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1.
J Korean Med Sci ; 35(5): e35, 2020 Feb 10.
Artigo em Inglês | MEDLINE | ID: mdl-32030922

RESUMO

BACKGROUND: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by arterial and venous thrombosis or pregnancy morbidity in patients with persistent antiphospholipid antibodies. However, nationwide population-based epidemiology studies regarding APS are still unavailable. METHODS: We analyzed claims data extracted from the Korean Health Insurance and Review Agency (HIRA) covering more than 52 million Koreans, between January 1, 2008, and December 31, 2017. Patients diagnosed with APS, as determined by the Korean Classification of Disease, 7th edition (D68.6), and a rare intractable disease program (V253), were identified in HIRA. RESULTS: A total of 3,088 newly diagnosed incident cases of 1,215 men and 1,873 women were identified during 2009-2016. The mean age was 44.6 ± 16.6 (men, 47.4 ± 16.3; women, 42.8 ± 16.6) years. The incidence was 0.75 per 105 person-year (95% confidence interval, 0.73-0.78). The prevalence in 2016 was 6.19 per 105 people. For incident cases, women showed incidence peak at ages of 30-39 years and 70-79 years, whereas for men, it was highest at ages of 70-79 years only. Of all patients, 1,766 (57%, 810 men and 956 women) had primary APS, 1,322 (43%, 405 men and 917 women) had secondary APS, and 845 (27%, 216 men and 629 women) were associated with systemic lupus erythematosus (SLE). CONCLUSION: The incidence of APS differs according to age groups and gender. The incidence of primary APS was higher than that of secondary APS in both gender. Furthermore, as already reported, secondary APS is highly associated with SLE; however, we observed that rheumatoid arthritis is also highly related.


Assuntos
Síndrome Antifosfolipídica , Lúpus Eritematoso Sistêmico , Adulto , Fatores Etários , Idoso , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/epidemiologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Masculino , Pessoa de Meia-Idade , Gravidez , Complicações na Gravidez/epidemiologia , Prevalência , República da Coreia/epidemiologia , Fatores Sexuais , Trombose Venosa/complicações
2.
Lupus ; 28(9): 1158-1166, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31299879

RESUMO

INTRODUCTION: Although extensively characterized in the outpatient setting, systemic lupus erythematosus (SLE) in the hospitalization wards is still scarcely portrayed, particularly in the perspective of its evolution over the years. METHODS: Retrospective analysis of SLE patients hospitalized in the Department of Autoimmune Diseases of a university hospital during a 20-year period (1995-2015), describing hospitalization characteristics, causes and predictors of outcome. RESULTS: A total of 814 hospitalizations concerning 339 patients were analysed. The main causes of admission were flare (40.2%), infection (19.2%), diagnostic procedures (18.8%) and thrombotic events (5.4%). Therapy with cyclophosphamide (odds ratio (OR) 1.908, p = 0.047) was associated with admission due to infection, while antimalarials displayed a protective effect (OR 0.649, p = 0.024). Nearly 3.9% of patients required admission to an intensive care unit, with associated antiphospholipid syndrome (OR 7.385, p = 0.04) standing as a predicting factor for this outcome. Readmission at 30 days occurred in 5.8% of patients, with thrombocytopenia (OR 6.007, p = 0.002) and renal involvement (OR 3.362, p = 0.032) featuring as predicting factors. Eight patients died, with antiphospholipid syndrome (OR 26.814, p = 0.02) and thrombocytopenia (OR 31.523, p = 0.01) being associated with mortality. There was no significant variation in patients' demographics or admission causes across the 20-year period, except for a decrease in admissions due to thrombotic and musculoskeletal causes. Recently, an increase in the use of mycophenolate mofetil and lower doses of glucocorticoids were noted. CONCLUSION: While demographics of SLE hospitalizations have not markedly changed over the past 20 years, changes in therapy patterns were observed. Thrombocytopenia, antiphospholipid syndrome and renal involvement featured as predictors of poor outcome.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Hospitalização/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/terapia , Trombocitopenia/epidemiologia , Adulto , Síndrome Antifosfolipídica/etiologia , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Pessoa de Meia-Idade , Readmissão do Paciente/estatística & dados numéricos , Estudos Retrospectivos , Espanha , Trombocitopenia/etiologia
3.
Thromb Haemost ; 119(9): 1403-1408, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31226720

