Epidemiological, clinical and immune factors that influence the persistence of antiphospholipid antibodies in leprosy

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.

Cirugía cardiaca en pacientes con lupus eritematoso sistémico: características clínicas y desenlaces / Cardiac surgery in systemic lupus erythematosus patients: Clinical characteristics and outcomes

Objetivos: Estudiar las características clínicas y desenlaces de los pacientes con lupus eritematoso sistémico (LES) intervenidos de cirugía cardiaca. Métodos: Se realizó un estudio retrospectivo de 30 pacientes con LES y cirugía cardiaca en un solo centro. Se registraron comorbilidades, características demográficas, clínicas, serológicas, riesgo cardiovascular, tratamiento, tipo de cirugía, complicaciones postoperatorias, mortalidad e histología. Resultados: La duración de LES al momento de la cirugía fue de 2 años. El procedimiento más frecuente fue recambio valvular (53%), seguido de ventana pericárdica (37%). Al menos una complicación postoperatoria se presentó en el 63% (principalmente infecciones). Un pinzamiento aórtico≥76 min se asoció con al menos una complicación (OR 6,4; IC 95% 1,1-35,4, p=0,03). La mortalidad temprana ocurrió en 5 pacientes (17%) y tardía en 3 (10%); siendo las causas principales sepsis e insuficiencia cardiaca. La actividad de la enfermedad se asoció a la realización de ventana pericárdica (OR 12,6; IC 95% 1,9-79; p=0,007), presencia de linfopenia≤1.200 (OR 10,1; IC 95% 1,05-97; p=0,04), edad≤30 años (OR 7,7; IC 95% 1,2-46,3; p=0,02) y NYHA clase III (OR 7,0; IC 95% 1,1-42, p=0,03). El desarrollo de infección postoperatoria se asoció con estancia hospitalaria≥2 semanas (OR 54,9; IC 95% 5,0-602,1; p=0,001), estancia en UCI≥10 días (OR 20; IC 95% 1,6-171,7, p=0,01), duración de ventilación mecánica ≥ 5 días (OR 16,9, IC 95% 1,5-171,7, p = 0,01) y PSAP≥50mmHg (OR 7,8; IC 95% 1,4-41,2; p=0,01). Conclusiones: La cirugía cardiaca en LES se asocia a alta morbimortalidad

Objectives: To study the clinical characteristics and outcomes in systemic lupus erythematosus (SLE) patients who underwent cardiac surgery. Methods: Retrospective analysis of 30 SLE patients who underwent cardiac surgery at a single center. Demographics, comorbidities, clinical and serologic characteristics, cardiovascular risk scores and treatment were recorded. Type of surgery, postoperative complications, mortality and histology were analyzed. Results: Disease duration at surgery was 2 years. Valve replacement was the procedure most frequently performed (53%), followed by pericardial window (37%). At least one postoperative complication developed in 63% (mainly infections). An aortic cross-clamp time≥76minutes was associated with at least one postoperative complication (OR 6.4, 95% CI 1.1-35.4, p=.03). Early death occurred in 5 patients (17%) and late in 3 (10%); main causes were sepsis and heart failure. Disease activity was associated with pericardial window (OR 12.6, 95% CI 1.9-79, p=.007); lymphopenia≤1.200 (OR 10.1, 95% CI 1.05-97, p=.04); age≤30 years (OR 7.7, 95% CI 1.2-46.3, p=.02); and New York Heart Association class III (OR 7.0, 95% CI 1.1-42, p=.03). Postoperative infection was associated with length of hospital stay≥2 weeks (OR 54.9, 95% CI 5.0-602.1, p=.001); intensive care unit stay≥10 days (OR 20, 95% CI 1.6-171.7, p=.01); duration of mechanical ventilation≥5 days (OR 16.9, 95% CI 1.5-171.7, p=.01); and pulmonary artery systolic pressure≥50mmHg (OR 7.8, 95% CI 1.4-41.2, p=.01). Conclusions: Cardiac surgery in SLE confers high morbidity and mortality. SLE-specific preoperative risk scores should be designed to identify prognostic factors

Controversias del síndrome de anticuerpos antifosfolipídicos en obstetricia / Controversies concerning the antiphospholipid syndrome in obstetrics

