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1.
Arch. argent. pediatr ; 119(6): e631-e635, dic. 2021. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1353055

ABSTRACT

El síndrome antifosfolipídico (SAF) es infrecuente en la edad pediátrica (3 %) y se presenta como eventos trombóticos de lechos vasculares y/o abortos espontáneos, asociado a la presencia de anticuerpos antifosfolipídicos (aFL). Este síndrome puede ser primario o asociado a alguna enfermedad sistémica subyacente. Se presenta el caso de una niña de 12 años con hemiparesia faciobraquiocrural derecha y alteración en la marcha de aparición aguda, en la cual se confirma un accidente cerebrovascular (ACV) isquémico por trombosis de la arteria cerebral media asociado a aFL positivos (anticuerpo anticardiolipina, anticoagulante lúpico y anticuerpo anti-ß2-glicoproteína). Cumple con los criterios para realizar diagnóstico de síndrome antifosfolipídico. Luego de iniciar el tratamiento, la paciente evoluciona de manera favorable. Se trata de una patología infrecuente y de presentación variable, por lo que requiere un alto sentido de alerta por parte del equipo de salud para evitar retrasos en el diagnóstico y el tratamiento, y disminuir su morbimortalidad


Antiphospholipid syndrome (APS) is infrequent at pediatric age (3 %) and is characterized by venous or arterial thrombosis and/or spontaneous abortions. APS occurs either as a primary condition or in the setting of an underlying disease. This is a case of a 12-year-old girl with a right hemiparesis and acute disturbance in gait, in which an ischemic cerebrovascular accident (CVA) due to middle cerebral artery thrombosis associated with positive antiphospholipid antibodies is confirmed (anticardiolipin antibody, lupus anticoagulant and anti-ß2-glycoprotein antibody), fulfilling the criteria for the diagnosis of antiphospholipid syndrome . After starting treatment accordingly, the patient evolves favorably. As this pathology is infrequent and of variable presentation, it requires a high sense of alert from the health team to avoid delays in diagnosis and treatment


Subject(s)
Humans , Female , Child , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Lupus Coagulation Inhibitor , Antibodies, Antiphospholipid , beta 2-Glycoprotein I
4.
Rev. Assoc. Med. Bras. (1992) ; 66(11): 1595-1601, Nov. 2020. tab
Article in English | SES-SP, LILACS, SES-SP | ID: biblio-1143628

ABSTRACT

SUMMARY The 2006 Revised Sapporo Classification Criteria for Definite Antiphospholipid Syndrome included as laboratory criteria the tests for antiphospholipid antibodies whose accuracy was regarded as satisfactory according to the evidence available at that time. In practice, however, the sensitivity and specificity of these "criteria" of antiphospholipid antibodies are sometimes insufficient for identifying or ruling out antiphospholipid syndrome. It has been studied whether the accuracy of the laboratory diagnosis of the syndrome could be improved by testing for non-criteria antiphospholipid antibodies. In this work, we review evidence on the clinical associations and diagnostic value of the most commonly studied non-criteria antibodies, namely: antiphosphatidylethanolamine, anti-annexin A5, anti-prothrombin, anti-phosphatidylserine/prothrombin complex, IgA anticardiolipin, and IgG anti-domain I of the β2 glycoprotein antibodies.


RESUMO A classificação de Sapporo revisada para a síndrome antifosfolipídica definida de 2006 incluiu como critérios laboratoriais aqueles testes para anticorpos antifosfolípides cuja acurácia era considerada satisfatória de acordo com a evidência então disponível. Porém, na prática, a sensibilidade e especificidade desses anticorpos antifosfolípides "critério" são por vezes insuficientes para identificar ou descartar a síndrome antifosfolípide. Tem-se estudado se a acurácia do diagnóstico laboratorial da síndrome poderia ser melhorada por meio da testagem de anticorpos antifosfolípides não critério. Neste trabalho revisamos a evidência a respeito das associações clínicas e valor diagnóstico dos anticorpos não critério mais estudados, nomeadamente: anticorpos antifosfatidiletanolamina, antianexina A5, antiprotrombina, anticomplexo fosfatidilserina/protrombina, IgA anticardiolipina e IgG antidomínio I da anti-β2 glicoproteína I.