RESUMO

Antiphospholipid syndrome (APS) is an acquired thrombophilia with an uncertain role in the development of chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to assess the association of APS with the clinical phenotype of CTEPH. We retrospectively reviewed data of CTEPH patients referred to our center. Clinical, angiographic, and hemodynamic data were available for all patients. APS was diagnosed in the presence of one or more positive antiphospholipid (aPL) tests confirmed more than 12 weeks apart. Data were compared between APS-positive and APS-negative patients. From May 2013 to December 2018, 297 patients with CTEPH were enrolled. Twenty-three (7.7%) were positive for laboratory tests exploring aPL antibodies. Among them, 17 patients (74%) had a triple positive aPL profile. When compared with the APS-negative group, APS patients were significantly younger (30.0 ± 11.1 vs. 55.6 ± 12.9 years, p < 0.0001), had more frequently a history of pulmonary embolism (95.6% vs. 65.7%, p = 0.003), and had more frequently associated autoimmune disease (43.5% vs. 2.9%, p < 0.0001). In APS-positive patients, pulmonary artery lesions were more proximal and hemodynamic profiles were less compromised. Our results show that patients with APS are a unique group of CTEPH patients with well-defined clinic and hemodynamic characteristics.


Assuntos
Fatores Etários , Síndrome Antifosfolipídica/epidemiologia , Doenças Autoimunes/epidemiologia , Hipertensão Pulmonar/epidemiologia , Embolia Pulmonar/epidemiologia , Tromboembolia/epidemiologia , Adulto , Idoso , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/complicações , China/epidemiologia , Doença Crônica , Feminino , Hemodinâmica , Humanos , Hipertensão Pulmonar/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Tromboembolia/complicações , Adulto Jovem
4.
Medicine (Baltimore) ; 98(20): e15733, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31096533

RESUMO

BACKGROUND: Antiphospholipid syndrome (APS) is a rare heterogenous autoimmune disorder with severe life-threatening complications shown during pregnancy. In this analysis, we aimed to systematically compare the pregnancy outcomes (both maternal and fetal) in patients with APS. METHODS: Web of Science, Google Scholar, Medicus, Cochrane Central, Embase, and Medline were searched for relevant English publications. The main inclusion criteria were based on studies that compared pregnancy outcomes in patients with APS vs a control group. Statistical analysis was carried out by the RevMan software version 5.3. This analysis involved dichotomous data, and risk ratios (RR) with 95% confidence intervals (CIs) were used to represent the analysis. RESULTS: Eight studies consisting of a total number of 212,954 participants were included. Seven hundred seventy participants were pregnant women with APS and 212,184 participants were assigned to the control group. Pregnancy-induced hypertension was significantly higher in women with APS (RR: 1.81, 95% CI: 1.33 - 2.45; P = .0002). The risks of fetal loss (RR: 1.33, 95% CI: 1.00-1.76; P = .05), abortion (RR: 2.42, 95% CI: 1.46-4.01; P = .0006), thrombosis (RR: 2.83, 95% CI: 1.47-5.44; P = .002), and preterm delivery (RR: 1.89, 95% CI: 1.52-2.35; P = .00001) were also significantly higher in women with APS. However, placental abruption (RR: 1.35, 95% CI: 0.78-2.34; P = .29) and pulmonary embolism were not significantly different (RR: 1.47, 95% CI: 0.11-19.20; P = .77). The risk of neonatal mortality (RR: 3.95, 95% CI: 1.98-7.86; P = .0001), infants small for gestational age (RR: 1.38, 95% CI: 1.04-1.82; P = .02), premature infants (RR: 1.86, 95% CI: 1.52-2.28; P = .0001), and infants who were admitted to neonatal intensive care unit (RR: 3.35, 95% CI: 2.29-4.89; P = .00001) were also significantly higher in women with APS. CONCLUSION: This analysis showed APS to be associated with significantly worse pregnancy outcomes when compared to the control group. A significantly higher risk of maternal and fetal complications was observed in this category of patients. Therefore, intense care should be given to pregnant women with APS to monitor unwanted outcomes and allow a successful pregnancy.


Assuntos
Síndrome Antifosfolipídica/complicações , Complicações na Gravidez/epidemiologia , Aborto Espontâneo/epidemiologia , Aborto Espontâneo/etiologia , Síndrome Antifosfolipídica/epidemiologia , Estudos de Casos e Controles , Feminino , Humanos , Hipertensão Induzida pela Gravidez/epidemiologia , Hipertensão Induzida pela Gravidez/etiologia , Lactente , Mortalidade Infantil , Recém-Nascido Prematuro , Recém-Nascido Pequeno para a Idade Gestacional , Gravidez , Complicações na Gravidez/classificação , Resultado da Gravidez , Nascimento Prematuro/epidemiologia , Nascimento Prematuro/etiologia
5.
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
6.
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
7.
Autoimmun Rev ; 18(5): 519-525, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30844560