El síndrome de anticuerpos antifosfolipídicos es una enfermedad autoinmune no inflamatoria, caracterizada por eventos trombóticos recurrentes y/o complicaciones obstétricas, asociados a la presencia de anticuerpos antifosfolipídicos circulantes: anticuerpos anticardiolipina, anti-β2 glucoproteína-i y/o anticoagulante lúpico. Los anticuerpos antifosfolipídicos son un grupo heterogéneo de autoanticuerpos asociados con morbilidad obstétrica, como pérdida gestacional recurrente, muerte fetal, parto pretérmino asociado a insuficiencia placentaria como enfermedad hipertensiva del embarazo y/o restricción del crecimiento intrauterino. Los procesos fisiopatológicos relacionados con la morbilidad obstétrica no se han comprendido del todo, involucrándose múltiples eventos inmunológicos, entre ellos los inflamatorios, la activación del complemento, el desbalance de los factores angiogénicos y, en alguna proporción de los casos, se ha demostrado trombosis e infarto. Debido a la controversia en los criterios clínicos y de laboratorio, así como a la repercusión en la mejora de los resultados perinatales en pacientes que inician tratamiento, decidimos llevar a cabo esta revisión sobre los conceptos de síndrome de anticuerpos antifosfolipídico relacionado con complicaciones obstétricas y síndrome de anticuerpos antifosfolipídico seronegativo, así como su manejo en obstetricia (AU)

Antiphospholipid antibody syndrome is a non-inflammatory autoimmune disease characterized by recurrent thrombotic events and/or obstetric complications associated with the presence of circulating antiphospholipid antibodies (anticardiolipin antibodies, anti-β2 glycoprotein-i antibodies, and/or lupus anticoagulant. Antiphospholipid antibodies are a heterogeneous group of autoantibodies associated with recurrent miscarriage, stillbirth, fetal growth restriction and premature birth. The diversity of the features of the proposed placental antiphospholipid antibodies fingerprint suggests that several disease processes may occur in the placentae of women with antiphospholipid antibody syndrome in the form of immune responses: inflammatory events, complement activation, angiogenic imbalance and, less commonly, thrombosis and infarction. Because of the disparity between clinical and laboratory criteria, and the impact on perinatal outcome in patients starting treatment, we reviewed the aspects of antiphospholipid antibody syndrome related to obstetric complications and seronegative antiphospholipid antibody syndrome, and their treatment in obstetrics (AU)

Diagnóstico en el síndrome antifosfolipídico: desde una perspectiva histórica a la aparición de nuevos autoanticuerpos / Diagnosis of antiphospholipid syndrome: From an historical perspective to the emergence of new autoantibodies

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Food anaphylaxis in antiphospholipid syndrome and thrombosis

Background: We have observed that some cases of food anaphylaxis were followed by severe thrombosis associated to anticardiolip in antibodies. Food anaphylaxis associated with antiphospholipid syndrome has seldom been published. Objective: The aims were: 1) to test anticardiolipin antibodies in an important number of patients with anaphylaxis due to vegetal foods and their relationship with possible thrombosis; and 2) to study seed and fruit hypersensitivity in patients with previous thrombotic events associated with antiphospholipid antibodies (aCL). Methods: We included 30 patients diagnosed of thrombosis associated with a CL, 52 patients who suffered from anaphylaxis due to seeds or fruits, and 120 control patients. Haematological, cardiopulmonary vascular and rheumatologic studies had been performed as needed. In vivo and in vitro allergy tests with a large battery of vegetal allergens were carried out in all the patients. Measurement of IgG a CL antibodies and specific IgE to vegetal food was done by ELISA and CAP-FEIA (Phadia). Immunodetection and inhibitions with lipoproteins belonging to seedswere performed. Results: Seventy-five percent of the patients diagnosed as having antiphospholipid primary syndrome had specific IgE against different proteins from different vegetable allergens, most of them seeds, and clearly against lipoproteins that were also recognised by the patients with food anaphylaxis but not by the control cases. Among the patients with anaphylaxis, 28% had anticardiolipin antibodies and 17.3% thrombosis. Conclusion: Our study suggests that seed lipoproteins which cause severe food anaphylaxis might have a potential role in the antiphospholipid syndrome and related thrombosis (AU)

Auto-anticorpos anti-β2-glicoproteína I e síndrome metabólica / Anti-beta2-glycoprotein I autoantibodies and metabolic syndrome / Anticuerpos anti-β2-glicoproteína I y síndrome metabólico

