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Current Rheumatology Reports Jan 2021Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome... (Review)
Review
PURPOSE OF REVIEW
Elucidating the pathogenic mechanisms mediated by antiphospholipid antibodies (aPL) might exert important clinical implications in pediatric antiphospholipid syndrome (APS).
RECENT FINDINGS
aPL are traditionally regarded as the main pathogenic players in APS, inducing thrombosis via the interaction with fluid-phase and cellular components of coagulation. Recent APS research has focused on the role of β2 glycoprotein I, which bridges innate immunity and coagulation. In pediatric populations, aPL should be screened in appropriate clinical settings, such as thrombosis, multiple-organ dysfunction, or concomitant systemic autoimmune diseases. Children positive for aPL tests often present non-thrombotic non-criteria manifestations or asymptomatic aPL positivity. In utero aPL exposure has been suggested to result in developmental disabilities, warranting long-term follow-up. The knowledge of the multifaceted nature of pediatric APS should be implemented to reduce the risk of underdiagnosing/undertreating this condition. Hopefully, recent pathogenic insights will open new windows of opportunity in the management of pediatric APS.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Child; Humans; Thrombosis
PubMed: 33511497
DOI: 10.1007/s11926-020-00976-7 -
Journal of Intensive Care Medicine 2006Catastrophic antiphospholipid syndrome is a rapidly progressive life-threatening disease that causes multiple organ thromboses and dysfunction in the presence of... (Review)
Review
Catastrophic antiphospholipid syndrome is a rapidly progressive life-threatening disease that causes multiple organ thromboses and dysfunction in the presence of antiphospholipid antibodies. A high index of clinical suspicion and careful investigation are required to make an early diagnosis so that treatment with anticoagulation and corticosteroids can be initiated; plasma exchange and/or intravenous immunoglobulins can be added if the life-threatening condition persists. Despite aggressive treatment and intensive care unit management, patients with catastrophic antiphospholipid syndrome have a 48% mortality rate, primarily attributable to cardiopulmonary failure. This article reviews the current information on the etiopathogenesis, clinical manifestations, diagnosis, management, and prognosis of catastrophic antiphospholipid syndrome.
Topics: Algorithms; Antiphospholipid Syndrome; Catastrophic Illness; Humans
PubMed: 16672637
DOI: 10.1177/0885066606287041 -
Current Opinion in Hematology Sep 2019The purpose of this review is to summarize the diagnosis and management of the antiphospholipid syndrome (APS) in children. (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to summarize the diagnosis and management of the antiphospholipid syndrome (APS) in children.
RECENT FINDINGS
APS is a rare, acquired autoimmune systemic disease that can result in significant morbidity in children related to vascular thrombosis. The diagnosis and management of APS in children can be challenging due to a lack of validated diagnostic criteria and the rarity of the disease. In addition, many healthy children have transient circulating antiphospholipid antibodies without thrombotic complications. Nevertheless, epidemiological studies suggest that APS represents a greater relative proportion of thrombotic disease in children than it does in adults. Management of pediatric APS is largely inferred from adult data despite unique characteristics of pediatric APS. The current recommendations include long-term anticoagulation, which can be problematic in young, active individuals. There is little data on potential benefits of nonantithrombotic therapy in the management of pediatric APS.
SUMMARY
Data on pediatric APS are limited, but evidence suggests that using current available diagnostic testing is valuable and, until further evidence is available, treating thrombotic complications with heparins or warfarin should be standard of care.
Topics: Adolescent; Antiphospholipid Syndrome; Child; Female; Heparin; Humans; Male
PubMed: 31348049
DOI: 10.1097/MOH.0000000000000523 -
Current Rheumatology Reports Oct 2003Catastrophic antiphospholipid syndrome, defined and documented in 1992, is a potentially fatal complication seen in patients with antiphospholipid antibody. It may arise... (Review)
Review
Catastrophic antiphospholipid syndrome, defined and documented in 1992, is a potentially fatal complication seen in patients with antiphospholipid antibody. It may arise de novo in patients not previously suspected as having an antiphospholipid syndrome, or it may complicate the course of patients currently treated for this syndrome. Precipitating or "trigger" factors have been identified in 55% of patients; the most common of these factors is infection. The precipitating factors should be avoided or energetically treated in patients with the antiphospholipid syndrome in order to prevent this "catastrophic" course. The clinical manifestations are those of multiorgan failure, and unusual vessels or organs can be involved. Treatment of the condition, with emphasis on effective anticoagulation, intravenous steroids, intravenous immunoglobulin, or plasma exchange, should be aggressive to achieve a satisfactory outcome. Regrettably, despite all available therapeutic options at this time, the mortality is still high (greater than 50%).