RESUMO

BACKGROUND: Anticardiolipin antibodies of the immunoglobulin G isotype (IgG aCL) have been suggested as risk factor for arterial and venous thrombosis. No conclusive data in patients with coronary artery disease (CAD) do exist. We investigate the risk of recurrent CAD according to the presence of IgG aCL. METHODS: We performed a systematic review and meta-analysis to evaluate the risk of recurrent major adverse cardiac events (MACE) associated with the presence of IgG aCL in patients with CAD. MEDLINE and Cochrane databases were searched. We conducted a meta-analysis of the relative risk (RR) both at 12 and 24 months. RESULTS: We included 11 eligible studies with a total of 2425 patients, 283 IgG aCL+ and 2142 IgG aCL-. The prevalence of IgG aCL+ ranged from 6.1% to 43.3%. A total of 341 cardiac events were reported: 71 (25.1%) in IgG aCL+ and 270 (12.6%) in IgG aCL- patients. We found an increased risk of recurrent MACE in patients with high IgG aCL both at 12 (RR 2.17, 2.5-97.5%CI, 1.54-3.00) and 24 months (RR 2.11, 2.5-97.5%CI, 1.62-2.66). This association was even stronger in patients with juvenile CAD (i.e. <50 years) at both 12 (RR 3.21, 2.5-97.5%CI, 1.74-5.41) and 24 months (RR 3.24, 2.5-97.5%CI, 1.84-5.21). CONCLUSION: Patients with CAD and elevated IgG aCL have a doubled risk of recurrent MACE at 12 and 24 months. The presence of aCL should be suspected in patients with recurrent CAD events or in patients with juvenile CAD.


Assuntos
Anticorpos Anticardiolipina/efeitos adversos , Síndrome Antifosfolipídica/imunologia , Doenças Cardiovasculares/etiologia , Imunoglobulina G/efeitos adversos , Adolescente , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/epidemiologia , Teorema de Bayes , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/imunologia , Doenças Cardiovasculares/patologia , Feminino , Humanos , Imunoglobulina G/imunologia , Masculino , Recidiva , Análise de Regressão , Fatores de Risco , Adulto Jovem
8.
Autoimmun Rev ; 18(4): 393-398, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30772492

RESUMO

OBJECTIVE: Recently, two studies demonstrated that a relevant percentage of primary antiphospholipid syndrome (PAPS) patients had an upregulation of interferon (IFN) genes. However, 20%-28% of these patients had anti-dsDNA, a highly specific systemic lupus erythematosus (SLE) autoantibody. This study aimed to determine the prevalence of the type I IFN signature in the peripheral blood mononuclear cells of PAPS patients without specific SLE autoantibodies and search for its clinical associations. METHODS: Fifty-three PAPS patients (Sydney's criteria) were consecutively selected and age-matched with 50 healthy controls. A third group of nonimmune-mediated thrombophilia patients was also included. The expression of 41 IFN-induced genes was analyzed using real time quantitative PCR. A principal component analysis determined which genes composed the IFN signature, and the z-score was calculated. An ROC curve defined the signature cut-off. RESULTS: Six genes remained in the IFN signature DNAJA1, IFIT5, IFI27, MX1, IFI6, and TYK2. The ROC cutoff was 3.9-fold (AUC = 0.706, S = 0.49, E = 0.86, PPV = 0.79, NPV = 0.61). The type I IFN signature was present in 49% of the patients with PAPS compared with 14.0% of the healthy controls and 17% of the nonimmune-mediated thrombophilia patients (p < .0001). The IFN signature was associated with a younger age at the first antiphospholipid syndrome event (p = .023) and with preeclampsia (p = .032). CONCLUSION: Our results indicate that PAPS patients without lupus-specific antibodies have an enhanced type I IFN gene signature that is not observed in nonimmune-mediated thrombophilia. Also, this overexpression of type I IFN-regulated genes associated with an earlier onset of antiphospholipid syndrome event and preeclampsia.


Assuntos
Síndrome Antifosfolipídica/genética , Interferon Tipo I/farmacologia , Pré-Eclâmpsia/etiologia , Transcriptoma/efeitos dos fármacos , Adulto , Idade de Início , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/imunologia , Autoanticorpos/imunologia , Estudos de Casos e Controles , Feminino , Expressão Gênica/efeitos dos fármacos , Humanos , Leucócitos Mononucleares/efeitos dos fármacos , Leucócitos Mononucleares/metabolismo , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Pré-Eclâmpsia/epidemiologia , Pré-Eclâmpsia/genética , Gravidez , Prevalência , Fatores de Risco , Adulto Jovem
9.
Autoimmun Rev ; 18(4): 406-414, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30772493

