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1.
Presse Med ; 48(11 Pt 2): 347-353, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31694791

RESUMO

Plasma exchange is a well-established therapeutic procedure commonly used in many autoimmune disorders. The beneficial effects of plasma exchange are thought to occur through the elimination of pathogenic mediators found in plasma, including autoantibodies, complement components, and cytokines. The catastrophic antiphsopholipid syndrome (CAPS) is a life-threatening variant of the antiphospholipid syndrome (APS) where several thrombosis take place in a short period of time in patients with circulating antiphospholipid antibodies. The triple therapy with anticoagulation, corticosteroids and plasma exchange or intravenous immunoglobulins has been proposed in CAPS. CAPS is a rare disease precluding the conduction of formal clinical trials. However, the observation of a better clinical course of patients who received this treatment supports their use. Plasma exchange has become an established therapeutic procedure in CAPS but there are no studies regarding the better approach and thus its use relies on the experience of the physicians in charge. The current article aims to review potential mechanisms of action of plasma exchange and the technical aspects of this procedure and will focus on its current role in CAPS, the experience published in treating this condition and the treatment protocol that we use in our institution.


Assuntos
Síndrome Antifosfolipídica/terapia , Troca Plasmática/métodos , Corticosteroides/uso terapêutico , Anticorpos Antifosfolipídeos/sangue , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Terapia Combinada/métodos , Hidratação/métodos , Humanos
3.
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
4.
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
5.
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
6.
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
7.
Autoimmun Rev ; 18(9): 102352, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31323355

RESUMO

Studies on last genetic and epigenetic predisposition to APS are summarized. It is well known that genetic predisposition is in HLA system (DR4 and DRw53) and that lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) are both associated with the same HLA antigens. Other genes, outside the MHC, give their contribution to the development of this autoimmune syndrome, such as IRF5, STAT4 and those related to inherited thrombophilia - factor V Leiden and G20210A prothrombin polymorphisms. Finally, post-transcriptional modifications of anti-beta2GPI antibodies could be implicated too. The most important discovery of last years is that altered microRNAs' expression is linked to autoimmunity, thrombosis, early atherosclerosis, and oxidative stress in APS.


Assuntos
Síndrome Antifosfolipídica/genética , Epigênese Genética/fisiologia , Antígenos HLA/genética , Resistência à Proteína C Ativada/sangue , Resistência à Proteína C Ativada/genética , Resistência à Proteína C Ativada/imunologia , Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Predisposição Genética para Doença , Genótipo , Humanos , Inibidor de Coagulação do Lúpus/sangue , Polimorfismo Genético , Trombose/complicações , Trombose/genética , Trombose/imunologia , beta 2-Glicoproteína I/imunologia
8.
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
9.
Methods Mol Biol ; 1967: 275-283, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31069777

RESUMO

ß2-Glycoprotein I is the major autoantigen in the antiphospholipid syndrome (APS), a prothrombotic disorder characterized by the occurrence of either venous or arterial thrombosis. In women it is also associated with an increased risk of obstetric complications such as recurrent miscarriages. We have identified that the plasma protein ß2-glycoprotein I in healthy individuals exists in an optimal ratio between two distinct forms, an oxidized and free thiol, reduced form. This ratio is disrupted in pathophysiological conditions associated with increased oxidative stress such as the APS, but also in the setting of age-related macular degeneration and gram-negative sepsis. We have developed assays that quantify plasma/serum levels of total and free thiol ß2-glycoprotein I which can potentially be used for risk stratification and prognostic purposes in the early stages of the aforementioned conditions.


