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Paediatric Drugs Jan 2022Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical... (Review)
Review
Pediatric antiphospholipid syndrome (APS) is a rare acquired multisystem autoimmune thromboinflammatory condition characterized by thrombotic and non-thrombotic clinical manifestations. APS in children and adolescents typically presents with large-vessel thrombosis, thrombotic microangiopathy, and, rarely, obstetric morbidity. Non-thrombotic clinical manifestations are frequently seen in pediatric APS and may be present even before the vascular thrombotic events occur. We review insights into the pathogenesis of APS and discuss potential targets for therapy. The identification of multiple immunologic abnormalities in patients with APS reveals molecular targets for current or future treatment. Management strategies, especially for APS in adolescents, require screening for additional prothrombotic risk factors and consideration of counseling regarding contraceptive strategies, lifestyle recommendations, treatment adherence, and mental health issues associated with this autoimmune thrombophilia. The main goal of therapy in pediatric APS is the prevention of thrombosis. The management of acute thrombosis events in children and adolescents is the same as for primary APS, which involves isolated occurrences, and secondary APS, which is seen in association with another autoimmune disease, e.g., systemic lupus erythematosus. A pediatric hematologist should be consulted so other differential thrombophilic conditions can be eliminated. Therapy includes unfractionated heparin or low-molecular-weight heparin followed by vitamin K antagonists. Treatment of catastrophic APS involves triple therapy (anticoagulation, intravenous corticosteroid pulse therapy, and plasma exchange) and may include intravenous immunoglobulin for children and adolescents with this condition. New drugs such as eculizumab and sirolimus seem to be promising drugs for APS.
Topics: Adolescent; Anticoagulants; Antiphospholipid Syndrome; Child; Female; Heparin; Humans; Lupus Erythematosus, Systemic; Pregnancy; Thrombosis
PubMed: 34904182
DOI: 10.1007/s40272-021-00484-w -
The Journal of the Association of... Mar 2010The 2006 International Consensus Statement on an Update of the Classification Criteria for Definite Antiphospholipid Syndrome has increased the time between the two... (Review)
Review
The 2006 International Consensus Statement on an Update of the Classification Criteria for Definite Antiphospholipid Syndrome has increased the time between the two laboratory studies required for diagnosis from 6 to 12 weeks. Antibody to beta2 glycoprotein 1 has been included as a criterion. Various non-criteria diagnostic clues such as livedo reticularis, heart valve disease, thrombocytopenia, renal thrombotic microangiopathy, neurological manifestations, non-criteria antibodies (IgA aCL, IgA anti-beta2 glycoprotein I) and some research laboratory-identified antibodies (antiphosphatidylserine antibodies, antiphosphatidylethanolamine antibodies, antibodies against prothrombin alone and antibodies to the phosphatidylserine-prothrombin complex) have been recognised. New concepts of pathogenesis now implicate complement activation and participation of the innate immune system upstream to thrombosis. Warfarin remains the treatment of choice for patients who have suffered thrombosis, but antiplatelet agents and heparin are other options. Target INR is 2.0-3.0. The other drugs which are used in resistant cases are: rituximab, hydroxychloroquine, thrombin inhibitors and statins.
Topics: Anticoagulants; Antiphospholipid Syndrome; Humans; Thrombosis; Warfarin
PubMed: 20848817
DOI: No ID Found -
Hamostaseologie Jun 2020Despite a lot of research on antiphospholipid antibodies (aPL), standardization of test systems, and better definition of its clinical symptoms, the pathomechanism of... (Review)
Review
Despite a lot of research on antiphospholipid antibodies (aPL), standardization of test systems, and better definition of its clinical symptoms, the pathomechanism of this acquired autoimmune disease is not yet fully explained. Progress in treatment increased the live birth rate in 70 to 80% of women suffering from obstetric antiphospholipid syndrome (OAPS). However, still 20 to 30% will develop adverse pregnancy outcome. Lack of awareness of this disorder as the cause for pregnancy complications is very harmful to mothers and to their newborns. Complications can be avoided or minimized by proper treatment. The aim of this article is to increase the awareness of gynecologists and medical personal for OAPS.
