Antiphospholipid Syndrome In Adults Research Articles

Management of Cardiovascular Complications in Antiphospholipid Syndrome: A Narrative Review with a Focus on Older Adults.

Antiphospholipid syndrome (APS), also known as Hughes syndrome, is an acquired autoimmune and procoagulant condition that predisposes individuals to recurrent thrombotic events and obstetric complications. Central is the role of three types of antiphospholipid antibodies that target phospholipid-binding proteins: lupus anticoagulant (LAC), anti-β2-glycoprotein I (β2-GPI-Ab), and anti-cardiolipin (aCL). Together with clinical data, these antibodies are the diagnostic standard. However, the diagnosis of APS in older adults may be challenging and, in the diagnostic workup of thromboembolic complications, it is an underestimated etiology. The therapeutic management of APS requires distinguishing two groups with differential risks of thromboembolic complications. The standard therapy is based on low-dose aspirin in the low-risk group and vitamin K antagonists in the high-risk group. The value of direct oral anticoagulants is currently controversial. The potential role of monoclonal antibodies is investigated. For example, rituximab is currently recommended in catastrophic antiphospholipid antibody syndrome. Research is ongoing on other monoclonal antibodies, such as daratumumab and obinutuzumab. This narrative review illustrates the pathophysiological mechanisms of APS, with a particular emphasis on cardiovascular complications and their impact in older adults. This article also highlights advancements in the diagnosis, risk stratification, and management of APS.

The prevalence and incidence of thrombotic primary antiphospholipid syndrome in adults aged 18–49 years: A population-based study in a mountain community in northern Italy

ObjectiveTo estimate prevalence and incidence of thrombotic Primary Antiphospholipid Syndrome (PAPS) in the general population aged 18–49 years. MethodsThe study was carried out in Valtrompia, a valley in northern Italy, in 2011–2015. The identification of PAPS cases leveraged three integrated sources: 1) Rheumatology Unit at the University Hospital; 2) General Practitioners; 3) hospital discharge codes of patients admitted for thrombotic events. ResultsPrevalence and incidence were estimated as 22.9 (95% C.I. 11.4–41.0) and 5.0 (2.6–8.7) cases per 100,000 individuals, respectively. The estimates were 28.3 and 4.8, and 17.2 and 5.1 in males and females, respectively. The type of disease onset was mainly of arterial type in men and venous in women. ConclusionsThrombotic PAPS was found to be a rare disease in this population-based study. Prevalence and incidence were not significantly different between males and females aged 18–49 years, but a different type of onset was observed.

Antifosfolipidni sindrom kod dece

Antiphospholipid syndrome is a multisystemic autoimmune disease characterized by the appearance of arterial and/or venous thrombosis, often in multiple locations, thrombocytopenia and repeated spontaneous abortions in affected pregnant women, due to the permanent presence of antiphospholipid antibodies. It can also occur in children and manifest itself in any period of childhood, from infancy, due to transplacental transfer of maternal antibodies, or de novo production of autoantibodies, to adolescence. Clinical manifestations described in adults can also occur in children but are modified by age characteristics, such as the immaturity of the immune or other organ systems, greater exposure to viral and bacterial infections, implementation of routine immunization, absence of thrombogenic risk factors common to adults (hypertension, arteriosclerosis, consumption of cigarettes, alcohol and/or oral contraceptives), as well as the absence of pregnancy. Antiphospholipid antibodies most often used in daily clinical work are anticardiolipin antibodies, anti-b2 glycoprotein I antibodies, and lupus anticoagulants. Pathogenic mechanisms have not been investigated in detail - it is assumed that they are similar to the adult form of the disease. However, there is rare evidence of the existence of specific patterns of immune response to individual antigenic components, which may lead to specific production of antiphospholipid antibodies during childhood. It is also important to note that these antibodies can be found in about 25% of healthy children. The Sapporo criteria are used to diagnose antiphospholipid syndrome in adults. They include clinical (vascular thrombosis, repeated fetal losses) and laboratory parameters (persistent presence of one of the antiphospholipid antibodies) in the IgG and/or IgM class, in medium or high titer. The updated Sapporo criteria also apply to children. They include the same laboratory and clinical criteria, except for pregnancy pathology. The differential diagnosis is very broad and requires extensive diagnostics. Considering the specificity of the disease in childhood, the recommendations valid for adults, modified to a certain degree, apply to the treatment of diseases in children.

The challenges in diagnosing pediatric primary antiphospholipid syndrome

Pediatric primary antiphospholipid syndrome (APS) is a very rare disease with significant distinctions from the APS in adults. Herein, we present our experience in the diagnosis and treatment of six pediatric primary APS patients, who met the updated Sapporo criteria for the APS diagnosis. One of them was also diagnosed as having probable catastrophic APS (CAPS) due to the involvement of three different organ systems simultaneously. Besides vascular involvement, four patients had thrombocytopenia, one had psychiatric disorder, and one had chorea and valvular heart disease. All patients received immunosuppressive treatment along with long-term anticoagulation therapy. Specific neurologic and hematologic manifestations that are not part of the classification criteria can be seen in children with primary APS. Therefore, using the adult criteria for diagnosing pediatric APS may result in missed or delayed diagnoses in children.

Treatment of thrombotic antiphospholipid syndrome in adults and children.

