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1.
Int J Hematol ; 110(2): 197-204, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31165409

RESUMO

Patients with lupus anticoagulant (LA), a thrombotic risk factor, along with decreased prothrombin (FII) activity are classified as lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) and occasionally show bleeding symptoms, although this is not essential for diagnosis. We treated 20 cases of LAHPS over a 3-year period. Median FII activity was 20.9% and the anti-prothrombin antibody (anti-II Ab), shown by ELISA findings, was detected in 55%. Bleeding symptoms were observed in 20%, although that finding was not correlated with FII activity or anti-FII Ab quantity. We also observed 21 LA cases with decreased activity of coagulation factors other than FII, which we have designated LAHPS-like syndrome (LLS). Among LLS patients, anti-FII Ab and bleeding symptoms were seen in 47.6% and 14.3%, respectively. Our findings suggest that bleeding in LAHPS and LLS cannot be explained only by FII activity decreased by anti-FII Ab. Low FVIII activity and the anti-FVIII antibody (anti-FVIII Ab) were detected in some LAHPS and LLS patients, making it difficult to distinguish those from acquired hemophilia A cases. Detection of anti-FVIII Ab quantity by ELISA may be useful for accurate determination, as that was not performed in our LAHPS or LLS patients.


Assuntos
Hipoprotrombinemias/epidemiologia , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/complicações , Trombofilia/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Autoanticorpos/sangue , Autoanticorpos/imunologia , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Fator VIII/imunologia , Feminino , Hemorragia/epidemiologia , Hemorragia/etiologia , Humanos , Hipoprotrombinemias/etiologia , Hipoprotrombinemias/imunologia , Japão/epidemiologia , Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Protrombina/análise , Protrombina/imunologia , Síndrome , Trombofilia/etiologia , Trombofilia/imunologia
2.
Lupus ; 28(7): 893-897, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31126213

RESUMO

OBJECTIVES: This study aims to inhibit antiphospholipid syndrome (APS) serum derived IgA anti-beta-2-glycoprotein I (aß2GPI) binding using Domain I (DI). METHODS: Serum from 13 APS patients was tested for IgA aß2GPI and Anti-Domain I. Whole IgA was purified by peptide M affinity chromatography from positive serum samples. Serum was tested for IgA aß2GPI binding in the presence and absence of either DI or of two biochemically modified variants containing either 20 kDa of poly(ethylene glycol) (PEG) or 40 kDa of PEG. RESULTS: Significant inhibition with DI was possible with average inhibition of 23% (N = 13). Further inhibitions using 20 kDa PEG-DI and 40 kDa PEG-DI variants showed significant inhibition (p = 0.0001) with both the 40 kDa PEG-DI and 20 kDa PEG-DI variants showing increased inhibition compared with DI alone (p = 0.0001 and p = 0.001, n = 10). CONCLUSIONS: Inhibition of IgA aß2GPI by DI is possible and can be enhanced by biochemical modification in a subset of patients.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Imunoglobulina A/imunologia , Inibidor de Coagulação do Lúpus/imunologia , beta 2-Glicoproteína I/metabolismo , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Londres , Lúpus Eritematoso Sistêmico/complicações , Masculino , Oxirredução , Turquia
3.
Hum Antibodies ; 27(2): 135-141, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30856108

RESUMO

BACKGROUND: Extractable nuclear antigen (ENA) and anti-double stranded DNA (anti-dsDNA) positivity and related diseases like systemic lupus erythematosus, Sjögren syndrome, and other autoimmune diseases are known to be associated with obstetrical complications and poor perinatal outcomes. OBJECTIVE: To demonstrate the importance of ENA, anti-dsDNA, antiphospholipid (APL), and anticardiolipin (ACL) antibody positivity on pregnancy outcomes. METHODS: Ninety one pregnant women with known ENA, anti-dsDNA, APL IgG and IgM, and ACL IgG and IgM antibody positivity were retrospectively compared with 91 randomly selected pregnant woman in terms of obstetrical complications and pregnancy outcomes. Beksac Obstetrics Index-pregnancy (BOIp), calculated as (number of children + (π/10))/gravidity in the current pregnancy, was used to compare the risk level between groups. RESULTS: Significant differences were found in the median maternal age, gravidity, number of previous miscarriages, and BOIp between the groups (p= 0.04, p< 0.001, p< 0.001, and p< 0.001, respectively). Significant differences were also found between the study and control groups in the median gestational age at birth, birth weight, and APGAR1 score (p< 0.001 for all). Similarly, significant differences were found between groups in the rates of intra-uterine growth restriction, oligohydramnios, and gestational hypertension (p< 0.001, p= 0.05, and p= 0.05, respectively). There were 3 (3.3%) stillbirths in the study group and none in the control group (p= 0.123). CONCLUSION: We evaluated the impact of anti-dsDNA, ENA, APL, and ACL antibody positivity, which may cause immunologic inflammation at placenta and thereby affect pregnancy outcomes.