FUNDAMENTO: A síndrome metabólica (SM) é uma entidade pró-aterogênica. Autoanticorpos tais como β2-glicoproteína I (β2-gpI) podem influenciar o aparecimento de ateromas. Estudos anteriores confirmaram uma associação entre anticorpos IgA anti-β2-gpI e isquemia cerebral, infarto do miocárdio, doença arterial periférica e doença da carótida. OBJETIVO: O objetivo desse estudo de caso-controle foi avaliar uma possível associação entre anticorpos anti-β2-gpI e anticardiolipina (aCL) com SM não-complicada. MÉTODOS: Pacientes com SM sem histórico de eventos vasculares e indivíduos-controle, consistindo em pacientes da Enfermaria de Ortopedia admitidos devido a doenças musculoesqueléticas foram incluídos no estudo. Idade, sexo, etnia, histórico de hipertensão, tabagismo, hipercolesterolemia e diabetes mellitus foram avaliados como fatores de risco em ambos os grupos. Anticorpos IgG, IgM, e IgA anti-β2-gpI e aCL foram detectados através de imunoensaios enzimáticos. RESULTADOS: Um total de 68 pacientes com SM e 82 controles foram estudados. Os pacientes com SM tinham média de idade superior à dos controles (P = 0,001), enquanto homens (P = 0,003; OR 0,31; IC95 por cento: 0,15-0,16) e etnia caucasiana (P = 0,004; OR 0,25; IC95 por cento:0,10-0,60) eram predominantes nos controles. Histórico de hipertensão, hipercolesterolemia e diabetes mellitus foi mais prevalente nos pacientes com SM do que nos controles (P < 0.05). A frequência de anticorpos aCL (todos os isotipos) e do IgG e IgM anti-β2 gpI não diferiu de forma significante nos pacientes com SM e controles. Anticorpos IgA anti-β2-gpI foram significantemente mais frequentes nos pacientes com SM (42,2 por cento) do que nos controles (10,9 por cento) (P < 0,001). O OR ajustado para anticorpos IgA anti-β2-gpI foi 3,60 (IC95 por cento: 1,55-8,37; P = 0,003). CONCLUSÃO: O presente estudo mostra que níveis elevados de autoanticorpos IgA para β2-gpI podem estar independentemente associados com SM.

BACKGROUND: The metabolic syndrome (MetS) is a proatherogenic entity. Autoantibodies to phospholipid cofactors such as beta2-glycoprotein I (beta2-gpI) can influence atheroma appearance. Previous studies confirmed an association of IgA anti-beta2-gpI antibodies with cerebral ischemia, myocardial infarction, peripheral artery disease and carotid disease. OBJECTIVE: This case-control study evaluates a possible association of anti-beta2-gpI and anticardiolipin (aCL) antibodies with non-complicated MetS. METHODS: Cases comprised patients with MetS without history of vascular events; controls included individuals from the Orthopedic Infirmary admitted due to musculoskeletal disorders. Age, sex, race, history of hypertension, smoking, hypercholesterolemia and diabetes mellitus were evaluated as risk factors in both groups. IgG, IgM, and IgA anti-beta2-gpI and aCL antibodies were detected by enzymatic immunoassay. RESULTS: Sixty-eight patients with MetS and 82 controls were studied. Patients with MetS showed mean age higher than controls (P = 0.001), while males (P = 0.003; OR 0.31; 95 percentCI 0.15-0.16) and Caucasian ethnicity (P = 0.004; OR 0.25; 95 percentCI 0.10-0.60) predominated in controls. History of hypertension, hypercholesterolemia and diabetes mellitus were more prevalent in cases than in controls (P < 0.05). The frequency of aCL antibodies (all isotypes) and of IgG and IgM anti-beta2 gpI did not significantly differ in cases and controls. IgA anti-beta2-gpI antibodies were significantly more frequent in MetS patients (42.2 percent) than controls (10.9 percent) (P < 0.001). The adjusted OR for IgA anti-beta2-gpI antibodies was 3.60 (95 percentCI 1.55-8.37; P = 0.003). CONCLUSION: The current study shows that elevated levels of IgA autoantibodies to β2-gpI might be independently associated to MetS.

