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1.
S D Med ; 72(10): 464-466, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31816208

RESUMO

Catastrophic antiphospholipid syndrome (CAPS) is a rare disorder characterized by acute, multi-system dysfunc- tion due to small-vessel thrombosis related to anti-phospholipid antibodies. Here we present an unusual case of CAPS presenting with genitourinary manifestations. A 72-year-old male developed a series of symptoms over the course of two weeks. His symptoms included testicular inflammation, scrotal edema, priapism, hematuria, penile eschar, elbow eschars, and acute kidney injury. He was found to have anti-phospholipid antibodies and treated with anticoagulation, high-dose steroids and plasma exchange. His symptoms resolved with minimal lasting effects. This case is unique to the literature because of the extensive genitourinary involvement.


Assuntos
Síndrome Antifosfolipídica , Trombose , Idoso , Anticorpos Antinucleares/sangue , Anticorpos Antifosfolipídeos , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Glucocorticoides/uso terapêutico , Humanos , Masculino , Troca Plasmática/métodos , Plasmaferese , Trombose/etiologia , Trombose/patologia
2.
Klin Lab Diagn ; 64(10): 603-606, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31742953

RESUMO

The objective of the study is to enhance sorption capacity of diagnostic agents by using cardiolipin antigens for antiphospholipid syndrome in patients with systemic lupus erythematosus (SLE). A technique of emulsion polimerization was used. Having integrated antigen nanoobjects we developed immobilized magnetocontrollable antigen nanosystems and put them to an evaluation test. The nanosystems are polyacrylamide granules with a built in antigen. To obtain stable immobilized multi-use biopharmaceuticals with targeted properties (shape, particle diameter, pore size, density) we used a modified version of emulsion polymerization method using polyacrylamide carrier gel. This method permitted a greater sorptive capacity, preserving the antigen in maximum native state, and opened up the possibility of controllable modification of nanoobjects. Cardiolipin was used as the antigen in question. Following the method described above we performed sorption of anticardiolipin antibodies from blood plasma of SLE patients who showed clinical presentations of antiphospholipid syndrome. All SLE patoents with signs of antiphospholipid syndrome showed reliably higher levels of cardiolipin antibodies compared with SLE patients without antiphospholipid syndrome signs; the antibody level was 0.365 ± 0.026 and 0.075 ± 0.003 on average, correspondingly (p < 0.001). Blood serum from 10 apparently healthy individuals served as control. The level of cardiolipin antibodies was determined before and after sorption by indirect solid phase immunoenzyme method. In the eluate we estimated total protein by Lowry method. In vitro testing showed that the obtained antigen nanosystems based on immobilized cardiolipin could effectively remove cardiolipin antibodies from whole blood of SLE patients with clinical presentations of APS to achieve the values of healthy individuals (before sorption cardiolipin antibodies 0.328 ± 0.0289; after sorption 0.059 ± 0.0170; p<0,001; sorption capacity 8.00 ± 0.390 mg/ml). The method of emulsion polymerization with consideration to hydrophobic and hydrophilic properties of lipid molecules permits obtaining and modifying biomolecules with certain properties, in a controlled fashion.


Assuntos
Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Síndrome Antifosfolipídica/complicações , Humanos , Lipídeos , Nanotecnologia
3.
Rinsho Shinkeigaku ; 59(10): 662-665, 2019 Oct 26.
Artigo em Japonês | MEDLINE | ID: mdl-31564704

RESUMO

A 55-year-old man presented with recurrent brain infarction which had increased multifocally mainly in the cerebral white matter over the course of one year. Antibodies associated with antiphospholipid syndrome (APS) were initially negative. The patient was admitted to our department because of the thickened meninges shown on gadolinium enhanced brain MRI, mimicking hypertrophic pachymeningitis. However, blood and cerebrospinal fluid analysis revealed no significant inflammatory changes. On histopathological examination of the biopsied meninges, the arachnoid membrane was thickened with fibrosis, and arachnoidal microvessels were enlarged without significant inflammatory changes. The dura mater was not thickened, and no inflammation or microvessel enlargement were revealed. Finally, serum IgG anticardiolipin antibody testing was positive twice at an interval of more than 12 weeks, confirming the diagnosis of APS. Since initiating antithrombotic therapy with warfarin, brain infarction has not recurred. Without inflammation in the arachnoid membrane, the congestion of blood flow caused by thrombosis of microvessels in the arachnoid membrane might have increased the thickness of the arachnoid membrane.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Aracnoide-Máter/patologia , Infarto Cerebral/etiologia , Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/tratamento farmacológico , Aracnoide-Máter/irrigação sanguínea , Aracnoide-Máter/diagnóstico por imagem , Biomarcadores/sangue , Cardiolipinas/imunologia , Infarto Cerebral/prevenção & controle , Humanos , Hipertrofia/etiologia , Imunoglobulina G/sangue , Imagem por Ressonância Magnética , Masculino , Microvasos , Pessoa de Meia-Idade , Recidiva , Trombose/complicações , Resultado do Tratamento , Varfarina/administração & dosagem
6.
Autoimmun Rev ; 18(12): 102407, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31639518