Assuntos
Anticorpos Anticardiolipina/imunologia , Anticorpos Antifosfolipídeos/imunologia , Antígenos Nucleares/imunologia , Complicações na Gravidez/imunologia , RNA de Cadeia Dupla/imunologia , Aborto Espontâneo/imunologia , Adulto , Síndrome Antifosfolipídica/imunologia , Doenças Autoimunes/imunologia , Feminino , Retardo do Crescimento Fetal/imunologia , Idade Gestacional , Humanos , Imunoglobulinas/imunologia , Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Placenta/imunologia , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Fatores de Risco , Natimorto , Adulto Jovem
4.
Clin Rheumatol ; 38(3): 885-893, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30515663

RESUMO

OBJECTIVE: To validate the association of thrombotic events with positive lupus anticoagulant (LA) and co-presence of anti-RNP/Sm, as well as the diagnostic accuracy of this combination of antibodies for thrombosis. METHODS: Case-control study of patients with systemic lupus erythematosus (SLE) who presented thrombosis after SLE diagnosis and controls with SLE without thrombosis. Comorbidities, traditional risk factors, clinical variables, and treatment were evaluated. Antiphospholipid (aPL) and anti-RNP/Sm antibodies were determined. RESULTS: Sixty-three cases and 63 controls were studied, 88% women, median age of 40 years, and disease duration of 135 months at study inclusion. No differences were found between groups regarding age, comorbidities, or clinical characteristics at SLE diagnosis. Patients with thrombosis were more frequently positive for anti-RNP/Sm (p = 0.001), IgG aCL (p = 0.02), IgG anti-B2GPI (p = 0.02), IgM anti-B2GPI (p = 0.02), LA (p < 0.001), the combination of anti-RNP/Sm + LA (p < 0.001), and aPL triple marker (p = 0.002), compared to controls. The combination of anti-RNP/Sm + LA, SLEDAI-2 K, and prednisone dose was associated with thrombosis (p < 0.05). The combination of anti-RNP/Sm + LA showed 56% sensitivity, 79% specificity, 73% positive predictive value, 64% negative predictive value, positive likelihood ratio (LR) 2.69, and negative LR 0.56 for predicting thrombosis. No difference was found in the comparison of area under the curve between LA alone and the combination of anti-RNP/Sm + LA (p = 0.73). CONCLUSION: Thrombosis was associated with disease activity, dose of prednisone, and the combination of anti-RNP/Sm antibodies and LA. This combination of antibodies could be useful in the identification of SLE patients at risk of thrombosis.


Assuntos
Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/imunologia , Trombose/imunologia , Proteínas Centrais de snRNP/imunologia , Adolescente , Adulto , Anticorpos Anticardiolipina/imunologia , Anticorpos Antinucleares/imunologia , Anticorpos Antifosfolipídeos/imunologia , Autoanticorpos/imunologia , Estudos de Casos e Controles , Feminino , Glucocorticoides/administração & dosagem , Humanos , Imunoglobulina G/imunologia , Modelos Logísticos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Prednisona/administração & dosagem , Fatores de Risco , Trombose/epidemiologia , Adulto Jovem , beta 2-Glicoproteína I/imunologia
5.
Arthritis Rheumatol ; 71(2): 281-289, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30375754

RESUMO

OBJECTIVE: To determine, in a large, multiethnic/multiracial, prospective inception cohort of patients with systemic lupus erythematosus (SLE), the frequency, attribution, clinical, and autoantibody associations with lupus psychosis and the short- and long-term outcomes as assessed by physicians and patients. METHODS: Patients were evaluated annually for 19 neuropsychiatric (NP) events including psychosis. Scores on the Systemic Lupus Erythematosus Disease Activity Index 2000, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and the Short Form 36 (SF-36) were recorded. Time to event and linear regressions were used as appropriate. RESULTS: Of 1,826 SLE patients, 88.8% were female and 48.8% were Caucasian. The mean ± SD age was 35.1 ± 13.3 years, the mean ± SD disease duration was 5.6 ± 4.2 months, and the mean ± SD follow-up period was 7.4 ± 4.5 years. There were 31 psychotic events in 28 of 1,826 patients (1.53%), and most patients had a single event (26 of 28 [93%]). In the majority of patients (20 of 25 [80%]) and events (28 of 31 [90%]), psychosis was attributed to SLE, usually either in the year prior to or within 3 years of SLE diagnosis. Positive associations (hazard ratios [HRs] and 95% confidence intervals [95% CIs]) with lupus psychosis were previous SLE NP events (HR 3.59 [95% CI 1.16-11.14]), male sex (HR 3.0 [95% CI 1.20-7.50]), younger age at SLE diagnosis (per 10 years) (HR 1.45 [95% CI 1.01-2.07]), and African ancestry (HR 4.59 [95% CI 1.79-11.76]). By physician assessment, most psychotic events resolved by the second annual visit following onset, in parallel with an improvement in patient-reported SF-36 summary and subscale scores. CONCLUSION: Psychosis is an infrequent manifestation of NPSLE. Generally, it occurs early after SLE onset and has a significant negative impact on health status. As determined by patient and physician report, the short- and long-term outlooks are good for most patients, although careful follow-up is required.