Assuntos
Síndrome Antifosfolipídica/sangue , Testes Diagnósticos de Rotina/métodos , Trombose/sangue , beta 2-Glicoproteína I/sangue , Aborto Habitual/sangue , Aborto Habitual/patologia , Síndrome Antifosfolipídica/patologia , Feminino , Humanos , Gravidez , Prognóstico , Compostos de Sulfidrila/sangue , Compostos de Sulfidrila/química , Trombose/patologia , beta 2-Glicoproteína I/isolamento & purificação
10.
Arthritis Rheumatol ; 71(9): 1545-1552, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30957430

RESUMO

OBJECTIVE: To estimate the annual incidence and prevalence of and frequency of mortality associated with antiphospholipid syndrome (APS). METHODS: An inception cohort of patients with incident APS in 2000-2015 from a geographically well-defined population was identified based on comprehensive individual medical records review. All cases met the 2006 Sydney criteria for APS (primary definition) or had a diagnosis of APS confirmed by physician consensus (secondary definition). Levels of lupus anticoagulant, IgM and IgG anticardiolipin antibodies, and anti-ß2-glycoprotein I antibodies were tested in a centralized laboratory. Incidence rates were age- and sex-adjusted to the 2010 US white population. Prevalence estimates were obtained from the incidence rates, assuming that there was no increased mortality associated with APS and that migration in or out of the area was independent of disease status. RESULTS: Among this cohort in 2000-2015, 33 cases of incident APS, as defined by the Sydney criteria, were identified (mean age of patients 54.2 years; 55% female, 97% white). The annual incidence of APS in adults ages ≥18 years was 2.1 (95% confidence interval [95% CI] 1.4-2.8) per 100,000 population. Incidence rates were similar in both sexes. The estimated prevalence of APS was 50 (95% CI 42-58) per 100,000 population, and was similar in both sexes. Six patients (18%) had a concurrent diagnosis of systemic lupus erythematosus. The most frequent clinical manifestation was deep vein thrombosis. The overall frequency of mortality among patients with APS was not significantly different from that in the general population (standardized mortality ratio 1.61, 95% CI 0.74-3.05). CONCLUSION: APS occurred in ~2 persons per 100,000 population per year. The estimated prevalence was 50 per 100,000 population. Overall mortality was not notably different from that observed in the general population.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Adulto , Anticorpos Anticardiolipina/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Austrália/epidemiologia , Autoanticorpos/sangue , Feminino , Humanos , Imunoglobulina M/sangue , Incidência , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Prevalência , Trombose Venosa/sangue , Trombose Venosa/epidemiologia , Trombose Venosa/imunologia , Adulto Jovem , beta 2-Glicoproteína I/imunologia
11.
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
12.
Rev Med Interne ; 40(6): 351-354, 2019 Jun.
Artigo em Francês | MEDLINE | ID: mdl-30905593

RESUMO

PURPOSE: Antiphospholipid syndrome (APS) is a clinico-biological syndrome, which associates vascular injury and persisting antiphospholipid antibodies (aPL). Patients with clinical symptoms of APS but without aPL are defined as "seronegative APS" (SNAPS). The aim of this study was to evaluate antiphosphatidylethanolamine antibody (aPE) investigation in patients with SNAPS suspicion. METHODS: This retrospective study was conducted in patients with SNAPS suspicion. A homemade enzyme-linked immunosorbent assay (ELISA) was used to search for aPE. The results of this homemade method were compared with those from a global screening ELISA. RESULTS: Two hundred twenty-eight patients with SNAPS suspicion were included. Among them, 58.3% had a thrombotic event. The homemade ELISA found positive persisting aPE in 23 patients (10%): 15 with a thrombotic event, 6 with obstetrical morbidity and 2 with a combined event. The global screening ELISA was positive in only 11 of these 23 patients (47.8%). CONCLUSION: These results suggest the implication of aPE in SNAPS.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/imunologia , Fosfatidiletanolaminas/imunologia , Adulto , Idoso , Síndrome Antifosfolipídica/diagnóstico , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
13.
Int J Lab Hematol ; 41(3): 412-417, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30860670