Subject(s)
Humans , Antiphospholipid Syndrome/diagnosis , Prothrombin , Sensitivity and Specificity , Antibodies, Antiphospholipid , Antibodies, Anticardiolipin , beta 2-Glycoprotein I
9.
Adv Rheumatol ; 60: 51, 2020. tab
Article in English | LILACS | ID: biblio-1130793

ABSTRACT

Abstract Background: Hemorrhoid disease (HD) is one of the most common gastrointestinal complaints worldwide, affecting 4.4% of the general population in the United States. Since antiphospholipid syndrome (APS) may lead to intra-abdominal thrombosis, one may expect that this condition can impact the risk for HD development. Additionally, as APS patients are more prone to thrombosis and treatment with anticoagulants may increase risk of bleeding, one may also infer that rates of HD complications may be higher in this scenario. Nevertheless, no data in these regards have been published until now. The objective of the present study is to evaluate frequency of HD and describe its complications rates in antiphospholipid syndrome APS patients. Methods: We consecutively invited patients who fulfilled APS criteria to undergo proctological examination. After examination, patients were divided in two groups, based on the presence of HD, and compared regarding different clinical manifestations and antiphospholipid profile. We performed the analysis of the data, using chi-square and Mann Whitney U when applicable and considering a significance level of 0.05. Multivariate regression analysis included age and variables with p < 0.10 in the bivariate analysis. Results: Forty-one APS patients agreed to undergo proctological examination. All were female and overall median age was 43 (36-49). Seventeen (41.4%) patients were diagnosed with HD, with the following frequency distribution: 7 internal (41.2%), 4 external (23.5%) and 5 mixed hemorrhoids (29.4%). Of the internal hemorrhoids, 5 patients were classified as grade I (71.4%), 1 grade II (14.3%), and 1 grade IV (14.3%). Prior gestation ( p = 0.067) and constipation ( p = 0.067) correlated with a higher frequency of HD. In multivariate analysis, constipation remained as an important risk factor (OR 3.92,CI95% 1.03-14.2, p = 0.037). Five out of 17 patients (29.4%) reported anal bleeding, but it did not correlate with warfarin dose ( p = 0.949). Surgical treatment was indicated for 10 patients (58.8%). Other anorectal findings were anal fissure, plicoma, condyloma and one chlamydial retitis. Conclusion: We found an unexpected high frequency of hemorrhoids in APS patients, with a great proportion requiring surgical treatment.(AU)


Subject(s)
Humans , Rectal Diseases/diagnosis , Antiphospholipid Syndrome/pathology , Antibodies, Antiphospholipid/blood , Cross-Sectional Studies , Colonoscopy
10.
Cambios rev. méd ; 18(2): 32-38, 2019/12/27. graf., tab.
Article in Spanish | LILACS | ID: biblio-1097727

ABSTRACT

INTRODUCCIÓN. El síndrome de superposición en pacientes con lupus eritematoso sistémico no ha sido caracterizado en nuestro país. OBJETIVO. Cuantificar la prevalencia de síndrome de superposición en pacientes con lupus eritematoso sistémico, analizar los factores de riesgo y ca-racterizar los pacientes lúpicos puros y con superposición. MATERIALES Y MÉTODOS. Estudio observacional, analítico de prevalencia, con población de 324 y una muestra de 308 pacientes que cumplieron con los criterios de inclusión de diagnóstico definitivo de lupus eritematoso sis-témico, de los cuales 118 tuvieron síndrome de superposición y 190 fueron lúpicos puros, en la consulta externa de la Unidad Técnica de Reumatología del Hospital de Especialidades Carlos Andrade Marín entre enero de 2015 y abril de 2018. RESULTADOS. La prevalencia global de superposición fue de 38,30% (118; 308), de éstos el 43,20% (51; 118) presentaron lupus eritema-toso sistémico con síndrome de anticuerpos antifosfolipídicos. Se encontró que los pacientes con síndrome de superposición presentó un mayor porcentaje diagnóstico en mujeres, edad mayor al hallazgo de lupus eritematoso sistémico, mayores manifestaciones articulares y cutáneas; y porcentajes mayores de positividad de anti-DNA e hipocomplementemia al diagnóstico de lupus eritematoso sistémico (p<0,05). No obstante, presentaron menores complicaciones como la ne-fropatía lúpica, alteraciones hematológicas y neuropsiquiátricas (p<0,05). La razón de momios de prevalencia estableció que la edad temprana al diagnóstico de lupus eritematoso sistémico, menor a 50 años, [RMP=0,51 IC 95% (0,26-0,98)] y la nefropatía lúpica [RMP=0,45 IC 95% (0,23-0,86)] constituyeron factores protectores para desarrollo de síndrome de superposición. CON-CLUSIÓN. Se cuantificó que el síndrome de superposición fue de alta frecuencia en pacientes con lupus eritematoso sistémico y de prevalencia similar en estudios internacionales. La edad, sexo fueron factores relevantes para el diagnóstico oportuno a edades tempranas, que no modifi-có la mortalidad con menor aparición de complicaciones renales y extrarrenales.