RESUMO

Antiphospholipid Syndrome (APS) is the commonest treatable cause of recurrent miscarriage and pharmacological treatment of pregnant patients with antiphospholipid antibodies (aPL) should aim at preventing obstetric complications and maternal thrombotic events. Conventional treatment for patients with an established diagnosis of obstetric APS (OAPS), generally resulting in over 70-80% successful pregnancies. Since seropositive (SP)-APS and seronegative (SN)-APS patients had shown similar clinical profiles, patients with SN- OAPS, as well as SP-OAPS, should receive combined treatment in order to improve the pregnancy prognosis; indeed, current standard of care increased good pregnancy outcome in SN-APS, with similar effect to confirmed APS. The above data suggest that there are patients with the clinical manifestations of OAPS but persistently negative to conventional aPL that need to be identified to ensure adequate therapy and therefore a better prognosis. The clinical utility of non-criteria aPL in the diagnosis of SN-APS is still a matter of debate. In the last decade more and more studies have reported the presence of patients suffering from SN-APS in which non-conventional ("non-criteria") aPL might be present or antibodies may be detected using methodological approaches different from the traditional assays. To improve test standardization large prospective, multicenter, and multinational studies are needed. Therefore, when assessing a patient with clinical manifestations consistent with OAPS but aPL negative using the conventional available assays, the clinician should consider the possibility that the patient is affected with SN-APS.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico , Complicações na Gravidez/sangue , Complicações na Gravidez/diagnóstico , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Feminino , Humanos , Gravidez , Complicações na Gravidez/imunologia , Estudos Prospectivos
8.
JNMA J Nepal Med Assoc ; 57(216): 133-145, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31477950

RESUMO

Anti-phospholipid Antibody Syndrome or Hugh's syndrome is a heterogeneous disorder, first fully described in 1980s. The syndrome is caused by the presence of specific antibodies against phospholipid binding plasma proteins in the serum of the patient, with or without underlying autoimmune diseases, that causes prolongation of tests of coagulation. High index of clinical suspicion is required for diagnosis of Anti-phospholipid Antibody Syndrome. Stroke or myocardial infarction in young, unprovoked recurrent deep vein thrombosis and recurrent pregnancy loss are typical scenarios where Anti-Phospholipid Antibody Syndrome should be suspected. Presence of non-criteria manifestations like livedo reticularis, skin ulcers, nephropathy, valvular heart disease and thrombocytopenia adds to diagnostic clue for presence of Anti-Phospholipid Antibody Syndrome. Treatment of Anti-Phospholipid Antibody Syndrome has preventive and therapeutic aspects that usually focus on thrombotic and obstetric manifestations of the disease. Therapeutic anti-coagulation with heparin followed by warfarin is required for patients presenting with acute thrombosis. Those with venous thrombosis are given moderate intensity warfarin International Normalized Ratio, 2-3), whereas those with arterial thrombosis or recurrent venous thrombosis even on warfarin are treated with high intensity warfarin (International Normalized Ratio, 3-4). Similarly, anticoagulation with heparin is advised in patients with obstetric Anti-Phospholipid Antibody Syndrome throughout pregnancy and up to six weeks postpartum. Treatment recommendations are still not clear for asymptomatic Anti-Phospholipid Antibody Syndrome positive patients and in those with non-criteria manifestations of the disease. Steroids, intravenous immunoglobulin and immunosuppressant are reported to be effective in severe cases of catastrophic antiphospholid syndrome characterized by rapid small vessel thrombotic involvement of multiple organ systems. Studies are evaluating the efficacy of direct thrombin inhibitors in the management of refractory cases. Keywords: anticoagulants; anti-phospholipid syndrome; obstetric APS; thrombotic APS.


