Síndrome antifosfolípido (SAF): diferencias clínicas e inmunoserológicas entre SAF primario y secundario en una cohorte colombiana / Antiphospholipid syndrome (APS): Clinical and immunoserological differences between primary and secondary APS in a Colombian cohort

RESUMEN Introducción: El síndrome antifosfolípido (SAF) es una enfermedad autoinmune sistémica, caracterizada por trombosis recurrente, que puede afectar la circulación arterial y venosa. Objetivo: Analizar las diferencias inmunológicas y farmacológicas, así como los desenlaces clínicos de una cohorte de pacientes con SAF primario y secundario. Materiales y métodos: Estudio de corte transversal que incluyó 352 pacientes con diagnóstico de SAF atendidos entre los arios 2014 y 2018. Se analizaron variables sociodemográficas, clínicas e inmunológicas y se realizó un análisis univariado y un análisis bivariado mediante la prueba chi-cuadrado para determinar diferencias entre los pacientes con SAF primario y SAF secundario. Finalmente, se hizo un análisis multivariado para buscar asociaciones con los desenlaces clínicos trombóticos en los pacientes con SAF. Resultados: La edad promedio de la población fue de 42,4 ± 14 años; el 84,6% correspondió a sexo femenino. El 67,6% de los pacientes tenía diagnóstico de SAF primario y un 32,4% de SAF secundario, siendo el lupus eritematoso sistémico (LES) la enfermedad asociada en un 84%. Dentro de los eventos trombóticos, el más frecuente fue la trombosis venosa profunda (17,3%), seguida por el ataque cerebrovascular (9,9%). En los eventos obstétricos existió una prevalencia del 39,4% para abortos. No se encontraron diferencias en el perfil sociodemográfico ni en el perfil inmunoserológico entre los pacientes con diagnóstico de SAF primario y aquellos con SAF secundario. Los eventos trombóticos tuvieron mayor frecuencia en el grupo de SAF primario, pero solo la tromboembolia pulmonar alcanzó significación estadís tica. Eventos obstétricos como los abortos no fueron diferentes entre ambos grupos. Dentro de los factores asociados a los eventos trombóticos, se encontró que el sexo femenino tiene una probabilidad 5 veces mayor de accidente cerebrovascular y 3 veces mayor de trombosis venosa profunda. Los anti- β2GPI tipo IgM aumentaron alrededor de 3 veces la probabilidad de presentar abortos en mujeres con SAF. Conclusión: Se presenta una de las cohortes colombianas más grandes de pacientes con SAF reportadas hasta el momento en la literatura. La población es comparable clínica y sociodemográficamente con lo encontrado en otros estudios, aunque la prevalencia de SAF primario fue mayor y las complicaciones trombóticas fueron menores. La tromboembolia pulmonar fue significativamente mayor en el grupo de SAF primario.

ABSTRACT Introduction: Antiphospholipid syndrome (APS) is a systemic autoimmune disease charac terized by recurrent thrombosis that can affect the arterial and venous circulation. Objective: To analyze the immunological and pharmacological differences, as well as the clinical outcomes of a cohort of patients with primary APS and secondary APS. Materials and methods: A retrospective cohort study was conducted that included 352 records of patients diagnosed with APS and treated between 2014 and 2018. A description is pre sented of the sociodemographic, clinical, and immunological profile of the population. A bivariate analysis performed using the chi-squared test to determine differences between groups with primary APS and secondary APS, and finally a multivariate analysis to search for associations with thrombotic clinical outcomes in patients with APS. Results: The mean age was 42.4 ± 14 years, and 84.6% were females. Two-thirds (67.6%) of the patients had a diagnosis of primary APS, and 32.4% of secondary APS, of which 84% were associated with systemic lupus erythematosus (SLE). Among the thrombotic events, the most frequent were deep vein thrombosis (17.3%) and stroke (9.9%). Obstetric events were frequent, with a prevalence of 39.4% for miscarriages. No differences were found in the sociodemographic or immunoserological profile when comparing the group of primary vs. secondary APS. Thrombotic events were more frequent in the primary APS group, although only pulmonary embolism reached statistical significance. There were no differences bet ween the two groups as regards obstetric events, such as miscarriages. Women were found to be 5 times more likely to have a stroke and 3 times more to have deep vein thrombosis. The anti-β2GPI type IgM increased the probability of presenting miscarriages about 3 times in women with APS. Conclusion: The study contains one of the largest Colombian cohorts with APS reported so far, and although it is both clinically and sociodemographically similar to other cohorts, there is a higher prevalence of primary APS. There was a lower frequency of thrombotic complications compared to other cohorts. Patients with primary APS had a tendency to develop thrombosis, as has already been reported in the literature.