FUNDAMENTO: El síndrome metabólico (SM) es una entidad pro-aterogénica. Autoanticuerpos tales como β2-glicoproteína I (β2-GPI) pueden influir en la aparición de ateromas. Estudios previos han confirmado una asociación entre anticuerpos IgA anti-β2-GPI y la isquemia cerebral, infarto de miocardio, enfermedad arterial periférica y enfermedad carotidea. OBJETIVO: El objetivo de este estudio de caso-control fue evaluar una posible asociación entre los anticuerpos anti-β2-GPI y anticardiolipina (aCL) con SM complicada. MÉTODOS: Se incluyeron en el estudio a los pacientes con SM sin antecedentes de eventos vasculares y los sujetos control, que consiste en pacientes de la Internación de Ortopedia ingresados debido a enfermedades musculoesqueléticas. Edad, sexo, origen étnico caucásico, antecedentes de hipertensión, tabaquismo, hipercolesterolemia y diabetes mellitus fueron evaluados como factores de riesgo en ambos grupos. Anticuerpos IgG, IgM, e IgA anti-β2-GPI y aCL se detectaron a través de inmunoensayos enzimáticos. RESULTADOS: Un total de 68 pacientes con SM y 82 controles se estudiaron. Los pacientes con SM tenían un promedio de edad superior de los controles (p = 0,001), mientras que los hombres (p = 0,003; OR 0,31; IC95 por ciento: 0,15-0,16) y origen étnico caucásica (p = 0,004; OR 0,25; IC95 por ciento:0,10-0,60) eran predominantes en los controles. Historia de hipertensión, hipercolesterolemia y diabetes mellitus fue más prevalente en los pacientes con SM que en los controles (p < 0,05). La frecuencia de anticuerpos aCL (todos los isotipos) y del IgG e IgM anti-β2 gpI no se distinguió de forma significante en los pacientes con SM y controles. Anticuerpos IgA anti-β2-gpI fueron significantemente más frecuentes en los pacientes con SM (42,2 por ciento) que en los controles (10,9 por ciento) (p < 0,001). El OR ajustado para anticuerpos IgA anti-β2-gpI fue 3,60 (IC95 por ciento: 1,55 a 8,37, p = 0,003). CONCLUSIÓN: El presente estudio muestra que los niveles elevados de autoanticuerpos IgA para β2-gpI pueden estar independientemente asociados con la SM.

Características clínicas y anticuerpos antifosfolipídicos en líquido cefalorraquídeo y suero en una muestra de pacientes con esclerosis múltiple en México / Clinical characteristics and presence of antiphospholipid antibodies cerebrospinal fluid and serum of in a series of patients with multiple sclerosis in Mexico

Introducción: El diagnóstico diferencial de la esclerosis múltiple (EM) incluye una gran variedad de enfermedades autoinmunitarias (lupus eritematoso sistémico, síndrome de Sjögren, síndrome antifosfolipídico, etc.). Los anticuerpos antifosfolipídicos (AAFL) en el suero de pacientes con EM se encuentran en cifras tan bajas como en el 10% y tan altas como en el 88% de los casos, pero su significación en la patogenia de la enfermedad o su utilidad diagnóstica aún no se han establecido. El objetivo fue conocer las características clínicas y demográficas de una muestra de pacientes con EM del Hospital General de México (HGM), y determinar presencia y frecuencia de anticuerpos antifosfolipídicos en líquido cefalorraquídeo y suero de estos pacientes. Pacientes y métodos: Se realizó un estudio prospectivo con pacientes captados en la consulta externa de neurología del HGM, con diagnóstico definido de EM a lo largo de 1 año. Se analizaron sus características clinicodemográficas y se determinó VDRL y anticuerpos anticomplejo cardiolipina-β2GP-1 en LCR y suero. Resultados: Se incluyó a 12 pacientes, con predominio del sexo femenino (58%). El cuadro clínico predominante fue la neuritis óptica (66,6%) seguida de la afección medular (58%). La mayoría de los pacientes fueron ambulatorios (< 4 puntos EDSS). Los títulos de los autoanticuerpos fueron negativos en todos los casos, tanto en LCR como en sangre. Conclusiones: Las características clinicodemográficas en la muestra de este estudio son semejantes a las comunicadas con anterioridad y las concentraciones de anticardiolipina-β2GP-1 fueron negativas, lo cual puede indicar que hay diversas variables clínicas y demográficas que influyen en su detección (AU)

