Antiphospholipid Syndrome

BACKGROUND: The genetic background plays an important role in thrombosis and pregnancy morbidities. Low levels of protein Z is associated with increased risk of thrombosis. The G79A polymorphism in the protein Z gene may be a genetic risk factor for thrombosis. AIM OF THE STUDY: To investigate the prevalence and clinical significance of the protein Z-79 G/A gene polymorphism in Egyptian patients with antiphospholipid syndrome (APS). METHODS: We genotyped 60 APS patients and 41 controls, for protein Z-79 G/A gene polymorphism using the PCR-restriction fragment length...

BACKGROUND: Heparin-induced thrombocytopenia (HIT) is a drug-induced, immunoglobulin G medicated autoimmune disorder associated with several negative clinical outcomes including increased morbidity, mortality, and increased medical costs. Previous studies have shown associations between comorbid autoimmune diseases, but there is little known about associations between HIT and autoimmunity. PURPOSE: To provide clinical data to suggest an association between HIT and autoimmunity...

OBJECTIVE: To assess outcomes after 20 weeks of pregnancy according to autoantibody profile and clinical presentation of maternal antiphospholipid syndrome (APS). METHODS: The present retrospective cohort analysis included women diagnosed with APS at a tertiary medical center in Israel between January 1, 2012, and December 31, 2016. Anticardiolipin antibodies, anti-β2-glycoprotein antibodies, and lupus anticoagulant were assessed. Participants were stratified by type of APS (obstetric vs thrombotic), antibody profile, and antibody titer (low vs high)...

Antiphospholipid syndrome (APS) patients have high cardiovascular risk. Speckle-tracking echocardiography (STE) detects myocardial function. To evaluate the myocardial function in primary APS (PAPS) patients using two-dimensional echocardiography (2-D) obtaining values of left ventricle global longitudinal strain (GLS) by STE. Patients with PAPS, > 16 years, both genders, without signs and symptoms of heart disease were recruited and matched with healthy controls by age and gender. Demographic, clinical data, and cardiovascular risk factors were recorded...

Background While thrombopoeitin (TPO) agonists that act to simulate platelet production have been approved for use in steroid-refractory chronic immune thrombocytopenia purpura (ITP), there are few data on the safety and efficacy of these medications in patients with concurrent systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS). Given that these agents can increase all hematopoietic cell lineages, it is unclear if there is an increased risk for exacerbation of the underlying lymphocyte-driven autoimmune disease in this population...

Objectives: APS is associated with arterial and venous thrombosis. The unfavourable fibrin clot phenotype, including formation of dense and poorly lysable clots, has been reported in thrombotic APS. We investigated whether abnormal plasma clot properties are predictive of recurrent thromboembolism in APS. Methods: We followed 126 consecutive patients with thrombotic APS and 105 control subjects, without APS, matched for thrombotic events. Plasma fibrin clot permeability (Ks), turbidity measurements and clot lysis time were evaluated ⩾5 months after a thrombotic event...

Atherosclerosis is regarded as one of the leading causes of mortality and morbidity in the world. Nowadays, it seems that atherosclerosis cannot be defined merely through the Framingham traditional risk factors and that autoimmunity settings exert a remarkable role in its mechanobiology. Individuals with autoimmune disorders show enhanced occurrence of cardiovascular complications and subclinical atherosclerosis. The mechanisms underlying the atherosclerosis in disorders like rheumatoid arthritis, systemic lupus erythematosus, antiphospholipid syndrome, systemic sclerosis and Sjögren's syndrome, seem to be the classical risk factors...

OBJECTIVE: Although systemic lupus erythematosus (SLE) is the most common autoimmune disease associated with antiphospholipid antibodies (aPL), limited data exist on the impact of SLE on the clinical phenotype of aPL-positive patients. The primary objective was to compare the clinical, laboratory, and treatment characteristics of aPL-positive patients with or without SLE. METHODS: A secure web-based data capture system stores patient demographics, and aPL-related clinical and laboratory characteristics...

An 83-year-old woman presented with acute-onset haemichorea and haemiballism particularly affecting the left side of the body. She was known to have type 2 diabetes, which was poorly controlled with sitagliptin. She was hyperglycaemic but not ketotic or acidotic. After she was started on insulin and good glycaemic control was achieved, her abnormal movements dramatically improved. MRI of the brain showed a T1-weighted hyperintense lesion on the right basal ganglia, which is typical of chorea-hyperglycaemia-basal ganglia syndrome...

Systemic lupus erythematosus (SLE) involves multiple organ systems and primarily affects women during their reproductive years. Pregnancy in a woman with SLE may lead to higher rates of disease flares. Little is known regarding which medications are safe to maintain remission and/or treat flares throughout such pregnancies. Here we retrospectively analyzed the efficacy of tacrolimus (TAC) in the pregnancy outcomes of SLE patients. We studied the 54 deliveries of 40 SLE patients over an eight-year period from 2008 to 2016...