INTRODUCTION. The overlap syndrome in patients with systemic lupus erythematosus has not been characterized in our country. OBJECTIVE. To quantify the prevalence of overlap syndrome in patients with systemic lupus erythematosus, to analyze risk factors and characterize pure and overlapping patients. MATERIALS AND METHODS. Study observational, analytical prevalence, with population of 324 and a sample of 308 patients who met the inclusion inclusion criteria for a definitive diagnosis of systemic lupus erythematosus, of whom 118 had overlap syndrome and 190 were pure lupus, in the external consultation of the Rheumatology Technical Unit of the Carlos Andrade Marín Specialties Hospital between January 2015 and April 2018. RESULTS. The overall prevalence of overlap was 38,30% (118; 308), 43,20% (51; 118) presented lupus erythematosus systemic with antiphospholipid antibody syndrome. It was found that patients with overlap syn-drome presented a higher diagnostic percentage in women, older than the finding of systemic lupus erythematosus, greater joint and skin manifestations; and higher percentages of anti-DNA positivy and hypocomplementemia at the diagnosis of lupus systemic erythematosus (p<0,05). However, they presented fewer complications such as kidney disease lupus, hematological and neuropsychiatric disorders (p <0.05). The Odds ratio of prevalence established that the early age at diagnosis of systemic lupus erythematosus, less than 50 years, [RMP = 0.51 IC 95% (0.26-0.98)] and lupus nephropathy [RMP = 0 , 45 IC 95% (0.23-0.86)] constituted protective factors for the development of overlap syndrome. CONCLUSION. Overlap syndrome was quantified as high frequency in patients with systemic lupus erythematosus and of similar prevalence in international studies. Age, sex were relevant factors for timely diagnosis at early ages, which did not change mortality with less occurrence of renal and extrarenal complications.


Subject(s)
Prevalence , Connective Tissue , Undifferentiated Connective Tissue Diseases , Immunity , Lupus Erythematosus, Systemic , Antibodies , Pathology , Arthritis, Rheumatoid , Scleroderma, Systemic , Autoimmune Diseases , Sjogren's Syndrome , Mortality , Antibodies, Antiphospholipid , Diagnosis , Immune System , Muscular Diseases
11.
Acta bioquím. clín. latinoam ; 53(4): 539-543, dic. 2019. graf
Article in Spanish | LILACS | ID: biblio-1124031

ABSTRACT

La certeza del valor de la relación internacional normalizada (RIN), ensayo para controlar la anticoagulación con dicumarínicos, en pacientes con anticoagulante lúpico positivo (AL) es desconocida especialmente para los dispositivos al lado del paciente (POCT). El objetivo de este trabajo fue investigar si existe correlación entre el valor del RIN obtenido por el método tradicional y el obtenido con un dispositivo portátil en pacientes con AL positivo. Se estudiaron 35 pacientes anticoagulados por enfermedad tromboembólica con diagnóstico de AL positivo persistente a los que se les determinó al mismo tiempo el RIN por el método tradicional y con CoaguChek durante 4 controles consecutivos. El rango del RIN fue 1,9 a 5,60 y el RIN-POCT estuvo entre 2,0 y 4,92. La comparación del RIN vs RIN-POCT mostró r=0,98, pendiente: 1,56 (0,98-1,12) y una ordenada al origen de -0,088 (-0,282-0,007). El sesgo fue 2,1%. Para un nivel del RIN menor de 3,5 (n=136 controles) la diferencia del RIN promedio fue de 0,17 con un rango de 0,01-0,56. Un paciente, con triple positividad, mostró una diferencia entre ambos métodos mayor de 0,4 en dos controles. Para un RIN mayor de 4,5 el grado de concordancia fue menor pero no tiene implicancia clínica. Los resultados del RIN obtenidos por CoaguChek en los pacientes estudiados con AL positivo son útiles para la práctica clínica. Los datos obtenidos demuestran que hay una buena correlación entre el RIN tradicional y el CoaguChek. Por la gran diversidad de los equipos POCT los resultados no son extrapolables a otros dispositivos. Dada la heterogeneidad de los anticuerpos antifosfolípidos, es recomendable probar en cada paciente si hay una buena concordancia entre el RIN tradicional y el RIN-POCT.


