scholarly journals EULAR recommendations for the management of antiphospholipid syndrome in adults

2019 ◽  
Vol 78 (10) ◽  
pp. 1296-1304 ◽  
Author(s):  
Maria G Tektonidou ◽  
Laura Andreoli ◽  
Marteen Limper ◽  
Zahir Amoura ◽  
Ricard Cervera ◽  
...  
Keyword(s):  
High Risk ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Low Dose ◽  
Antiphospholipid Antibody ◽  
Adequate Treatment ◽  
Thrombosis Risk ◽  
Long Term Treatment ◽  
Obstetric Aps

The objective was to develop evidence-based recommendations for the management of antiphospholipid syndrome (APS) in adults. Based on evidence from a systematic literature review and expert opinion, overarching principles and recommendations were formulated and voted. High-risk antiphospholipid antibody (aPL) profile is associated with greater risk for thrombotic and obstetric APS. Risk modification includes screening for and management of cardiovascular and venous thrombosis risk factors, patient education about treatment adherence, and lifestyle counselling. Low-dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and non-pregnant women with a history of obstetric APS only, all with high-risk aPL profiles. Patients with APS and first unprovoked venous thrombosis should receive long-term treatment with vitamin K antagonists (VKA) with a target international normalised ratio (INR) of 2–3. In patients with APS with first arterial thrombosis, treatment with VKA with INR 2–3 or INR 3–4 is recommended, considering the individual’s bleeding/thrombosis risk. Rivaroxaban should not be used in patients with APS with triple aPL positivity. For patients with recurrent arterial or venous thrombosis despite adequate treatment, addition of LDA, increase of INR target to 3–4 or switch to low molecular weight heparin may be considered. In women with prior obstetric APS, combination treatment with LDA and prophylactic dosage heparin during pregnancy is recommended. In patients with recurrent pregnancy complications, increase of heparin to therapeutic dose, addition of hydroxychloroquine or addition of low-dose prednisolone in the first trimester may be considered. These recommendations aim to guide treatment in adults with APS. High-quality evidence is limited, indicating a need for more research.

The antiphospholipid (antibody) syndrome

2011 ◽  
pp. 343-352
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Lupus Anticoagulant ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Pregnancy Morbidity

Introduction 344 Epidemiology and pathology 345 Clinical features of antiphospholipid syndrome 346 Treatment of antiphospholipid syndrome 348 Catastrophic antiphospholipid syndrome 350 The antiphospholipid syndrome (APS) was first described in the 1980s and comprises arterial and venous thrombosis with or without pregnancy morbidity in the presence of anticardiolipin (ACL) antibodies or the lupus anticoagulant (LAC). It can be primary, or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) (...

scholarly journals Genetic Risk Factors for Thrombosis in Systemic Lupus Erythematosus

2012 ◽  
Vol 39 (8) ◽  
pp. 1603-1610 ◽  
Author(s):  
RACHEL KAISER ◽  
YONGHONG LI ◽  
MONICA CHANG ◽  
JOSEPH CATANESE ◽  
ANN B. BEGOVICH ◽  
...  
Keyword(s):  
Risk Factors ◽  
Systemic Lupus Erythematosus ◽  
Venous Thrombosis ◽  
Lupus Erythematosus ◽  
Genetic Risk ◽  
Genetic Risk Factors ◽  
Antiphospholipid Antibody ◽  
Systemic Lupus ◽  
Discovery Cohort ◽  
Thrombosis Risk

Objective.Thrombosis is a serious complication of systemic lupus erythematosus (SLE). We investigated whether genetic variants implicated in thrombosis pathways are associated with thrombosis among 2 ethnically diverse SLE cohorts.Methods.Our discovery cohort consisted of 1698 patients with SLE enrolled in the University of California, San Francisco, Lupus Genetics Project and our replication cohort included 1361 patients with SLE enrolled in the PROFILE cohort. Patients fulfilled American College of Rheumatology SLE criteria, and data relevant to thrombosis were available. Thirty-three single nucleotide polymorphisms (SNP) previously shown to be associated with risk of deep venous thrombosis in the general population or implicated in thrombosis pathways were genotyped and tested for association with thrombosis in bivariate allelic analyses. SNP with p < 0.1 in the bivariate analyses were further tested in multivariable logistic regression models adjusted for age, sex, disease duration, antiphospholipid antibody status, smoking, nephritis, and medications.Results.In the discovery cohort, 23% of patients with SLE experienced a thrombotic event. SNP in the following genes demonstrated association with thrombosis risk overall in the discovery or replication cohorts and were assessed using metaanalytic methods: factor V Leiden (FVL) rs6025 (OR 1.85, p = 0.02) and methylenetetrahydrofolate reductase (MTHFR) rs1801133 (OR 0.75, p = 0.04) in whites, and fibrinogen gamma (FGG) rs2066865 (OR 1.91, p = 0.01) in Hispanic Americans. SNP in these genes showed association with venous thrombosis risk in whites: MTHFR rs1801131 (OR 1.51, p = 0.01), MTHFR rs1801133 (OR 0.70, p = 0.04), FVL rs6025 (OR 2.69, p = 0.002), and FGG rs2066865 (OR 1.49, p = 0.02) in whites. A SNP in FGG rs2066865 (OR 2.19, p = 0.003) demonstrated association with arterial thrombosis risk in Hispanics.Conclusion.Our results implicate specific genetic risk factors for thrombosis in patients with SLE and suggest that genetic risk for thrombosis differs across ethnic groups.

