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Journal of the American College of... May 2017Antiphospholipid syndrome (APS) is an autoimmune disease characterized by venous thromboembolism, arterial thrombosis, and obstetric morbidities in the setting of... (Review)
Review
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by venous thromboembolism, arterial thrombosis, and obstetric morbidities in the setting of persistently positive levels of antiphospholipid antibodies measured on 2 different occasions 12 weeks apart. Patients with APS are at increased risk for accelerated atherosclerosis, myocardial infarction, stroke, and valvular heart disease. Vascular endothelial cell dysfunction mediated by antiphospholipid antibodies and subsequent complement system activation play a cardinal role in APS pathogenesis. Improved understanding of their pathogenic function could help in the risk stratification of patients with APS and provide new molecular therapeutic targets.
Topics: Antiphospholipid Syndrome; Endothelial Cells; Endothelium, Vascular; Heart Valve Diseases; Humans; Risk Assessment
PubMed: 28473138
DOI: 10.1016/j.jacc.2017.02.058 -
Clinical and Applied... Oct 2001Antiphospholipid antibodies are strongly associated with thrombosis and are the most common of the acquired blood protein defects causing thrombosis. Although the... (Review)
Review
Antiphospholipid antibodies are strongly associated with thrombosis and are the most common of the acquired blood protein defects causing thrombosis. Although the precise mechanism(s) whereby antiphospholipid antibodies alter hemostasis to induce a hypercoagutable state remain unclear, numerous theories, as previously discussed, have been advanced. The most common thrombotic events associated with anticardiolipin antibodies are deep vein thrombosis and pulmonary embolus (type I syndrome), coronary or peripheral artery thrombosis (type II syndrome), or cerebrovascular/retinal vessel thrombosis (type II syndrome); occasionally, patients present with mixtures of these types (type IV syndrome). Type V patients are those with antiphospholipid antibodies and RMS. It is as yet unclear how many seemingly normal individuals who may never develop manifestations of antiphospholipid syndrome (type VI) harbor asymptomatic antiphospholipid antibodies. The relative frequency of anticardiolipin antibodies in association with arterial and venous thrombosis strongly suggests that these should be looked for in any individual with unexplained thrombosis; all three idiotypes (IgG, IgA, and IgM) should be assessed. Also, the type of syndrome (I through VI) should be defined if possible, as this may dictate both type and duration of both immediate and long-term anticoagulant therapy. Unlike those with anticardiolipin antibodies, patients with primary lupus anticoagulant thrombosis syndrome usually experience venous thrombosis. Because the aPTT is unreliable inpatients with lupus anticoagulant (prolonged in only about 40 to 50% of patients) and is not usually prolonged in patients with anticardiolipin antibodies, definitive tests, including ELISA for anticardiolipin antibodies, the dRVVT for lupus anticoagulant, hexagonal phospholipid neutralization procedure, and beta-2-GP-I (IgG, IgA, and IgM) should be immediately ordered when suspecting antiphospholipid syndrome or in individuals with otherwise unexplained thrombotic or thromboembolic events. If results of these tests are negative, in the appropriate clinical setting, subgroups should also be assessed. Finally, most patients with antiphospholipid thrombosis syndrome will fail warfarin therapy and, except for retinal vascular thrombosis, may fail some types of antiplatelet therapy; thus it is of major importance to make this diagnosis so that patients can be treated with the most effective therapy for secondary prevention--LMWH or UH in most instances, and clopidogrel in some instances.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Female; Humans; Male; Pregnancy; Thrombophilia; Thrombosis
PubMed: 11697705
DOI: 10.1177/107602960100700401 -
Journal of Internal Medicine Aug 2011The antiphospholipid syndrome (APS) identifies a condition at increased risk of vascular occlusion and/or pregnancy complications. Patients are defined as having APS if... (Review)
Review
The antiphospholipid syndrome (APS) identifies a condition at increased risk of vascular occlusion and/or pregnancy complications. Patients are defined as having APS if they have at least one clinical (vascular occlusion and/or pregnancy complications) and one laboratory criterion at the same time. The laboratory criteria that define APS are repeated positivity (confirmed 12 weeks apart) for lupus anticoagulants and/or antibodies targeted against cardiolipin or β(2) -glycoprotein I immobilized on solid surfaces. Over the years, APS has attracted the interest of many medical specialties. The aim of this review is to provide an update on (i) the laboratory criteria that determine the presence of APS, (ii) how the antibodies increase the risk of vascular occlusion and foetal loss and (iii) the treatment of the related clinical events.
Topics: Anticoagulants; Antiphospholipid Syndrome; Biomarkers; Female; Humans; Lupus Coagulation Inhibitor; Pregnancy; Pregnancy Complications, Cardiovascular; beta 2-Glycoprotein I
PubMed: 21323768
DOI: 10.1111/j.1365-2796.2011.02362.x -
Pediatric Rheumatology Online Journal Feb 2022Pediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic...