The certainty of the value of the international normalized relation (INR) assay to control dicoumarin anticoagulation in patients with positive lupus anticoagulant (LA) is unknown especially for the point of care testing (POCT). The aim of this work was to investigate if there was a correlation between the INR values obtained by the traditional method and those obtained with a POCT in patients with positive LA. The population under study were 35 patients anticoagulated by thromboembolic disease with a persistent positive LA, whose INR was determined at the same time by the traditional method and with CoaguChek during 4 consecutive controls. The INR range was 1.9 to 5.60 and the RIN-POCT was between 2.0-4.92. The comparison of INR vs. INR - POCT showed r=0.98, slope: 1.56 (0.98-1.12) and ordered to the origin -0.088 (-0.282-0.007). The bias was 2.1%. For an INR level lower than 3.5 (n=136 controls) the average INR difference was 0.17 with a range of 0.01-0.56. One patient, with triple positivity showed a difference between both methods greater than 0.4. in two controls. For INR greater than 4.5, the degree of concordance is lower but has no clinical implications. The data obtained show that there is a good correlation between the traditional INR and the CoaguChek. The results of INR obtained by CoaguChek in patients studied with positive LA are useful for clinical practice. Due to the large diversity of POCT, the results cannot be extrapolated to other devices. Given the heterogeneity of antiphospholipid antibodies, it is advisable to test in each patient whether there is a good agreement between the traditional INR and INR-POCT.


A certeza do valor da razão internacional normalizada (RIN ou IIN), ensaio que controla a anticoagulação com dicumarínicos, em pacientes com anticoagulante lúpico positivo (AL) é desconhecida especialmente para os dispositivos de teste do tipo point-of-care (POCT). Este trabalho teve como objetivo pesquisar se existe correlação entre o valor de RIN obtido pelo método tradicional e aquele obtido com um dispositivo portátil em pacientes com AL positivo. Foram estudados 35 pacientes anticoagulados por doença tromboembólica com diagnóstico de AL positivo persistente aos quais lhes determinaram, ao mesmo tempo, a RIN pelo método tradicional e com CoaguChek durante 4 controles consecutivos. O intervalo de RIN foi de 1,9 a 5,60 e o de RIN-POCT ficou entre 2,0 e 4,92. A comparação de RIN vs RIN-POCT mostrou r=0,98, pendente: 1,56 (0,98-1,12) e uma ordenada à origem de -0,088 (-0,282-0,007). O viés foi 2,1%. Para um nível de RIN menor a 3,5 (n=136 controles) a diferença de RIN em média foi de 0,17 com um intervalo de 0,01-0,56. Um paciente, com tríplice positividade, mostrou uma diferença entre ambos os métodos maior a 0,4 em dois controles. Para um RIN de mais de 4,5, o grau de concordância foi menor, mas não tem consequências clínicas. Nos pacientes estudados com AL positivo, os resultados da RIN obtidos por CoaguChek são úteis para a prática clínica. Os dados obtidos demonstram que existe uma boa correlação entre a RIN tradicional e o CoaguChek. Devido à grande diversidade dos equipamentos POCT, os resultados não são extrapoláveis a outros dispositivos. É recomendável, visto a heterogeneidade dos anticorpos antifosfolípídes, provar em cada paciente a existência de uma boa concordância entre a RIN tradicional e a RIN-POCT.


Subject(s)
Lupus Coagulation Inhibitor/analysis , Antibodies, Antiphospholipid , Antibodies , Anticoagulants , Time , Work , Bias , Disease , Lupus Coagulation Inhibitor , International Normalized Ratio , Diagnosis , Equipment and Supplies , Control , Point-of-Care Testing , Methods
12.
Rev. bras. ginecol. obstet ; 41(10): 621-627, Oct. 2019. tab
Article in English | LILACS | ID: biblio-1042317

ABSTRACT

Abstract Antiphospholipid antibody syndrome (APS) is a systemic, autoimmune, prothrombotic disease characterized by persistent antiphospholipid antibodies (aPLs), thrombosis, recurrent abortion, complications during pregnancy, and occasionally thrombocytopenia. The objective of the present study was to review the pathophysiology of APS and its association with female infertility. A bibliographic review of articles of the past 20 yearswas performed at the PubMed, Scielo, and Bireme databases. Antiphospholipid antibody syndrome may be associated with primary infertility, interfering with endometrial decidualization and with decreased ovarian reserve. Antiphospholipid antibodies also have direct negative effects on placentation, when they bind to the trophoblast, reducing their capacity for invasion, and proinflammatory effects, such as complement activation and neutrophil recruitment, contributing to placental insufficiency, restricted intrauterine growth, and fetal loss. In relation to thrombosis, APS results in a diffuse thrombotic diathesis, with global and diffuse dysregulation of the homeostatic balance. Knowing the pathophysiology of APS, which is closely linked to female infertility, is essential for new therapeutic approaches, specialized in immunomodulation andinflammatory signaling pathways, to provide important advances in its treatment.