Clinical and sociodemographic factors associated with lupus nephritis in Colombian patients: A cross-sectional study / Factores clínicos y sociodemográficos asociados a nefritis lúpica en pacientes colombianos: resultados de un estudio transversal

Introduction: Over the past decades, incidence of SLE (Systemic Lupus Erythematosus) has increased due to early case detection and improved survival of patients. SLE presents at an earlier age and has a more severe presentation in African-American, Native American, Asian, and Hispanic populations. Worldwide, lupus nephritis (LN) is observed in 29–60% of SLE patients, it has a negative impact in renal survival and patient mortality. Several cohorts have established potential risk factors associated with lupus nephritis, such as male sex, serological markers, and some extra-renal manifestations. Objectives: To describe sociodemographic, clinical, immunological, and environmental risk factors in Colombian SLE patients and to compare the population with and without nephritis, in order to establish risk factors and possible associations. Materials and methods: A total of 1175 SLE patients participated in this study. During medical care, an interview and structured survey was conducted and later registered in a database. Sociodemographic, clinical, immunological, and environmental exposure variables were analyzed. Bivariate and multivariate analyses were performed using presence of LN as an outcome. Results: Prevalence of LN was 38.7%. Variables significantly associated with LN included being male (OR 1.98), a duration of SLE>10 years (OR 1.48), positive anti-DNA (OR 1.34), positive anti-Sm (OR 1.45), and smoking (OR 1.66). Being non-smoker was a protective factor (OR 0.52). Conclusion: This study describes potential factors associated with lupus nephritis in a Latin American population. Smoking status could be a target for intervention as it is a modifiable risk factor. The association between being male and LN is observed in Latin-American populations such as presented here. Further research in other large-scale population studies and more efforts are needed to gain better insights to explicate these relationships.(AU)

Introducción: En las últimas décadas la incidencia del Lupus eritematoso sistémico (LES) se ha incrementado debido a la detección temprana y su mejoría en la supervivencia. La nefritis lúpica (NL) se observa en el 29% a 60% de los pacientes con LES, teniendo un impacto negativo en la supervivencia renal y la mortalidad. Varias cohortes han establecido factores de riesgo asociados con la NL, como el sexo masculino, marcadores serológicos y algunas manifestaciones extrarrenales. Objetivos: Describir los factores de riesgo sociodemográficos, clínicos, inmunológicos y ambientales en pacientes colombianos con LES y comparar la población con y sin NL para establecer posibles asociaciones. Materiales y métodos: Se incluyeron 1175 pacientes con LES, se analizaron variables sociodemográficas, clínicas, inmunológicas y ambientales, tomadas de bases de datos de registros cínicos. Los análisis bivariados y multivariados se realizaron utilizando la presencia de NL como desenlace. Resultados: La prevalencia de NL fue del 38,7%. Las variables significativamente asociadas con NL incluyeron sexo masculino (OR 1.98), una duración del LES>10 años (OR 1.48), anti-ADN positivo (OR 1.34), anti-Sm positivo (OR 1.45) y tabaquismo (OR 1.66), mientras que la ausencia de exposición al tabaco se comportó como factor protector (OR 0.52). Conclusión: Se describen los factores potenciales asociados con NL en una población latinoamericana. El tabaquismo se presenta como un factor de riesgo susceptible de intervención. El sexo masculino y su asociación con NL ya ha sido reportado en otras poblaciones latinoamericanas. Se requieren investigaciones a gran escala en otras poblaciones para explicar mejor estas asociaciones.(AU)

Clinical and sociodemographic factors associated with lupus nephritis in Colombian patients: A cross-sectional study.

