different sections of the cardiovascular system, most commonly venous, but also arterial. This clinical condition could be confirmed by performing laboratory genetic tests. We studied a group of forty-one young patients with first appearance of ACS ≤ 55 years old included for a five-year period. All of them were evaluated with CA and received EVT. According to the thrombotic risk, we defined a high-risk group, treated with anticoagulant (AC) on top of dual antiplatelet therapy (DAPT). The patients were followed-up for recurrent ischemic and bleeding events. We performed laboratory tests for the most frequent TF gene mutations in Bulgarian population. There is a conflicting data about this issue in different ethnic origins. The aim of our study is to estimate the possible relationship between the TF and the arterial thrombosis in young ACS patients, to defi ne specific treatment strategies, improving the prognosis of the patients.

The significance of autoantibodies against β2-glycoprotein I

Blood ◽  
2012 ◽  
Vol 120 (2) ◽  
pp. 266-274 ◽  
Author(s):  
Philip G. de Groot ◽  
Rolf T. Urbanus
Keyword(s):  
Autoimmune Disease ◽  
Infectious Diseases ◽  
Antiphospholipid Syndrome ◽  
Plasma Protein ◽  
Antiphospholipid Antibodies ◽  
Fetal Loss ◽  
Thrombotic Risk ◽  
Pregnancy Morbidity ◽  
Glycoprotein I ◽  
History Of

AbstractThe antiphospholipid syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies in patients with a history of thrombosis and/or pregnancy morbidity, including fetal loss. APS is an autoimmune disease with a confusing name because the pathologic auto-antibodies are shown to be directed against the plasma protein β2-glycoprotein I and not against phospholipids. In fact, auto-antibodies that recognize phospholipids themselves are not associated with thrombosis but with infectious diseases. One of the intriguing questions is why autoantibodies against β2-glycoprotein I are so commonly found in both patients and the healthy. Several potential mechanisms have been suggested to explain the increased thrombotic risk in patients with these autoantibodies. In this overview, we will summarize our knowledge on the etiology of the autoantibodies, and we will discuss the evidence that identify autoantibodies against β2-glycoprotein I as the culprit of APS.

The Coinicidence of Venous and Arterial Thrombosis in Patients with Antiphospholipid Syndrome - Correlation with Laboratory Findings.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4045-4045
Author(s):  
Wolfgang A. Miesbach ◽  
Robert Hudeck ◽  
Martina Boehm ◽  
Inge Scharrer
Keyword(s):  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Arterial Thrombosis ◽  
Clinical Manifestations ◽  
Anticardiolipin Antibodies ◽  
Enzyme Linked Immunosorbent Assay ◽  
Normal Range ◽  
Laboratory Findings ◽  
Lupus Anticoagulants ◽  
Vein Thrombosis

Abstract The antiphospholipid syndrome (APS) is one of the most common acquired causes of thrombosis on the venous or arterial site. The initial type of the thrombosis appears to be the most likely type of event to recur. In general, APS patients present more common venous thrombosis, especially deep vein thrombosis of the legs. Arterial thromboses are less common and mostly manifest with ischemia or infarction. It is not clear whether a combination of phospholipid antibodies or the additional presence of lupus anticoagulants in patients with anticardiolipin antibodies increase the risk of manifestation of APS. Methods: We investigated retrospectively 300 patients with elevated aCL antibodies. IgG and IgM aCL antibodies were tested by enzyme-linked immunosorbent assay (ELISA). LA were tested by more than 2 different methods according to the proposed criteria of the SSC of the ISTH. Results: Of these patients, 61 % suffered from manifestations of APS, 35 % from venous and 28 % from arterial thrombosis, 7 % of the patients had abortions. In 39 % no thromboembolic disease was found. The coincidence of venous and arterial thrombosis was found in 25/300 (8 %) of the patients. An increased titer of IgG aCL was found in 135 of 300 patients (45 %, median 49 GPL-U/ml, normal range: - 11,3 GPL-U/ml), and an increased titer of IgM aCL in 260 patients (87 %, median 13 MPL-U/ml, normal range: 5,6 MPL-U/ml). Lupus anticoagulants were additionally detected in 130/300 patients (43 %). The rate of clinical manifestations did not differ between LA-positive patients (78 of 130 patients, 60 %) and LA-negative patients (105 of 170, 62 %) patients with elevated aCL antibodies. Regarding the patients with the coincidence of venous and arterial thrombosis, we found a significantly higher rate of positive lupus anticoagulants than in the patients with APS manifestation of one site only (17/25 vs. 113/275, p < 0.05, OR 3.04; 95 % CI 1.31 – 7.06). An elevation of IgG-aCL titres were more frequently found, too. Table 1: Characteristics of the patients with a coincidence of venous and arterial thrombosis compared to patients with thrombosis of one site only Conclusion: The additional presence of lupus anticoagulants in patients with anticardiolipin antibodies identifies a group of patients with high risk of recurrent manifestations of the antiphospholipid syndrome. Particularly the risk of manifestation of both, arterial and venous thrombosis in these patients should be early recognized to initiate a careful follow-up and antithrombotic therapy in these patients. Venous and Arterial Thrombosis Thrombosis of One Site No APS Manifestation n 25/300 (8 %) 157/300 (59 %) 118/300 (39 %) Age (yrs) median 48.5 50 46 Sex (F/M) 17/8 (2.1) 62/157 (2.1) 51/118 (2.3) LA 17/25 (68 %) 62/157 (39 %) 51/118 (43 %) IgG-aCL 15/25 (60 %) 73/157 (46 %) 47/118 (40 %) median titre 108 GPL 87 GPL 37 GPL IgM-aCL 24/25 (96 %) 129/157 (82 %) 104/118 (88 %) median titre 13 MPL 14 MPL 14 MPL