Resumo A Síndrome do anticorpo antifosfolípide (SAF) é uma doença sistêmica, autoimune e prótrombótica caracterizada por anticorpos antifosfolípides, trombose, aborto recorrente, complicações durante a gestação, e, ocasionalmente, trombocitopenia. O objetivo do presente estudo foi revisar a fisiopatologia da SAF e sua associação com a infertilidade feminina. Foi feita uma revisão bibliográfica dos últimos 20 anos nas bases de dados PubMed, Scielo e Bireme. A SAF pode estar associada à infertilidade primária, interferindo na decidualização endometrial e combaixas reservas ovarianas. Os anticorpos antifosfolípides também apresentam efeito negativo direto na placentação, se ligando ao trofoblasto e diminuindo sua capacidade de invasão, além de efeitos pró-inflamatórios, tais como ativação do sistema de complemento e recrutamento de neutrófilos, contribuindo para a insuficiência placentária, crescimento intrauterino restrito e perda fetal.Quanto a trombose, a SAF resulta em distúrbios trombóticos difusos, com uma desregulação do balanço homeostático. Conhecer a fisiopatologia da SAF, que apresenta associação importante com a infertilidade feminina, é essencial para novas abordagens terapêuticas, principalmente no que tange imunomodulação e os caminhos de ativação inflamatórios.


Subject(s)
Humans , Female , Pregnancy , Adult , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/physiopathology , Infertility, Female/complications , Infertility, Female/physiopathology , Abortion, Habitual , Antibodies, Antiphospholipid/blood , Middle Aged
13.
Rev. colomb. reumatol ; 26(3): 204-208, jul.-set. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1126336

ABSTRACT

Resumen El síndrome de anticuerpos antifosfolípidos es una condición de reciente descripción, cuyo diagnóstico se basa en la presencia de eventos trombóticos sin predisposición previa, con positividad de anticuerpos antifosfolípidos. Su presentación clínica incluye gran variedad de patrones, algunos de ellos no incluidos dentro de los criterios clínicos de diagnóstico, pero que deben ser conocidos. El compromiso dérmico es usual como livedo, sin embargo, la necrosis dérmica no es usual. Se presenta un caso de síndrome de anticuerpos antifosfolípidos con necrosis dérmica como manifestación primaria de la enfermedad.


Abstract Antiphospholipid antibodies syndrome is a recently described condition. The diagnosis of this condition is based on the presence of thrombotic events without previous predisposition and the positivity of anti-phospholipid antibodies. Its clinical presentation includes a variety of patterns, some of which are not included within the clinical criteria of the diagnosis, but must be known. Dermal involvement as livedo reticularis is common. However, dermal necrosis is not usual. Thus, a case of antiphospholipid syndrome with dermal necrosis is presented as a primary manifestation of the disease.


Subject(s)
Humans , Female , Aged , Antiphospholipid Syndrome , Necrosis , Thrombosis , Antibodies, Antiphospholipid , Diagnosis
14.
Rev. méd. Urug ; 35(1): 59-65, mar. 2019. tab
Article in Spanish | LILACS | ID: biblio-982060

ABSTRACT

Introducción: el síndrome de Budd-Chiari es una entidad rara definida por la obstrucción al flujo de salida venoso hepático. Se asocia frecuentemente a un estado protrombótico. El diagnóstico y tratamiento oportuno representan un reto para el médico clínico. El objetivo del presente trabajo es reportar un caso clínico de síndrome de Budd-Chiari secundario a síndrome antifosfolípido, asociación infrecuente en la literatura. Caso clínico: mujer de 31 años. Ascitis de seis años de evolución, actualmente refractaria. Repercusión general en el último año. Examen físico: lúcida, ictericia universal, hepatomegalia dolorosa, esplenomegalia y ascitis a tensión. Laboratorio: hepatograma con patrón colestásico. Colinesterasa y tasa de protrombina descendidas. Ecografía Doppler abdominal: ascitis severa, hepatomegalia irregular, ecogenicidad aumentada. Esplenomegalia. Obstrucción de vena suprahepática media, estrechamiento de vena suprahepática derecha. Flujo escasamente fásico de baja velocidad, invertido en algunas ramas, hepatófugo. Fibrogastroduodenoscopía: várices esofágicas grado III. Se planteó ascitis secundaria a hipertensión portal, probable síndrome de Budd-Chiari. Los anticuerpos IgM beta2 glicoproteína 1 fueron positivos. Diagnóstico de síndrome antifosfolípido. Se realizó trasplante hepático con buena evolución posterior. Discusión y conclusiones: el síndrome de Budd-Chiari es más frecuente en mujeres entre los 30 y 50 años. Debe considerarse como causa de enfermedad hepática, fundamentalmente cuando no existe otra causa evidente. Los estados protrombóticos que más se asocian al síndrome de Budd-Chiari en Occidente son las enfermedades hematológicas. El tratamiento debe ser individualizado según la presentación clínica. El trasplante hepático se plantea como medida de salvataje cuando el resto de los tratamientos han fracasado.