Assuntos
Vasculite Associada ao Lúpus do Sistema Nervoso Central/epidemiologia , Transtornos Psicóticos/epidemiologia , Adulto , Fatores Etários , Anticorpos Anticardiolipina/imunologia , Autoanticorpos/imunologia , Estudos de Coortes , Feminino , Humanos , Estimativa de Kaplan-Meier , Modelos Lineares , Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Vasculite Associada ao Lúpus do Sistema Nervoso Central/imunologia , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Prospectivos , Transtornos Psicóticos/imunologia , Receptores de N-Metil-D-Aspartato/imunologia , Fatores Sexuais , Adulto Jovem , beta 2-Glicoproteína I/imunologia
6.
Br J Haematol ; 183(5): 703-716, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30488425

RESUMO

Chronic venous disease (CVD) represents a significant healthcare burden. Thrombophilia is proposed as a risk factor, particularly for post-thrombotic CVD. A systematic review was performed to determine the relationship between thrombophilia and non-thrombotic CVD. MEDLINE® and Embase® databases were searched from 1946 up to March 2018. Case-control studies, cohort studies or randomised clinical trials reporting on thrombophilias in non-thrombotic lower limb CVD in adult patients were included. Non-English and post-thrombotic syndrome studies were excluded. Study selection and data extraction were performed by two reviewers. Fifteen studies were included, reporting on 916 cases and 1261 controls. Studies largely focused on venous ulceration and investigated multiple haemostatic factors. An association between thrombophilia and non-thrombotic CVD was identified, with greater prevalence and factor concentration alteration reported in patients compared to controls. Concomitant thrombophilia presence was associated with earlier CVD onset. Relationship strength varied, with commoner aetiologies showing clearer correlation than rarer ones. Thrombophilia is associated with non-thrombotic CVD but the mechanism is unclear and causation cannot be determined. Future research should focus on prospective studies with larger populations and identify adjunct therapies targeting thrombophilia.


Assuntos
Extremidade Inferior/irrigação sanguínea , Trombofilia/complicações , Doenças Vasculares/etiologia , Veias , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos/sangue , Antitrombinas/metabolismo , Fatores de Coagulação Sanguínea/metabolismo , Doença Crônica , Feminino , Fibrinólise/fisiologia , Humanos , Hiper-Homocisteinemia/complicações , Inibidor de Coagulação do Lúpus/imunologia , Masculino , Pessoa de Meia-Idade , Mutação/genética , Protrombina/genética , Fatores de Risco , Úlcera Varicosa/etiologia , Adulto Jovem
7.
Thromb Res ; 172: 172-178, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30466070

RESUMO

BACKGROUND: Whether antibodies directed to ß2-Glycoprotein I (aß2GPI) are responsible for LA activity is not well defined. However, in the absence of such antibodies the molecule responsible for LA phenomenon is unknown. OBJECTIVE: The aim of this study was the biochemical identification of the target antigen epitope of aPL responsible of LA activity in the absence of aß2GPI antibodies together with the biological and clinical characteristics of these patients in comparison with classical triple positive patients. PATIENTS/METHODS: A comparison of patients with LA without (LA+/aß2GPI-) and those with (LA+/aß2GPI+) associated aß2GPI antibodies was performed. Size exclusion chromatography and analytical chromatography were used to identify the molecule with LA activity in patients LA+/aß2GPI-. RESULTS AND CONCLUSIONS: Analytical size-exclusion chromatography revealed a peak of 996Kd with LA activity perfectly overlapping that of IgM anti phosphatidylserine/prothrombin (aPS/PT) antibodies. Similarly, all the 25 LA+/aß2GPI- patients were positive for aPS/PT antibodies. LA+/aß2GPI- compared to 33 LA+/aß2GPI+ patients turned out to be significantly older, with a lower rate of previous thromboembolic events and a weaker LA activity. Search for aPS/PT and aß2GPI antibodies in patients with LA is useful to identify two subgroups of LA at different risk of thromboembolic events.