Introduction: The differential diagnosis of multiple sclerosis (MS) includes a wide variety of autoimmune diseases (systemic lupus erythematosus, Sjögren syndrome, antiphospholipid syndrome, etc.). The presence of antiphospholipid antibodies (APLA) in serum of MS patients has been reported to be as low as 10%, or sometimes as high as 88% of the cases, although its significance in the pathogenesis of the disease, or its diagnostic usefulness is still unknown. The goal of this study was to describe the clinic and demographic characteristics of a sample of patients with MS from the Hospital General de México (HGM), as well as to determine the presence and frequency of APLA in cerebrospinal fluid (CSF) and serum samples of these patients. Patients and methods: A prospective study with patients from the Neurology Department at the HGM was performed. These patients were diagnosed with MS over a one-year period. Clinical and demographic characteristics were compiled. VDRL and anti-cardiolipin-β2GP-1 complex antibodies were analyzed in CSF and serum samples. Results: Twelve patients were included in the study, the majority females (58%). The predominant clinic feature was optic neuritis (66.6%) followed by medullary involvement (58%). Most of patients were ambulatory (< 4 EDSS points). Auto-antibody levels were found in negative ranges in all cases, both in CSF and serum. Conclusions: The clinical-demographic characteristics in patients studied in this work were similar to those previously reported, and the levels of anti-cardiolipin-β2GP-1 were negative, thus indicating the existence of different clinical and demographic variables influencing their detection (AU)

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.

Encefalopatía subaguda con respuesta a esteroides sin evidencia de enfermedad autoinmune tiroidea: un caso de meningoencefalitis autoinmune no vasculítica / Sub-acute encephalopathy that responds to sterorids without any evidence of autoinmune thyroid disease a case of non-vasculitic autoimmune nm

Aim. To report a case of sub-acute encephalopathy with all the extension study negative and with response to steroid therapy. Case report. The study involves a 22-year-old female with no relevant past history who presented symptoms of sub-acute encephalopathy consisting in behavioural disorders, generalised seizures and bradypsychia, which gradually progressed to a state of low-level consciousness. While she was in hospital all kinds of diagnostic tests were conducted, the results of which were either normal or negative; the electroencephalogram was repeatedly abnormal and detection of protein 14-3-3 in cerebrospinal fluid was positive. Empirical corticoid therapy was begun with clinical and electrophysiological improvements and the patient recovered completely without any sequelae. Conclusions. With no evidence of autoimmune thyroid disease, although non-specific autoimmunity was present, the patient was diagnosed as having non-vasculitic autoimmune meningoencephalitis (AU)

Objetivo. Presentar un caso de encefalopatía subaguda con todo el estudio de extensión negativo y con respuesta a terapia esteroidea. Caso clínico. Mujer de 22 años sin antecedentes de interés que presentó un cuadro de encefalopatía subaguda consistente en alteraciones del comportamiento, crisis generalizadas y bradipsiquia, que evolucionó progresivamente a un estado de bajo nivel de conciencia. Durante su ingreso se realizaron todo tipo de pruebas diagnósticas, que fueron normales o negativas; el electroencefalograma fue repetidamente anormal y se detectó positividad de la proteína 14-3-3 en el líquido cefalorraquídeo. Se inició corticoterapia empírica con mejoría clínica y electrofisiológica, y recuperación ad integrum sin secuelas. Conclusiones. Sin la evidencia de enfermedad autoinmune tiroidea, aunque con presencia de autoinmunidad no específica, se diagnosticó a la paciente meningoencefalitis autoinmune no vasculítica (AU)

Usefulness of Silica Clotting Time for Detection of Lupus Anticoagulants / 대한진단검사의학회지

BACKGROUND: The presence of lupus anticoagulants (LA) is a strong risk factor for thrombosis in antiphospholipid syndrome. We investigated the usefulness of addition of silica clotting time (SCT) to the pre-existing dilute Russell's viper venom test (dRVVT) for detection of LA. Also, we analyzed differences in the thrombotic features and the characteristics of antiphospholipid antibodies between dRVVT and SCT. METHODS: A total of 167 patients positive for LA or anti-cardiolipin (anti-CL) antibody and 76 healthy controls were enrolled. The dRVVT and SCT were used for detection of LA. Anti-CL, anti-beta2-glycoprotein I (anti-beta2 GPI) and anti-prothrombin (anti-PT) antibodies were measured using commercial ELISA kits. RESULTS: In detection of thrombosis, the sensitivity of the combined test of SCT and dRVVT was 56.4%, which was higher than that of dRVVT alone (46.2%) or SCT alone (23.1%). The specificity of the combined test (80.9%) was comparable to that of dRVVT (81.9%). Also, odds ratio for predicting thrombosis was higher in the combined test than in dRVVT or SCT alone. When normalized LA ratio of the two tests was compared, the group of patients with higher ratio of SCT showed significantly higher prevalence of recurrent abortion and higher positivity of IgG types of anti-CL, anti-beta2 GPI and anti-PT than the group with higher ratio of dRVVT. CONCLUSIONS: Addition of SCT to dRVVT can improve the detection sensitivity of thrombosis in LA test. And the high normalized LA ratio of SCT may be a useful parameter for detection of recurrent abortion.