INTRODUCTION: Over the past decades, incidence of SLE (Systemic Lupus Erythematosus) has increased due to early case detection and improved survival of patients. SLE presents at an earlier age and has a more severe presentation in African-American, Native American, Asian, and Hispanic populations. Worldwide, lupus nephritis (LN) is observed in 29-60% of SLE patients, it has a negative impact in renal survival and patient mortality. Several cohorts have established potential risk factors associated with lupus nephritis, such as male sex, serological markers, and some extra-renal manifestations. OBJECTIVES: To describe sociodemographic, clinical, immunological, and environmental risk factors in Colombian SLE patients and to compare the population with and without nephritis, in order to establish risk factors and possible associations. MATERIALS AND METHODS: A total of 1175 SLE patients participated in this study. During medical care, an interview and structured survey was conducted and later registered in a database. Sociodemographic, clinical, immunological, and environmental exposure variables were analyzed. Bivariate and multivariate analyses were performed using presence of LN as an outcome. RESULTS: Prevalence of LN was 38.7%. Variables significantly associated with LN included being male (OR 1.98), a duration of SLE>10 years (OR 1.48), positive anti-DNA (OR 1.34), positive anti-Sm (OR 1.45), and smoking (OR 1.66). Being non-smoker was a protective factor (OR 0.52). CONCLUSION: This study describes potential factors associated with lupus nephritis in a Latin American population. Smoking status could be a target for intervention as it is a modifiable risk factor. The association between being male and LN is observed in Latin-American populations such as presented here. Further research in other large-scale population studies and more efforts are needed to gain better insights to explicate these relationships.

Bloqueo de rama alternante: reporte de una complicación inusual tras implante valvular aórtico transcatéter / Alternating bundle branch block: report of an unusual complication after transcatheter aortic valve implantation

Resumen Los trastornos de la conducción representan una potencial complicación de los reemplazos valvulares percutáneos. Los bloqueos alternantes de rama, aunque son inusuales, reflejan daño extenso del sistema His Purkinje con riesgo potencial de bloqueo auriculoventricular completo, por lo cual es importante su identificación y abordaje oportuno. Se presenta el caso de una paciente femenina de 82 años con múltiples comorbilidades y estenosis aórtica severa, en seguimiento médico desde hacía un año, en quien no se había realizado reemplazo quirúrgico por negativa de la paciente y por el alto riesgo intraoperatorio. Ingresó a la institución con un cuadro clínico de disnea progresiva de un mes de evolución, se realizó ecocardiograma transtorácico documentando estenosis aórtica crítica y fue llevada a implante valvular aórtico percutáneo. Por el alto riesgo de necesidad de marcapasos peri o postprocedimiento, se realizó seguimiento con monitoreo Holter, documentando bloqueo alternante de rama, el cual no progresó a bloqueo auriculoventricular completo luego de 72 horas de seguimiento, por lo que no se implantó marcapasos permanente. MÉD.UIS.2020;33(3): 37-42

Abstract Conduction disorders represent a potential complication of percutaneous valve replacements. Alternating bundle branch block, although unusual, reflect extensive damage of the His-Purkinje system with potential risk of complete atrioventricular block, therefore, its identification and timely approach is important. We present the case of an 82 years old female patient with multiple comorbidities and a medical history of severe aortic stenosis in medical follow-up for a year, in whom surgical replacement had not been conducted due to the patient's refusal and to the high surgical risk. She was admitted to the institution referring one month of progressive shortness of breath and an echocardiogram documented critical aortic stenosis. Percutaneous aortic valve implantation was performed. Due to her high risk of needing a peri or post-procedure pacemaker, Holter monitoring was performed, documenting alternating bundle branch block, which did not progress to complete atrioventricular block after 72 hours of follow-up, in consequence, a permanent pacemaker was not implanted. MÉD.UIS.2020;33(3): 37-42

Disseminated Streptococcus bovis infection after consumption of manatee meat: A case report and review of the literature.