Assuntos
Síndrome Antifosfolipídica/terapia , Trombose/etiologia , Anticorpos Antifosfolipídeos/imunologia , Anticoagulantes/administração & dosagem , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/fisiopatologia , Feminino , Humanos , Gravidez , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/fisiopatologia , Complicações na Gravidez/terapia , Trombose/tratamento farmacológico
9.
Vasc Health Risk Manag ; 15: 253-258, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31496715

RESUMO

Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia characterized by venous and/or arterial thromboses, pregnancy morbidity (predominantly repeated fetal losses), and the presence of phospholipid antibodies. The estimated annual incidence of APS is 5 new cases per 100,000 people. The most common thrombotic events in patients with APS in order of frequency are stroke, transient ischemic attack, deep vein thrombosis, and pulmonary embolism. Patients with APS may develop an intracardiac thrombus, which is a life-threatening complication with a high risk of increased morbidity and mortality; however, it is treatable by surgical removal, extensive anticoagulant administration, and prevention of other complications. Catastrophic APS, which is a rare and severe condition diagnosed based on rapidly progressive thromboembolic events involving three or more organs, systems, or tissues, occurs in less than 1% of all patients with APS. We herein report an autopsy case of catastrophic APS in a 12-year-old Thai boy with multiple thromboembolic events including intracardiac thrombus formation with a positive lupus anticoagulant test result. To the best of our knowledge, this is the youngest reported patient with APS to date.


Assuntos
Síndrome Antifosfolipídica/complicações , Cardiopatias/etiologia , Tromboembolia/etiologia , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Autopsia , Biomarcadores/sangue , Doença Catastrófica , Criança , Evolução Fatal , Cardiopatias/sangue , Cardiopatias/diagnóstico , Cardiopatias/terapia , Humanos , Inibidor de Coagulação do Lúpus/sangue , Masculino , Tromboembolia/sangue , Tromboembolia/diagnóstico , Tromboembolia/terapia
10.
J Clin Neurosci ; 70: 247-249, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31540859

RESUMO

We present a case demonstrating histopathological clot findings after endovascular thrombectomy for acute ischaemic stroke in a 38 year-old male with systemic lupus erythematosus and antiphospholipid syndrome (APS). The differential diagnosis was embolism of a suspected Libman-Sacks vegetation or less likely an in-situ thrombosis. Clot analysis provided guidance with patient management and anticoagulation was commenced. The utility of clot analysis in this case provides support for routine clot analysis, which has been standard practice at our institution, and is likely to evolve as endovascular thrombectomy becomes more widely accessible.


Assuntos
Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Acidente Vascular Cerebral/etiologia , Trombose/patologia , Adulto , Procedimentos Endovasculares , Humanos , Masculino , Acidente Vascular Cerebral/cirurgia , Trombectomia
11.
Neurosciences (Riyadh) ; 24(3): 240-244, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31380826

RESUMO

New-onset refractory status epilepticus (NORSE) is a drug-resistant status epilepticus that often has a catastrophic outcome. Our patient was diagnosed with NORSE and had an EEG reading that showed status epilepticus persisting for 8 months in general anesthesia. After autoimmune workup showed positive antiphospholipid antibodies, his seizure was controlled, and he was discharged with good condition apart from moderate cognitive impairment. However, he later developed schizophrenia. Although psychiatric disorders have been associated with antiphospholipid syndrome, to the best of our knowledge, it has not been reported to be associated with status epilepticus. We recommend vigilance of psychological complications of refractory status epilepticus patients for early psychiatric referral, diagnosis, and treatment.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Esquizofrenia/diagnóstico , Estado Epiléptico/diagnóstico , Adulto , Síndrome Antifosfolipídica/complicações , Humanos , Masculino , Esquizofrenia/etiologia , Estado Epiléptico/etiologia
12.
Zhonghua Nei Ke Za Zhi ; 58(7): 496-500, 2019 Jul 01.
Artigo em Chinês | MEDLINE | ID: mdl-31269565