Anticuerpos antifosfolipídicos, síndrome antifosfolipídico y hematomas suprarrenales / No disponible

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Retirada de la anticoagulación en el síndrome antifosfolípido primario cuando se negativizan los anticuerpos anticardiolipina / Anticoagulation treatment withdrawal in primary antiphospholipid syndrome when anticardiolipin antibodies become negative

Objetivos. La anticoagulación a largo plazo es el tratamiento recomendado en el síndrome antifosfolípido primario (SAF) tras un evento trombótico. Sin embargo, no se conoce con precisión cómo se deben manejar los enfermos que permanecen estables durante años, y cuos anticuerpos antifosfolípido (AAF) descienden hasta negativizarse. El objetivo de este trabajo es valorar la evolución del SAF primario, tras la suspensión de la anticoagulación, en este grupo de pacientes. Población y métodos. Se incluyeron diez pacientes diagnosticados de SAF primario que habían sufrido una trombosis venosa profunda en las extremidades (9) y en la aorta(1). Tras un período mínimo de 12 meses de anticoagulación se retiró el tratamiento a los pacientes que presentaron AAF negativos a lo largo del seguimiento en dos determinaciones consecutivas. Resultados. Los AAF se negativizaron de forma persistente en seis enfermos (60%). Cuatro presentaban factores de riesgo transitorios (2 gestación, 1 inmovilización, 2 anticonceptivos), y tras la retirada de la coagulación ninguno desarrolló nuevos eventos trombóticos. Conclusiones. Nuestros datos sugieren que la retirada del tratamiento anticoagulante puede ser adecuada en un grupo de pacientes con SAF primario que, tras un periodo prolongado de anticoagulación, negativizan los AAF de forma estable. Esta actitud sería más segura si el evento trombótico era venoso y ocurrió durante la presencia de un factor de riesgo transitorio. No obstante, son necesarios estudios más amplios que confirmen estos hallazgos

Objectives. The recommended treatment in patients with primary antiphospholipid syndrome (APS) after a thrombotic event is long-term anticoagulation. However, it is still not exactly known how to manage patients who remain stable for years and whose antiphospholipid antibodies (APA) decrease until becoming negative. This study aims to assess the course of the primary APS in a group of patients after anticoagulation therapy is discontinued. Patients and method. Ten patients with primary APS who had developed deep venous thrombosis in the limbs (9) or in the aorta (1) were included. After a minimum period of 12 months of anticoagulation therapy, this was discontinued if the patients were negative APA during the follow-up in two consecutive measurements. Results. Six patients (60%) developed persistent negative APA. Four had transient risk factors (2 pregnant, 1 immobilization, 2 oral contraceptives). No new thrombosis episode was observed after a follow-up period of 21 ± 4.9 months. Conclusions. Our data suggest that anticoagulation can be discontinued in those patients with primary APS and persistent negative APA, especially if the thrombotic event was venous and occurred in association with a transient risk factor, such as immobilization or pregnancy. Extensive studies are required to confirm these results

Varón de 46 años con hematoma suprarrenal bilateral / Bilateral suprarenal hematoma in a 46-year-old male

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Monitorización inmunológica en mujeres con aborto recurrente / Immunological monitoring in women with recurrent spontaneous abortions