RESUMO

AIM: To analyse the clinical features, laboratory data and foetal-maternal outcomes, and follow them up on a cohort of 1000 women with obstetric antiphospholipid syndrome (OAPS). METHODS: The European Registry of OAPS became a registry within the framework of the European Forum on Antiphospholipid Antibody projects and was placed on a website in June 2010. Thirty hospitals throughout Europe have collaborated to carry out this registry. Cases with obstetric complaints related to antiphospholipid antibodies (aPL) who tested positive for aPL at least twice were included prospectively and retrospectively. The seven-year survey results are reported. RESULTS: 1000 women with 3553 episodes were included of which 2553 were historical and 1000 were latest episodes. All cases fulfilled the Sydney classification criteria. According to the laboratory categories, 292 (29.2%) were in category I, 357 (35.7%) in IIa, 224 (22.4%) in IIb and 127 (12.7%) in IIc. Miscarriages were the most prevalent clinical manifestation in 386 cases (38.6%). Moreover, the presence of early preeclampsia (PE) and early foetal growth restriction (FGR) appeared in 181 (18.1%) and 161 (16.1%), respectively. In this series, 448 (44.8%) women received the recommended OAPS treatment. Patients with recommended treatment had a good live-birth rate (85%), but worse results (72.4%) were obtained in patients with any treatment (low-dose aspirin (LDA) or low-molecular-weight heparin (LMWH) not on recommended schedule, while patients with no treatment showed a poor birth rate (49.6%). CONCLUSION: In this series, recurrent miscarriage is the most frequent poor outcome. To avoid false-negative diagnoses, all laboratory category subsets were needed. OAPS cases have very good foetal-maternal outcomes when treated. Results suggest that we were able to improve our clinical practice to offer better treatment and outcomes to OAPS patients.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Complicações na Gravidez/epidemiologia , Aborto Habitual/tratamento farmacológico , Aborto Habitual/epidemiologia , Aborto Habitual/etiologia , Adulto , Anticorpos Antifosfolipídeos/imunologia , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/imunologia , Aspirina/uso terapêutico , Estudos de Coortes , Europa (Continente)/epidemiologia , Feminino , Seguimentos , Heparina de Baixo Peso Molecular/uso terapêutico , Humanos , Recém-Nascido , Gravidez , Complicações na Gravidez/tratamento farmacológico , Complicações na Gravidez/imunologia , Resultado da Gravidez , Sistema de Registros , Estudos Retrospectivos , Adulto Jovem
10.
Thromb Res ; 176: 67-73, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30780006

RESUMO

INTRODUCTION: Thrombophilia screening has limited detection efficiency. We assessed the detection rate when a standardized approach to thrombophilia-screened outpatients was used. METHODS: We analyzed 1185 patients (36.5% males, median age: 43 years [IQR 33-54]) referred to a single center from January 2014 to October 2017 with 11 different clinical indications for thrombophilia screening, which was performed in the adherence to published guidelines. Factor V Leiden, prothrombin G20210A mutation, antithrombin (AT), protein C, protein S deficiencies and antiphospholipid syndrome (APS) were determined. RESULTS: The overall positivity rate was 37.1% (95% CI 34.3%-39.7%). The highest positivity rate was found in women following VTE during pregnancy/childbirth (64.1%) and provoked VTE patients with positive family history (52.9%). In patients aged >50 years (32.5%), APS was found at a similar rate as in younger subjects (11.4% vs 10.1%), while AT deficiency was detected more frequently in the older group (5.7% vs 2.4%, p = 0.003). CONCLUSIONS: Standard indications for thrombophilia screening lead to detection rates of 37% or more. Frequent detection of APS and AT deficiency among older patients, which often implies a need for long-term anticoagulation and could impact clinical practice patterns, suggests a benefit of thrombophilia screening in this population in selected clinical circumstances.


Assuntos
Síndrome Antifosfolipídica/diagnóstico , Deficiência de Antitrombina III/diagnóstico , Trombofilia/diagnóstico , Adulto , Fatores Etários , Idoso , Síndrome Antifosfolipídica/epidemiologia , Deficiência de Antitrombina III/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pacientes Ambulatoriais , Trombofilia/epidemiologia , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/epidemiologia
11.
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
12.
J Clin Lab Anal ; 33(1): e22617, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29992646

RESUMO

INTRODUCTION: The patients with antiphospholipid syndrome (APS) associate an increased risk of atherosclerosis. OBJECTIVE: To determine the predictors of an abnormal ankle-brachial index (ABI), surrogate measure of atherosclerosis, in patients with APS. METHODS: The ABI was measured according to standard recommendations in 106 patients. Traditional cardiovascular risk factors were assessed in all cases. A large spectrum of APS antibodies was determined in 73 patients. RESULTS: A total of 106 patients diagnosed with APS were included. 28.3% patients included were found to have low ABI. Anti-beta 2-glycoprotein I (aß2GPI) IgG antibodies [4.00 (1.00-79.00) vs 3.00 (0.00-29.00) U/mL, P = 0.02] and antiprothrombin (aPT) IgM antibodies [4.50 (0.00-82.00) vs 3.00 (0.00-14.00) U/mL, P = 0.05] titers were found to be higher in patients with abnormal ABI. However, after multivariate regression analysis, only the aß2GPI IgG titer remained predictor of low ABI (P = 0.04). CONCLUSIONS: aß2GPI IgG associated with impaired ABI in patients with APS. This relation might reflect their involvement in the atherosclerosis occurrence.