RESUMO

Antiphospholipid antibodies (aPLs) are a group of autoantibodies that target phospholipids and/or phospholipid-binding proteins. aPLs are important serum markers of anti phospholipid syndrome and are also risk factors for thrombosis and pathological pregnancy. Standardization of aPLs detection is critical to its clinical application.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Trombose/imunologia , Anticorpos Anticardiolipina/sangue , Anticorpos Anticardiolipina/imunologia , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Biomarcadores/sangue , Consenso , Feminino , Humanos , Gravidez , Trombose/prevenção & controle , beta 2-Glicoproteína I
13.
Med J Aust ; 211(4): 184-188, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31271468

RESUMO

Antiphospholipid syndrome is characterised by recurrent thrombosis (arterial, venous, microvascular) and/or pregnancy complications in the presence of persistent antiphospholipid antibodies (lupus anticoagulant, anti-ß2-glycoprotein 1 and anticardiolipin). It can be a primary disease or associated with another autoimmune disease (especially systemic lupus erythematosis). Testing for antiphospholipid antibodies should be considered in patients < 50 years of age with unprovoked venous or arterial thromboembolism, thrombosis at unusual sites or pregnancy complications. The mainstay of treatment is antithrombotic therapy and recommendations vary based on arterial, venous or pregnancy complications. If associated with systemic lupus erythematosis, hydroxychloroquine is recommended both as primary and secondary prophylaxis. Antithrombotic treatment is gold standard and effective.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Complicações na Gravidez/sangue , Anticoagulantes/uso terapêutico , Feminino , Humanos , Imunomodulação , Lúpus Eritematoso Sistêmico/complicações , Masculino , Pessoa de Meia-Idade , Gravidez , Complicações na Gravidez/terapia , Prevenção Primária , Ensaios Clínicos Controlados Aleatórios como Assunto , Prevenção Secundária , Trombose/prevenção & controle
14.
PLoS One ; 14(7): e0220033, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31339913

RESUMO

BACKGROUND: Persistent antiphospholipid antibodies (aPL) constitute the serological hallmark of the antiphospholipid syndrome (APS). Recently, various new assay technologies for the detection of aPL better suited to multiplex reaction environments than ELISAs emerged. We evaluated the diagnostic performance of such a novel line immunoassay (LIA) for the simultaneous detection of 10 different aPL. METHODS: Fifty-three APS patients and 34 healthy controls were investigated for criteria (antibodies against cardiolipin [aCL], ß2-glycoprotein I [aß2-GPI]) and non-criteria aPL (antibodies against phosphatidic acid [aPA], phosphatidyl-choline [aPC], -ethanolamine [aPE], -glycerol [aPG], -inositol [aPI], -serine [aPS], annexin V [aAnnV], prothrombin [aPT]) IgG and IgM by LIA. Criteria aPL were additionally determined with the established Alegria (ALE), AcuStar (ACU), UniCap (UNI), and AESKULISA (AES) systems and non-criteria aPL with the AES system. Diagnostic performance was evaluated with a gold standard for criteria aPL derived from the results of the four established assays via latent class analysis and with the clinical diagnosis as gold standard for non-criteria aPL. RESULTS: Assay performance of the LIA for criteria aPL was comparable to that of ALE, ACU, UNI, and AES. For non-criteria aPL, sensitivities of the LIA for aPA-, aPI-, aPS-IgG and aPA-IgM were significantly higher and for aPC-, aPE-, aAnnV-IgG and aPC- and aPE-IgM significantly lower than AES. Specificities did not differ significantly. CONCLUSIONS: The LIA constitutes a valuable diagnostic tool for aPL profiling. It offers increased sensitivity for the detection of aPL against anionic phospholipids. In contrast, ELISAs exhibit strengths for the sensitive detection of aPL against neutral phospholipids.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/diagnóstico , Testes Sorológicos/métodos , Adulto , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Feminino , Humanos , Imunoensaio/métodos , Imunoensaio/normas , Masculino , Pessoa de Meia-Idade , Fosfolipídeos/imunologia , Sensibilidade e Especificidade , Testes Sorológicos/normas
15.
J Am Podiatr Med Assoc ; 109(3): 235-240, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31268794