Para evaluar la prevalencia de alteraciones inmunológicas en pacientes con aborto espontáneo recurrente realizamos un estudio retrospectivo transversal. Las mujeres estudiadas habían sido evaluadas previamente mediante cariotipo de pareja y ecografía para investigar alteración anatómica. La monitorización inmunológica de rutina incluyó anticuerpos antifosfolípidos (in = 333), anticuerpos anticardiolipina (ACA), anticoagulante lúpico], anticuerpos anti-beta-2 glucoproteína-I (n = 139) [anticuerpos antinucleares (n = 333), anticuerpos antitiroideos (n = 180) y evaluación del porcentaje de células natural killer (NK) en sangre periférica (n = 189) en 333 pacientes consecutivas con 2 o más (intervalo 2-12) abortos espontáneos recurrentes. Se detectaron anticuerpos antifosfolípidos (ACA a valor moderado-alto o anticoagulante lúpico) en un 15,9% de pacientes. Un 6% de las pacientes tuvo anticuerpos antinucleares a título 1:160 o mayor. Se observó un porcentaje elevado de linfocitos CD56+/CD16+CD3­ (> 18%) en el 11,1% de las pacientes. Se documentó positividad de anticuerpos antitiroideos en el 4% de las mujeres. Globalmente, un 28,5% de las mujeres tuvo al menos una alteración inmunológica de las estudiadas. En un estudio realizado en mujeres con aborto espontáneo recurrente se observaron alteraciones autoinmunitarias y aloinmunitarias que involucran distintos autoanticuerpos y células NK de sangre periférica (AU)

We performed a retrospective cross-sectional study to ascertain the prevalence of immunological abnormalities in women with recurrent spontaneous abortions (RSA). The women had previously undergone karyotyping of the couple and ultrasonography to investigate anatomic uterine anomalies before immunological study. Routine immunologic monitoring for circulating antiphospholipid antibodies (aPL, n=333) [anticardiolipin antibodies (aCL), lupus anticoagulant (LA)], anti-beta-2 glycoprotein-I antibodies (n=139), antinuclear antibodies (n=333), antithyroid antibodies (n=180) and percentages of natural killer (NK) cells in peripheral blood (n=189) was carried out in 333 consecutive patients with two or more (range 2-12) RSA. aPL positivity (mean-high value aCL or LA) was 15.9%. The frequency of positive antinuclear antibody tests at a titer of 1:160 or higher was 6%. Elevated levels of CD56+/CD16+CD32 lymphocytes (>18%) were observed in 11.1% of the patients studied. Anti-thyroid antibody positivity was found in 4% of women. Overall, 28.5% of women had at least one immunological abnormality. Women with RSA had significant autoimmune and alloimmune alterations involving functionally distinct autoantibodies and peripheral blood NK cells (AU)

Síndrome antifosfolípido primario infantil / Primary child antiphospholipid syndrome

El síndrome antifosfolípido es una patología autoinmune de origen desconocido que se caracteriza por la aparición de trombosis tanto arteriales como venosas, abortos de repetición y trombocitopenia, entre otras manifestaciones, junto a la presencia de anticuerpos antifosfolípidos. El síndrome es definido como primario cuando aparece en pacientes que no padecen lupus eritematoso sistémico u otras conectivopatías. Los anticuerpos hallados más frecuentemente son el anticoagulante lúpico y los anticuerpos anticardiolipina. Presentamos el caso de una niña que sufrió una trombosis venosa del seno longitudinal superior como consecuencia de un síndrome antifosfolípido primario describiendo su sintomatología, evolución y el tratamiento realizado. Hacemos una revisión bibliográfica sobre este síndrome resaltando el significado clínico y pronóstico de la presencia de anticuerpos antifosfolípidos en niños

The antiphospholipid syndrome is an autoimmune disorder whose etiology is unknown. It is characterized by arterial and venous thrombosis, recurrent pregnancy loss and thrombocytopenia, among other symptoms, together with antiphospholipid antibodies. The syndrome is classified as primary when it occurs in the absence of systemic lupus erythematosus or other connective tissue diseases. The two most frequent antibodies are the lupus anticoagulant and the anticardiolipin antibody. We present a case of a sagittal sinus venous thrombosis associated with primary antiphospholipid syndrome in a girl, describing its symptomatology, course and treatment applied. We made a bibliographical review on this syndrome, stressing the clinical significance and prognosis of the presence of antiphospholipid antibodies in children

Anticorpos anticardiolipina fixadores de complemento em pacientes com e sem história de trombose / The complement fixing activity of anticardiolipin antibodies in patients with without trombosis

Vários estudos têm sugerido a associação dos anticorpos anticardiolipina (aCL) com eventos trombóticos na síndrome do anticorpo antifosfolipídeo (SAF) porém, existe evidência que nem todos os pacientes aCL positivos apresentam episódios trombóticos / A strong link between anticardiolipin antibodies (aCL) and thrombosis events in antiphospholipid syndrome (APS) is suggested by many studies but there is evidence that not all aCL positive patients present thrombosis episodes...