Topics: Antiphospholipid Syndrome; Catastrophic Illness; Humans
PubMed: 12967527
DOI: 10.1007/s11926-003-0031-7 -
Current Opinion in Hematology Sep 2000In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of... (Review)
Review
In its classic presentation, the antiphospholipid syndrome manifests a combination of venous or arterial thrombosis and fetal loss, accompanied by elevations of antibodies directed toward negatively charged phospholipids, as measured by anticardiolipin antibody assays and/or positive lupus anticoagulant tests. The manifestations often include a moderate thrombocytopenia and, less commonly, hemolysis. In contrast, a less frequently encountered subset of the antiphospholipid syndrome, termed the "catastrophic" antiphospholipid syndrome, affects mainly small vessels predominantly supplying organs. The thrombocytopenia is usually marked, and a Coombs positive microangiopathic-type anemia may accompany the condition. Features of disseminated intravascular coagulation may be evident in some patients. It is fatal in approximately 50% of cases reported. Treatment should include not only adequate anticoagulation with intravenous heparin but also full doses of intravenous corticosteroids, to offset the systemic inflammatory response syndrome that occurs as a result of the extensive tissue damage, and plasmapheresis, using fresh frozen plasma. Parenteral antibiotics should be administered early if infection is suspected.
Topics: Antiphospholipid Syndrome; Catastrophic Illness; Humans; Microcirculation; Multiple Organ Failure; Thrombosis
PubMed: 10961585
DOI: 10.1097/00062752-200009000-00012 -
Nature Clinical Practice. Rheumatology Feb 2006Antiphospholipid syndrome (APS) is a thrombotic disorder associated with autoantibodies that target membrane phospholipids and phospholipid-binding proteins, which... (Review)
Review
Antiphospholipid syndrome (APS) is a thrombotic disorder associated with autoantibodies that target membrane phospholipids and phospholipid-binding proteins, which regulate coagulation. APS is usually characterized by major arterial or venous occlusions, pregnancy complications, or both. In 1992, Asherson described an unusual variant of APS termed the catastrophic antiphospholipid syndrome (also known as Asherson's syndrome), the hallmark of which is rapid multiorgan failure caused by widespread small-vessel thrombi. Empiric treatments have improved the prognosis of patients, but half still die from thrombotic diathesis, even though those who survive the acute stages frequently remain well. Given the persistently high mortality rate, efforts have been underway to facilitate early diagnosis, institute effective treatments in a timely manner and to better understand the cause (or causes) of this extreme condition in order to improve outcomes.
Topics: Anemia, Hemolytic; Antiphospholipid Syndrome; Diagnosis, Differential; Disease Progression; Humans; Multiple Organ Failure; Plasma Exchange; Thrombosis; Treatment Outcome
PubMed: 16932661
DOI: 10.1038/ncprheum0069 -
The Journal of Maternal-fetal &... Dec 2016Catastrophic antiphospholipid syndrome (CAPS) is an accelerated form of the antiphospholipid antibody syndrome resulting in multi-organ ischemia and failure. It is a... (Review)
Review
Catastrophic antiphospholipid syndrome (CAPS) is an accelerated form of the antiphospholipid antibody syndrome resulting in multi-organ ischemia and failure. It is a rare and life-threatening condition that can be easily mistaken with hemolysis elevated liver enzymes low platelets syndrome, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome. In order to make a diagnosis, it is required to have multi-organ thrombosis over 1 week affecting at least three organs or systems, and to have positive antiphospholipid antibody on two occasions (6 weeks apart), and histopathologic confirmation of small vessel occlusion. However, due to similarities in clinical and laboratory findings between CAPS and some other obstetric complications, potential misdiagnosis or delay in diagnosis are common, increasing the risk of adverse maternal and perinatal outcomes. In this review we summarized information presented in previous studies, focusing on CAPS related to pregnancy. We reviewed diagnostic criteria, differential diagnosis, and common presentation ranging from malaise, abdominal pain, dyspnea, hypertension, to altered mental status and seizures. We also discussed management in pregnancy and included a detailed algorithm with steps to take. Of note, the most significant reduction in mortality was seen in patients receiving triple therapy which will be discussed in this review.