Assuntos
Índice Tornozelo-Braço/estatística & dados numéricos , Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/fisiopatologia , Adulto , Aterosclerose , Autoanticorpos/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Fatores de Risco , beta 2-Glicoproteína I/imunologia
13.
Clin Rheumatol ; 38(2): 545-553, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30255283

RESUMO

Antiphospholipid syndrome (APS) is the most common acquired pro-thrombotic disorder, also associated with obstetric complications. Phosphatidylserine/Prothrombin complex antibody (aPSPT) though associated with various APS manifestations, is not included in the revised Sapporo Criteria. To study the prevalence of aPSPT in Asian-Indian patients with suspected APS and compare its performance with the criteria anti-phospholipid antibodies (APLs). Electronic charts of 372 individuals whose sera was tested for aPSPT in suspected APS between June 2014 and May 2016 were retrieved and analyzed. aPSPT was assayed by ELISA. aPSPT tested individuals were categorized into cases-seropositive and seronegative APS (SNAPS) and controls. aPSPT was positive in 24/58 (41.3%) cases and 17/314 (5.4%) controls (p < 0.001). aPSPT positivity was seen in 44.5%, 38.7%, and 58.4% in primary, secondary and SNAPS patients respectively. aPSPT had the best performance among all APLs, in obstetric APS with 31% sensitivity, 97.7% specificity, and an odds ratio of 18.8. It showed 41.4% sensitivity, 94.6% specificity for the classification/diagnosis of primary APS and 38.7% sensitivity, 91.5% specificity for secondary APS. Addition of aPSPT to current APS criteria to SNAPS patients led to reclassification of additional 12.1% patients as APS overall and 42.8% in obstetric APS category. In Asian-Indian patients with suspected APS, aPSPT outperformed all classical APLs in diagnosis/classification of obstetric APS and both isotypes of beta 2-glycoprotein-I antibodies in diagnosis/classification of APS. aPSPT could reclassify additional 12.1 and 42.8% patients as APS overall and obstetric APS respectively, over and above the cases satisfying revised Sapporo criteria.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/epidemiologia , Adulto , Síndrome Antifosfolipídica/sangue , Biomarcadores/sangue , Estudos de Casos e Controles , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Índia/epidemiologia , Modelos Logísticos , Masculino , Fosfatidilserinas/imunologia , Protrombina/imunologia , Sensibilidade e Especificidade , Adulto Jovem , beta 2-Glicoproteína I/imunologia
14.
Asian Pac J Allergy Immunol ; 37(3): 171-178, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29981567

RESUMO

OBJECTIVES: To describe the characteristics of patients with antiphospholipid syndrome (APS) in an Asian clinical practice setting. METHODS: We conducted a single-center, retrospective study of APS patients attending the rheumatology or hematology clinics, between January 2012 and December 2016. RESULTS: There were 450 patients suspected of having APS referred to our clinics. Seventy-four (16.4%) were diagnosed of APS, 51% of which were definite. Fifty-two (70%) patients were classified as primary APS, 50% of which were definite APS. The most common clinical manifestation was stroke (33%), followed by deep vein thrombosis of the lower extremities (30%) and pulmonary embolism (19%). Hypertension and the presence of at least one established cardiovascular risk factor were independently associated with stroke. Seven (9%) patients had multiorgan thrombosis as their first presentation of APS, 71% of which ultimately suffered from permanent organ damage or died of severe thrombosis, despite not fulfilling the criteria for 'definite' catastrophic APS (CAPS). Late fetal loss was the most prevalent obstetric complication. The majority of patients (79%) tested positive for lupus anticoagulant (LAC), while only 32% tested positive for anti-cardiolipin antibodies. Triple positive profile was documented in 14% of the cohort. Overall, recurrent thrombosis and bleeding complications were recorded in 9% and 28%, respectively. CONCLUSION: APS patients in central Thailand demonstrated high prevalence of stroke, late fetal loss, LAC positivity, and multiorgan thrombosis at first presentation, leading to poor outcomes.