scholarly journals Improvements in diagnosis and risk assessment of primary and secondary antiphospholipid syndrome

Hematology ◽  
2019 ◽  
Vol 2019 (1) ◽  
pp. 415-420 ◽  
Author(s):  
Michelle Petri
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Risk Identification ◽  
Classification Criteria ◽  
Antiphospholipid Antibody ◽  
Systemic Lupus Erythematosus Disease ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Glycoprotein I ◽  
Thrombosis Risk

Abstract Classification criteria for antiphospholipid syndrome have not been updated since the revised Sapporo classification criteria were published in 2006. These criteria have limitations in that they omit nonclassical manifestations (hematologic and neurologic), include anticardiolipin and anti–β2-glycoprotein I immunoglobulin (Ig)M isotypes, and do not separately consider primary (no autoimmune disease) or secondary (usually systemic lupus erythematosus) disease. Recent findings in antiphospholipid antibody include fluctuation of antiphospholipid antibodies, recognition that IgA isotypes do confer risk, identification of the role of complementopathy in catastrophic antiphospholipid syndrome, and elucidation of the role of thrombosis risk equations.

scholarly journals Upper extremity deep venous thrombosis: risk factors, diagnosis, treatment

2016 ◽  
Vol 11 (1) ◽  
pp. 28-32
Author(s):  
Camelia C. DIACONU ◽  
◽  
Mădălina ILIE ◽  
Mihaela Adela IANCU ◽  
◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Embolism ◽  
High Risk ◽  
Venous Thrombosis ◽  
Deep Venous Thrombosis ◽  
Upper Extremity ◽  
Brachiocephalic Vein ◽  
Risk Of Death ◽  
Thrombosis Risk ◽  
Effort Thrombosis

Upper extremity deep venous thrombosis is a condition with increasing prevalence, with high risk of morbidity and mortality, due to embolic complications. In the majority of the cases, thrombosis involves more than one venous segment, most frequently being affected the subclavian vein, followed by internal jugular vein, brachiocephalic vein and basilic vein. Upper extremity deep venous thrombosis in patients without risk factors for thrombosis is called primary deep venous thrombosis and includes idiopathic thrombosis and effort thrombosis. Deep venous thrombosis of upper extremity is called secondary when there are known risk factors and it is encountered mainly in older patients, with many comorbidities. The positive diagnosis is established only after paraclinical and imaging investigations, ultrasonography being the most useful diagnostic method. The most important complication, with high risk of death, is pulmonary embolism. Treatment consists in anticoagulant therapy, for preventing thrombosis extension and pulmonary embolism.

Determinants of Risk for Venous and Arterial Thrombosis in Primary Antiphospholipid Syndrome and in Antiphospholipid Syndrome with Systemic Lupus Erythematosus

2009 ◽  
Vol 36 (6) ◽  
pp. 1195-1199 ◽  
Author(s):  
ADRIANA DANOWSKI ◽  
MARIO NEWTON LEITÃO de AZEVEDO ◽  
JOSE ANGELO de SOUZA PAPI ◽  
MICHELLE PETRI
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Pregnancy Loss ◽  
Fold Increase ◽  
Systemic Lupus ◽  
Hereditary Thrombophilia

Objective.Antiphospholipid syndrome (APS) is characterized by thrombosis (venous and arterial) and pregnancy loss in conjunction with the lupus anticoagulant, IgG or IgM anticardiolipin, or IgG or IgM anti-ß2-glycoprotein I. In most series, only a minority of patients with antiphospholipid antibodies develop a clinical manifestation.Methods.A cross-sectional study of consecutive patients in the Hopkins Lupus Center was performed. Interviews were done and records were reviewed for the following variables: gender, ethnicity, hypertension, triglycerides, cholesterol, smoking, diabetes mellitus, homocysteine, cancer, hepatitis C, hormone replacement therapy/oral contraceptives, hereditary thrombophilia, anticardiolipin antibodies IgG, IgM and IgA, and lupus anticoagulant (LAC). Our aim was to identify risk factors associated with thrombosis and pregnancy loss in patients with antiphospholipid antibodies.Results.A total of 122 patients (84% female, 74% Caucasian) were studied. Patients were divided into 3 groups: primary APS, APS associated with systemic lupus erythematosus, and patients with systemic lupus erythematosus (SLE) with antiphospholipid antibodies but no thrombosis or pregnancy loss. Venous thrombosis was associated with high triglycerides (p = 0.001), hereditary thrombophilia (p = 0.02), anticardiolipin antibodies IgG > 40 (p = 0.04), and LAC (p = 0.012). Hypertriglyceridemia was associated with a 6.4-fold increase, hereditary thrombophilia with a 7.3-fold increase, and anticardiolipin IgG > 40 GPL with a 2.8-fold increase in the risk of venous thrombosis. Arterial thrombosis was associated with hypertension (p = 0.008) and elevated homocysteine (p = 0.044). Hypertension was associated with a 2.4-fold increase in the risk of arterial thrombosis. No correlations were found for pregnancy loss.Conclusion.The frequency of thrombosis and pregnancy loss is greater in APS associated with SLE than in primary APS. Risk factors differ for venous and arterial thrombosis in APS. Treatment of hypertension may be the most important intervention to reduce arterial thrombosis. Elevated triglycerides are a major associate of venous thrombosis, but the benefit of treatment is not known. Hereditary thrombophilia is an associate of venous but not arterial thrombosis, making it cost-effective to investigate only in venous thrombosis.