Introduction: [/sectitle][p]Budd-Chiari is a rare condition characterized by the occlusion of the hepatic vein flow out. It is frequently associated to a pro-thrombotic state. Diagnosis and treatment constitute a challenge for clinical doctors. This study aims to report a clinical case of Budd-Chiari syndrome secondary to an antiphospholipid syndrome, a rather unusual association in literature.[/p] Clinical case: 31 year old woman, with 6 years of evolution ascites, evidencing a general affection in the last year. Clinical examination: lucid, universal hyperbilirubinemia (jaundice), painful hepatomegaly, splenomegaly and tension ascites. Laboratory tests: hepatogram with cholestatic pattern, cholinesterase, and reduced prothrombin rate. Abdominal Doppler revealed: severe ascites, irregular hepatomegaly, increased echogenicity. Splenomegaly. Occlusion of the middle suprahepatic vein, narrowing of the right suprahepatic vein. Llow speed scarcely phasic flow, inverse in a few branches, hepatofugal. Fibrogastroduodenoscopy: esophagic varices grade III. Ascites secondary to portal hypertension was suspected, probable Budd Chiari syndrome. Antibodies IgM â2glicoprotein 1 were positive. Diagnosis of antiphospholipid syndrome. Liver transplant was performed with a good evolution of the patient. Discussion and conclusions:Budd-Chiari syndrome is more frequent in women between 35 and 50 years old. It needs to be regarded as a cause of liver conditions, in particular when there is no other evident cause. Hematologic diseases are the prothrombotic states more frequently associated to Budd-Chiari syndrome in the western world. Treatment must be based on the clinical presentation. Liver transplant is a rescue/salvage measure when all other treatments fail.


Introdução: a síndrome de Budd-Chiari é uma entidade rara definida pela obstrução do fluxo de saída venoso hepática. Frequentemente está associado a um estado protrombótico. O diagnóstico e o tratamento oportuno são um desafio para o médico clínico. O objetivo deste trabalho é descrever um caso clínico de síndrome de Budd-Chiari secundário à síndrome antifosfolípidica, uma associação pouco frequente na literatura. Caso clínico: mulher de 31 anos. Ascite com seis anos de evolução, atualmente refrataria. Repercussão geral no último ano. Exame físico: lúcida, icterícia universal, hepatomegalia dolorosa, esplenomegalia e ascite sob tensão. Laboratório: hepatograma com padrão colestásico. Colinesterase e taxa de protrombina diminuídas. Ultrassonografia Doppler abdominal: ascite severa, hepatomegalia irregular, ecogenicidade aumentada. Esplenomegalia. Obstrução de veia supra-hepática média, estreitamento de veia supra-hepática direita. Fluxo escassamente fásico de baixa velocidade, invertido em algumas ramas, hematófago. Fibrogastroduodenoscopia: varizes esofágicas de grau 3. Diagnóstico presuntivo: ascite secundaria à hipertensão portal, provável síndrome de Budd-Chiari. Anticorpos IgM alfa 2glicoproteína 1 positivos. Diagnóstico de síndrome antifosfolípido. Foi realizado um transplante hepático com boa evolução.


Subject(s)
Humans , Antibodies, Antiphospholipid , Budd-Chiari Syndrome
15.
Article in English | WPRIM | ID: wpr-719617

ABSTRACT

BACKGROUND: Antiphospholipid antibody syndrome (APS), an important cause of acquired thrombophilia, is diagnosed when vascular thrombosis or pregnancy morbidity occurs with persistently positive antiphospholipid antibodies (aPL). APS is a risk factor for unprovoked recurrence of pulmonary embolism (PE). Performing laboratory testing for aPL after a first unprovoked acute PE is controversial. We investigated if a specific phenotype existed in patients with unprovoked with acute PE, suggesting the need to evaluate them for APS. METHODS: We retrospectively reviewed patients with PE and APS (n=24) and those with unprovoked PE with aPL negative (n=44), evaluated 2006–2016 at the Asan Medical Center. We compared patient demographics, clinical manifestations, laboratory findings, and radiological findings between the groups. RESULTS: On multivariate logistic regression analysis, two models of independent risk factors for APS-PE were suggested. Model I included hemoptysis (odds ratio [OR], 12.897; 95% confidence interval [CI], 1.025–162.343), low PE severity index (OR, 0.948; 95% CI, 0.917–0.979), and activated partial thromboplastin time (aPTT; OR, 1.166; 95% CI, 1.040–1.307). Model II included age (OR, 0.930; 95% CI, 0.893–0.969) and aPTT (OR, 1.104; 95% CI, 1.000–1.217). CONCLUSION: We conclude that patients with first unprovoked PE with hemoptysis and are age <40; have a low pulmonary embolism severity index, especially in risk class I–II; and/or prolonged aPTT (above 75th percentile of the reference interval), should be suspected of having APS, and undergo laboratory testing for aPL.