Objective: Therapeutic plasma exchange (TPE) offers an alternative therapeutic modality for patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). However, there is conflicting evidence regarding its efficacy in different sub-phenotypes. This study aimed to investigate the main clinical characteristics and outcomes of patients with different phenotypes of SLE and APS treated with TPE at a tertiary care center. Methods: The database of the Blood and Apheresis Unit between 2001 and 2013 was screened for patients with SLE and primary APS...

Libman-Sacks endocarditis is a relatively rare sterile verrucous vegetative lesion observed in systemic lupus erythematosus (SLE)/antiphospholipid syndrome (APLS) patients. Most patients with this condition are asymptomatic. Here we report a case of a 46-year-old woman with APLS secondary to SLE complicated with frequent thromboembolic events due to a mitral valve mass. We performed minimally invasive mitral valve replacement with a mechanical prosthetic valve, and she was successfully discharged 14 days after surgery...

OBJECTIVE: Determination of lupus anticoagulants (LA) is an important, but still challenging test in the diagnosis of antiphospholipid syndrome (APS). This is especially the case in patients using one of the direct oral anticoagulants (DOACs). The aim of our study was to examine the influence of these drugs on DRVVT assays from two companies (in each case: screening test, confirming test and calculated ratio) and on aPTT and lupus-sensitive aPTT. METHODS: We used plasma samples from healthy volunteers spiked with the DOACs dabigatran, rivaroxaban and apixaban (0, 10, 30, 50, 100 ng/mL) for testing...

The direct oral anticoagulants (DOACs) are therapeutic alternatives to warfarin and other vitamin K antagonists (VKAs), and the standard of care for many indications. VKAs are conventional therapy for the treatment and secondary thromboprophylaxis of thrombotic antiphospholipid syndrome (APS), but are often problematic due to the variable sensitivity of thromboplastins to lupus anticoagulant. Thus, the International Normalised Ratio may not accurately reflect anticoagulation intensity, or be clinically effective...

Antiphospholipid antibody (aPL)‑mediated antiphospholipid syndrome (APS) is an autoimmune disease. Upon binding to aPL, the primary antigen of aPL, β2‑glycoprotein I (β2‑GP I), induces abnormal immune function, which further activates downstream signaling pathways in the cell and eventually leads to APS. The present study aimed to determine whether β2‑GP I antigen and anti‑β2‑glycoprotein I antibody (aβ2‑GP I), which belong to the aPL class of antibodies, may affect human chorionic epithelium cell (JEG‑3) proliferation, migration and invasion...

The clinical picture of systemic lupus and antiphospholipid syndrome is remarkably varied and disease manifestations are commonly very heterogeneous. Relatively often both diseases are associated with severe, acute and life threatening manifestations, which places demands on the knowledge of differential diagnostics and experience of the physicians. This article deals with the serious and mostly acute impairment of cardiovascular, respiratory, renal, gastrointestinal, hematopoietic or nervous systems, briefly discusses the acute pregnancy complication and summarizes the basic therapeutic option...

Objective: To report a case of a habitual aborter that had a pregnancy reach near term and successfully delivered a viable female infant. Design: Report of a unique case of a G10P1 that was successfully able to maintain a pregnancy by maintaining serum levels of estradiol and progesterone at or above 200 pg/dL and 25 ng/dL respectively. This case provides a benchmark for exogenous support of estradiol and progesterone throughout pregnancy. Setting: A private advanced reproductive center...

Acute mesenteric venous thrombosis (MVT) is the rarest cause of acute mesenteric ischaemia, so thrombosis of a variant inferior mesenteric vein (IMV) is especially uncommon in the setting of antiphospholipid syndrome (APS). Here, we present such a case of seronegative APS initially manifesting as an anomalous IMV thrombosis in a 76-year-old woman. Although guidelines support anticoagulation with vitamin K antagonists in these patients, we anticoagulated with rivaroxaban (a direct oral anticoagulant (DOAC)) due to patient preference, which resulted in complete clinical and endoscopic resolution...

Background: Recurrent miscarriage, also referred to as recurrent spontaneous abortion (RSA), affects 1 - 5% of couples and has a multifactorial genesis. Acquired and congenital thrombophilia have been discussed as hemostatic risk factors in the pathogenesis of RSA. Method: This review article was based on a selective search of the literature in PubMed. There was a special focus on the current body of evidence studying the association between RSA and antiphospholipid syndrome and hereditary thrombophilia disorders...

BACKGROUND: Increasing evidence has highlighted the role of non-criteria antiphospholipid antibodies (aPLs) as important supplements to the current criteria aPLs for the diagnosis of antiphospholipid syndrome (APS). In this retrospective study, we evaluated the clinical relevance of antibodies to phosphatidylserine/prothrombin (aPS/PT) in Chinese patients with APS. METHODS: A total of 441 subjects were tested, including 101 patients with primary APS (PAPS), 140 patients with secondary APS (SAPS), 161 disease controls (DCs) and 39 healthy controls (HCs)...