Topics: Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications
PubMed: 32455458
DOI: 10.1055/a-1113-0689 -
Immunologic Research Feb 2017Headaches have been extensively reported in Antiphospholipid syndrome (APS)/Antiphospholipid antibodies (aPL)-positive patients. The aim of this study was to highlight... (Review)
Review
Headaches have been extensively reported in Antiphospholipid syndrome (APS)/Antiphospholipid antibodies (aPL)-positive patients. The aim of this study was to highlight the prevalence of headaches among APS/aPL-positive patients and discuss its association with laboratory, clinical and imaging findings. We searched the literature through Google Scholar and PubMed for publications on the epidemiology, pathogenesis, laboratory, imaging and clinical findings, and management of headaches in APS/aPL-positive patients. The following keywords were used: Antiphospholipid, Hughes syndrome, anticardiolipin, lupus anticoagulant, anti-β2 glycoprotein I, headache, migraine, tension, and cluster. All reports published between 1969 and 2015 were included. Migraine is the most commonly reported type of headache in APS/aPL-positive patients. Thrombotic and platelet dysfunction hypotheses have been studied to uncover the pathogenic role of aPL in the development of headaches. Several studies are reporting higher levels of aPL in primary and secondary APS migraineurs, but only few reached statistical significance. Migraine patients without clinical signs/symptoms of cerebral infarction rarely show positive imaging findings. Digital subtraction angiography shows promise in demonstrating small vascular lesions otherwise not detected on computed tomography, magnetic resonance imaging, or cerebral angiograms. Although it may be solitary and harmless in many cases, the deleterious effect of migraine on the quality of life of APS patients prompts rapid diagnosis and proper management. An anticoagulation trial is advisable in APS patients with migraine as many cases of severe, refractory migraine resolved with anticoagulation therapy. The profile of migraine headaches discussed in this study permits its candidacy for inclusion in future APS classification criteria.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Humans; Migraine Disorders
PubMed: 27423434
DOI: 10.1007/s12026-016-8831-9 -
Southern Medical Journal Jun 2005Antiphospholipid syndrome spans many medical disciplines. Classic criteria include the presence of anticardiolipin antibody or lupus anticoagulant with typical... (Review)
Review
Antiphospholipid syndrome spans many medical disciplines. Classic criteria include the presence of anticardiolipin antibody or lupus anticoagulant with typical complications of thrombosis or pregnancy loss. Other common associated manifestations include livedo reticularis, thrombocytopenia, valvular heart disease, and nephropathy with renal insufficiency, hypertension, and proteinuria. Treatment of serious complications with anticoagulation is standard; generally warfarin for thrombosis and aspirin/heparin for pregnancy prophylaxis. Detailed recommendations regarding precise intensity and duration of anticoagulation are still a subject of debate.
Topics: Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Female; Humans; Lupus Coagulation Inhibitor; Pregnancy; Pregnancy Complications; Pregnancy Outcome
PubMed: 16004169
DOI: 10.1097/01.smj.0000166748.90089.65 -
Journal of the American Academy of... Jun 1997The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for... (Review)
Review
The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for the syndrome are the lupus anticoagulant and anticardiolipin antibodies. Clinical features include recurrent thrombotic events (arterial or venous), repeated fetal loss, and thrombocytopenia. Cutaneous manifestations may occur as the first sign of antiphospholipid syndrome. These include livedo reticularis, necrotizing vasculitis, livedoid vasculitis, thrombophlebitis, cutaneous ulceration and necrosis, erythematous macules, purpura, ecchymoses, painful skin nodules, and subungual splinter hemorrhages. Antiphospholipid syndrome may also be associated rarely with anetoderma, discoid lupus erythematosus, cutaneous T-cell lymphoma, or disorders that closely resemble Sneddon or Degos syndromes. Noninflammatory vascular thrombosis is the most frequent histopathologic feature observed. Prophylaxis and treatment of thrombosis in patients with antiphospholipid syndrome relies principally on anticoagulant and antiplatelet agents.