Assuntos
Anticorpos/imunologia , Inibidor de Coagulação do Lúpus/imunologia , beta 2-Glicoproteína I/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Feminino , Humanos , Imunoglobulina M/imunologia , Inibidor de Coagulação do Lúpus/análise , Masculino , Pessoa de Meia-Idade , Fosfatidilserinas/imunologia , Protrombina/imunologia , Tromboembolia/imunologia
8.
Haemophilia ; 24(5): 807-814, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30004159

RESUMO

INTRODUCTION: Accurate diagnosis of an inhibitor, a neutralizing antibody to infused factor VIII (FVIII), is essential for appropriate management of haemophilia A (HA). Low-titre inhibitors may be difficult to diagnose due to high rates of false-positive inhibitor results in that range. Transient low-titre inhibitors and false-positive inhibitors may be due to the presence of a lupus anticoagulant (LA) or other non-specific antibodies. Fluorescence immunoassay (FLI) to detect antibodies to FVIII is a sensitive method to identify inhibitors in HA. Evaluations of antibody profiles by various groups have demonstrated that haemophilic inhibitors detected by Nijmegen-Bethesda (NBA) and chromogenic Bethesda (CBA) assays correlate with positivity for anti-FVIII immunoglobulin (Ig) G1 and G4. AIM: This study sought to determine whether FLI could distinguish false-positive FVIII inhibitor results related to LAs from clinically relevant FVIII inhibitors in HA patients. METHODS: Samples from haemophilic and non-haemophilic subjects were tested for LA, specific FVIII inhibitors by NBA and CBA, and anti-FVIII immunoglobulin profiles by FLI. RESULTS: No samples from LA-positive non-haemophilic subjects were positive by FLI for anti-FVIII IgG4. Conversely, 91% of NBA-positive samples from haemophilia subjects were positive for anti-FVIII IgG4. Two of 11 haemophilia subjects had samples negative for anti-FVIII IgG4 and CBA, which likely represented LA rather than FVIII inhibitor presence. CONCLUSIONS: Assessment of anti-FVIII profiles along with the CBA may be useful to distinguish a clinically relevant low-titre FVIII inhibitor from a transient LA in HA patients.


Assuntos
Fator VIII/antagonistas & inibidores , Hemofilia A/imunologia , Inibidor de Coagulação do Lúpus/imunologia , Adolescente , Adulto , Idoso , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
9.
Clin Rheumatol ; 37(12): 3359-3364, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-29948348

RESUMO

Vascular antiphospholipid syndrome (VAPS) and obstetric (OAPS) are different entities because some patients only develop thrombosis (without recurrent pregnancy losses) and vice versa. Only two articles have reported that low 25-hydroxy-cholecalciferol (vitamin D3, VD3) levels were not correlated with the presence of conventional antiphospholipid antibodies (aPL Abs: anticardiolipin (aCL), anti-beta2glycoprotein I (aß2gpI), and lupus anticoagulant (LA)), but no article analyzed the association of VD3 and anti-annexin A5 (aanxA5) Abs. The aim of our study was to investigate the association between VD3, multiple positivity of conventional aPL and aanxA5 Abs levels only in female OAPS vs. VAPS. Our study included 62 consecutive female PAPS patients. Concentrations of Abs were measured by ELISA, while VD3 levels were determined by immunochemiluminescence. Only 10/62 (16.13%) had sufficient (≥ 30 ng/ml) VD3 levels, while 48/62 (77.42%) and 4/62 (6.45%) had insufficiency and VD3 deficiency, respectively. Statistically significant VD3 deficiency was noticed in VAPS (vs. OAPS, P = 0.013). A negative correlation between VD3 levels and the age of patients was noticed (r = - 0.493, P = 0.032) only in VAPS subgroup. Multiple positivity of aPL and aanxA5 Abs was not associated with VD3 deficiency. Newly emerging aPL Abs, such as aanxA5 Abs, or their combinations with classical aPL Abs are not associated with VD3 deficiency in neither OAPS nor VAPS patients. Due to its immunomodulatory roles in B-Ly homeostasis, supplementation with VD3 should be considered in APS, at least in subgroup with severe form of the disease, i.e., VAPS.


Assuntos
Anexina A5/química , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Calcifediol/química , Aborto Habitual , Adulto , Anexina A5/imunologia , Anticorpos Anticardiolipina/imunologia , Síndrome Antifosfolipídica/sangue , Calcifediol/imunologia , Cardiolipinas/imunologia , Colecalciferol/sangue , Colecalciferol/deficiência , Feminino , Humanos , Imunoglobulina G/imunologia , Imunoglobulina M/imunologia , Inibidor de Coagulação do Lúpus/imunologia , Pessoa de Meia-Idade , Gravidez , Estudos Retrospectivos , Trombose/imunologia , Trombose/patologia , beta 2-Glicoproteína I/imunologia
10.
Int J Gynaecol Obstet ; 142(2): 214-220, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29676461