Subject(s)
Antibodies, Antiphospholipid , Antiphospholipid Syndrome , Demography , Hemoptysis , Humans , Logistic Models , Partial Thromboplastin Time , Phenotype , Pregnancy , Pulmonary Embolism , Recurrence , Retrospective Studies , Risk Factors , Thrombophilia , Thrombosis
16.
Article in English | WPRIM | ID: wpr-719458

ABSTRACT

The catastrophic variant of antiphospholipid syndrome (APS) is a very rare and life-threatening condition of APS. This condition is characterized by thrombosis in multiple organs within a short period of time in the presence of positive antiphospholipid antibodies (aPL). Over the past few decades, considerable progress has been made in the treatment of patients with catastrophic APS; however, the mortality rate still remains very high. Although some cases of rituximab treatment in patients with catastrophic APS have been reported, there is no clear treatment protocol. A 14-year-old girl with systemic lupus erythematosus was diagnosed with catastrophic APS. She received several medications: corticosteroids, intravenous immunoglobulin, and plasmapheresis with anticoagulants. Unfortunately, she did not improve, and rituximab was started with four courses. After the rituximab treatment, she did not experience further thrombotic events during the follow up. This paper reports a pediatric case of catastrophic APS treated successfully with rituximab in Korea.


Subject(s)
Adolescent , Adrenal Cortex Hormones , Antibodies, Antiphospholipid , Anticoagulants , Antiphospholipid Syndrome , Clinical Protocols , Female , Follow-Up Studies , Humans , Immunoglobulins , Korea , Lupus Erythematosus, Systemic , Mortality , Plasmapheresis , Rituximab , Thrombosis
17.
Article in English | WPRIM | ID: wpr-741869

ABSTRACT

Antiphospholipid antibodies may be produced in cases involving autoimmune diseases and can sometimes be caused by infections, such as Mycoplasma pneumoniae infection. However, antiphospholipid antibodies causing thrombosis associated with M. pneumoniae pneumonia in children have rarely been reported. We report a case of an 8-year-old boy with M. pneumoniae pneumonia with antiphospholipid antibodies, complicated by brachial artery thrombosis. He was found to have antiphospholipid antibodies and low protein S levels. The brachial artery thrombus was removed via thrombectomy. The titers of antiphospholipid antibodies turned normal within 5 months. This is a rare case of M. pneumoniae infection with brachial artery thrombosis associated with transient antiphospholipid antibodies.


Subject(s)
Antibodies, Antiphospholipid , Autoimmune Diseases , Brachial Artery , Child , Humans , Male , Mycoplasma pneumoniae , Mycoplasma , Pneumonia , Pneumonia, Mycoplasma , Protein S , Thrombectomy , Thrombosis
18.
Adv Rheumatol ; 59: 52, 2019. tab, graf
Article in English | LILACS | ID: biblio-1088611

ABSTRACT

Abstract Introduction: Antiphospholipid antibodies (aPL) are described in individuals with leprosy without the clinical features of antiphospholipid antibody syndrome (APS), a condition involving thromboembolic phenomena. We have described the persistence of these antibodies for over 5 years in patients with leprosy after specific treatment. Objectives: To determine whether epidemiological, clinical and immunological factors played a role in the longterm persistence of aPL antibodies in leprosy patients after multidrug therapy (MDT) had finished. Methods: The study sample consisted of 38 patients with a diagnosis of leprosy being followed up at the Dermatology and Venereology Outpatient Department at the Alfredo da Matta Foundation (FUAM) in Manaus, AM. ELISA was used to detect anticardiolipin (aCL) and anti-β2 glycoprotein I (anti-β2GPI) antibodies. Patients were reassessed on average of 5 years after specific treatment for the disease (MDT) had been completed. Results: Persistence of aPL antibodies among the 38 leprosy patients was 84% (32/38), and all had the IgM isotype. Mean age was 48.1 ± 15.9 years, and 23 (72.0%) were male. The lepromatous form (LL) of leprosy was the most common (n = 16, 50%). Reactional episodes were observed in three patients (9.4%). Eighteen (47.37%) were still taking medication (prednisone and/or thalidomide). Mean IgM levels were 64 U/mL for aCL and 62 U/mL for anti-β2GPI. In the multivariate binary logistic regression the following variables showed a significant association: age (p = 0.045, OR = 0.91 and CI 95% 0.82-0.98), LL clinical presention (p = 0.034; OR = 0.02 and CI 95% = 0.0-0.76) and bacterial index (p = 0.044; OR = 2.74 and CI 95% = 1.03-7.33). We did not find association between prednisone or thalidomide doses and positivity for aPL (p = 0.504 and p = 0.670, respectively). No differences in the variables vascular thrombosis, pregnancy morbidity, diabetes, smoking and alcoholism were found between aPL-positive and aPL-negative patients. Conclusion: Persistence of positivity for aPL antibodies was influenced by age, clinical presentation and bacterial index. However, further studies are needed to elucidate the reason for this persistence, the role played by aPL antibodies in the disease and the B cell lineages responsible for generation of these antibodies.


