Clinical and laboratory characteristics of Brazilian versus non-Brazilian primary antiphospholipid syndrome patients in AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) clinical database and repository

Abstract Background: Antiphospholipid syndrome (APS) is characterized by episodes of thrombosis, obstetric morbidity or both, associated with persistently positive antiphospholipid antibodies (aPL). Studying the profile of a rare disease in an admixed population is important as it can provide new insights for understanding an autoimmune disease. In this sense of miscegenation, Brazil is characterized by one of the most heterogeneous populations in the world, which is the result of five centuries of interethnic crosses of people from three continents. The objective of this study was to compare the clinical and laboratory characteristics of Brazilian vs. non-Brazilian primary antiphospholipid syndrome (PAPS) patients. Methods: We classified PAPS patients into 2 groups: Brazilian PAPS patients (BPAPS) and PAPS patients from other countries (non-BPAPS). They were compared regarding demographic characteristics, criteria and non-criteria APS manifestations, antiphospholipid antibody (aPL) profile, and the adjusted Global Antiphospholipid Syndrome Score (aGAPSS). Results: We included 415 PAPS patients (88 [21%] BPAPS and 327 [79%] non-BPAPS). Brazilian patients were significantly younger, more frequently female, sedentary, obese, non-white, and had a higher frequency of livedo (25% vs. 10%, p < 0.001), cognitive dysfunction (21% vs. 8%, p = 0.001) and seizures (16% vs. 7%, p = 0.007), and a lower frequency of thrombocytopenia (9% vs. 18%, p = 0.037). Additionally, they were more frequently positive for lupus anticoagulant (87.5% vs. 74.6%, p = 0.01), and less frequently positive to anticardiolipin (46.6% vs. 73.7%, p < 0.001) and anti-ß2-glycoprotein-I (13.6% vs. 62.7%, p < 0.001) antibodies. Triple aPL positivity was also less frequent (8% vs. 41.6%, p < 0.001) in Brazilian patients. Median aGAPSS was lower in the Brazilian group (8 vs. 10, p < 0.0001). In the multivariate analysis, BPAPS patients still presented more frequently with livedo, cognitive dysfunction and sedentary lifestyle, and less frequently with thrombocytopenia and triple positivity to aPL. They were also less often white. Conclusions: Our study suggests a specific profile of PAPS in Brazil with higher frequency of selected non-criteria manifestations and lupus anticoagulant positivity. Lupus anticoagulant (not triple positivity) was the major aPL predictor of a classification criteria event.

Multiple Spontaneous Intracerebral Hematoma without Presenting Risk Factors

The incidence of intracerebral hemorrhage in those aged 45–84 years is 0.3-0.5%. In people over 80 years of age, this incidence increases 25-fold compared with that of the total population. The most common causes of spontaneous intracerebral hemorrhage in the younger population are vascular malformation, aneurysm, and overuse of drugs. In contrast, common causes in the elderly include hypertension, tumors, and coagulation disorders. Here, we present a case involving a 72-year-old male patient who, without any of these predisposing conditions, was admitted to the hospital with spontaneous intracerebral hemorrhage and showed signs of multifocal intracerebral hemorrhage during his stay. We conclude that spontaneous intracerebral hemorrhage can occur without any predisposing factors, and can lead to a patient's death. Therefore, the possibility of recurrent spontaneous intracerebral hemorrhage must be considered in patients with primary spontaneous intracerebral hemorrhage.

Resultados maternos y perinatales en mujeres con diabetes gestacional según criterios de pesquisa / Maternal and perinatal health in women with gestational diabetes research results according to criteria

OBJETIVO: Conocer los resultados maternos y perinatales de mujeres con diabetes gestacional diagnosticada según los Criterios de la Asociación Internacional de Grupos de Estudio de Diabetes y Embarazo y la Asociación Latinoamericana de Diabetes. MÉTODOS: Se estudiaron ciento veintidós embarazadas con diagnóstico de diabetes gestacional, aplicando los criterios Asociación Internacional de Grupos de Estudio de Diabetes y Embarazo y Asociación Latinoamericana de Diabetes. Se compararon datos socio-demográficos, factores de riesgo para diabetes gestacional, parámetros clínicos y metabólicos durante el embarazo y la pesquisa posparto de diabetes en ambos grupos. Las determinaciones de glucemias de las pruebas de tolerancia oral a la glucosa se realizaron mediante el método enzimático. Se utilizó Chi cuadrado y t de Student para la asociación de variables. Se consideró significativo un valor de P < 0,05. RESULTADOS: Al usar los criterios de la Asociación Internacional de Grupos de Estudio de Diabetes y Embarazo, el número de pacientes con diabetes gestacional aumentó 22,95 %. No hubo diferencias en cuanto a factores de riesgo para diabetes gestacional, resultados maternos y perinatales entre los dos grupos. En el grupo según los criterios Asociación Latinoamericana de Diabetes, las glucemias 2 horas poscarga en el posparto eran más altas (P< 0,02) y resultaron casos de prediabetes. CONCLUSIONES: Se incrementó la proporción de casos con diabetes gestacional, al emplear los criterios de la Asociación Internacional de Grupos de Estudio de Diabetes y Embarazo y se detectaron más alteraciones glucémicas maternas en el posparto con los criterios Asociación Latinoamericana de Diabetes. Se requieren investigaciones ulteriores, cuyos resultados puedan contribuir a las decisiones institucionales sobre los criterios para la pesquisa de diabetes gestacional.