Assuntos
Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/etiologia , Grupo com Ancestrais do Continente Asiático , Adulto , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Biomarcadores , Suscetibilidade a Doenças/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Estudos Retrospectivos , Trombose/diagnóstico , Trombose/etiologia , Trombose/terapia , Resultado do Tratamento , Adulto Jovem
15.
World J Biol Psychiatry ; 20(1): 51-63, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-28532221

RESUMO

OBJECTIVES: Case reports describe neuropsychiatric manifestations associated with antiphospholipid antibodies (aPlAbs). In patients sharing the same symptoms fulfilling the antiphospholipid syndrome (APS) clinical criteria, the prevalence of common mental disorders has, however, never been studied. METHODS: We observed women with three consecutive abortions before the 10th week of gestation or one foetal loss at or beyond the 10th week. We compared the prevalence of common psychiatric disorders detected through screening using the Mini International Neuropsychiatric Interview, 10 years after inclusion, in women with APS (n = 506), women negative for aPlAbs but carrying the F5rs6025 or F2rs1799963 thrombogenic polymorphism (n = 269), and women with negative thrombophilia screening results as controls (n = 764). RESULTS: Similar prevalence values were obtained for controls and women bearing one of the two thrombogenic polymorphisms. Women with APS more frequently had mood disorders (relative risk (RR) 1.57 (1.262-1.953), P = .0001) and anxiety (RR 1.645 (1.366-1.979), P < .0001). Within the APS group, lupus anticoagulant (LA) and anti-ß2GP1 IgG, or triple positivity, were strong risk factors for mood disorders. CONCLUSIONS: Women with obstetric APS have a higher risk of positive screening for common mental disorders than women without APS.


Assuntos
Aborto Espontâneo , Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica , Transtornos de Ansiedade , Transtornos do Humor , Trombofilia , Aborto Habitual/sangue , Aborto Habitual/epidemiologia , Aborto Habitual/imunologia , Aborto Espontâneo/sangue , Aborto Espontâneo/epidemiologia , Aborto Espontâneo/imunologia , Adulto , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/epidemiologia , Síndrome Antifosfolipídica/imunologia , Transtornos de Ansiedade/sangue , Transtornos de Ansiedade/epidemiologia , Transtornos de Ansiedade/imunologia , Feminino , Seguimentos , Humanos , Incidência , Pessoa de Meia-Idade , Transtornos do Humor/sangue , Transtornos do Humor/epidemiologia , Transtornos do Humor/imunologia , Gravidez , Prevalência , Transtornos Psicóticos/sangue , Transtornos Psicóticos/epidemiologia , Transtornos Psicóticos/imunologia , Transtornos Relacionados ao Uso de Substâncias/sangue , Transtornos Relacionados ao Uso de Substâncias/epidemiologia , Transtornos Relacionados ao Uso de Substâncias/imunologia , Trombofilia/sangue , Trombofilia/epidemiologia , Trombofilia/imunologia
16.
Zhonghua Fu Chan Ke Za Zhi ; 53(12): 855-859, 2018 Dec 25.
Artigo em Chinês | MEDLINE | ID: mdl-30585025

RESUMO

Objective: To compare the etiological constitution of recurrent miscarriage (RM) between patients with consecutive two and three or more miscarriages through combining the routine examination results and embryonic karyotype. Methods: Patients with a history of two or more consecutive clinical miscarriages (≤12 weeks of gestation) consulting in the RM clinic of the First Affiliated Hospital of Sun Yat-sen University from March 2011 to January 2016 were collected. Six hundred and ninety-six with detailed history recorded, routine clinical examinations of RM and at least once embryonic karyotype were ultimately enrolled in this study. Their etiological constitution of RM were analyzed in groups of consecutive two and three or more miscarriage. The etiologies of RM in analysis consisted of women age, body mass index (BMI) , chromosome abnormalities of couples, uterine abnormalities, endocrinology abnormalities and antiphospholipid syndrome (APS) . Results: (1) Among 696 patients, the abnormal embryonic karyotypes was 60.6% (422/696) and routine RM etiologies was 32.2% (224/696) , leaving the ratio of unexplained RM was only 29.0% (202/696). (2) A total of 717 embryo karyotype were found in 696 patients, included21 cases with twice embryo karyotype results the percentage of normal embryo was 39.7% (285/717) , while abnormal ones was 60.3% (432/717). Among the types of abnormal karyotype, the most common ones (>10%) were trisomy 16 (19.2%, 83/432) , monosome X (11.3%, 49/432) and trisomy 22 (10.9%, 47/432). (3) Among the 696 RM patients, the number of two and three or more miscarriages were respectively 446 (64.1%, 446/696) and 250 (35.9%, 250/696). Comparing groups of three or more miscarriages with two miscarriages, there were significant differencein older age as well as uterine adhesion (P<0.05). But no difference was found in body mass index (BMI) , the rates of chromosome abnormalities of couples, uterine abnormalities except uterine adhesion, endocrinology abnormalities and APS (all P>0.05) between two groups. Conclusions: The abnormal embryonic karyotype is the most common cause of first-trimester RM. The etiological constitution of two and three or more recurrent miscarriages is accordant, suggesting that routine clinical examination and the embryonic karyotype should be started following two consecutive clinical early miscarriages.