Subject(s)
Humans , Leprosy/pathology , Enzyme-Linked Immunosorbent Assay/instrumentation , Antibodies, Antiphospholipid/analysis , Antibodies, Anticardiolipin/analysis , Drug Therapy, Combination/adverse effects , beta 2-Glycoprotein I/analysis
20.
Rev. colomb. reumatol ; 25(2): 85-91, abr.-jun. 2018. tab
Article in Spanish | LILACS | ID: biblio-990932

ABSTRACT

RESUMEN El daño irreversible de órgano es predictor de morbilidad, mortalidad, mayor acúmulo de daño y mala calidad de vida en los pacientes con lupus eritematoso sistémico. Objetivos: Caracterizar el daño y los factores que mejor lo explican, en una población de pacientes colombianos con lupus eritematoso sistémico. Métodos: Estudio retrospectivo de seguimiento a una cohorte. El daño se midió con el SLICC/ACR (índice de Systemic Lupus International Collaborating Clinics y del American College of Rheumatology) y la actividad de la enfermedad por SELENA SLEDAI. La caracterización del daño se hizo mediante estadística descriptiva, los factores asociados con el desenlace se evaluaron con Chi2 de Pearson o Fisher, t de Student o U de Mann-Whitney; la proporción de pacientes que acumularon daño se evaluó con el test de Friedman y el puntaje acumulado con el test de Wilcoxon. La determinación de los factores asociados independientemente con el desenlace se hizo con una regresión logística. Resultados: Se incluyeron 161 pacientes con diagnóstico de novo y seguimiento mínimo de un año; el 28,9% sufrió daño. Los dominios más representados fueron el neuropsiquiátrico, renal y vascular. Los anticuerpos antifosfolípido, las dosis promedio de prednisolona mayores a 12,5 mg/día y presentar 2 o más recaídas se asociaron independientemente al daño orgánico. Conclusiones: Los anticuerpos antifosfolípido, la dosis de esteroides y la frecuencia de recaídas se asocian al daño orgánico en una población colombiana de pacientes con lupus eritematoso sistémico.


ABSTRACT Irreversible organ damage is a predictive factor of morbidity, mortality, increased accumulation of damage, and poor quality of life in patients with systemic lupus erythematosus. Objectives: To describe the damage, and the factors that best explain it, in a population of Colombian patients. Methods: A retrospective follow-up study of a patient cohort. The damage was measured using the Systemic Lupus International Collaborating Clinics (SLICC) and the American College of Rheumatology (ACR) index, and disease activity by SELENA SLEDAI. Descriptive statistics were used to describe the damage. The factors associated with the outcome were evaluated with Pearson's or Fisher's Chi2, Student's t or Mann-Whitney's U. The proportion of patients that accumulated damage was evaluated with the Friedman test, and the cumulative score with the Wilcoxon test. The determination of the factors independently associated with the outcome was performed using logistic regression. Results: A total of 161 patients with recent diagnosis, and followed for one year or more, were included, 28.9% of whom had suffered damage. The most represented domains were neuropsychiatric, renal and vascular. Anti-phospholipid antibodies, mean doses of prednisolone greater than 12.5 mg/day, and suffering 2 or more relapses were independently associated with organ damage. Conclusions: Anti-phospholipid antibodies, steroid doses and frequency of relapses are associated with organ damage in a Colombian population of patients with systemic lupus erythematosus.


Subject(s)
Humans , Adolescent , Adult , Middle Aged , Aged , Aged, 80 and over , Lupus Erythematosus, Systemic , Quality of Life , Prednisolone , Antibodies, Antiphospholipid , Lupus Vasculitis, Central Nervous System , Antibodies
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