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Performance of Automated Chemiluminescence Assay for Antiphospholipid Antibody Testing

BACKGROUND: Detection of antiphospholipid antibodies (aPL) can be considered problematic due to assay variability and reagent sensitivity, high false-positive and false-negative rates, and lack of assay standardization. Therefore, utilizing an automated system can improve reproducibility and reduce interlaboratory variation. Here, we evaluated the analytical performance of the new automated ACL AcuStar chemiluminescence assay (Instrumentation Laboratory, USA). This was compared to the results of a panel analyzed with the QUANTA Lite ELISA (INOVA Diagnostics Inc., USA). METHODS: We evaluated the inter-assay precision, linearity, and carry-over between the two methods, ACL and ELISA. A reference range study for each of the anticardiolipin (aCL) and anti-beta2 glycoprotein-I (abeta2GPI) IgG and IgM antibodies were performed using 135 healthy patient samples, which served as controls. We then compared the accuracy among the AcuStar and ELISA systems via four aPL tests. For this comparison, 69 patient samples suspected of an autoimmune disorder were used as the experimental panel. RESULTS: The AcuStar analyzer showed excellent precision, linearity, and carry-over for all four assays. The calculated cutoff values were 20.3 U/mL for aCL IgG, 20.3 U/mL for aCL IgM, 26.3 U/mL for abeta2GPI IgG, and 11.9 U/mL for abeta2GPI IgM. The consensus between AcuStar and ELISA results were generally comparable. Total agreement varied between 82.6% and 95.7%, and kappa values showed moderate to good agreement. CONCLUSIONS: Our study demonstrates that the new AcuStar chemiluminescence assay showed better performance. This automated system leads to improved reproducibility and reduces interlaboratory variability.

Discussing the Correlation between BA,EMAb, ACA and Unexplained Recurrent Spontaneous Abortion / 现代检验医学杂志

Objective To discuss the correlation between the joint detection of BA,EMAb,ACA and unexplained recurrent spontaneous abortion(URSA).Methods Selected 144 URSA cases,166 SA(sporadic abortion)cases,188 normal pregnant women,and detected their BA,EMAb,ACA and statistical analysis of parallelism.Results Comparing URSA group with SA group and control group respectively,there were significant statistical difference between BA negative rate (74.31%, 25.30% and 50.53%),EMAb positive rate (35.42%,7.23% and 6.91%)and ACA positive rate (26.39%,5.42% and 5.32%)(χ2 = 19.344 ~ 74.180;P < 0.05;χ2 = 37.837 ~ 42.586,P < 0.05),(χ2 = 26.355 ~ 29.270,P < 0.05).URSA group’s BA,ACA and EMAb joint detection rate of positive (84.72%)was significantly higher than SA group and the con-trol group (χ2 =35.532~93.076,P <0.05).Conclusion There was a close relationship between the lack of BA,the genera-tion of EMAb,ACA and unexplained recurrent spontaneous abortion.Joint detection has important clinical significance in auxiliary diagnosis of unexplained recurrent spontaneous abortion and prognosis judgement.

Doença de Behçet, anticorpos anticardiolipina, forame oval patente e síndrome de Sweet ? uma associação incomum / Behçet?s disease, anticardiolipin antibodies, patent foramen ovale, and Sweet syndrome ? an uncommon association

Doença de Behçet é condição inflamatória e autoimune infrequente, caracterizada por úlceras orais recorrentes e dois ou mais dos seguintesachados ? úlceras genitais, alterações oculares, lesões cutâneas e teste de patergia positivo. A ocorrência de vasculites e de anticorpos antifosfolípides pode contribuir para o aparecimento de tromboembolismos na doença de Behçet. Mulher de 49 anos, com doença de Behçet associada com diagnóstico prévio de síndrome de anticorpos antifosfolípides, apresentou erupção cutânea consistente com a síndrome de Sweet em um local de punção venosa. Foi introduzida prednisona 20mg/dia ao tratamento com warfarina 5 mg/dia que já utilizava, e a resposta satisfatória foi rápida. Pesquisa de sangue oculto foi positiva nas fezes, com concentração de antígeno carcinoembrionário discretamente elevada, e a colonoscopia revelou pólipos no cólon e no sigmoide. O câncerde cólon é descrito em portadores de doença de Behçet com lesões intestinais. Forame oval patente com shunt esquerdo-direito foi achado incidental no presente caso, e essa anomalia propicia embolias paradoxais em pacientes com estados hipercoaguláveis. A associação de doença de Behçet com síndrome de Sweet é raramente descrita e, embora sem o necessário esclarecimento, alguns autores admitem a possibilidade de mecanismo etiopatogênico comum a essas entidades.

Behçet?s disease is an inflammatory and autoimmune condition, characterized by recurrent oral ulcers and, at least, two of the following findings ? genital ulcers, ocular changes, cutaneous lesions, and positivetest for pathergy. The occurrence of vasculitis and antiphospholipid antibodies may contribute to thromboembolisms in Behçet?s disease. A 49-yearold woman with Behçet?s disease associated with a previous diagnosis of antiphospholipid antibodies syndrome presented with an abrupt cutaneous eruption consistent with the Sweet syndrome at the site of venipuncture. Prednisone 20 mg/day was introduced to the treatment with warfarin 5 mg/day that she was undergoing, resulting in a rapid favorable response. Fecal occult blood test was positive, and the level ofcarcinoembryonic antigen was mildly elevated, and the colonoscopy evaluation revealed colon and sigmoid polyps. Colon cancer may develop in patients with intestinal lesions of Behçet?s disease. Patent foramen ovale with left to right shunt was an incidental finding in the present case study, and this anomaly predisposes to paradoxal embolism in patients with thrombophilic conditions. Association between Behçet?s disease andSweet syndrome has been rarely described and, although without the necessary clarification, some authors believe in a possible common etiopathogenic mechanism.