RESUMO

Antiphospholipid syndrome is an autoimmune disease characterized by vascular thrombosis involving both the arterial and venous systems that can lead to tissue ischemia or end-organ damage. Much of the literature describes various symptoms at initial presentation, but isolated tissue ischemia manifesting as a solitary blue toe is unusual. We discuss a case of a 23-year-old man who presented to the emergency department with a solitary blue fourth digit with minimal erythema and edema, who was suffering from exquisite pain. Following an extensive workup, the patient was diagnosed with antiphospholipid syndrome with thrombi of the vasculature in their lower extremity. With therapeutic anticoagulation, the patient's symptoms subsided and amputation of the digit was prevented.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/diagnóstico , Síndrome do Artelho Azul/etiologia , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Angiografia por Tomografia Computadorizada , Diagnóstico Diferencial , Enoxaparina/uso terapêutico , Pé/irrigação sanguínea , Pé/diagnóstico por imagem , Humanos , Masculino , Dor/etiologia , Artérias da Tíbia/diagnóstico por imagem , Dedos do Pé/irrigação sanguínea , Varfarina/uso terapêutico , Adulto Jovem
17.
Lupus ; 28(7): 868-877, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31132916

RESUMO

Introduction: Previous studies suggested different obstetric outcomes between patients with thrombotic or obstetric antiphospholipid syndrome, but the data are inconclusive. Aims: To investigate obstetric outcomes and their relation to the antiphospholipid antibody profile in primary thrombotic or obstetric antiphospholipid syndrome patients and compare those to a control population. Materials and methods: A retrospective single-centre study on a cohort of 30 pregnant women with primary antiphospholipid syndrome treated at Karolinska University Hospital Solna, Sweden between 2000 and 2016. The pregnancy outcomes were compared to the outcomes of all pregnancies in Stockholm County during the same period. Results: Preeclampsia (p < 0.001), low birth weight at delivery (p = 0.001), Apgar < 7 at 5 minutes (p < 0.001) and small infants (p < 0.001) were more common in antiphospholipid syndrome patients compared to controls. Obstetric antiphospholipid syndrome patients had a higher incidence of small infants (p = 0.023), lower birth weight (p = 0.013) and infants born with complications (p=0.004) compared to thrombotic antiphospholipid syndrome. Mothers with triple antibody positivity had a higher incidence of preeclampsia (p = 0.03), preterm delivery (p = 0.011), small infants (p=0.002) and infants born with complications (p = 0.012). Conclusions: Patients with primary antiphospholipid syndrome, especially those with obstetric antiphospholipid syndrome and triple antibody positivity, are at higher risk for adverse pregnancy outcomes, even under antithrombotic treatment. More frequent antenatal controls in high-risk patients can further improve outcomes.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/epidemiologia , Complicações na Gravidez/sangue , Resultado da Gravidez/epidemiologia , Trombose/sangue , Adulto , Síndrome Antifosfolipídica/diagnóstico , Peso ao Nascer , Feminino , Idade Gestacional , Heparina de Baixo Peso Molecular , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Inibidores da Agregação de Plaquetas/uso terapêutico , Pré-Eclâmpsia/epidemiologia , Gravidez , Complicações na Gravidez/epidemiologia , Nascimento Prematuro , Estudos Retrospectivos , Suécia , Trombose/imunologia , beta 2-Glicoproteína I/imunologia
18.
BMJ Case Rep ; 12(5)2019 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-31092489

RESUMO

A 58 year-old left-handed woman was transferred to our hospital with an evolving left middle cerebral artery stroke, severe thrombocytopenia and elevated inflammatory markers. She had a history of chronic Budd-Chiari syndrome (BCS) 16 months prior, attributed to a calcified web in the inferior vena cava that was stented. No thrombophilia testing was performed at that time. The current presentation demonstrated dense right-sided facial and arm paresis and neglect. Erythrocyte sedimentation rate and C-reactive protein were elevated, an autoimmune workup was consistent with a new diagnosis of systemic lupus erythematosus and triple-positive antiphospholipid antibodies. A transesophageal echocardiogram demonstrated a vegetation consistent with Libman-Sacks endocarditis (LSE), thought to have embolised to the brain. The patient was treated acutely with steroids, intravenous immunoglobulin and clopidogrel. This case demonstrates an atypical constellation of the antiphospholipid syndrome, with a novel presentation of BCS and LSE, and reinforces the importance of hypercoagulability screening in this population.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Endocardite não Infecciosa/etiologia , Lúpus Eritematoso Sistêmico/diagnóstico , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/complicações , Síndrome de Budd-Chiari/diagnóstico , Diagnóstico Diferencial , Endocardite não Infecciosa/diagnóstico , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/complicações , Pessoa de Meia-Idade , Acidente Vascular Cerebral/etiologia
19.
Neth J Med ; 77(3): 98-108, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31012427