RESUMO

OBJECTIVE: To assess outcomes after 20 weeks of pregnancy according to autoantibody profile and clinical presentation of maternal antiphospholipid syndrome (APS). METHODS: The present retrospective cohort analysis included women diagnosed with APS at a tertiary medical center in Israel between January 1, 2012, and December 31, 2016. Anticardiolipin antibodies, anti-ß2-glycoprotein antibodies, and lupus anticoagulant were assessed. Participants were stratified by type of APS (obstetric vs thrombotic), antibody profile, and antibody titer (low vs high). Primary composite outcomes were rated as severe (stillbirth, fetal growth restriction at <34 weeks, severe pre-eclampsia, or delivery at <32 weeks) and mild (stillbirth, any fetal growth restriction, any pre-eclampsia, or delivery at <34 weeks). RESULTS: A total of 99 women were included in the analysis. The primary composite outcomes were similar regardless of stratification. Lupus anticoagulant positivity was associated with delivery before 37 weeks. When compared with low antibody titer, high antibody titer was associated delivery at or before 32 weeks (P=0.045) and 34 weeks (P=0.029). CONCLUSION: High antibody titer might be associated with an increased risk of severe prematurity among pregnant women with APS.


Assuntos
Síndrome Antifosfolipídica/imunologia , Retardo do Crescimento Fetal/imunologia , Pré-Eclâmpsia/imunologia , Complicações na Gravidez/imunologia , Nascimento Prematuro/imunologia , Adulto , Anticorpos Anticardiolipina/sangue , Anticorpos Anticardiolipina/imunologia , Síndrome Antifosfolipídica/sangue , Autoanticorpos/sangue , Autoanticorpos/imunologia , Feminino , Humanos , Recém-Nascido de Baixo Peso/imunologia , Israel , Inibidor de Coagulação do Lúpus/sangue , Inibidor de Coagulação do Lúpus/imunologia , Gravidez , Complicações na Gravidez/sangue , Resultado da Gravidez , Glicoproteínas beta 1 Específicas da Gravidez/análise , Glicoproteínas beta 1 Específicas da Gravidez/imunologia , Estudos Retrospectivos , Natimorto , Adulto Jovem
11.
Semin Thromb Hemost ; 44(5): 445-452, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28898901

RESUMO

Lupus anticoagulant (LA) represents the most enigmatic antibody population in patients with antiphospholipid syndrome and represents a paradox that is still unsolved. This class of antiphospholipid antibody causes a phospholipid-dependent prolongation of the clotting time but is associated with an increased risk of thrombosis and pregnancy morbidity. In this review, we will provide an overview of the different antibodies that have been associated with LA activity, their importance based on clinical studies, and address the question why this prolongation of the clotting time is associated with thrombosis rather than a bleeding tendency.


Assuntos
Síndrome Antifosfolipídica/imunologia , Testes de Coagulação Sanguínea/métodos , Inibidor de Coagulação do Lúpus/imunologia , Feminino , Humanos , Gravidez
12.
Pan Afr Med J ; 31: 150, 2018.
Artigo em Francês | MEDLINE | ID: mdl-31037210

RESUMO

Antiphospholipid antibody syndrome is a thrombophilia characterized by the association of a clinical or obstetric arterial and/or venous thromboembolic event with persistent antiphospholipid antibodies. We here report the case of a young patient admitted with acute renal failure associated with bilateral renal artery thrombosis. Immunology tests showed lupus-like circulating anticoagulant on the basis of which the diagnosis of antiphospholipid antibodies was retained. Early angioplasty was performed enabling renal function recovery.


Assuntos
Lesão Renal Aguda/etiologia , Síndrome Antifosfolipídica/diagnóstico , Artéria Renal/patologia , Trombose/etiologia , Adolescente , Angioplastia/métodos , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/imunologia , Feminino , Humanos , Inibidor de Coagulação do Lúpus/imunologia
13.
J Pediatr Hematol Oncol ; 39(8): e521-e524, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-28787393

RESUMO

Lupus anticoagulant, also known as lupus antibody, is generally associated with thrombosis rather than bleeding events. Lupus anticoagulant-hypoprothrombinemia syndrome in children is rather rare but can lead to mild to life-threatening bleeding. Here, we report 3 cases of lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus. They initially presented with mucocutaneous bleedings, and subsequently developed other symptoms fulfilling the laboratory criteria for systemic lupus erythematosus. Case 2 and 3 had significant epistaxis and intracerebral hemorrhage responded to systemic corticosteroid along with fresh frozen plasma. Three cases demonstrated acquired hypoprothrombinemia with no correction of mixing studies. Case 1 had low factor X level, which has never been reported previously. In all 3 cases, their coagulogram returned to normal level after corticosteroid treatment.