Topics: Antiphospholipid Syndrome; Combined Modality Therapy; Diagnosis, Differential; Female; Humans; Multiple Organ Failure; Pregnancy; Pregnancy Complications; Pregnancy Outcome
PubMed: 27023104
DOI: 10.3109/14767058.2016.1160047 -
Best Practice & Research. Clinical... Oct 2008The antiphospholipid syndrome is an acquired autoimmune syndrome characterized by arterial and/or venous thrombosis and/or pregnancy morbidity in association with the... (Review)
Review
The antiphospholipid syndrome is an acquired autoimmune syndrome characterized by arterial and/or venous thrombosis and/or pregnancy morbidity in association with the prolonged presence of serum autoantibodies, including the so-called lupus anticoagulant and anticardiolipin antibodies, which are mainly directed against complexes of proteins and anionic phospholipids.
Topics: Adult; Antiphospholipid Syndrome; Female; Humans; Pregnancy; Young Adult
PubMed: 19028366
DOI: 10.1016/j.berh.2008.08.008 -
Current Opinion in Rheumatology Sep 2008Antiphospholipid syndrome is considered as the most common acquired hypercoagulation state of autoimmune disorder in children. Besides vascular occlusion,... (Review)
Review
PURPOSE OF REVIEW
Antiphospholipid syndrome is considered as the most common acquired hypercoagulation state of autoimmune disorder in children. Besides vascular occlusion, antiphospholipid antibodies have been associated with various nonthrombotic clinical manifestations. This review highlights recent clinical advances in the field of neonatal and pediatric antiphospholipid syndrome and emphasizes differences in relation to the antiphospholipid syndrome in adult population.
RECENT FINDINGS
Neonatal antiphospholipid syndrome is a rare clinical entity characterized by neonatal thrombotic disease due to the transplacental passage of maternal antiphospholipid antibodies. There is growing evidence that transplacentally transferred antiphospholipid antibodies act as a risk factor, but are not usually a sufficient condition for thrombosis and other thrombophilic risk factors should be systematically evaluated. Long-term studies of children born to antiphospholipid-antibody-positive mothers provided the evidence of possible neurodevelopmental changes in these children and regular neuropsychological assessments are recommended. Data from the pediatric antiphospholipid syndrome studies have confirmed that antiphospholipid-antibody-related thromboses in children are frequently associated with multiple antiphospholipid antibodies positivity and concomitant presence of inherited prothrombotic disorders. Children with antiphospholipid syndrome have frequently demonstrated associated nonthrombotic manifestations, particularly hematological, skin and neurological manifestations. Comparisons between children with primary antiphospholipid syndrome and antiphospholipid syndrome associated with autoimmune disease have recognized certain differences that suggest two distinct subgroups with specific clinical characteristics.
SUMMARY
Clinical and laboratory characterization of pediatric patients with antiphospholipid syndrome continues to improve and implies some important differences between antiphospholipid syndrome in pediatric and adult populations.
Topics: Antiphospholipid Syndrome; Child; Humans; Infant, Newborn; Registries; Risk Factors; Thrombosis
PubMed: 18698184
DOI: 10.1097/BOR.0b013e3283060a8c -
Journal of Internal Medicine Apr 2020The antiphospholipid syndrome (APS) was fully recognized as a clinical entity in the early 1980s. Still, more than 30 years later, the epidemiology of APS is not well... (Review)
Review
The antiphospholipid syndrome (APS) was fully recognized as a clinical entity in the early 1980s. Still, more than 30 years later, the epidemiology of APS is not well described, and furthermore, APS remains a challenge in terms of both diagnostic issues and clinical praxis involving a wide range of specialties. To date, there are no diagnostic criteria for APS. The present classification criteria rely on a combination of clinical manifestations and persistently positive tests for antiphospholipid antibodies (aPL). Clinical symptoms comprise vascular thrombosis, which can affect any vascular bed, including venous, microvascular and arterial vessels, and a set of pregnancy morbidities including early and late miscarriages, foetal death and preeclampsia. APS is more frequent among patients with other autoimmune diseases, and it is especially common in systemic lupus erythematosus (SLE). Importantly, APS symptoms can present in almost any medical specialty, but general knowledge and most previous clinical studies have essentially been confined to haematology, rheumatology and obstetrics/gynaecology. However, recent data demonstrate a relatively high prevalence of aPL also in patients from the general population who suffer from vascular occlusions or pregnancy complications. It is important that these patients are recognized by the general health care since APS is a treatable condition. This review aims to summarize the present knowledge on the history, pathogenesis, clinical manifestations and treatment of APS in order to urge a wide range of clinicians to consider comprehensive assessment of all patients where the diagnosis APS may be conceivable.
Topics: Antiphospholipid Syndrome; Arterial Occlusive Diseases; Humans; Vascular Diseases
PubMed: 31957081
DOI: 10.1111/joim.13022