RESUMO

INTRODUCTION: Lupus anticoagulant (LAC) testing is a multistep procedure including screening, mixing, and confirmation tests. STA Coag Expert is a software module for STA R Max and STA Compact Max analyzers which includes an on-demand LAC algorithm, based on ISTH guidelines, for automatic interpretation, calculation, and launch of assays in LAC interpretation ("Stago coag algorithm"). MATERIALS AND METHODS: One hundred ninety four patient samples were analyzed in parallel and interpreted manually and automatically by LAC algorithms. LAC algorithms use identical flowcharts and cutoff values as in daily practice. Differently, it only uses index of circulating anticoagulant (ICA), whereas in routine also normalized ratios were assessed for interpretation of mixing tests. Interpretation of dRVVT and aPTT pathways and final conclusions were compared between both approaches. RESULTS: Compared to routine interpretation, LAC algorithm showed a sensitivity of 94% and a specificity of 100% for LAC detection, when discrepancies due to measured clotting times between both analyzers were excluded. Three false negatives were due to different interpretation of dRVVT mixing test. Discrepancies in interpretation of the aPTT mixing test (n = 11) did not result in discrepant final LAC result, all having negative confirmation tests. No false positives were observed. With LAC algorithm, hands-on time reduced from 200 to 80 minutes. CONCLUSION: The LAC algorithm of the STA Coag Expert shows good comparability to the manual interpretation of LAC and may be used to assist laboratories in automatic launching of additional tests and in interpretation of LAC according to ISTH guidelines. This way the STA Coag Expert LAC algorithm may improve interlaboratory and STA comparability of LAC results.


Assuntos
Imunoensaio/métodos , Inibidor de Coagulação do Lúpus/sangue , Algoritmos , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/tratamento farmacológico , Automação Laboratorial , Testes de Coagulação Sanguínea/métodos , Testes de Coagulação Sanguínea/normas , Humanos , Imunoensaio/normas , Tempo de Tromboplastina Parcial , Reprodutibilidade dos Testes , Software
14.
Int J Rheum Dis ; 22(4): 677-685, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30729698

RESUMO

AIM: Dysregulated apoptosis has been implicated in autoimmune diseases. In the present study, we investigated the apoptosis-related cytokines and apoptosis in patients with primary antiphospholipid syndrome (pAPS). METHOD: We prospectively recruited 12 pAPS patients, 17 antiphospholipid antibody (APA)-positive systemic lupus erythematosus (SLE) patients without APS manifestations (APA+ SLE), 13 SLE patients with secondary APS (APS+ SLE) and 10 healthy controls (HCs). Plasma levels of soluble apoptosis-inducing ligands and cytokines, and the expression levels of apoptosis-inducing ligands in peripheral blood mononuclear cells, were determined. In addition, blood lymphocytes/monocytes apoptosis were determined in six pAPS patients and six HCs, using flow cytometric analysis of caspase 3, 8 and 9 activities. RESULTS: There was a trend toward higher plasma levels of soluble tumor necrosis factor (TNF)-related apoptosis-inducing ligand (sTRAIL), interleukin-10 (IL-10) and TNF-α in pAPS patients when compared with HCs. We also observed higher plasma levels of IL-10 and TNF α in APA+ SLE and APS+ SLE patients when compared with HCs. However, there was no significant difference in blood lymphocytes/monocytes apoptosis between pAPS patients and HCs. CONCLUSION: There was a trend toward elevated plasma levels of sTRAIL, IL-10 and TNF-α, but no evidence for dysregulated apoptosis in pAPS patients.