Assuntos
Hipoprotrombinemias/diagnóstico , Hipoprotrombinemias/etiologia , Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/imunologia , Adolescente , Contagem de Células Sanguíneas , Testes de Coagulação Sanguínea , Medula Óssea/patologia , Criança , Feminino , Humanos , Hipoprotrombinemias/tratamento farmacológico , Imunossupressores/uso terapêutico , Inibidor de Coagulação do Lúpus/sangue , Lúpus Eritematoso Sistêmico/sangue , Troca Plasmática , Síndrome , Resultado do Tratamento
14.
J Pak Med Assoc ; 67(7): 1004-1008, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28770876

RESUMO

OBJECTIVE: Immune thrombocytopenia (ITP) is an immune haematologic disorder causing platelet destruction mediated by anti-platelet antibodies. In this study we aimed to evaluate the clinical and laboratory variables of ITP patients in southeast of Turkey. METHODS: In this retrospective study 167 ITP patients between 2005 and 2015 were evaluated. All patients were screened for immunological parameters including ANA (antinuclear antibodies), anti dsDNA (anti-double-stranded-DNA), ACA(anti-cardiolipin) IgM and IgG, LA (lupus anticoagulants). All patients were screened for Helicobacter pylori, HBsAg (Hepatitis B surface antigen), anti-HCV (hepatitis C virus antibody), and anti-HIV ½ (HIV antibody) and brucellosis.. RESULTS: Among the patients, 50 (29.9%) patients were male, 117 (70.1%) were female. The age range of patients was 18-86 (mean 38.16±14). In 56 patients (33.5%) splenectomy was performed. 36 patients (21.6%) were positive for ANA, 5 (3%) were positive for anti dsDNA, 14 (8.4%) for ACA Ig G, and 14 (8.4%) patients for ACA IgM. LA was tested in 165 patients and 30 (18%) patients were positive for LA. Microbiologic evaluation was as follows: 16 patients (9.6%) were positive for HbsAg, 109 (65.3%) positive for Anti-HBs, 5 positive for anti-HCV (3%), 56 (33.5%) patients were positive for Helicobacter pylori antigen, 5 (2.9%) for Brucella and one patient was positive for anti-HIV ½. CONCLUSIONS: Immune thrombocytopenia patients have to be evaluated according to their demographic characteristics and laboratory results. Secondary causes of ITP were HIV, HCV, Helicobacter pylori, brucellosis, tuberculosis, and autoimmune diseases in our region. Management of ITP patients can change in different regions.


Assuntos
Anticorpos Anticardiolipina/imunologia , Anticorpos Antinucleares/imunologia , Inibidor de Coagulação do Lúpus/imunologia , Púrpura Trombocitopênica Idiopática/imunologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Brucelose/complicações , Brucelose/imunologia , DNA/imunologia , Feminino , Infecções por HIV/complicações , Infecções por HIV/imunologia , Infecções por Helicobacter/complicações , Infecções por Helicobacter/imunologia , Helicobacter pylori/imunologia , Hepatite B/complicações , Hepatite B/imunologia , Anticorpos Anti-Hepatite B/imunologia , Antígenos de Superfície da Hepatite B/imunologia , Hepatite C/complicações , Hepatite C/imunologia , Anticorpos Anti-Hepatite C/imunologia , Humanos , Imunoglobulina G/imunologia , Imunoglobulina M/imunologia , Masculino , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/etiologia , Estudos Retrospectivos , Esplenectomia , Turquia , Adulto Jovem
15.
Isr Med Assoc J ; 19(6): 382-386, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28647939

RESUMO

BACKGROUND: Obstetric antiphospholipid syndrome (Obs-APS) is one of the most commonly identified causes of recurrent pregnancy loss and its accurate diagnosis is a requirement for optimal treatment. Some patients do not fulfill the revised Sapporo classification criteria, the original APS classification criteria, and are considered to be non-criteria Obs-APS. In these patients with non-criteria, there is controversy about their inclusion within the spectrum of APS and eventually their treatment as having Obs-APS. A subset of patients may also have clinical characteristics of Obs-APS even though lupus anticoagulant (LA), anticardiolipin antibodies, and anti-ß2-glycoprotein I (aß2GPI) antibodies are consistently negative. These patients are recognized as seronegative Obs-APS. We reviewed evidence of non-criteria Obs-APS and discuss a case of a woman with a diagnosis of active systemic lupus erythematosus (SLE) and non-criteria Obs-APS with four consecutive pregnancy losses. After an accurate diagnosis the patient received prenatal counseling and benefited from the optimal treatment of Obs-APS that led to a successful pregnancy. The applicability of this successful experience about outcomes in women with non-criteria, or seronegative, Obs-APS is also evaluated.