Assuntos
Aborto Habitual/etiologia , Aborto Habitual/genética , Síndrome Antifosfolipídica/complicações , Índice de Massa Corporal , Aberrações Cromossômicas , Idade Materna , Doenças Uterinas/complicações , Aborto Habitual/epidemiologia , Síndrome Antifosfolipídica/epidemiologia , Cromossomos Humanos Par 16 , Cromossomos Humanos Par 22 , Feminino , Humanos , Cariotipagem , Mosaicismo , Gravidez , Trissomia , Doenças Uterinas/epidemiologia
17.
Front Immunol ; 9: 2457, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30464761

RESUMO

Objectives: Antiphospholipid antibodies (aPL) can be present in the sera of systemic sclerosis (SSc) patients. This study aimed to determine the prevalence of aPL in a cross-sectional study of SSc patients, to assess their clinical associations, to perform a systematic review of published reports and a meta-analysis to estimate the worldwide prevalence of aPL in SSc. Methods: Two-hundred and forty-nine SSc patients were consecutively tested once for lupus anticoagulant (LA), anticardiolipin (aCL), and anti-ß2glycoprotein I (anti-ß2GpI) antibodies. Clinical associations with aPL positivity were studied using a logistic regression model. A systematic review of the literature was carried out in PubMed and Embase. Meta-analysis was performed using number of aPL positive (at least one of the three antibodies positive) and negative patients. Meta-regression was used to study potential factors explaining the heterogeneity between studies. Results: In our cross-sectional study, aPL positivity was found in 16 patients (prevalence 6.4%; 95%CI [3.8-10.4]). In multivariate analysis, there was a significant association between aPL positivity and venous thrombosis (VT) (OR 6.25 [1.18-33.00]; p = 0.028) and miscarriage (OR 5.43; 95%CI [1.31-22.13]; p = 0.017). Twenty-four studies were included in the meta-analysis, representing a total population of 3036 SSc patients. The overall pooled prevalence of aPL in SSc was 14% (9-20) with a high degree of heterogeneity among studies. Conclusion: This study found a prevalence of aPL positivity in our SSc population of 6.4% (3.8-10.4) and an overall worldwide pooled prevalence of 14% (9-20). In our SSc population, aPL positivity was associated with VT and miscarriage. These data provide additional insights into the role of aPL in the vasculopathy observed in SSc.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/epidemiologia , Escleroderma Sistêmico/sangue , Escleroderma Sistêmico/epidemiologia , Aborto Espontâneo/epidemiologia , Idoso , Anticorpos Anticardiolipina/sangue , Anticorpos Antifosfolipídeos/imunologia , Estudos Transversais , Feminino , França/epidemiologia , Humanos , Inibidor de Coagulação do Lúpus/sangue , Masculino , Pessoa de Meia-Idade , Gravidez , Escleroderma Sistêmico/imunologia , Trombose Venosa/epidemiologia , beta 2-Glicoproteína I/imunologia
18.
Arthritis Res Ther ; 20(1): 249, 2018 Nov 06.
Artigo em Inglês | MEDLINE | ID: mdl-30400982

RESUMO

OBJECTIVE: Although one of the three obstetric manifestations of antiphospholipid syndrome (APS) is intrauterine fetal death (IUFD), little is known about it in this context. We report the first large series of patients with APS and IUFD. METHODS: We retrospectively analyzed the history and clinical data of women at four French hospitals. All had (1) APS diagnosis (Sydney criteria) and (2) IUFD at or after 10 weeks of gestation (weeks) between 2000 and 2016. RESULTS: The study included 65 women. Their median age at the index IUFD was 29 years (IQR 26-33); 38 (58%) were primigravidas. The index IUFD was the first APS clinical manifestation in 48 women (74%). Overall, 35% had a triple-positive antibody profile. IUFD occurred at a median gestational age of 24 weeks (IQR 18-27) and was associated with maternal obstetric complications in 16 women (25%), namely, preeclampsia (n = 12), hemolysis, elevated liver enzymes, and low platelet syndrome (HELLP) (n = 6), and/or placental abruption (n = 5). Half of the 50 women with available data had a small-for-gestational-age fetus. Overall, including during the follow-up period of 4 years (IQR 2-9), 28 women (43%) had at least one thrombosis, and 29% were diagnosed with systemic lupus erythematosus (SLE). Ultimately, 54 women (83%) had at least one live birth. Only one woman had three consecutive early miscarriages. CONCLUSION: IUFD was most often the inaugural sign of APS. Of the APS classification criteria, IUFD, preeclampsia, and thromboses were common in this cohort, while the "3 consecutive early miscarriages" criterion was met only once. With treatment, most of the women successfully had at least one live birth.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/sangue , Síndrome Antifosfolipídica/diagnóstico por imagem , Morte Fetal/etiologia , Complicações na Gravidez/sangue , Complicações na Gravidez/diagnóstico por imagem , Adulto , Síndrome Antifosfolipídica/epidemiologia , Feminino , Seguimentos , França/epidemiologia , Humanos , Gravidez , Complicações na Gravidez/epidemiologia , Estudos Retrospectivos
19.
Stroke ; 49(11): 2770-2772, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30355196