Topics: Antiphospholipid Syndrome; Humans; Skin Diseases; Thrombosis
PubMed: 9204065
DOI: 10.1016/s0190-9622(97)80283-6 -
Scottish Medical Journal Jun 1998The antiphospholipid syndrome is now well established as an important, acquired prothrombotic disorder. Advances are being made in the understanding of the condition and... (Review)
Review
The antiphospholipid syndrome is now well established as an important, acquired prothrombotic disorder. Advances are being made in the understanding of the condition and the paradox of the in vitro anticoagulation, and the in vivo prothrombotic tendency. Newer and better assays for the diagnosis of antiphospholipid syndrome can be expected in the near future. Large, multi-centre, controlled trials to determine the optimum treatment of patients who test positive for the antiphospholipid syndrome, but have not suffered a thrombotic event, are required.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Clinical Trials as Topic; Female; Humans; Incidence; Male; Prognosis; Risk Factors; Scotland
PubMed: 9682289
DOI: 10.1177/003693309804300302 -
Nature Reviews. Rheumatology Jun 2024Antiphospholipid syndrome (APS) consists of thrombotic, non-thrombotic and obstetric clinical manifestations developing in individuals with persistent antiphospholipid... (Review)
Review
Antiphospholipid syndrome (APS) consists of thrombotic, non-thrombotic and obstetric clinical manifestations developing in individuals with persistent antiphospholipid antibodies (aPL). Although researchers have made progress in characterizing different clinical phenotypes of aPL-positive people, the current approach to clinical management is still mostly based on a 'one size fits all' strategy, which is derived from the results of a limited number of prospective, controlled studies. With the 2023 publication of the ACR-EULAR APS classification criteria, it is now possible to rethink APS, to lay the groundwork for subphenotyping through novel pathophysiology-informed approaches, and to set a future APS research agenda guided by unmet needs in clinical management.
Topics: Antiphospholipid Syndrome; Humans; Antibodies, Antiphospholipid; Biomedical Research
PubMed: 38702511
DOI: 10.1038/s41584-024-01110-y -
Journal of Clinical Rheumatology :... Aug 2023Strategies to prevent thrombosis in antiphospholipid antibody (aPL)-positive patients are of the utmost importance. The risk of thrombosis in patients with aPLs varies,...
Strategies to prevent thrombosis in antiphospholipid antibody (aPL)-positive patients are of the utmost importance. The risk of thrombosis in patients with aPLs varies, depending on additional venous thrombosis and cardiovascular risk factors, as well as associated comorbidities. Recurrent thrombosis despite treatment with vitamin K antagonists is relatively common in daily practice. In this context, the effectiveness of the new direct oral anticoagulants in antiphospholipid syndrome is debated, as well as that of low-dose aspirin for primary thromboprophylaxis. There is an urgent unmet need to recognize the subgroup of patients that may benefit from low-dose aspirin use. Here we also discuss different points of view on primary and secondary thrombosis preventions in aPL-positive patients, which were presented as a debate during the 2021 PANLAR Congress (Pan-American League of the Association of Rheumatology) and that was organized by GESAF (Argentine Society of Rheumatology APS Study Group). It is the intention of this article to provide a useful discussion to aid treatment decision-making in daily clinical practice.
Topics: Humans; Antiphospholipid Syndrome; Anticoagulants; Venous Thromboembolism; Thrombosis; Aspirin
PubMed: 37478021
DOI: 10.1097/RHU.0000000000001961 -
Medizinische Klinik (Munich, Germany :... Feb 1999Antiphospholipid antibodies comprise a family of auto-antibodies mainly characterized by the presence of the lupus anticoagulant (LA) and anticardiolipin antibodies... (Review)
Review
BACKGROUND
Antiphospholipid antibodies comprise a family of auto-antibodies mainly characterized by the presence of the lupus anticoagulant (LA) and anticardiolipin antibodies (ACA).
CLINICAL APPEARANCE
The antiphospholipid antibody syndrome is defined by the appearance of frequent thromboses, repeated fetal losses and thrombocytopenia. Other clinical manifestations associated with APA include migraine, chorea, hemolytic anemia, heart valve disease, Budd-Chiari syndrome, perpetual pancreatitic episodes, intestinal infarctions, malignant hypertension, livedo reticularis, pre-eclampsia, fetal growth retardation or catastrophic antiphospholipid syndrome. LA and ACA occur in a variety of clinical conditions (secondary antiphospholipid antibody syndrome, SAPS), including other autoimmune disorders, infectious diseases, neoplastic disorders, in association with the use of certain drugs or in otherwise healthy individuals (primary antiphospholipid antibody syndrome, PAPS).
TREATMENT
Patients with thrombosis associated with APA should receive long-term anticoagulation therapy, whereas treatment of asymptomatic patients seems to be not indicated, because only approximately 10% of patients with APA may develop thrombotic complications. In patients with PAPS there is no evidence that the prophylactic administration of immunosuppressive drugs will prevent thromboembolic events.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Diagnosis, Differential; Humans
PubMed: 10194954
DOI: 10.1007/BF03044707