Assuntos
Aborto Habitual/etiologia , Síndrome Antifosfolipídica/imunologia , Anticorpos Anticardiolipina/análise , Anticorpos Antifosfolipídeos/análise , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/terapia , Feminino , Humanos , Inibidor de Coagulação do Lúpus/imunologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/terapia , Gravidez , beta 2-Glicoproteína I/imunologia
16.
Autoimmun Rev ; 16(7): 730-734, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28478081

RESUMO

AIM: To describe the consecutive pregnancy outcome and treatment in refractory obstetrical antiphospholipid syndrome (APS). METHODS: Retrospective multicenter open-labelled study from December 2015 to June 2016. We analyzed the outcome of pregnancies in patients with obstetrical APS (Sydney criteria) and previous adverse obstetrical event despite low-dose aspirin and low-molecular weight heparin LMWH (LMWH) conventional treatment who experienced at least one subsequent pregnancy. RESULTS: Forty nine patients with median age 27years (23-32) were included from 8 European centers. Obstetrical APS was present in 71%, while 26% had obstetrical and thrombotic APS. Lupus anticoagulant was present in 76% and triple antiphospholipid antibody (APL) positivity in 45% of patients. Pregnancy loss was noted in 71% with a median age of gestation of 11 (8-21) weeks. The presence of APS non-criteria features (35% vs 17% in pregnancies without adverse obstetrical event; p=0.09), previous intrauterine death (65% vs 38%; p=0.06), of LA (90% vs 65%; p=0.05) were more frequent in pregnancies with adverse pregnancy outcome, whereas isolated recurrent miscarriage profile was more frequent in pregnancies without any adverse pregnancy outcome (15% vs 41%; p=0.04). In univariate analysis considering all pregnancies (index and subsequent ones), an history of previous intrauterine death was associated with pregnancy loss (odds-ratio 2.51 (95% CI 1.274.96); p=0.008), whereas previous history of prematurity related to APS (odds-ratio 0.13 95%CI 0.04 0.41, P=0.006), steroids use during the pregnancy (odds-ratio 0.30 95% CI 0.11-0.82, p=0.019) and anticardiolipids isolated profile (odds-ratio 0.51 95% CI 0.26-1.03, p=0.0588) were associated with favorable outcome. In multivariate analysis, only previous history of prematurity, steroids use and anticardiolipids isolated profiles were associated with live-birth pregnancy. CONCLUSION: The main features of refractory obstetrical APS were the high rates of LA and triple APL positivity. Steroids could be effective in this APS profile, but prospective studies are necessary.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Aspirina/uso terapêutico , Heparina de Baixo Peso Molecular/uso terapêutico , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/imunologia , Feminino , Humanos , Inibidor de Coagulação do Lúpus/imunologia , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Resultado do Tratamento
17.
Thromb Res ; 153: 90-94, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28363116

RESUMO

BACKGROUND: Antiphospholipid syndrome (APS) is characterized by the presence of circulating antiphospholipid antibodies (aPL) in patients with thrombosis and/or pregnancy morbidity. In APS patients anti-domain 1 ß2-glycoprotein I (anti-D1 ß2GPI) IgG antibodies correlate strongly with thrombosis and to the lesser extent, with pregnancy complications. The aim of this study was to assess clinical utility of the anti-D1 ß2GPI antibodies in the diagnosis and risk stratification of antiphospholipid syndrome. PATIENTS/METHODS: In this retrospective study 202 autoimmune patients were studied (primary APS - 58, secondary - 45 SLE - 99). Anticardiolipin (aCL) and anti-ß2GPI (aß2GPI antibodies) (IgG and IgM class) together with anti-D1 IgG were tested with QUANTA Flash chemiluminescent immunoassay and lupus anticoagulant (LA) with coagulometric methods. RESULTS: The highest anti-D1 values were observed in triple positive patients as compared to patients with other antiphospholipid antibody profiles. A strong correlation was found between levels of anti-D1 IgG and a ß2GPI IgG antibodies for all patients analyzed (Spearman's ρ=0.87; p<0.0001). Anti-D1 IgG antibodies increase specificity resulting from classic aPL positivity but at the expense of sensitivity. Anti-D1 test does not add accuracy in predicting APS thrombotic complications on the top of accuracy offered by classic aPL tests and their profiles. CONCLUSIONS: Anti-D1 IgG antibodies did not add diagnostic power to the standard laboratory aPL tests as assessed by this retrospective study. A true clinical significance of anti-D1 antibodies in thrombotic risk stratification of aPL positive patients will require a properly designed clinical prospective trials.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/imunologia , Trombose/etiologia , beta 2-Glicoproteína I/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Anticardiolipina/imunologia , Síndrome Antifosfolipídica/diagnóstico , Feminino , Humanos , Imunoglobulina G/imunologia , Imunoglobulina M/imunologia , Inibidor de Coagulação do Lúpus/imunologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Trombose/diagnóstico , Trombose/imunologia , Adulto Jovem
18.
Lupus ; 26(12): 1297-1303, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28355988