Assuntos
Síndrome Antifosfolipídica/patologia , Proteínas Reguladoras de Apoptose/sangue , Apoptose , Citocinas/sangue , Linfócitos/patologia , Monócitos/patologia , Adulto , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/genética , Síndrome Antifosfolipídica/imunologia , Proteínas Reguladoras de Apoptose/genética , Biomarcadores/sangue , Estudos de Casos e Controles , Feminino , Humanos , Interleucina-10/sangue , Linfócitos/imunologia , Linfócitos/metabolismo , Masculino , Pessoa de Meia-Idade , Monócitos/imunologia , Monócitos/metabolismo , Estudos Prospectivos , Ligante Indutor de Apoptose Relacionado a TNF/sangue , Fator de Necrose Tumoral alfa/sangue
15.
Lupus ; 28(3): 427-431, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30717622

RESUMO

OBJECTIVE: The study aims to investigate the ovarian reserve in premenopausal women with antiphospholipid syndrome (APS) and to evaluate whether it is associated with cumulative organ damage or the risk of clinical complications. METHODS: This single-center study was conducted in 23 premenopausal female patients (10 with primary APS and 13 with secondary APS) and 24 healthy volunteers. Serum anti-Müllerian hormone (AMH) levels were measured by enzyme-linked immunoassay. Disease-specific organ damage (DIAPS score) and the risk of clinical complications (aGAPSS score) were additionally evaluated in APS patients. RESULTS: Serum AMH levels were similar in APS patients (median 6.06, interquartile range 4.31-7.54 ng/ml) and in controls (4.87, 2.64-6.40 ng/ml; P = 0.116), and no differences were observed between the primary (6.60, 5.49-8.88 ng/ml) and secondary (6.06, 3.91-7.30 ng/ml; P = 0.532) forms of the syndrome. In individuals with APS, serum AMH levels correlated inversely with the aGAPSS score (rho-0.421, 95% confidence intervals -0.716 to -0.001; P = 0.045), while no associations were observed with the DIAPS score (rho-0.001, -0.423 to 0.422; P = 0.996). CONCLUSIONS: Ovarian reserve is not reduced in premenopausal women with APS. In addition, serum AMH levels may reflect the risk of APS-related clinical complications but not the burden of disease-specific organ damage.


Assuntos
Síndrome Antifosfolipídica/sangue , Reserva Ovariana/imunologia , Adulto , Hormônio Antimülleriano/sangue , Síndrome Antifosfolipídica/complicações , Estudos de Casos e Controles , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Pessoa de Meia-Idade , Pré-Menopausa
16.
BMJ Case Rep ; 12(1)2019 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-30635311

RESUMO

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterised by thromboembolic events including venous thromboembolism (VTE) in association with the presence of antiphospholipid antibodies. The standard treatment of VTE historically consists of anticoagulation therapy with warfarin, a vitamin K antagonist. Recently, direct oral anticoagulants, including rivaroxaban have become available for the treatment of VTE. However, the choice of anticoagulant, and the duration of anticoagulation in patients with APS has not been determined yet due to lack of evidence. Here, we report a case of recurrent venous thrombosis after discontinuation of rivaroxaban therapy and avoiding sedentary lifestyle in a patient with APS. We suggest that indefinite anticoagulation therapy might be needed even in low-risk APS cases.


Assuntos
Síndrome Antifosfolipídica/complicações , Rivaroxabana/uso terapêutico , Tromboembolia Venosa/etiologia , Trombose Venosa/etiologia , Assistência ao Convalescente , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/patologia , Inibidores do Fator Xa/uso terapêutico , Produtos de Degradação da Fibrina e do Fibrinogênio/análise , Humanos , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/tratamento farmacológico , Embolia Pulmonar/etiologia , Recidiva , Rivaroxabana/administração & dosagem , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Tromboembolia Venosa/diagnóstico por imagem , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/tratamento farmacológico
17.
Pathology ; 51(3): 292-300, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30665674

