antiphospholipid guidelines

OBJECTIVE: Hepatic infarction is a rare complication of pregnancy most often associated with hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. The objective of this review is to identify risk factors, presenting signs and symptoms, methods of diagnosis, and best management practices based on published case reviews. DATA SOURCES: PUBMED and MEDLINE: OVID databases were searched for citations regarding hepatic infarction in pregnancy or the postpartum period since database inception until the study date of December 18, 2023...

Venous thromboembolism (VTE) is frequently encountered across various specialties. The management of VTE has become more nuanced, requiring consideration of several factors when deciding on the choice and duration of anticoagulation. This evidence-based review article summarises the current practice and evidence behind anticoagulation in VTE, incorporating national and international guidelines. Factors influencing decision-making around the choice and duration of anticoagulation, along with special circumstances such as cancer and antiphospholipid syndrome, are discussed...

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality...

Background: Thromboembolic disease is associated with a high rate of disability or death and gravely jeopardizes people's health and places considerable financial pressure on society. The primary treatment for thromboembolic illness is anticoagulant medication. Fondaparinux, a parenteral anticoagulant medicine, is still used but is confusing due to its disparate domestic and international indications and lack of knowledge about its usage. Its off-label drug usage in therapeutic settings and irrational drug use are also common...

Thrombocytopenia is one of the most common manifestations of antiphospholipid syndrome (APS). There is little evidence or definitive guidelines regarding the treatment of APS with thrombocytopenia. We describe a patient with APS and moderate-to-severe thrombocytopenia and the challenges of balancing anticoagulation with thrombocytopenia. A 19-year-old male patient presented with right lower limb swelling to the emergency department with a history of gradually worsening right leg swelling for 1 week and was diagnosed with right leg proximal deep vein thrombosis...

Purpose These recommendations issued by the AGG (Section Maternal Diseases in Pregnancy) were developed as a rapid orientation on maternal rheumatic diseases for counselling and disease management in pregnancy and breastfeeding. Methods The standard literature, consensus and position papers, guidelines and recommendations by other specialist associations were evaluated by a task force of the Section and summarized in these recommendations following a joint consensus process. Recommendations This paper provides an orientating overview of the physiology, pathophysiology and definitions of rheumatic diseases which is relevant for gynecologists and obstetricians...

BACKGROUND: Primary hypercoagulable disorders pose a significant challenge to microsurgeons and have traditionally been regarded as a relative contraindication to free tissue transfer. Since free flaps offer numerous advantages in breast reconstruction, there is an effort to expand the population to whom these operations can be safely offered. The purpose of this study is to describe our chemoprophylaxis regimen in cases of primary hypercoagulability, as well as to compare flap outcomes and complications between women with and without hypercoagulability...

In this review, we discuss the current evidence on classic and newer oral anticoagulant therapy, older drugs such as HCQ and statins, and new potential treatment targets in APS. Vitamin K antagonists (VKAs) remain the cornerstone treatment for thrombotic events in APS. In patients fulfilling criteria for definite APS presenting with a first venous thrombosis, treatment with VKAs with a target international normalized ratio (INR) 2.0-3.0 is recommended. In patients with arterial thrombosis, treatment with VKA with target INR 2...

BACKGROUND Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome characterized by widespread thrombotic complications leading to multiorgan ischemia and failure. Although there are no standard treatment guidelines for CAPS, it often involves triple therapy with anticoagulation, corticosteroids, and plasma exchange. Recently, biologics such as rituximab and eculizumab have also shown promise as potential new therapies for CAPS, as observed in our case. CASE REPORT We describe a 59-year-old female patient who presented with altered mental status and diffuse weakness...

Multiple factors contribute to recurrent pregnancy loss (RPL). This review highlights the latest international guidelines for RPL workup, including immunological testing, by the American Society for Reproductive Medicine (ASRM), the European Society of Human Reproduction and Embryology (ESHRE), and the Royal College of Obstetricians and Gynaecologists (RCOG). These three societies recommend testing for antiphospholipid syndrome. ESHRE and RCOG also recommend thyroid peroxidase antibody testing, whereas ASRM does not...

Antiphospholipid syndrome (APS) is a rare autoimmune disease characterized by arterial, venous, or microvascular thrombosis, pregnancy morbidities, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies. These antibodies bind cellular phospholipids and phospholipid-protein complexes resulting in cellular activation and inflammation that lead to the clinical features of APS. Our evolving understanding of APS has resulted in more specific classification criteria. Patients meeting these criteria should be treated during pregnancy according to current guidelines...