Patrón clínico en gestantes con síndrome antifosfolipídico (SAF) / Clinical pattern in pregnant women with antiphospholipid syndrome (APS)

Objetivo: Describir las características epidemiológicas, clínicas y analíticas de gestantes portadoras del síndrome antifosfolipídico que fueron tratadas en la consulta de medicina interna durante el período 2001-2012. Métodos: Estudio de campo, descriptivo, longitudinal. La población que se estudió fue de 157 pacientes portadoras del síndrome antifosfolipídico, sin tomar en cuenta el criterio del intervalo de al menos 12 semanas para el diagnóstico definitivo. Resultados: Se identificaron como síndrome antifosfolipídico primario 143 pacientes, mientras que como secundario 14 pacientes. Ochenta y seis pacientes solo tuvieron títulos positivos de anticuerpos anticardiolipina, seguido de 62 pacientes con títulos positivos para anticuerpos anticardiolipinas y beta- 2glicoproteína-1, 4 con anticuerpos anticardiolipinas, beta-2glicoproteína-1 y anticoagulante lúpico positivos, 3 solo anticoagulante lúpico positivo y 2 solo beta-2glicoproteína-1 positivo. El tratamiento anticoagulante triple con heparina de bajo peso molecular más ácido acetilsalicílico y prednisona a bajas dosis fue el más frecuentemente utilizado en 99 pacientes. Conclusión: Además de un tratamiento adecuado, el control prenatal y la vigilancia materno-fetal estrecha fue la clave para obtener un resultado favorable en cuanto al bienestar y salud materno-fetal.

Objective: Describe characteristics epidemiological, clinical and analytical for pregnant carrying the antiphospholipid syndrome that were treated in internal medicine, period 2001-2012. Methods: Descriptive, longitudinal, non-experimental and field study. The population that was studied in 157 patients carrying the antiphospholipid syndrome, was not taken into account the criterion interval of at least 12 weeks for definitive diagnosis. Results: We identified 143 patients as primary antiphospholipid syndrome, secondary antiphosphilipid syndrome while as 14 patients. Eighty-six patients had only positive titers of anticardiolipin antibodies, followed 62 patients with positive titers for anticardiolipin antibodies and beta 2 glycoprotein 1, 4 with anticardiolipin antibodies and lupus anticoagulant, beta 2 glycoprotein 1 positive, 3 only lupus anticoagulant positive and 2 beta 2 glycoprotein 1 only positive. The triple anticoagulant therapy with low molecular weight heparin plus acetylsalicylic acid and low-dose prednisone was the most frequently used in 99 patients. Conclusion: In addition to proper treatment, prenatal and maternal-fetal monitoring closely a favorable result was obtained for the welfare and maternal and fetal health.

Comportamiento de anticardiolipinas en gestantes / Anticardiolipins behavior in pregnant

Objetivo: Evaluar los niveles de anticuerpos anticardiolipina en gestantes portadoras del síndrome antifosfolipídico que fueron tratadas en la consulta de medicina interna durante el período 2001-2012. Métodos: Estudio de campo, descriptivo, longitudinal. La población que se estudió fue de 86 pacientes portadoras del síndrome antifosfolipídico. No se tomó en cuenta el criterio del intervalo de al menos 12 semanas para el diagnóstico definitivo. Resultados: En relación con los niveles de IgM e IgG de anticuerpos anticardiolipinas el promedio más alto (30,98) y (19,62) predominó en el primer trimestre del embarazo. El nivel mayor de IgM que se evidenció fue 198, mientras que el menor fue 0,6 durante toda la gestación. Los anticuerpos IgM en el tercer trimestre disminuyeron en 55,81 % con respecto al primer trimestre. Conclusiones: A pesar de que los niveles de anticuerpos son impredecibles, en las últimas etapas de la gestación los anticuerpos anticardiolipinas IgM disminuyeron con respecto a las primeras etapas.

Objective: Evaluate the levels of anticardiolipin antibodies for pregnant carrying the antiphospholipid syndrome that were treated in internal medicine, period 2001-2012. Methods: A descriptive, longitudinal non-experimental and field study. The population studied was 86 patients carrying the SAF, did not take into account the criteria in the interval of at least 12 weeks for definitive diagnosis. Results: In relation to the levels of IgM and IgG anticardiolipin antibodies the highest average (30.98) and (19.62) prevailed in the first trimester of pregnancy. The higher level of IgM was 198, while the lowest was 0.6 during the entire pregnancy. IgM anticardiolipin antibodies in the third quarter decreased by 55.81 % over the first quarter. Conclusions: Although antibody levels are unpredictable, in the latter stages of gestation IgM anticardiolipin antibodies decreased with respect to the early stages.

Impact of Anziheji against ERK1/2 signaling pathways on the maternal-fetal interface of ACA positive abortion in mice / 中国生化药物杂志

Objective To study the role of ERK1/2 signaling pathways on the process of ACA positive abortion and explore the effect of Anziheji on ERK1/2 signaling pathways from cell proliferation.Methods Levels of ERK1/2 on maternal-fetal interface of every mice in ACA positive abortion model group,blank control group,Anziheji low,mediate,high dose group,aspirin group were detected by immunohistochemical. Results The average optical density value of ERK1 in Anziheji low dose group(0.678 ±0.097)was close to normal group(0.727 ±0.047),there was no significant difference between them.Compared with blank control group,the ERK1 average optical density value in model group(0.434 ±0.066),Anziheji dose group(0.613 ±0.041),high dose group(0.487 ±0.061)and aspirin group(0.551 ±0.056)were significantly lower,and the differences were all statistically significant(P<0.01).Compared with blank control group(0.708 ±0.038),the ERK2 average optical density values in model group(0.430 ±0.058), Anziheji low dose group(0.621 ±0.041),mediate dose group(0.562 ±0.047),high dose group(0.449 ±0.062)and aspirin group(0.515 ±0.045) were significantly lower,and the differences were all statistically significant (P <0.01 ).Conclusion Anziheji can improve the activity of cell proliferation,improve the function of the placenta and change the outcome of pregnancy through ERK signal pathway.