Streptococcus bovis bacteremia (currently called S. gallolyticus) represents an infrequent condition, but associated with a high rate of morbidity and mortality Amado et al. (2015). The clinical presentation is characterized by symptoms associated with disseminated infection and with a higher frequency of endocarditis and gastrointestinal neoplasms, often occult Amado et al. (2015), Olmos et al. (2016). Probable sources of infection and zoonotic transmission have not been explored in the literature. We present a case of disseminated infection after ingestion of a marine mammal.

Enfermedad relacionada con IgG4: ¿es el rituximab la mejor estrategia terapéutica en los casos refractarios a terapia convencional? Resultados de una revisión sistemática / IgG4-related disease: Is rituximab the best therapeutic strategy for cases refractory to conventional therapy? Results of a systematic review

INTRODUCCIÓN: La enfermedad relacionada con IgG4 es una condición fibroinflamatoria caracterizada por infiltración linfoplasmocítica con predominio de células plasmáticas IgG4+, fibrosis y en la mayoría de los casos niveles séricos elevados de IgG4. Puede afectar cualquier órgano y llevar a manifestaciones clínicas variables. El tratamiento con esteroides es la piedra angular, sin embargo, el porcentaje de recidivas es alto. Estudios recientes han mostrado efectos favorables con rituximab. OBJETIVO: Evaluar la efectividad relacionada con la respuesta al tratamiento con rituximab en los pacientes con enfermedad relacionada con IgG4 refractarios a uso de esteroides y otras terapias inmunosupresoras. MATERIALES Y MÉTODOS: Se realizó una búsqueda sistemática en bases de datos especializadas EMBASE, LILACS, PUBMED y OVID-Cochrane de publicaciones hasta diciembre de 2017. RESULTADOS: Posteriormente al análisis de calidad se seleccionaron 27 artículos (264 pacientes en total) para la revisión final de los cuales 23 correspondían a reportes de caso y series de caso (105 pacientes), 3 a estudios observacionales de seguimiento a una cohorte (129 pacientes) y un ensayo clínico (30 pacientes). La enfermedad relacionada con IgG4 se presenta predominantemente en pacientes de sexo masculino con una edad promedio entre 50 y 70 años. El compromiso orgánico es múltiple con un promedio de 3,5 órganos comprometidos, siendo lo más frecuente el compromiso orbitario, glandular y ganglionar. Los pacientes de los diferentes estudios incluidos recibieron diversos tratamientos previo al inicio de rituximab, entre ellos glucocorticoides y fármacos antirreumáticos modificadores de la enfermedad. El porcentaje de respuesta en aquellos casos donde el rituximab se empleó como terapia de segunda línea fue del 90,7%; en el 10% de los pacientes se empleó rituximab como primera línea con un porcentaje de respuesta del 100%. CONCLUSIÓN: El uso de rituximab en pacientes refractarios a terapias de primera línea se asocia a un alto porcentaje de respuesta y a menor dependencia a glucocorticoides