BACKGROUND/PURPOSE
Pediatric antiphospholipid syndrome (APS) is a thromboinflammatory disease characterized by the presence of circulating antiphospholipid antibodies and either thrombotic events or pregnancy morbidity. The objective of this study was to review a large institution's experience to better understand the characteristics of children with APS.
METHODS
We conducted a retrospective review of pediatric APS at a tertiary referral center. The electronic medical record system was queried from 2000 through 2019, and 21 cases were included based on meeting the revised Sapporo Classification criteria by age 18 or younger. Comparisons between primary and secondary APS patients were made with two-tailed t-tests.
RESULTS
Twenty-one patients were included with a median age at diagnosis of 16 years and median follow-up of 5.8 years. Secondary APS was slightly more common than primary APS (11 vs. 10 cases) and was primarily diagnosed in the context of systemic lupus erythematosus. Two thirds of patients (67%) also had "non-criteria" manifestations of APS including thrombocytopenia, autoimmune hemolytic anemia, and livedo reticularis/racemosa. Almost half of patients (43%) had recurrent thrombosis, typically when patients were subtherapeutic or non-adherent with anticoagulation. Damage Index in Patients with Thrombotic APS (DIAPS) scores indicated a chronic burden of disease in both primary and secondary APS patients.
CONCLUSION
This case series of pediatric APS provides important context regarding disease phenotypes displayed by children with APS. High prevalence of non-criteria clinical manifestations highlights the need to consider these characteristics when developing pediatric-specific classification criteria and when considering this relatively rare diagnosis in pediatric practice.
Topics: Adolescent; Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Cost of Illness; Female; Humans; Immunomodulating Agents; Lupus Erythematosus, Systemic; Male; Patient Compliance; Platelet Aggregation Inhibitors; Retrospective Studies; Severity of Illness Index; Symptom Assessment; Tertiary Care Centers; Thrombosis; Treatment Outcome
PubMed: 35197077
DOI: 10.1186/s12969-022-00677-8 -
Missouri Medicine 2023Antiphospholipid syndrome (APS) is an autoimmune condition affecting young patients, primarily women, negatively impacting their quality of life. APS is under-recognized...
Antiphospholipid syndrome (APS) is an autoimmune condition affecting young patients, primarily women, negatively impacting their quality of life. APS is under-recognized and underdiagnosed and can have devastating results if untreated, mainly due to uncontrolled thrombosis. Research in the past decades has led to several breakthroughs with important implications for clinical practice. Here, we summarize the state of APS diagnosis, treatment, pathophysiology, and research directions that hold promise for advancing diagnosis and treatment.
Topics: Humans; Female; Antiphospholipid Syndrome; Quality of Life; Thrombosis
PubMed: 37841574
DOI: No ID Found -
Turkish Journal of Haematology :... Mar 2024Antiphospholipid syndrome (APS) is a systemic autoimmune disorder resulting in thrombosis, microvascular disease, morbidity in pregnancy, and/or non-thrombotic...
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder resulting in thrombosis, microvascular disease, morbidity in pregnancy, and/or non-thrombotic manifestations. The recently introduced 2023 American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) APS classification criteria, with significantly higher specificity compared to the revised Sapporo criteria, now reflect the current thinking about APS and provide a new foundation for future APS research. The purpose of this short commentary is to discuss the appropriate circumstances under which the 2023 ACR/EULAR classification criteria could be used and to demonstrate how the new criteria can be applied to simple case scenarios.
Topics: Female; Pregnancy; Humans; Antiphospholipid Syndrome; Rheumatology; Thrombosis
PubMed: 38284227
DOI: 10.4274/tjh.galenos.2024.2024.0003 -
Best Practice & Research. Clinical... Aug 2012Catastrophic antiphospholipid syndrome (CAPS) is a very severe variant of the classic APS, characterised by clinical evidence of multiple organ involvement developing... (Review)
Review
Catastrophic antiphospholipid syndrome (CAPS) is a very severe variant of the classic APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of antiphospholipid antibodies (aPL), usually in high titre. Although patients with catastrophic APS represent less than 1% of all patients with APS, this is usually a life-threatening condition. In this article, we aimed to review the state-of-the art about current knowledge in pathogenesis, clinical manifestations, diagnosis and treatment strategies in CAPS.
Topics: Antiphospholipid Syndrome; Catastrophic Illness; Humans; Prognosis; Rare Diseases
PubMed: 23040365
DOI: 10.1016/j.berh.2012.07.005 -
Annals of the Rheumatic Diseases Jun 2007The catastrophic variant of the antiphospholipid syndrome (APS) is a life-threatening form of presentation of this syndrome that can be triggered by several factors. (Review)
Review
BACKGROUND
The catastrophic variant of the antiphospholipid syndrome (APS) is a life-threatening form of presentation of this syndrome that can be triggered by several factors.