Correlation of serum IL-33, AECA-IgA and ACA-IgA in acute stage of Henoch-Sch?nlein purpura in children / 临床儿科杂志

Objectives To explore the changes of serum interleukin-33 (IL-33), IgA anti-endothelial cell antibodies (AECA) and IgA anticardiolipin antibodies (ACA) in acute stage of Henoch-Sch?nlein purpura (HSP) in children and its clinical signiif-cance. Methods Thirty-seven children with acute HSP admitted to hospital were selected from Nov.2012 to Jan.2013. Twenty healthy children were selected as healthy controls. The serum levels of IL-33 were measured by double anti-body sandwich en-zyme-linked immunosorbent assay (ELISA) and the serum levels of AECA-IgA and ACA-IgA were detected by double antigen sandwich ELISA. Results Serum levels of IL-33, AECA-IgA and ACA-IgA in patients with acute HSP were signiifcantly higher than those in healthy controls (P<0.001). According to different clinical types, the serum levels of IL-33, AECA-IgA and ACA-IgA were signiifcantly higher in mixed-type HSP (with joint involvement and gastrointestinal symptoms and/or kidney damage) than those in HSP patients with only joint, abdomen or renal involvement (P<0.05). Serum levels of IL-33 in acute stage of HSP had a positive correlation with AECA-IgA (r=0.752, P<0.001) and ACA-IgA (r=0.788, P<0.001). Conclusions The immune pathological injury in vessels caused by the interaction of IL-33, AECA-IgA and ACA-IgA may be the important pathogenesis of HSP, which may provide a clue for the early diagnosis and treatment for HSP.

Descripción de la presencia de anticuerpos antianexina V, cardiolipina y B2-glicoproteína I en mujeres con pérdidas gestacionales recurrentes en la ciudad de Medellín, Colombia / Describing the presence of anti-annexin V antibodies, cardiolipin and B2-glycoprotein 1 in females suffering recurrent pregnancy loss in Medellin, Colombia

Objetivo: describir la frecuencia de anticuerpos antianexina V en una serie de mujeres que presentaron aborto recurrente en la ciudad de Medellín. Materiales y métodos: se llevó a cabo una serie de casos constituida por mujeres sanas que acudían a consulta ginecológica por historia de pérdidas gestacionales recurrentes caracterizadas por dos o más abortos en el primer trimestre de gestación, en dos instituciones prestadoras de servicios de salud de segundo y tercer nivel de complejidad, y a la consulta privada de algunos ginecólogos de la ciudad de Medellín que aceptaron participar en el estudio. Se excluyeron las mujeres con diagnóstico de síndrome antifosfolípido secundario. Se evaluaron las características sociodemográficas, los antecedentes obstétricos y médicos con énfasis en hipertensión arterial y trombosis. También se evaluaron los anticuerpos anticardiolipina, anticoagulante lúpico, número de anticuerpos antiß2-GPI y número de anticuerpos antianexina V. La información se tabuló en una hoja de cálculo de Microsoft Excel y se procesó en el programa estadístico Stata 10.0, en el análisis estadístico se utilizó estadística descriptiva. Resultados: se encuestaron 65 mujeres con edades entre los 18 y 46 años, la mitad de las pacientes reportaron 2 abortos antes de la décima semana, y la otra mitad después de esta. En los resultados de los anticuerpos anticardiolipina se encontró que el 4,6% (3) de las pacientes presentaron anticuerpos positivos IgM y el 3,1% (2) tenían anticuerpos positivos IgG. El 3,1% (2) de las mujeres presentaron anticuerpos antiß2-GPI IgG e IgM. El 4,6% (3) de las pacientes presentaron anticuerpos positivos antianexina IgG y un 12,3% (8) de estas presentaron un resultado indeterminado.Conclusiones: se evidencia la presencia de anticuerpos antianexina en las pacientes estudiadas, aunque un subgrupo de mujeres presentó un resultado indeterminado; surge la hipótesis de que la anexina V podría ser un factor importante e independiente de los anticuerpos cardiolipina y β2-GPI en las pérdidas gestacionales recurrentes.

Objective: Describing anti-annexin V antibody frequency in a series of females suffering recurrent abortion in Medellin. Materials and methods: A series of cases was compiled, consisting of healthy females attending gynecological consultation who had a background of recurrent pregnancy loss (RPL), characterized by two or more abortions during the first three months of pregnancy. The females were attending a second- and third-level complexity healthcareproviding institution and/or private consultation with some gynecologists in Medellin who agreed to participate in the study. Females diagnosed with secondary antiphospholipid syndrome were excluded. Socio-demographic characteristics, obstetric and medical background emphasizing hypertension and thrombosis were evaluated, as were anti-cardiolipin antibodies, lupus anticoagulant, number of anti-â2-GPI antibodies and number of anti-annexin V antibodies. Such information was tabulated on a Microsoft Excel sheet and processed using Stata 10.0 statistical software; descriptive statistics were used for statistical analysis. Results: 65 females were surveyed, their ages ranging from 18 to 46 years. Half the patients reported 2 abortions before the tenth week and the other half following such time. Anti-cardiolipin antibody results revealed that 4.6% (3) of the patients proved positive for IgM antibodies and 3.1% (2) positive for IgG antibodies. 3.1% (2) of the females had IgG and IgM anti-â2-GPI antibodies. 4.6% (3) of the patients were positive for anti-annexin IgG antibodies and 12.3% (8) had an indeterminate result. Conclusions: Anti-annexin antibodies were detected in the patients surveyed in this study, although a subgroup of females had an indeterminate result. This led to advancing a hypothesis that annexin V could be an important and independent factor for cardiolipin and â2-GPI antibodies in recurrent pregnancy loss.Conclusions: Anti-annexin antibodies were detected in the patients surveyed in this study, although a subgroup of females had an indeterminate result. This led to advancing a hypothesis that annexin V could be an important and independent factor for cardiolipin and β2-GPI antibodies in recurrent pregnancy loss.