Antiphospholipid antibodies (aPL) are autoimmune antibodies directed toward phospholipids or phospholipid-protein complexes, particularly those containing β2-glycoprotein I (β2GPI). Persistently positive aPL accompanied by arterial or venous thrombosis, or recurrent pregnancy loss, constitutes the antiphospholipid syndrome (APS). Several types of aPL with different specificities have been defined and may be detected in the clinical lab, including lupus anticoagulants (detected using clotting assays) and anticardiolipin, anti-β2GPI and anti-prothrombin/phosphatidylserine antibodies (detected by ELISA); each of the last 3 aPL may be either IgG, IgM, or IgA, though IgA antibodies are not included in criteria for APS...

Lupus anticoagulant (LA) is one of three tests identified as laboratory criteria for definite antiphospholipid syndrome (APS). The other two tests are anticardiolipin antibody (aCL) and anti-β2-glycoprotein I (aβ2GPI) antibody. The presence of LA is assessed using clot-based tests, while the presence of aCL and aβ2GPI is assessed by immunological assays. Since no test can be considered 100% sensitive or specific for LA, current guidelines recommend using two different clot-based assays reflecting different principles, with the dilute Russell viper venom time (dRVVT) and activated partial thromboplastin time (aPTT) recommended...

RATIONALE: Thrombotic microangiopathy (TMA) is a spectrum of rare diseases characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ damage. Differentiating pre-eclampsia, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelets) syndrome and atypical hemolytic uremic syndrome (aHUS) during pregnancy may be diagnostically challenging yet important as the treatment pathways differ. Most cases of aHUS are associated with dysregulation of the complement alternative pathway, for which current guidelines recommend prompt treatment with complement C5 inhibitor to prevent chronic sequelae...

Recurrent pregnancy loss (RPL) can be a devastating experience for individuals trying to have children. Various potential causes contribute to the multifactorial pathogenesis of RPL, including chromosomal anomalies, endocrine conditions, autoimmunity, thrombophilias, and infectious agents. Antinuclear antibodies (ANAs) offer an unspecific clue to the underlying autoimmune pathogenic etiology of RPL. This case details a 40-year-old female with a history of RPL, fibromyalgia, and ANA positivity, who spontaneously developed lupus anticoagulant antibodies during her second trimester of pregnancy...

PROBLEM: The current standard prevention of obstetric complications in patients with antiphospholipid antibody syndrome (APS) is the use of combination low-dose aspirin and low molecular weight heparin. However, 20-30% of women still experience refractory obstetrical APS. Hydroxychloroquine (HCQ) is an immunomodulatory agent that has been shown in laboratory studies to decrease thrombosis risk, support placentation, and minimize the destructive effects of antiphospholipid antibodies. The objective of this study was to evaluate the risk of pregnancy loss upon treatment with HCQ among women with refractory obstetrical APS...

Cerebral venous thrombosis (CVT) is a rare disorder predominantly affecting young women. Clinical presentation is not specific and varies depending on the location of the thrombus. The diagnosis requires clinical suspicion with confirmation by images. Guidelines for treatment recommend heparin during the acute phase even in patients with intraparenchymal hemorrhage. It is associated with a good prognosis when diagnosed and treated promptly. We present a case of CVT and intraparenchymal hemorrhage in a 17-year-old female with severe headache, nausea, vomiting, and altered mental status...

BACKGROUND: To examine blood pressure (BP) and lipid treatment eligibility in antiphospholipid syndrome (APS) according to European Alliance of Associations for Rheumatology (EULAR) and European Society of Cardiology (ESC) recommendations. METHODS: Systematic Coronary Risk Evaluation (SCORE), modified-SCORE, diabetes mellitus (DM)-equivalent risk classifiers (DIME) and disease-related classifiers -type of thrombotic events (APSevents ), antiphospholipid-antibody profile (aPLprofile ) and adjusted Global APS Score for cardiovascular disease- were used to calculate predicted low-moderate, high and very-high cardiovascular risk (CVR) in 111 patients with APS without prior atherosclerotic cardiovascular events or DM...

BACKGROUND: Practice variation in recurrent pregnancy loss (RPL) care is common. International guidelines vary in their recommendations for the management of RPL couples, which could lead to an increase of cross border reproductive care. Currently, the Dutch RPL guideline is being adapted from the European Society for Human Reproduction and Embryology (ESHRE) guideline. We aim to identify discrepancies between RPL guidelines and RPL practice. These discrepancies could be considered in the development of a new guideline and implementation strategies to promote adherence to new recommendations...

Even though diagnosis and management pathways have been substantially improved over the last years, autoimmune rheumatic diseases (AIRDs) such as rheumatoid arthritis, systemic sclerosis, systemic lupus erythematosus, antiphospholipid syndrome, Sjögren's syndrome, and systemic vasculitides have been linked to elevated rates of cardiovascular morbidity and mortality, primarily secondary to accelerated atherosclerosis. This phenomenon can be partially attributed to the presence of established cardiovascular risk factors but may also be a result of other inflammatory and autoimmune mechanisms that are enhanced in AIRDs...