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CMAJ : Canadian Medical Association... Jun 2003Antiphospholipid antibodies are a heterogeneous group of autoantibodies that are detected by immunoassays and functional coagulation tests. The antigenic targets are... (Review)
Review
Antiphospholipid antibodies are a heterogeneous group of autoantibodies that are detected by immunoassays and functional coagulation tests. The antigenic targets are negatively charged phospholipids and serum phospholipid-binding proteins. The latter antibodies are frequently associated with thrombosis, fetal loss and other clinical manifestations of the antiphospholipid syndrome. These antibodies are felt to be etiologically important in the syndrome, although the precise pathogenic mechanisms are still being determined. Proposed mechanisms include antibody-mediated interference with coagulation homeostasis, activation of platelets and endothelial cells and a T-cell immune response to serum phospholipid-binding proteins. The mainstay of therapy is anticoagulation, whereas immunosuppression is ineffective.
Topics: Abortion, Spontaneous; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Female; Humans; Pregnancy; Pregnancy Complications; Venous Thrombosis
PubMed: 12821621
DOI: No ID Found -
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 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 -
In Vivo (Athens, Greece) 2023Catastrophic antiphospholipid syndrome (CAPS) may be the first manifestation ("de novo") of antiphospholipid syndrome (APS) or a complication in the clinical course of... (Review)
Review
BACKGROUND/AIM
Catastrophic antiphospholipid syndrome (CAPS) may be the first manifestation ("de novo") of antiphospholipid syndrome (APS) or a complication in the clinical course of patients known to have this syndrome. Approximately 40% of patients had an associated autoimmune disease, mainly, systemic lupus erythematosus (SLE). The trigger can be one of the following: infections, surgical interventions, neoplasms, pregnancy, discontinuation of anticoagulant treatment, and others. CAPS is a medical emergency in which early identification and prompt initiation of aggressive therapy is extremely important. According to the Guidelines for the use of Therapeutic Apheresis in Clinical Practice developed by the American Society for Apheresis (ASFA), last updated in April 2023, in CAPS, the indication for therapeutic plasma exchange (TPE) is category I, grade 2C.
CASE REPORT
We present a case of probable CAPS secondary to systemic lupus erythematosus (SLE) in an elderly patient in whom clinical and biological improvement was achieved through a multidisciplinary approach and prompt initiation of TPE. Because TPE is considered first-line therapy in CAPS, we initiated the procedure as soon as the attending rheumatologist raised this suspicion. Four plasmapheresis sessions were performed in the Intensive Care Unit. We used TPE by membrane filtration. Following the therapeutic intervention with TPE, corticotherapy (Solumedrol in puls-therapy), cyclophosphamide and anticoagulant treatment, the evolution was favourable, with clinical and biological improvement.
CONCLUSION
The prompt initiation of TPE, because of the suspicion of CAPS, increases the chances of a favourable evolution.
Topics: Humans; Aged; Antiphospholipid Syndrome; Plasma Exchange; Catastrophic Illness; Plasmapheresis; Lupus Erythematosus, Systemic; Anticoagulants
PubMed: 37369472
DOI: 10.21873/invivo.13286 -
Thrombosis and Haemostasis Feb 2014Antibodies to prothrombin are detected by directly coating prothrombin on irradiated ELISA plates (aPT) or by using the phosphatidylserine/prothrombin complex as antigen... (Review)
Review
Antibodies to prothrombin are detected by directly coating prothrombin on irradiated ELISA plates (aPT) or by using the phosphatidylserine/prothrombin complex as antigen (aPS/PT). Although these antibodies have both been associated with antiphospholipid syndrome (APS) and a correlation between the two assays have been reported, it seems that aPT and aPS/PT belong to different populations of autoantibodies. It was our objective to systematically review the available evidence on aPT and aPS/PT antibodies and the risk of thrombosis in APS. Medline-reports published between 1988 and 2013 investigating aPT and aPS/PT as a risk factor for thrombosis were included. Whenever possible, antibody isotype(s) and site of thrombosis were analysed. This systematic review is based on available data from more than 7,000 patients and controls from 38 studies analysing aPT and 10 aPS/PT. Antibodies to prothrombin (both aPT and aPS/PT) increased the risk of thrombosis (odds ratio [OR] 2.3; 95% confidence interval [CI] 1.72-3.5). aPS/PT seemed to represent a stronger risk factor for thrombosis, both arterial and/or venous than aPT (OR 5.11; 95%CI 4.2-6.3 and OR 1.82; 95%CI 1.44-2.75, respectively). In conclusion, routine measurement of aPS/PT (but not aPT) might be useful in establishing the thrombotic risk of patients with previous thrombosis and/or systemic lupus erythematosus. Their inclusion as laboratory criteria for the APS should be indisputably further explored.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Biomarkers; Humans; Odds Ratio; Phosphatidylserines; Predictive Value of Tests; Prothrombin; Risk Assessment; Risk Factors; Thrombosis
PubMed: 24172938
DOI: 10.1160/TH13-06-0509 -
Journal of Immunology Research 2014A major cause of morbidity and mortality in the context of the antiphospholipid syndrome (APS) is the occurrence of thrombotic events. Besides the pathogenic roles of... (Review)
Review
A major cause of morbidity and mortality in the context of the antiphospholipid syndrome (APS) is the occurrence of thrombotic events. Besides the pathogenic roles of antiphospholipid antibodies (aPL), other risk factors and medical conditions, which are conditions for traditional risk of an individual without the APS, can coexist in this patient, raising their risk of developing thrombosis. Therefore, the clinical and laboratory investigation of comorbidities known to increase cardiovascular risk in patients with antiphospholipid antibody syndrome is crucial for the adoption of a more complete and effective treatment. Experimental models and clinical studies show evidence of association between APS and premature formation of atherosclerotic plaques. Atherosclerosis has major traditional risk factors: hypertension, diabetes mellitus, obesity, dyslipidemia, smoking, and sedentary lifestyle that may be implicated in vascular involvement in patients with APS. The influence of nontraditional risk factors as hyperhomocysteinemia, increased lipoprotein a, and anti-oxLDL in the development of thromboembolic events in APS patients has been studied in scientific literature. Metabolic syndrome with all its components also has been recently studied in antiphospholipid syndrome and is associated with arterial events.