Anticardiolipin antibodies and lupus anticoagulants among women undergoing in vitro fertilization in Jamaica / Anticuerpos anticardiolipinas y anticoagulantes lúpicos entre mujeres a las que se les realiza FIV en Jamaica

OBJECTIVE: The aims of this study were to evaluate the effect of anticardiolipin antibody (aCL) and lupus anticoagulant (LA) on the outcome of the in vitro ferlitization (IVF) cycles and to determine the prevalence of these antibodies in infertile women seeking IVF in Jamaica. METHODS: A retrospective cohort study was performed to determine if screening patients for aCL and LA had any significant impact on the outcome of the IVF process. Each patient's hospital record, between March 2000 and March 2010, was collected and the relevant data extracted. RESULTS: The prevalence of aCL in this cohort of Jamaican women was moderate/high positive 3.88%, low positive 0.68% and those with negative aCL results 95.4%. The prevalence of women who were LA positive was 4.1% and 0.9% of the women were positive for both LA and aCL. Of the patients who were LA and/or aCL positive, eight out of 30 patients (26.7%) had a positive pregnancy test in comparison to 61 out of 181 patients (33.7%) who were LA and/or aCL negative (p = 0.5787). CONCLUSION: The prevalence of positive aCL and/or lA in infertile women seeking IVF in Jamaica is 7.76%. The presence of these antibodies did not affect the pregnancy rate of these women nor did it demonstrate an increased risk for IVF cycle cancellation or ovarian hyperstimulation syndrome. Screening women undergoing IVF for these antibodies is not justified.

OBJETIVO: Los objetivos de este estudio fueron evaluar el efecto del anticuerpo anticardiolipina (aCL) y el anticoagulante lúpico (LA) sobre el resultado de los ciclos de la fertilización en vitro (FIV), así como determinar la prevalencia de estos anticuerpos en mujeres estériles que buscan tratamiento de FIV en Jamaica. MÉTODOS: Se realizó un estudio de cohorte para determinar si el tamizaje de pacientes para detectar el anticuerpo anticardiolipina y el anticoagulante lúpico tenía un impacto significativo en el resultado del proceso de FIV. Se obtuvieron las historias clínicas hospitalarias de cada una de las pacientes, entre marzo de 2000 y marzo de 2010, y se extrajeron los datos pertinentes. RESULTADOS: La prevalencia de aCL en esta cohorte de mujeres jamaicanas fue 3.88% moderada/alta positiva, 0.68% positiva baja, y aquellas con resultados negativos de aCL, 95.4%. La prevalencia de mujeres con resultados de anticoagulante lúpico positivos fue 4.1%, y 0.9% de las mujeres resultaron positivas con respecto tanto al LA como al aCL. De las pacientes que fueron positivas al LA y/o al aCL, ocho de cada 30 pacientes (26.7%) tuvieron una prueba de embarazo positiva, en comparación con 61 de cada 181 pacientes (33.7%) negativas al LA y/o al aCL (p = 0.5787). CONCLUSIÓN: La prevalencia de resultados positivos en relación con anticuerpos anticardiolipinas y/o anticoagulantes lúpicos en mujeres estériles que buscan FIV en Jamaica es 7.76%. La presencia de estos anticuerpos no afectó la tasa de embarazo de estas mujeres, ni mostró un aumento de riesgo de la cancelación del ciclo FIV, o riesgo de síndrome de hiperestimulación ovárica. El tamizaje en busca de estos anticuerpos en mujeres que buscan tratamiento de FIV, no está justificado.

Anticorpos antinucleossomo e síndrome antifosfolipídica: estudo observacional / Antinucleosome antibodies and primary antiphospholipid syndrome: an observational study

OBJETIVO: Avaliar a associação entre a presença de anticorpos antinucleossomo (anti-NCS) e a síndrome antifosfolipídica primária (SAFP) e o posterior desenvolvimento de lúpus eritematoso sistêmico (LES). MATERIAIS E MÉTODOS: Trinta e seis mulheres com o diagnóstico de SAFP foram avaliadas prospectivamente para manifestações de doenças reumáticas autoimunes e para a presença de anticorpos antifosfolípides, anticorpos antinucleares e anti-NCS/cromatina. RESULTADOS: Após um período médio de seguimento de 45,7 meses, anticorpos anti-NCS/cromatina foram detectados em apenas uma paciente (2,8%), que desenvolveu manifestações de LES tais como poliartrite, linfopenia, neurite óptica, lesões compatíveis com esclerose múltipla em substância branca cerebral e perfil de autoanticorpos altamente sugestivo de LES. CONCLUSÃO: A frequência de anticorpos anti-NCS/cromatina é baixa em pacientes com SAFP, e sua presença pode associar-se ao desenvolvimento de manifestações de LES.

OBJECTIVE: To study the association of anti-nucleosome (anti-NCS) antibodies in primary antiphospholipid syndrome (APS) and the development of systemic lupus erythematosus (SLE) during follow-up. MATERIALS AND METHODS: Thirty-six women with primary APS were evaluated prospectively for clinical features of systemic autoimmune diseases and for the presence of antiphospholipid antibodies, antinuclear antibodies and anti-NCS/chromatin antibodies. RESULTS: After a mean follow-up period of 45.7 months, anti-NCS/chromatin antibodies were detected in only one patient (2.8%), who developed features of SLE including polyarthritis, lymphopenia, optic neuritis, multiple sclerosis-like lesions, and an autoantibody profile suggestive of SLE. CONCLUSION: The frequency of anti-NCS/chromatin antibodies in primary APS patients is very low, and they may be associated with the development of SLE manifestations.

Gangrena de pavilhão auricular como primeira manifestação de síndrome do anticorpo antifosfolípide / Gangrene of the auricle as the first sign of antiphospholipid antibody syndrome

A síndrome do anticorpo antifosfolípide (SAF), mais comum em mulheres, manifesta-se clinicamente como trombose e/ou abortamentos de repetição. Anemia hemolítica autoimune e manifestações neurológicas, cardíacas e cutâneas são comuns. Relata-se o caso de um paciente do gênero masculino cuja manifestação inicial da doença foi gangrena em pavilhão auricular, e o diagnóstico de SAF se deu por meio de biópsia de pele do membro inferior, que mostrava vasculopatia trombótica, sem evidência de vasculite. Esse resultado é um dos dois critérios maiores que, associados a um critério menor, fecham o diagnóstico dessa doença. Discutem-se neste caso os possíveis diagnósticos diferenciais e como eles se diferenciam da doença em foco, além da importância que a biópsia teve no diagnóstico de SAF nesse indivíduo.