RESUMO

INTRODUCTION: The antiphospholipid syndrome (APS) is defined by the occurrence of venous and/or arterial thrombosis and/or pregnancy-related morbidity, combined with the presence of antiphospholipid antibodies (aPL) and/or a lupus anticoagulant (LAC). Large, controlled, intervention trials in APS are limited. This paper aims to provide clinicians with an expert consensus on the management of APS. METHODS: Relevant papers were identified by literature search. Statements on diagnostics and treatment were extracted. During two consensus meetings, statements were discussed, followed by a Delphi procedure. Subsequently, a final paper was written. RESULTS: Diagnosis of APS includes the combination of thrombotic events and presence of aPL. Risk stratification on an individual base remains challenging. 'Triple positive' patients have highest risk of recurrent thrombosis. aPL titres > 99th percentile should be considered positive. No gold standard exists for aPL testing; guidance on assay characteristics as formulated by the International Society on Thrombosis and Haemostasis should be followed. Treatment with vitamin K-antagonists (VKA) with INR 2.0-3.0 is first-line treatment for a first or recurrent APS-related venous thrombotic event. Patients with first arterial thrombosis should be treated with clopidogrel or VKA with target INR 2.0-3.0. Treatment with direct oral anticoagulants is not recommended. Patients with catastrophic APS, recurrent thrombotic events or recurrent pregnancy morbidity should be referred to an expert centre. CONCLUSION: This consensus paper fills the gap between evidence-based medicine and daily clinical practice for the care of APS patients.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , 4-Hidroxicumarinas/uso terapêutico , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/complicações , Técnica Delfos , Feminino , Humanos , Indenos/uso terapêutico , Gravidez , Complicações na Gravidez/imunologia , Trombose/imunologia , Trombose/terapia , Vitamina K/antagonistas & inibidores , Vitamina K/uso terapêutico
20.
Clin Chim Acta ; 495: 205-209, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31002781

RESUMO

BACKGROUND: Increasing evidence suggests the role of non-criteria aPLs as important supplements to the current criteria aPLs in APS. In this study, we evaluated the clinical performance of a panel of non-criteria antibodies to phospholipid antigens, including, phosphatidylserine (aPS), phosphatidylinositol (aPI), sphingomyelin (aSM), phosphatidylcholine (aPC) and phosphatidylethanolamine (aPE) in a well-defined Chinese APS cohort. METHODS: A total of 229 subjects were tested, including 86 patients with APS, 104 disease controls (DCs) and 39 healthy controls (HCs). Serum IgG/IgM aCL, IgG/IgM aß2GP1, IgG/IgM aPS, IgG/IgM aPI, IgG/IgM aSM, IgG/IgM aPC, and IgG/IgM aPE were tested by ELISA. RESULTS: The presence of aPE, aPS, aPI, aPC, and aSM in patients with APS and Disease Controls were 8.1% (7/86) and 1.0% (1/104), 37.2% (32/86) and 9.6% (10/104), 50.0% (43/86) and 8.7% (9/104), 23.3% (20/86) and 1.0% (1/104), and 18.6% (16/86) and 1.9% (2/104), respectively. In criteria aPLs, aCL IgG demonstrated the highest positive likelihood ratio (LR+) of 35.75, followed by LA (LR+ of 13.51) and aCL IgM (LR+ of 11.64). In non-criteria aPLs, aPC IgG demonstrated the highest LR+ of 24.94 followed by aSM IgM (LR+ of 14.97). Importantly, the non-criteria aPLs were detected in 18.8% (3/16) of seronegative APS patients. The criteria aPLs, including LA, IgG aCL and IgG aß2GPI, were significantly correlated with both arterial thrombosis and venous thrombosis, while the non-criteria aPLs, including IgG aPS, IgM aPS, IgG aPI and IgG aPC were significantly associated with arterial thrombosis but not venous thrombosis. CONCLUSIONS: In summary, our findings indicate that those non-criteria aPLs may be particularly helpful for patients in whom APS is highly suspected, but conventional aPLs are repeatedly negative as well as for predicting APS patients with arterial thrombosis.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Síndrome Antifosfolipídica/diagnóstico , Estudos de Casos e Controles , Criança , China , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
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