AIM
To describe the characteristics of patients who developed catastrophic APS triggered during pregnancy and puerperium.
METHODS
A review of the first 255 cases collected in the website-based "CAPS Registry" was undertaken. Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added.
RESULTS
Fifteen cases were identified. The mean (range) age was 27 (17-38) years. Most patients had a previous unsuccessful obstetric history. In 7 of 14 (50%) cases with available medical history, the catastrophic APS appeared during pregnancy, in 6 (43%) during the puerperium and in 1 (7%) after curettage for a fetal death. The main clinical and serological characteristics were similar to those patients with catastrophic APS triggered by other factors, except for a history of a higher prevalence of previous abortions (p<0.01). Several specific features were found, including the HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome in 8 (53%) patients, placental infarctions in 4 (27%) patients, and pelvic vein thrombosis and myometrium thrombotic microangiopathy in 1 (7%) patient each. Mortality rate was high for the mothers (46%), and for the babies (54%).
CONCLUSIONS
It is important to consider the possibility of the development of catastrophic APS in those patients with signs of HELLP syndrome and multiorgan failure during pregnancy or puerperium, especially in those patients with previous history of abortions and/or thrombosis.
Topics: Adolescent; Adult; Antiphospholipid Syndrome; Catastrophic Illness; Female; HELLP Syndrome; Humans; Multiple Organ Failure; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Puerperal Disorders; Registries
PubMed: 17223653
DOI: 10.1136/ard.2006.061671 -
Rheumatology (Oxford, England) Dec 2021Cognitive dysfunction is common in patients with aPL (including primary APS or APS associated with SLE). Neuroimaging biomarkers may contribute to our understanding of...
OBJECTIVES
Cognitive dysfunction is common in patients with aPL (including primary APS or APS associated with SLE). Neuroimaging biomarkers may contribute to our understanding of mechanisms of cognitive dysfunction in these cohorts. This review aimed to investigate: (i) the prevalence of cognitive dysfunction in studies including neuroimaging biomarkers; and (ii) associations between cognition and neuroimaging biomarkers in patients with APS/aPL.
METHODS
We conducted a systematic search of electronic databases PubMed, Science Direct, Scopus and PsycINFO, and included studies with descriptions of neuroimaging findings, cognitive dysfunction or both, in patients with aPL positivity (LA, IgG and IgM aCL and anti-β2 glycoprotein-I antibodies).
RESULTS
Of 120 search results we included 20 eligible studies (6 APS, 4 SLE with APS/aPL and 10 NPSLE). We identified a medium risk of bias in 6/11 (54%) of cohort studies and 44% of case-control studies, as well as marked heterogeneity in cognitive assessment batteries, APS and aPL definitions, and neuroimaging modalities and protocols. The prevalence of cognitive dysfunction ranged between 11 and 60.5%. Structural MRI was the most common imaging modality, reporting cognitive dysfunction to be associated with white matter hyperintensities, ischaemic lesions and cortical atrophy (four with cerebral atrophy, two with white matter hyperintensities and two with cerebral infarcts).
CONCLUSION
Our findings confirm that cognitive impairment is commonly found in patients with aPL (including APS, SLE and NPSLE). The risk of bias, and heterogeneity in the cognitive and neuroimaging biomarkers reported does not allow for definitive conclusions.
Topics: Antiphospholipid Syndrome; Biomarkers; Cognition; Cognitive Dysfunction; Dementia; Humans; Neuroimaging; Prevalence
PubMed: 34003972
DOI: 10.1093/rheumatology/keab452 -
Reumatologia Clinica 2017Antiphospholipid antibody syndrome is a non-inflammatory autoimmune disease characterized by recurrent thrombotic events and/or obstetric complications associated with... (Review)
Review
Antiphospholipid antibody syndrome is a non-inflammatory autoimmune disease characterized by recurrent thrombotic events and/or obstetric complications associated with the presence of circulating antiphospholipid antibodies (anticardiolipin antibodies, anti-β glycoprotein-i antibodies, and/or lupus anticoagulant. Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with recurrent miscarriage, stillbirth, fetal growth restriction and premature birth. The diversity of the features of the proposed placental antiphospholipid antibodies fingerprint suggests that several disease processes may occur in the placentae of women with antiphospholipid antibody syndrome in the form of immune responses: inflammatory events, complement activation, angiogenic imbalance and, less commonly, thrombosis and infarction. Because of the disparity between clinical and laboratory criteria, and the impact on perinatal outcome in patients starting treatment, we reviewed the aspects of antiphospholipid antibody syndrome related to obstetric complications and seronegative antiphospholipid antibody syndrome, and their treatment in obstetrics.
Topics: Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications
PubMed: 27291869
DOI: 10.1016/j.reuma.2016.04.011