Antiphospholipid syndrome (APS), more common in females, manifests clinically as thrombosis and/or recurrent fetal loss. Hemolytic autoimmune anemia and neurological, cardiac and cutaneous manifestations are common. This is the case report of a male patient whose first manifestation of the disease was gangrene of the auricle. The diagnosis of APS was established by biopsy of the lower limb skin, which showed thrombotic vasculopathy with no evidence of vasculitis. This is one of the two major criteria, which, along with a minor criterion, establishes the diagnosis of APS. Possible differential diagnoses are discussed. The importance of the biopsy in the APS diagnosis of this male patient is emphasized.

Characterization of antiphospholipid antibodies in chronic hepatitis B infection

BACKGROUND: Many infections are associated with antiphospholipid antibodies (aPLs). The purpose of this study was to investigate the prevalence, persistence, clinical significance, and characteristics of aPLs in hepatitis B virus (HBV)-infected patients. METHODS: This study included 143 patients with HBV infection and 32 healthy individuals as controls. The presence of anticardiolipin antibodies (aCL Ab), anti-beta2-glycoprotein I antibodies (beta2GPI Ab), and lupus anticoagulant (LA) was assessed. RESULTS: The total prevalence of aPLs in HBV-infected patients was 12.6% (18 of 143). Of these 18 patients, 15 had low to medium titers of aCL Ab (10 with IgM, 4 with IgG, and 1 with both isotypes). beta2GPI Ab and LA were detected in 3 (2.1%) and 2 (1.4%) patients with HBV infection, respectively. In follow-up specimens from 14 patients with elevated levels of aCL Ab or beta2GPI Ab, 10 (71.4%) showed the persistent presence of aPLs. No clinical manifestations related to aPLs were identified. CONCLUSION: In HBV-infected patients, the most frequently detected antiphospholipid antibodies were IgM aCL Ab, which have a weak association with the clinical manifestations of APS. Unlike the transient presence reported for other infection-associated aPLs, most aPLs were persistently detected over a 12-week period in patients with HBV infection.

Mutação G20210A no gene da protrombina, fator V de Leiden e anticorpos anticardiolipina não influenciam a sobrevida do enxerto renal após o transplante / Prothrombin G20210A gene mutation, factor V Leiden and anticardiolipin antibodies do not influence renal graft survival after transplantation

Introdução: Complicações tromboembólicas são importantes fatores de risco para perda do enxerto e pior evolução após o transplante renal. pacientes com defeito trombofílico apresentam maior risco de complicações tromboembólicas. Foram analisados, entre receptores de transplante renal, a prevalência de defeito trombofílico e o risco atribuído a esta condição para a perda do enxerto e para o desenvolvimento de tromboses intravasculares. Métodos: estudo do tipo coorte incluindo 388 receptores adultos analisados quanto à presença de trombofilia de acordo com a pesquisa de anticorpos anticardiolipidina (aCL) por ELISA e das mutações G1691A no gene do fator V (FV) e G20210A no gene da protrombina (PT) por PCR multiplex. Resultados: Defeito trombofílico foi identificado em 25,8% dos pacientes. As taxas de sobrevida de 2 anos do enxerto foram semelhantes entre os pacientes com e sem defeito trombofílico (94%, p=0,53), bem como a sobrevida dos enxertos livres de tromboses intravasculares (97% versus 97%, p=0,83). pacientes com defeito trombofílico apresentaram prevalência de tromboses intravasculares semelhante à do grupo-controle (3% versus 3,5%, p=0,82). O transplante renal anterior foi associado a maior risco de perda de enxerto (OR 20,8, p<0,001) e de ocorrência de trombose intravasculares (OR 6,8, p=0,008). Conclusões: As prevalências das mutações FVG1691A e PTG20210A na população estudada foram semelhantes às da população geral não transplantada, e a prevalência de anticorpos aCL superou a observada entre os indivíduos sadios. Não houve associação entre os marcadores de trombofilia estudados e a sobrevida em médio prazo do transplante renal.

Introduction: Thromboembolic complications are important risk factors for graft loss and poor outcome after renal transplantation. patients with thrombophilic defects are at increased risk of thromboembolic complications. Were analyzed, among kidney transplant recipients, the prevalence of thrombophilic defects and the risk attributed to this condition for graft loss and the development of intravascular thrombosis. Methods: A cohort study including 388 adult recipients analyzed for the presence of thrombophilia according to anticardiolipidina antibodies (aCL) by ELISA and gene mutations G1691A in factor V (FV) and prothrombin gene G20210A (PT) by multiplex PCR. Results: thrombophilic defect was identified in 25.8% of patients. The survival rates of two years of the graft were similar between patients with and without thrombophilic defect (94%, p = 0.53), and the survival of free grafts of intravascular thrombosis (97% versus 97%, p = 0 , 83). patients with an increased prevalence of thrombophilic defect intravascular thrombosis similar to the control group (3% versus 3.5%, p = 0.82). Previous renal transplantation was associated with increased risk of graft loss (OR 20.8, p <0.001) and intravascular thrombosis (OR 6.8, p = 0.008). Conclusions: The prevalence of mutations and FVG1691A PTG20210A in this study were similar to those of the general population not transplanted, and the prevalence of aCL antibodies exceeded that observed among healthy individuals. There was no association between markers of thrombophilia studied and medium-term survival in renal transplantation.