RESUMO

We and others have previously highlighted the potential problems with testing of lupus anticoagulants (LA) in patients on anticoagulant therapy, including most recently as related to the direct oral anticoagulants (DOACs). Thus, current DOACs in use (e.g., dabigatran, a direct thrombin inhibitor, and apixaban and rivaroxaban, both direct Xa inhibitors), affect a wide variety of coagulation assays, including those used in LA investigation. The Russell viper venom time (RVVT) assay in particular, key to the investigation of LA, is highly sensitive to DOACs. LA is a marker of thrombophilia, and patients who have had a thrombosis may be placed on a DOAC. Thus, there is a high likelihood that LA testing will be requested on patients whilst they are on DOACs. In the current report, we have assessed data from our facility for the past two and a half years for all LA tests performed by RVVT testing, and have evaluated this data with respect to patient anticoagulant status. In total, there were 7170 test requests for RVVT associated testing during the period of data capture. Most LA-RVVT screen results (5008; ∼70%) were within normal limits, thereby excluding LA by RVVT method in most of the patient cohort. All DOACs led to a prolongation in both RVVT screen and confirm assays. However, rivaroxaban affected the screen more than the confirm, leading to higher RVVT ratios, whereas apixaban affected the confirm more than the screen, leading to lower RVVT ratios. LA testing in the presence of DOACs also led to lower intra-patient consistency in LA test results. We conclude that ex-vivo data appears to confirm the potential for false positive (with rivaroxaban) and potential for false negative (with apixaban) identification of LA in patients on DOAC treatment. We also make some recommendations in regards to such testing.


Assuntos
Anticoagulantes/farmacologia , Síndrome Antifosfolipídica/diagnóstico , Testes de Coagulação Sanguínea , Coagulação Sanguínea/efeitos dos fármacos , Inibidor de Coagulação do Lúpus/análise , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/sangue , Dabigatrana/farmacologia , Dabigatrana/uso terapêutico , Reações Falso-Negativas , Reações Falso-Positivas , Humanos , Tempo de Protrombina , Rivaroxabana/farmacologia , Rivaroxabana/uso terapêutico , Trombofilia/sangue , Trombofilia/tratamento farmacológico
18.
Intern Emerg Med ; 14(4): 521-527, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30603858

RESUMO

How thrombophilia may contribute to the development of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. We searched on PubMed and EMBASE (until 15 April 2018), studies on CTEPH reporting data on inherited or acquired thrombophilia. Starting from 367 articles mentioning the search terms, 347 were excluded mainly as duplicate articles or articles not in English. After reading the full text of remaining articles, ten were excluded for being reviews, editorials, letters or case reports, and two were further removed from the analysis because of the potential selection bias. All the eight considered studies provided the proportion of patients positive for antiphospholipid (aPL) antibodies. The crude rate of aPL in CTPEH patients is 11.8% (95% CI 10.09-13.8%). The meta-analysis considering the weighted mean proportion and 95% confidence intervals (CIs) yields a rate of aPL antibody-positive profile of 12.06% (95% CI 8.12-16.65%) among the patients with CTEPH in the random effects model (I2 76.33%; 95% CI 52.75-88.14%, p = 0.0001). The sensibility analysis confirms the result. No predictors of heterogeneity are found in a meta-regression analysis. Our results suggest that aPL antibodies are frequently associated with CTEPH underlining the need to test for aPL antibodies in young patients with "idiopathic" and "provoked" PE caused by mild provoking risk factors.


Assuntos
Anticorpos Antifosfolipídeos/análise , Hipertensão Pulmonar/complicações , Prevalência , Trombose/etiologia , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/fisiopatologia , Humanos , Hipertensão Pulmonar/sangue , Hipertensão Pulmonar/fisiopatologia , Fatores de Risco , Trombose/sangue , Trombose/fisiopatologia
19.
Curr Rheumatol Rev ; 15(1): 59-66, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-29756580