RESUMO

Background and Purpose- International classification criteria for antiphospholipid syndrome (APS) include IgM (immunoglobulin M), aCL (anticardiolipin), and aB2GPI (anti-ß2-glycoprotein-I) antibodies, but their relevance is still debated. We aimed to assess whether patients with isolated IgM aCL and/or aB2GPI at diagnosis have specific characteristics and outcomes. Methods- We retrospectively included APS patients with isolated IgM antiphospholipid antibodies (isolated-IgM-APS) and compared them to APS patients with IgG and IgM, or IgG alone and/or lupus anticoagulant (nonisolated-IgM-APS). Results- Among the 168 APS patients included, 24 (14.3%) had isolated IgM. Median follow-up was 92.5 months (36-151.5). Isolated-IgM-APS patients were 9.5 years older. At diagnosis, stroke was more frequent in isolated-IgM-APS after adjustment for cardiovascular risk factors (odds ratio, 3.8; 95% CI, 1.3-11.5). IgM isotype remained isolated in 17 of 24 (70.8%) patients over time. Global relapse-free survival did not differ between the two groups. In thrombotic APS, monotherapy with antiplatelet agents was more frequently used in isolated-IgM-APS group with 14 of 20 versus 28 of 134 patients ( P<0.0001), with a higher relapse rate with antiplatelet agent alone compared to vitamin K antagonists, especially for patients presenting with a stroke (hazard ratio, 7.37; 95% CI, 1.19-19.0). Conclusions- Isolated IgM APS patients should not be disregarded because they represent 14.3% of an APS population. They have some characteristics: older age at diagnosis and a strong association with stroke. Clinicians must be aware of this situation because antiplatelet agent do not seem to well prevent relapses compared to vitamin K antagonist.


Assuntos
Anticorpos Anticardiolipina/imunologia , Síndrome Antifosfolipídica/imunologia , Imunoglobulina M/imunologia , Acidente Vascular Cerebral/imunologia , beta 2-Glicoproteína I/imunologia , Adulto , Anticoagulantes/uso terapêutico , Síndrome Antifosfolipídica/tratamento farmacológico , Síndrome Antifosfolipídica/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Inibidores da Agregação de Plaquetas/uso terapêutico , Acidente Vascular Cerebral/epidemiologia
20.
Atherosclerosis ; 278: 60-65, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30253290

RESUMO

BACKGROUND AND AIMS: Cardiovascular disease (CVD), including coronary artery disease and stroke/peripheral artery disease, is less commonly reported than venous thromboembolism in subjects with antiphospholipid antibodies (aPLs) and little is known about the association of CVD with adjusted Global AntiphosPholipid Syndrome Score (aGAPSS). METHODS: Consecutive aPLs subjects were enrolled to assess the association of CVD with aGAPSS. Moreover, additional risk factors of CVD were identified by means of multivariate analysis to design an aGAPSS specific for CVD (aGAPSSCVD). RESULTS: A total of 192 aPLs subjects (34 males, 158 females, mean age 49.84 ±â€¯12.0 years) were enrolled. CVD was reported in 52 subjects (27.1%), 26 episodes of coronary artery disease and 26 stroke/peripheral artery disease. The prevalence of CVD increased for increasing aGAPSS ranging from 20.5% in the lowest aGAPSS category, up to 37.9% in the highest category (p = 0.027). ROC analysis showed that aGAPSS detected 63.0% of CVD and was associated with OR for CVD of 2.52 (95%CI: 1.24-5.10, p = 0.010). When including obesity, diabetes and smoking habit in the score, we found that aGAPSSCVD detected 71.4% of CVD (72.4% for early-CVD and 69.0% for CVD after 50 years) with an OR for CVD of 4.68 (95%CI: 2.31-9.51, p < 0.001). CONCLUSIONS: The aGAPSSCVD, obtained after adding obesity, smoking habit and diabetes to the standard aGAPSS, showed a higher detection rate of CVD in aPLs subjects, particularly of early-CVD. These results need to be validated in ad hoc designed prospective studies.


Assuntos
Anticorpos Antifosfolipídeos/sangue , Síndrome Antifosfolipídica/diagnóstico , Doença da Artéria Coronariana/diagnóstico , Doença Arterial Periférica/diagnóstico , Medição de Risco/métodos , Acidente Vascular Cerebral/diagnóstico , Adulto , Síndrome Antifosfolipídica/epidemiologia , Cardiologia/normas , Doença da Artéria Coronariana/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença Arterial Periférica/epidemiologia , Estudos Prospectivos , Fatores de Risco , Índice de Gravidade de Doença , Acidente Vascular Cerebral/epidemiologia , Tromboembolia Venosa
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