Autoantibodies in leprosy patients, with and without joint involvement, in the state of Amazonas / Autoanticorpos em pacientes com hanseníase, com e sem comprometimento articular, no Estado do Amazonas

OBJETIVOS: Determinar a frequência do fator reumatoide (FR-IgM), anticorpos antipeptídeos citrulinados cíclicos (anti-CCP), antinucleares (AAN), anticitoplasma de neutrófilos (ANCA), anticardiolipina (aCL) e anti-β2 glicoproteína I (anti-β2GPI) em pacientes com hanseníase, com e sem comprometimento articular, avaliando a possível associação entre estes autoanticorpos e as manifestações articulares, a forma clínica, a reação hansênica, o tratamento com poliquimioterapia (PQT) e a alta. PACIENTES E MÉTODOS: 158 pacientes com hanseníase foram distribuídos em dois grupos; 73 pacientes com (Grupo I) e 82 sem comprometimento articular (Grupo II). Compuseram o Grupo III 129 indivíduos saudáveis. MÉTODOS: aglutinação com partículas de látex para FR-IgM, imunofluorescência indireta para AAN e ANCA, e, ELISA para anti-CCP, aCL e anti-β2GPI. RESULTADOS: Dentre 158 pacientes com hanseníase, 56 apresentavam a forma virchowiana (VV). A frequência de anticorpos anti-CCP, FR e AAN nos Grupos I e II foi semelhante à do Grupo III. ANCA não foi detectado em nenhum dos grupos. Anticorpos aCL foram mais frequentes nos pacientes com hanseníase (Grupos I e II) que em controles sadios (15,8 por cento vs. 3,1 por cento; P < 0,001), não sendo observada diferença entre Grupos I e II (P = 0,67). Anticorpos anti-β2GPI também foram mais frequentes nos pacientes que nos controles (46,2 por cento vs. 9,4; P < 0,001), sem diferença significativa entre os Grupos I e II. Houve predomínio do isotipo IgM com relação ao IgG tanto para aCL (88 por cento vs. 16 por cento, P = 0,001), quanto para anti-β2GPI (97,3 por cento vs. 12,3 por cento, P < 0,001). Nenhum paciente apresentou manifestações sugestivas de trombose vascular. CONCLUSÃO: A frequência de anticorpos aCL e anti-β2GPI foi significativamente maior nos pacientes com hanseníase que nos controles saudáveis. Entretanto, a positividade dos demais autoanticorpos foi semelhante à dos controles. Não foi observada associação entre autoanticorpos...

OBJECTIVE: Determine the frequency of rheumatoid factor (IgM-RF) and anti-cyclic citrullinated peptide antibodies (anti-CCP), antinuclear antibodies (ANA), antineutrophil cytoplasmic antibodies (ANCA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein I antibodies (anti-β2GPI) in leprosy patients, with and without joint involvement, and to evaluate the possible association among those antibodies and articular manifestations, clinical type, reactional episodes, polychemotherapic treatment (PCT), and discharge from PCT. PATIENTS AND METHODS: One hundred and fifty-eight leprosy patients were divided in two groups of 73 patients (Group I) and 82 patients (Group II). Group III was composed of 129 healthy individuals. Methods: Semi-quantitative latex agglutination test for IgM-RF, indirect immunofluorescence for ANA and ANCA, and ELISA for anti-CCP, aCL, and anti-β2GPI. RESULTS: Fifty-six (35.4 percent) of 158 leprosy patients had lepromatous leprosy (LL). The frequency of anti-CCP, RF, and ANA antibodies in Groups I and II was similar to that of Group III. Antineutrophil cytoplasmic antibodies were not detected in any patient. Anticardiolipin antibodies were more frequent in leprosy patients (Groups I and II) than in control group (15.8 percent vs. 3.1 percent; P < 0.001), and differences between Groups I and II (P = 0.67) were not observed. Anti-β2GPI antibodies were also more common in leprosy patients than in control group (46.2 percent vs. 9.4 percent; P < 0.001), without differences between Groups I and II. A predominance of IgM isotype over IgG isotype was observed both for aCL (88 percent vs. 16 percent; P = 0.001) and anti-β2GPI (97.3 percent vs. 12.3 percent; P < 0.001). Patients did not present manifestations suggestive of vascular thrombosis. CONCLUSION: The frequency of aCL and anti-β2GPI antibodies was significantly increased in leprosy patients than in healthy individuals. However, positivity to other autoantibodies...

Leishmaniasis cutánea asociada a síndrome antifosfolipídico / Cutaneous leishmaniasis associated to antiphospholipid syndrome

Presentamos el caso de un paciente masculino de 24 años, con diagnóstico de leishmaniasis cutánea, que muestra cuadro clínico caracterizado por lesiones ulcerosas, de bordes definidos, edematoso y eritematoso en brazo derecho y párpado superior de ojo izquierdo, acompañado de dolor intenso en el mismo miembro, documentándose por ecografía y flebografía trombo en el nivel de la vena basílica en el brazo derecho. Se diagnostica trombosis venosa y se solicitan anticuerpos anticardiolipinas, los cuales reportan positivos, llegando a la conclusión de que el paciente es portador de un síndrome antifosfolipídico secundario, siendo esta asociación poco frecuente.

We present a 24 years old male patient, with diagnosis of cutaneous leishmaniasis, characterized by clinical erythematous, edematous and ulcerating lesions of well defined edges, in his right arm and upper left eyelid, accompanied by intense pain at the same extremity; a thrombus was documented by ultrasound and phlebography at the basilica vein in his right arm. It was diagnosed venous thrombosis and the anticardiolipin-antibodies was reported was positive. We made the diagnosis of secondary antiphospholipid syndrome secondary, being this a rare association.