RESUMO

Background Direct oral anticoagulants (DOACs) demonstrate a lower risk-benefit ratio than vitamin K antagonists (VKAs) for secondary thromboprophylaxis of thrombotic events. But there are no data on the efficacy of DOACs for the prevention of thrombotic recurrence in patients with antiphospholipid syndrome (APS). In this study, we evaluated the efficacy of DOACs to prevent recurrences of thrombotic events in patients with APS. Methods This was a single-center pilot, using a multi-step Fleming design. If seven or fewer patients presented treatment failure with rivaroxaban, the study could conclude efficacy. Results A total of 23 patients were included. APS involved the veins only ( n = 19), arteries only ( n = 2) or both ( n = 1) and 1 patient exhibited catastrophic antiphospholipid syndrome (CAPS). Overall, two patients were positive for lupus anticoagulant, anti-beta-2 glycoprotein I antibodies and anticardiolipid antibodies (triple positivity). The mean duration of follow up was 35.6 (range, 29-40) months. A total of six treatment failures were reported: one patient, with triple positivity, developed bilateral distal pulmonary embolism (PE) after 20 months of treatment with rivaroxaban, two patients refused to take rivaroxaban, the treatment was stopped in three other patients: two with adverse effects and one with chronic iron-deficiency anemia. Conclusions Rivaroxaban may represent an alternative for secondary thromboprophylaxis for thrombo-embolism in patients with APS, in particular, those with poor international normalized ratio (INR) control and those who are not at the highest risk of recurrent thrombosis, such as those with triple positivity.


Assuntos
Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Rivaroxabana/uso terapêutico , Trombose/prevenção & controle , Administração Oral , Adulto , Anticorpos Anticardiolipina/imunologia , Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/imunologia , Estudos de Coortes , Feminino , Seguimentos , Humanos , Inibidor de Coagulação do Lúpus/imunologia , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Embolia Pulmonar/etiologia , Embolia Pulmonar/prevenção & controle , Rivaroxabana/efeitos adversos , Trombose/etiologia , Adulto Jovem , beta 2-Glicoproteína I/imunologia
19.
Thromb Res ; 151 Suppl 1: S34-S37, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28262231

RESUMO

Antiphospholipid antibodies (APLAbs) are generally considered as risk factors for foetal death, for premature birth ≤34weeks due to severe pre-eclampsia or severe placental insufficiency and for recurrent consecutive spontaneous abortions <10weeks. Among these three obstetrical morbidities, only the first one is however not regularly questioned. The coexistence of an inflammatory disease and/or of thrombotic manifestations increases the obstetrical risks. Among the three criteria APLAbs, i.e. lupus anticoagulant (LA), anticardiolipin (aCL) Abs, anti-ß2 glycoprotein-I (aß2GP1)Abs, LA seems the more widely associated to clinical risks, the clinical impact of aß2GP1Abs is progressively defined and the pejorative impact of triple positivity is still discussed. High quality prospective multicentric epidemiological studies are still awaited. The identification of predictors of pregnancy outcome is necessary to streamline the design and use of new treatments acting on pathophysiological molecular targets.


Assuntos
Anticorpos Antifosfolipídeos/imunologia , Morte Fetal/etiologia , Pré-Eclâmpsia/etiologia , Aborto Habitual/etiologia , Aborto Habitual/imunologia , Anticorpos Anticardiolipina/imunologia , Feminino , Humanos , Inibidor de Coagulação do Lúpus/imunologia , Pré-Eclâmpsia/imunologia , Gravidez , Resultado da Gravidez , Nascimento Prematuro/etiologia , Nascimento Prematuro/imunologia , Estudos Prospectivos , beta 2-Glicoproteína I/imunologia
20.
Acta Neurol Taiwan ; 25(2): 65-69, 2016 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-27854095

RESUMO

BACKGROUND: Although Sjögren's syndrome has been known to complicate with white matter lesions, encephalopathy, or stroke, reports of cerebral venous sinus thrombosis due to Sjögren's syndrome with atypical antibodies are rare. CASE REPORT: A 50-year-old woman was admitted to our neurological ward with nausea and vomiting following acute onset of severe headache in the left occipital region. Brain computed tomography revealed no abnormalities. The patient was fully conscious, with normal cognitive functioning, but exhibited unsteady tandem gait. Both magnetic resonance venography and computed tomography venography suggested left transverse sinus blockage. Intravenous enoxaparin, followed by oral warfarin, was initiated as treatment for cerebral venous sinus thrombosis. After investigation, Sjögren's syndrome was diagnosed and lupus anticoagulant antibody test was positive. The patient was treated with hydroxychloroquine, and appeared fully recovered at the 6-month follow-up, with no clinical or radiological signs of relapse. CONCLUSION: This case reports the relationship between cerebral venous sinus thrombosis and Sjögren's syndrome. It is necessary to screen autoimmune disorders in patients with cerebral venous sinus thrombosis that present with no common risk factors of venous thrombosis in order to prevent inappropriate management, and potentially adverse outcomes.


Assuntos
Inibidor de Coagulação do Lúpus/imunologia , Trombose dos Seios Intracranianos , Síndrome de Sjogren , Feminino , Humanos , Pessoa de Meia-Idade , Trombose dos Seios Intracranianos/diagnóstico , Trombose dos Seios Intracranianos/etiologia , Trombose dos Seios Intracranianos/imunologia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/imunologia
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