scholarly journals The Wolf Hidden behind the Clots: Catastrophic Antiphospholipid Antibody Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Myat Han Soe ◽  
Krishna Adit Agarwal ◽  
Alueshima Akough-Weir
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Anticoagulation Therapy ◽  
Multiple Organ ◽  
Clinical Syndrome ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Case Review ◽  
Cmv Infection ◽  
Thrombophilic Condition

Catastrophic antiphospholipid syndrome (CAPS) is a rare but highly fatal clinical syndrome that occurs in up to 1% of patients with antiphospholipid syndrome (APS). The diagnosis of CAPS is often delayed because its presentation with multiple organ thromboses can be confused with other thrombotic microangiopathies and severe sepsis. We report a case of CAPS in a patient with APS and systemic lupus erythematosus (SLE) presenting with thrombotic storm precipitated by trauma, cytomegalovirus (CMV) infection, and noncompliance with anticoagulation therapy. Our case reflects the “two-hit hypothesis” of APS in which the presence of antiphospholipid antibodies (first hit) increases the thrombophilic risk, and thromboses take place in the presence of another thrombophilic condition such as CMV infection in our case. In this case review, we discuss the diagnostic challenges and management of CAPS. In clinical practice, we aim to stress the importance of thorough evaluation and management of precipitating events such as infections in addition to timely diagnosis and treatment of this catastrophic clinical entity.

scholarly journals Digital ischaemia secondary to adalimumab-induced antiphospholipid syndrome

2020 ◽  
Vol 13 (2) ◽  
pp. e232907 ◽  
Author(s):  
Shashank Cheemalavagu ◽  
Sara S McCoy ◽  
Jason S Knight
Keyword(s):  
Antiphospholipid Syndrome ◽  
Low Dose ◽  
Antiphospholipid Antibody ◽  
Antibody Testing ◽  
Left Hand ◽  
Dose Aspirin ◽  
Low Dose Aspirin ◽  
History Of ◽  
Digital Ischaemia ◽  
Necrosis Factor

A 50-year-old woman with a history of Crohn’s disease treated with adalimumab presented with left hand pain and duskiness. Angiogram showed non-filling of the radial and digital arteries of the hand. Antiphospholipid antibody testing was positive, leading to a diagnosis of antitumour necrosis factor-induced antiphospholipid syndrome. Adalimumab was discontinued, and she was treated with the vitamin K antagonist warfarin and low-dose aspirin. Upon resolution of the antiphospholipid antibodies, she was transitioned to aspirin alone without recurrence of thrombosis.

Detection and Prevention of Deep Venous Thrombosis

1988 ◽  
Vol 22 (2) ◽  
pp. 107-114 ◽  
Author(s):  
Andra J. Melamed ◽  
Jeanette Suarez
Keyword(s):  
High Risk ◽  
Venous Thrombosis ◽  
Deep Venous Thrombosis ◽  
Low Dose ◽  
Diagnostic Techniques ◽  
Prior History ◽  
Dose Heparin ◽  
Prophylactic Measures ◽  
Patients At Risk ◽  
Low Dose Heparin

Deep venous thrombosis (DVT) is a significant problem in the postoperative course of high-risk patients. Risk factors that further predispose patients to DVT include obesity, age over 40 years, smoking, dehydration, and a prior history of thromboembolism. Diagnosis of DVT by physical examination and medical history is difficult; objective diagnostic techniques are often required. Considerable emphasis has been placed on the cost-effectiveness of implementing prophylactic measures in patients who are at high risk for developing DVT. Physical maneuvers attempt to reduce stasis and enhance venous return and pharmacologic approaches alter blood coagulability. The drug therapy used in preventing DVT consists of dextran, low-dose heparin, a combination of low-dose heparin and dihydroergotamine, and warfarin. Effective prophylactic regimens differ according to the type of patients at risk. Prophylactic therapy should be tailored according to the patient's disease and degree of risk.

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