Evaluation of antiphospholipid antibodies testing for the diagnosis of antiphospholipid syndrome / Avaliação da pesquisa de anticorpos antifosfolipídios para o diagnóstico da síndrome antifosfolípide

OBJETIVO: Determinar a prevalência de anticoagulante lúpico (LAC) e dos isótipos de anticardiolipina (ACL) e suas eventuais associações clínicas. PACIENTES E MÉTODOS: Estudo retrospectivo que avaliou manifestações clínicas e laboratoriais em indivíduos que apresentaram positividade para anticorpos antifosfolipídios no Hospital Edmundo Vasconcelos entre março de 2005 e junho de 2006. RESULTADOS: Cento e seis indivíduos (média de idade 42,2 ± 14,1 anos, 84 por cento do sexo feminino) foram incluídos no estudo. A prevalência de trombose foi de 17,9 por cento (19/106) e de morbidade gestacional foi de 12,3 por cento (13/106). O diagnóstico de Síndrome Antifosfolípide (SAF) foi feito em 23,6 por cento (25/106), sendo primária em 68 por cento (17/25) e secundária em 32 por cento (8/25). A prevalência de ACL foi de 97,1 por cento (103/106) e de LAC foi de 11,4 por cento (5/44 dos exames realizados). ACL isótipos IgM, IgG e IgA foram encontrados em 100 por cento, 23,3 por cento e 4,9 por cento dos 103 soros ACL positivos, respectivamente. Para o diagnóstico de SAF, a ACL IgM apresentou sensibilidade de 92 por cento e especificidade de 1,2 por cento, enquanto a ACL IgG teve sensibilidade de 40 por cento e especificidade de 82,5 por cento. A ausência de ACL IgG teve alto valor preditivo negativo (81,4 por cento) para SAF. O LAC apresentou sensibilidade de 18,7 por cento e especificidade de 92,8 por cento. A curva Receiver Operating Characteristic (ROC) demonstrou maior área abaixo da curva para ACL IgG e LAC. CONCLUSÃO: Em amostra aleatória de indivíduos com anticorpos antifosfolipídios positivos, ACL IgG e LAC apresentaram maior especificidade para o diagnóstico de SAF, que se caracterizou pela maior prevalência de trombose.

OBJECTIVE: To evaluate the prevalence of lupus anticoagulant (LAC) and isotypes of anticardiolipin (ACL) antibodies and its possible clinical associations. PATIENTS AND METHODS: A retrospective study analyzed clinical and laboratorial manifestations in individuals who showed positive antiphospholipid antibodies followed-up at Hospital Edmundo Vasconcelos from March 2005 to June 2006. RESULTS: 106 participants (mean age of 42.2 ± 14.1 years at inclusion and female gender in 84 percent of patients) were included in the study. The prevalence of thrombosis was 17.9 percent (19/106 patients) and pregnancy morbidity was 12.3 percent (13/106 patients). The antiphospholipid syndrome (APS) was confirmed in 23.6 percent (25/106 patients), and it was primary in 68 percent (17/25 patients) and secondary in 32 percent (8/25 patients). The ACL antibodies were found in 97.1 percent (103/106) and LAC in 11.4 percent (5/44 of the serum samples tested). IgM, IgG and IgA ACL isotypes were respectively found in 100 percent, 23.3 percent and in 4.9 percent of these ACL positive sera. For APS diagnosis the sensitivity of IgM ACL was 92 percent and its specificity was 1.2 percent, while IgG ACL had a sensitivity of 40 percent and specificity of 82.5 percent. The absence of IgG ACL had a high negative predictive value for APS diagnosis (81.4 percent).The analysis of the Receiver Operating Characteristic (ROC) curve showed larger area under the curve for ACL IgG and LAC. CONCLUSION: In a random sample of individuals with positive antiphospholipid antibodies, IgG ACL and LAC showed a larger specificity for APS diagnosis which had been characterized by a higher prevalence of thrombosis.

Apresentação pediátrica da síndrome antifosfolípide / Paediatric antiphospholipid syndrome presentation

OBJETIVO: Descrever as características clínicas, laboratoriais e de desfecho de uma série de casos com diagnóstico definido de síndrome antifosfolípide (SAF) pediátrica. MÉTODOS: Estudo observacional-retrospectivo de referência pediátrica terciária, que identificou os casos por meio de evento vascular, trombose venosa ou oclusão arterial, determinação de anticorpos anticardiolipina (IgG e IgM) e teste do anticoagulante lúpico. RESULTADOS: Foram identificados cinco casos atendidos nos últimos cinco anos, sendo dois meninos e três meninas. A trombose venosa ocorreu em seios venosos cerebrais (2), fibular (2), poplítea (1), femoral (1), intestinal (1), renal (1), acompanhados por oclusão arterial intestinal (1), de artéria renal (1) e artéria digital (1), esta resultando gangrena periférica como evento recorrente durante anticoagulação com warfarina. Um abortamento espontâneo ocorreu em uma adolescente em vigência de púrpura trombocitopênica, evoluindo com anemia hemolítica (síndrome de Evans) e desfecho fatal por hemorragia. A investigação laboratorial em todos os casos resultou, pelo menos, uma determinação positiva de anticardiolipina IgG e/ou IgM, sendo considerados como SAF primária. Três dos casos estão em seguimento com anticoagulação oral. CONLUSÃO: A trombose venosa cerebral e de extremidades foram os eventos mais freqüentes. A presente série alerta para a investigação e o diagnóstico precoces, com abordagem multidisciplinar para o tratamento.

OBJECTIVE: To describe clinical and laboratorial features as well as outcome in a paediatric series with defined diagnosis of antiphospholipid syndrome. METHODS: A descriptive-retrospective report from a pediatric tertiary referral, with case ascertainment by vascular events identification, either venous thrombosis or arterial occlusion, anti-cardiolipin antibodies (IgG and IgM) titres and lupus anticoagulant tests. RESULTS: Five cases, being two boys and three girls, were identified in the last five years. Venous thrombosis occurred in brain sinus vessels (2), fibular (2), popliteal (1), femoral (1), intestinal (1) and renal (1) veins, followed by arterial occlusion in the gut (1), kidneys (1) halux (1) this resulted in digit gangrene as a recurrent event occurring during warfarin treatment. A spontaneous abortion occurred in an adolescent with thrombocytopenic purpura evolving into haemolytic anemia (Evans Syndrome) and bleeding fatal outcome. Laboratory investigation resulted in at least one positive titre of IgG and/or IgM anticardiolipin antibodies in all of the cases, being considered as primary antiphospholipid syndrome. Three cases have been followed under oral anticoagulation treatment. CONCLUSION: Cerebral and extremities venous thrombosis were the most frequent events in this series. This points to early diagnosis and team approach for investigation and treatment.