Topics: Adipocytes; Animals; Antiphospholipid Syndrome; Comorbidity; Cytokines; Humans; Metabolic Syndrome; Risk Factors
PubMed: 25133195
DOI: 10.1155/2014/621270 -
Actas Dermo-sifiliograficas 2007Antiphospholipid syndrome is an acquired autoimmune thrombophilia that produces significant morbidity and mortality. Its diagnosis requires the presence of... (Review)
Review
Antiphospholipid syndrome is an acquired autoimmune thrombophilia that produces significant morbidity and mortality. Its diagnosis requires the presence of antiphospholipid antibodies and clinical manifestations that include thrombotic phenomena and/or recurrent miscarriages. Antiphospholipid antibodies may be detected in many instances, including healthy subjects. Clinical manifestations are varied and may occur in episodes and also appear in other situations. Therefore, it is important to have clear guidelines in order to establish a correct diagnosis, carry out an adequate treatment, and to know which are the prophylactic measures and when they should be undertaken. In this article we explain the most recent diagnostic criteria reviewed in the 11th International Congress on Antiphospholipid Antibodies (Sydney 2004), comment on the varied clinical manifestations with special focus on cutaneous lesions, and review current guidelines for the treatment and prophylaxis of thrombotic and obstetric pathology.
Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Humans
PubMed: 17374329
DOI: No ID Found -
The Israel Medical Association Journal... Sep 2021
Topics: Adult; Antiphospholipid Syndrome; Female; Gangrene; Humans; Livedo Reticularis
PubMed: 34472238
DOI: No ID Found -
RMD Open Jan 2020High-risk patients with antiphospholipid syndrome (APS) experience increased risk of thrombosis when treated with direct oral anticoagulant (DOAC) therapy compared with... (Review)
Review
High-risk patients with antiphospholipid syndrome (APS) experience increased risk of thrombosis when treated with direct oral anticoagulant (DOAC) therapy compared with warfarin. It is essential to establish the APS diagnosis to choose therapy and determine treatment duration. It requires testing for antiphospholipid antibodies, including lupus anticoagulant (LAC). In this viewpoint, we discuss the options for timing of LAC testing, which includes testing before starting anticoagulant treatment (DOAC or warfarin), after switching to heparin or after withdrawal of anticoagulant treatment. DOACs interfere with LAC testing and recommendations emerge stating not to conduct on-therapy LAC testing. All approaches are to some extent currently practised, but have limitations and the area is therefore seemingly a catch 22. We put forward that the anticoagulant effect of DOAC can be eliminated in the laboratory and therefore patients can be tested on-therapy. While it may not eliminate all cases of interference, it could aid the interpretation in these situations and this approach is attractive from the patient and clinician's perspective. Nevertheless, to prevent misdiagnosis the diagnostic workup for APS requires collaboration between the clinician and the laboratory. We advocate for standardisation in laboratory and clinical practice when diagnosing APS.
Topics: Antibodies, Antiphospholipid; Antibody Specificity; Anticoagulants; Antiphospholipid Syndrome; DiGeorge Syndrome; Diagnosis, Differential; Humans
PubMed: 32144138
DOI: 10.1136/rmdopen-2019-001156 -
Nihon Rinsho Men'eki Gakkai Kaishi =... 2012Antiphospholipid syndrome (APS) is an autoimmune disorder defined by the presence of antiphospholipid antibodies in plasma of patients with vascular thrombosis and/or... (Review)
Review
Antiphospholipid syndrome (APS) is an autoimmune disorder defined by the presence of antiphospholipid antibodies in plasma of patients with vascular thrombosis and/or pregnancy morbidity. APS is considered the major cause of brain infarction in young adults and is a generally accepted cause of recurrent pregnancy loss. Antiphospholipid antibodies are a heterogeneous group of autoantibodies strongly related to the clinical manifestations of APS and with a widely recognized pathogenic role in thrombosis. In this article, recent clinical and pathophysiological advances in APS are discussed.
Topics: Abortion, Habitual; Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Autoimmunity; Biomarkers; Female; Humans; Male; Molecular Targeted Therapy; NF-kappa B; P-Selectin; Pregnancy; Pregnancy Complications; Risk Assessment; Signal Transduction; Thromboplastin; Thrombosis; p38 Mitogen-Activated Protein Kinases
PubMed: 23291483
DOI: 10.2177/jsci.35.481