RESUMO

BACKGROUND AND OBJECTIVES: Concomitant neurologic manifestations and positive antiphospholipid antibodies (APAs) have been investigated in different manners. The present study aimed to investigate the association between neurologic manifestations and APAs. MATERIALS AND METHODS: This cross-sectional descriptive study was conducted on 100 consecutive patients with selected neurological manifestations and at least one positive APAs within the age range of 20-50 years, referred to the Rheumatic Diseases Research Center from the Northeast Central Neurology Department of Iran during August 2012 to March 2014. RESULTS: According to the results, 89% of the participants were persistently positive for APAs, including lupus anticoagulant, IgG anticardiolipin (aCL), IgM aCL, IgG ß-2 glycoprotein 1 (ß2- GP1), and IgM ß2-GP1, observed in 16%, 41%, 42%, 17%, and 15% of the patients, respectively. Furthermore, 10% of the patients had concomitant lupus manifestations, and 37% of them showed anti-DNA. The IgG and IgM aCL were the most prevalent antibodies. Cerebral vascular accident (33%), retinal artery/vein occlusion (21%), and seizure (20%) were the most frequent presentations among the patients. In addition, the patients with multiple sclerosis (composing 3% of the subjects) were 100% positive for IgG and IgM aCL, as well as lupus anticoagulant. In addition, IgM anti-ß2- GP1 was 100% positive in optic neuritis patients (composing 5% of the subjects) and was significantly associated with this neurologic disorder. IgM anti-ß2-GP1 was also prevalent in the cases with Guillain-Barré syndrome. The most prevalent persistently positive antibody in the patients with cerebrovascular accident was IgM aCL. CONCLUSION: The findings of this study revealed some associations between the subtypes of APAs and incidence of neurologic disorders. However, the exact correlation between those symptoms and APAs needs further investigations.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/imunologia , Doenças do Sistema Nervoso/imunologia , Adulto , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/sangue , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/sangue , Adulto Jovem
20.
Arthritis Care Res (Hoboken) ; 71(1): 134-141, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29669399

RESUMO

OBJECTIVE: Although systemic lupus erythematosus (SLE) is the most common autoimmune disease associated with antiphospholipid antibodies (aPL), limited data exist regarding the impact of SLE on the clinical phenotype of aPL-positive patients. The primary objective of this study was to compare the clinical, laboratory, and treatment characteristics of aPL-positive patients with SLE with those of aPL-positive patients without SLE. METHODS: A secure web-based data capture system was used to store patient demographic characteristics and aPL-related clinical and laboratory characteristics. Inclusion criteria included positive aPL according to the updated Sapporo classification criteria. Antiphospholipid antibody-positive patients fulfilling the American College of Rheumatology criteria for the classification of SLE ("aPL with SLE") and those with no other autoimmune diseases ("aPL only") were included in the analysis. RESULTS: Six hundred seventy-two aPL-positive patients were recruited from 24 international centers; 426 of these patients did not have other autoimmune disease, and 197 had SLE. The frequency of thrombocytopenia, hemolytic anemia, low complement levels, and IgA anti-ß2 -glycoprotein I (anti-ß2 GPI) antibodies was higher in the aPL-positive patients with SLE, whereas the frequency of cognitive dysfunction and IgG anti-ß2 GPI antibodies was higher in the aPL-only group. The frequency of arterial and venous thromboses (including recurrent) as well as pregnancy morbidity was similar in the 2 groups. The prevalence of cardiovascular disease risk factors at the time of entry into the registry entry did not differ between the 2 groups, with the exception of current smoking, which was more frequent in aPL-positive patients with SLE. CONCLUSION: Although the frequencies of thrombosis and pregnancy morbidity are similar in aPL-positive patients with and those without SLE, the diagnosis of SLE in patients with persistently positive aPL is associated with an increased frequency of thrombocytopenia, hemolytic anemia, low complement levels, and positive IgA anti-ß2 GPI antibodies.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/epidemiologia , Bases de Dados Factuais , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/epidemiologia , Fenótipo , Adulto , Síndrome Antifosfolipídica/diagnóstico , Feminino , Humanos , Internacionalidade , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Pessoa de Meia-Idade , Gravidez , Sistema de Registros
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