Antiphospholipid syndrome (APS), more common than once believed, is an autoimmune disease best known for its high risk of incident and recurrent thrombotic events. The approach to treatment potentially differs from treatment of thrombosis in the general population, and this article endeavors to review the latest updates on this topic. The epidemiology of APS is being increasingly elucidated by large population-based studies, with APS perhaps affecting as many as 1 in 2000 individuals. Vitamin K antagonists, aspirin, and heparinoids continue to have obvious roles in the management of patients with APS. There has recently been intensive study of direct oral anticoagulants in APS, with the most recent randomized studies raising concerns about their inferiority to vitamin K antagonists, at least in some subgroups. Other approaches to treating APS beyond anticoagulants and antiaggregants are also receiving increased attention in mechanistic and preclinical studies with an eye toward future roles in patients with refractory and/or microvascular disease. Pediatric APS is identified as an area in desperate need of additional prospective research. Progress continues to be made in pursuit of improving the lives of individuals afflicted with APS. The most important future directions would seem to involve leveraging modern molecular technologies in order to improve subphenotyping of antiphospholipid antibody-positive individuals. This will help personalize risk profiles and ideally define the optimal approach to therapy based on future risk, rather than past morbid events.

EULAR recommendations for the management of antiphospholipid syndrome in adults

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...

Preporuke Evropske lige protiv reumatizma (EULAR) za profilaksu i lečenje antifosfolipidnog sindroma kod odraslih

Antiphospholipid syndrome (APS) is a rare disease characterised by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid (aPL) antibodies. The European League Against Rheumatism, EULAR, has published a set of recommendations for the management of antiphospholipid syndrome in adults, based on the systematic literature reviews (SLRs) results and expert opinions. Low dose aspirin (LDA) is recommended for asymptomatic aPL carriers, patients with systemic lupus erythematosus without prior thrombotic or obstetric APS, and nonpregnant women with a history of obstetric APS only, all with high-risk aPL profiles. Patients with APS and the 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 the first arterial thrombosis, treatment with VKA with INR 2-3 or INR 3-4 is recommended, considering the individual's bleeding/ thrombosis risk. 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.

The Ped-APS Registry: the antiphospholipid syndrome in childhood

In recent years, antiphospholipid syndrome (APS) has been increasingly recognised in various paediatric autoimmune and nonautoimmune diseases, but the relatively low prevalence and heterogeneity of APS in childhood made it very difficult to study in a systematic way. The project of an international registry of paediatric patients with APS (the Ped-APS Registry) was initiated in 2004 to foster and conduct multicentre, controlled studies with large number of paediatric APS patients. The Ped-APS Registry is organised as a collaborative project of the European Forum on Antiphospholipid Antibodies and Juvenile Systemic Lupus Erythematosus Working Group of the Paediatric Rheumatology European Society. Currently, it documents a standardised clinical, laboratory and therapeutic data of 133 children with antiphospholipid antibodies (aPL)-related thrombosis from 14 countries. The priority projects for future research of the Ped-APS Registry include prospective enrollment of new patients with aPL-related thrombosis, assessment of differences between the paediatric and adult APS, evaluation of proinflammatory genotype as a risk factor for APS manifestations in childhood and evaluation of patients with isolated nonthrombotic aPL-related manifestations.

Review: Antiphospholipid antibodies in pediatric systemic lupus erythematosus and the antiphospholipid syndrome

The antiphospholipid syndrome (APS) is recognized increasingly as the most common acquired hypercoagulation state of autoimmune etiology and may occur as an isolated clinical entity (primary APS) or in association with an underlying systemic disease, particularly systemic lupus erythematosus (SLE). The major differences between pediatric and adult APS include absence of common acquired risk factors for thrombosis, absence of pregnancy-related morbidity, increased incidence of infection-induced antibodies, differences in cut-off values for determination of aPL and specific factors regarding long-term therapy in children. APS in children has been largely reported in patients with arterial or venous thromboses and less frequently in association with neurological or hematological manifestations. The presence of aPL in pediatric SLE can modify the disease expression and may be an important predictor of the development of irreversible organ damage. Two recently established international registries of neonates and children with APS provide a good opportunity to conduct large, prospective studies on the clinical significance of aPL and long-term outcome of pediatric APS.

Cardiovascular Surgical Outcomes in Patients with the Antiphospholipid Syndrome—A Case-Series

Patients with the antiphospholipid syndrome (APLS) have severe involvement of the cardiovascular apparatus and often need surgical interventions to correct these manifestations. Few studies that have looked at the outcomes of cardiothoracic surgeries in APLS patients have reported high rates of perioperative mortality and thromboembolic events. Our goal was to examine the outcomes of adult APLS patients undergoing coronary artery bypass surgery (CABG) and/or valvular surgery. We also wanted to determine whether aggressive anticoagulation therapy could prevent life threatening thromboembolic complications in these patients. We retrospectively reviewed medical records of nine patients with primary APLS undergoing cardiothoracic surgery between 1985 and 2005 at our institution. Patient demographics, operative procedures and one-year clinical outcomes were obtained. Forty-five percent of our patients had more than three cardiovascular risk factors other than APLS. There were no mortalities in our case series. However, 89% of our patients developed major complications. Despite aggressive anticoagulation, 37.5% developed thromboembolic events including cerebrovascular accidents, myocardial infarctions and vena caval thrombosis. Other complications included heparin-induced thrombocytopenia, redo of CABG surgery and sepsis. Despite aggressive anticoagulation and lack of significant pre-operative co-morbidities, APLS patients undergoing cardiothoracic surgery appear to have high rates of post-operative clinical events.