Induction of Thrombosis in a Mouse Model by IgG, IgM and IgA Immunoglobulins from Patients with the Antiphospholipid Syndrome

1995 ◽  
Vol 74 (05) ◽  
pp. 1361-1367 ◽  
Author(s):  
Silva S Pierangeli ◽  
Xiao Wei Liu ◽  
John H Barker ◽  
Gary Anderson ◽  
E Nigel Harris
Keyword(s):  
Mouse Model ◽  
Antiphospholipid Syndrome ◽  
Thrombus Formation ◽  
Anticardiolipin Antibodies ◽  
Significant Delay ◽  
Recurrent Thrombosis ◽  
Immunoglobulin Preparations ◽  
Normal Controls

SummaryAntiphospholipid syndrome is a disorder of recurrent thrombosis and pregnancy losses associated with production of anticardiolipin antibodies and lupus anticoagulant positivity. Recently, we have adapted a mouse model of induced venous thrombosis to study the role of autoantibodies in thrombus formation. To determine whether immunoglobulins from patients with the antiphospholipid syndrome play a role in thrombosis, we injected groups of CDI mice either with immunoglobulins purified from seven patients with the antiphospholipid syndrome (nine preparations studied: four IgG, three IgM and two IgA) or with immunoglobulins of the same isotype from healthy controls. Seventy- two h after injection, a non-occlusive thrombus was induced in the femoral veins of experimental mice by a pinch injury; the thrombus areas as well as times of formation and disappearance of the thrombi were measured. Eight of the nine antiphospholipid syndrome immunoglobulin preparations caused a significant increase in mean thrombus area and a significant delay in mean thrombus disappearance time as compared with normal controls. To determine whether anticardiolipin antibodies might be involved, separate groups of mice were injected with affinity-purified IgG (n = 2) or IgM (n = 2) anticardiolipin antibodies or with normal immunoglobulins of the same isotype, and the effects on thrombus formation compared. Mean thrombus area and mean disappearance times were again significantly increased in all four groups injected with affinity-purified antibodies. This is the first study to show that anticardiolipin antibodies of IgG, IgM and IgA isotypes may play a role in thrombosis in vivo.

scholarly journals Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage

2019 ◽  
Vol 12 (3) ◽  
pp. e227171 ◽  
Author(s):  
Anju Adhikari ◽  
Mohammad Muhsin Chisti ◽  
Sanjog Bastola ◽  
Ojbindra KC
Keyword(s):  
Antiphospholipid Syndrome ◽  
Intracranial Haemorrhage ◽  
Clinical Course ◽  
Multiorgan Failure ◽  
Severe Form ◽  
High Dose ◽  
Cmv Infection ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Potential Trigger ◽  
Acute Cytomegalovirus

Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe form of antiphospholipid syndrome (APS). The syndrome manifests itself as a rapidly progressive multiorgan failure that is believed to be caused by widespread micro-thrombosis. Seldom does bleeding comanifest with thrombosis. We present a patient with APS who presented with nausea, vomiting and fatigue, and rapidly progressed into multiorgan failure before being diagnosed with CAPS. The clinical course was complicated by an atraumatic intracranial haemorrhage which demanded discontinuation of anticoagulation. The patient was treated with high dose steroid, intravenous immunoglobulin, followed by weekly rituximab infusion. Although the trigger for CAPS was not obvious during her hospital stay, she was diagnosed with acute cytomegalovirus (CMV) infection soon after discharge. In this case report, we explore the differential diagnoses of CAPS, investigate the possibility of CMV infection as a potential trigger, present the therapeutic challenges of anticoagulation and discuss the emerging use of rituximab.

Sign in / Sign up

Export Citation Format

Share Document