INTRODUCTION: IgG4 related disease is a fibroinflammatory condition characterised by lymphoplasmocytic infiltration with predominance of IgG4+ plasma cells, fibrosis, and in most cases elevated IgG4 serum levels. It can affect any organ and result in varying clinical manifestations. Steroids are the cornerstone of treatment, however there is a high percentage of relapse. Recent studies have demonstrated favourable effects with rituximab. OBJECTIVE: To evaluate effectiveness related to the response to treatment with rituximab in patients with IgG4 related disease refractory to steroids and other immunosuppressant therapies. MATERIALS AND METHODS: We undertook a systematic search of the specialist databases EMBASE, LILACS, PUBMED and OVID-Cochrane for publications up until December 2017. RESULTS: After the quality analysis, we selected 27 articles (264 patients in total) for the final review, of which 23 were case reports and case series (105 patients), 3 were observational follow-up cohort studies (129 patients), and there was one clinical trial (30 patients). IgG4 related disease presents predominantly in male patients aged between 50 and 70 years on average. Multiple organs are compromised with an average of 3.5 compromised organs. Orbital, glandular and lymph-node compromise is most frequent. Patients in the different studies we included had received various treatments prior to starting rituximab, including glucocorticoids and disease-modifying anti-rheumatic drugs. There was 90.7% response in the cases where rituximab was used as second line therapy; rituximab was used as first line treatment for 10% of the patients with a 100% response rate. CONCLUSION: The use of rituximab for patients refractory to first-line treatments was associated with a high response percentage and less dependence on glucocorticoids

IgG4-related disease: Is rituximab the best therapeutic strategy for cases refractory to conventional therapy? Results of a systematic review. / Enfermedad relacionada con IgG4: ¿es el rituximab la mejor estrategia terapéutica en los casos refractarios a terapia convencional? Resultados de una revisión sistemática.

INTRODUCTION: IgG4 related disease is a fibroinflammatory condition characterised by lymphoplasmocytic infiltration with predominance of IgG4+ plasma cells, fibrosis, and in most cases elevated IgG4 serum levels. It can affect any organ and result in varying clinical manifestations. Steroids are the cornerstone of treatment, however there is a high percentage of relapse. Recent studies have demonstrated favourable effects with rituximab. OBJECTIVE: To evaluate effectiveness related to the response to treatment with rituximab in patients with IgG4 related disease refractory to steroids and other immunosuppressant therapies. MATERIALS AND METHODS: We undertook a systematic search of the specialist databases EMBASE, LILACS, PUBMED and OVID-Cochrane for publications up until December 2017. RESULTS: After the quality analysis, we selected 27 articles (264 patients in total) for the final review, of which 23 were case reports and case series (105 patients), 3 were observational follow-up cohort studies (129 patients), and there was one clinical trial (30 patients). IgG4 related disease presents predominantly in male patients aged between 50 and 70 years on average. Multiple organs are compromised with an average of 3.5 compromised organs. Orbital, glandular and lymph-node compromise is most frequent. Patients in the different studies we included had received various treatments prior to starting rituximab, including glucocorticoids and disease-modifying anti-rheumatic drugs. There was 90.7% response in the cases where rituximab was used as second line therapy; rituximab was used as first line treatment for 10% of the patients with a 100% response rate. CONCLUSION: The use of rituximab for patients refractory to first-line treatments was associated with a high response percentage and less dependence on glucocorticoids.

Factores, clínicos, sociodemográficos, inmunológicos y exposicionales en Lupus Eritematoso Sistémico asociados a Nefritis Lúpica en población colombiana / Factors, clinical, sociodemographic, immunological and expositional in Systemic Lupus Erythematosus associated with lupus nephritis in Colombian population

Introducción: El lupus eritematoso sistémico (LES) es una enfermedad autoinmune caracterizada por compromiso de múltiples órganos, siendo la nefritis lúpica (NL)1 una de las manifestaciones mas graves. Objetivo: Establecer factores asociados a nefritis lúpica en los pacientes con LES. Métodos: Estudio de corte trasversal tomado de una cohorte de 1175 pacientes con LES que cumplieron criterios clasificatorios para ACR 1997 o SLICC 2012, atendidos en una IPS especializada entre 2007 y 2015. Se realizó análisis bivariado de múltiples características entre pacientes con presencia y ausencia de NL a través de chi2 para variables cuantitativas y U de Mann Whitney para variables politómicas o cuantitativas sin distribución normal en búsqueda de asociación estadística.