Síndrome de anticuerpos antifosfolípidos / Antiphospholipid antibody syndrome

La vasculitis necrosante es una de las manifestaciones cutáneas del síndrome de anticuerpos antifosfolípidos, ya que dos terceras partes de los pacientes, que desarrollan este síndrome, presentan úlceras necróticas que pueden confundirse con úlceras de cualquier otra etiología. La aparición de estas úlceras está asociada con más frecuencia a la presencia de anticuerpos anticardiolipina que a la de anticoagulante lúpico en sangre. Se presenta el caso de una paciente de 52 años de edad con vasculitis necrosante asociada al síndrome de anticuerpos antifosfolípidos (AU)

Hipertensión arterial por displasia fibromuscular en dos hermanas / Hypertension for fibromuscular dysplasia in two sisters

Presentamos a dos hermanas con hipertensión arterial descubierta cuando se encontraban embarazadas y producida en ambos casos por displasia fibromuscular de las arterias renales. Comentamos la infrecuente incidencia familiar de la misma y sus posibles causas y revisamos los procedimientos diagnósticos y terapéuticos utilizados (AU)

Agrupacion diagnostica del sindrome antifosfolipidico con base en los niveles sericos de IgG anticardiolipina / Classificatory limits to diagnosis antiphospholipid syndrome (SAF)

Objetivo: proponer, mediante métodos matemáticos de agrupamiento, límites para la clasificación diagnóstica del síndrome antifosfolipídico (SAF), en pacientes con trombosis venosa o arterial, en relación con los niveles séricos de anticuerpos IgG anticardiolipina (anti-cLP), única variable continua capaz de discriminar el diagnóstico de SAF. Tipo de estudio: estudio descriptivo prospectivo. Lugar y tiempo de estudio: Unidades de Hematología y Reumatología de la Facultad de Medicina de la Universidad Nacional de Colombia con sede en el Hospital San Juan de Dios de Bogotá, entre el 5 de enero de 1998 y el 12 de julio de 2000. Material y métodos: se incluyeron todos los pacientes con edad igual o mayor a 17 años, con eventos incidentes que cumplieran con la definición operacional de evento oclusivo vascular, arterial o venoso. Fueron evaluados en total 42 pacientes, a quienes se les documentó edad, sexo, diagnóstico de lupus eritematoso sistémico y se les cuantificó en unidades GPL, mediante técnica de micro ELISA, los niveles séricos de anticuerpos IgG anti-cLP. Se realizó un análisis de conglomerados, combinando un análisis jerárquico aglomerante inicial con un análisis ulterior no jerárquico (de "K-medias"). La matriz de proximidad del análisis jerárquico aglomerante empleó el cálculo de distancias euclidianas para todos los pares de casos, con base en una única variable estandarizada ( IgG anti- cLP). La regla de agrupamiento utilizada fue el promedio entre grupos. Se emplearon la media, la mediana y los valores mínimos y máximos de la variable analizada, para describir las medidas de resumen que determinaron las diferencias entre los grupos. Resultados: la relación mujer: hombre del total de 42 pacientes incluidos, fue 3.7: 1. La interpretación del dendograma elaborado a partir de la matriz de los diferentes coeficientes de aglomeración, indicó que podrían ser tres los conglomerados a formar. Los valores mínimos y máximos de unidades GPL de IgG anti-cLP para cada uno de los tres conglomerados formados fueron: Grupo 1, 7.5 y 49.8; Grupo 2, 61.7 y 109; Grupo 3,174 y 190. Discusión: se propone el diagnóstico de SAF inequívoco en pacientes con trombosis vascular con niveles séricos de anticuerpos IgG anti-cLP iguales o superiores a 50 unidades GPL y considerar otros diagnósticos para títulos inferiores, o bien, complementar el estudio con pruebas para la detección de...

Retinal Vein Occlusion in Two Patients with Primary Antiphospholipid Syndrome

Primary antiphopholipid syndrome (APS) is a disease producing vascular thrombus with antiphospholipid antibody without association with autoimmune diseases as systemic lupus erythematosus. Retinal vein occlusion is a rare vascular manifestation in primary APS. We describe 2 cases of primary APS presenting with developing blurred vision. Each had central retinal vein occlusion and high titer of IgG anticardiolipin antibody.