Frecuencia de eventos cardiovasculares en pacientes con lupus eritematoso sistémico / Frequency of cardiovascular events in patients with systemic lupus erythematosus

Resumen Introducción: el progreso en los tratamientos para el lupus eritematoso sistémico (LES) resultó en una disminución de la mortalidad; sin embargo, la enfermedad cardiovascular y las complicaciones infecciosas aún son las principales causas de muerte. La evidencia apoya la participación del sistema inmunológico en la generación de la placa aterosclerótica, así como su conexión con las enfermedades autoinmunes. Objetivos: describir la frecuencia de eventos cardiovasculares (ECV) en el Registro de Lupus Eritematoso Sistémico de la Sociedad Argentina de Reumatología (RELESSAR) transversal, así como sus principales factores de riesgo asociados. Materiales y métodos: estudio descriptivo y transversal para el cual se tomaron los pacientes ingresados en el registro RELESSAR transversal. Se describieron las variables sociodemográficas y clínicas, las comorbilidades, score de actividad y daño. ECV se definió como la presencia de al menos una de las siguientes patologías: enfermedad arterial periférica, cardiopatía isquémica o accidente cerebrovascular. El evento clasificado para el análisis fue aquel posterior al diagnóstico del LES. Se conformaron dos grupos macheados por edad y sexo 1:2. Resultados: 1515 pacientes mayores de 18 años participaron del registro. Se describieron 80 pacientes con ECV (5,3%). En este análisis se incluyeron 240 pacientes conformando dos grupos. La edad media fue de 47,8 (14,4) y 47,6 (14,2) en el grupo con y sin ECV respectivamente. Los pacientes con ECV tuvieron mayor duración del LES en meses, mayor índice de Charlson, mayor SLICC (Systemic Lupus International Collaborating Clinics/American College of Rheumatology), mayor frecuencia de manifestaciones neurológicas, síndrome antifosfolípido, hospitalizaciones y uso de ciclofosfamida. Las únicas variables asociadas en el análisis multivariado fueron el índice de Charlson (p=0,004) y el SLICC (p<0,001). Conclusiones: los ECV influyen significativamente en nuestros pacientes, y se asocian a mayor posibilidad de daño irreversible y comorbilidades.

Abstract Introduction: progress in treatments for systemic lupus erythematosus (SLE) has resulted in a decrease in mortality; however, cardiovascular and infectious diseases remain the leading causes of death. Evidence supports the involvement of the immune system in the generation of atherosclerotic plaque, as well as its connection to autoimmune diseases. Objectives: to describe the frequency of cardiovascular disease (CVD) in the cross-sectional RELESSAR registry, as well as its associated variables. Materials and methods: a descriptive and cross-sectional study was performed using patients admitted to the cross-sectional RELESSAR registry. Sociodemographic variables, clinical variables, comorbidities, activity and damage scores were described. CVD was defined as at least one of the following: peripheral arterial disease, ischemic heart disease, or cerebrovascular accident. All patients with at least one CVD were included in our analysis (heart attack, central nervous system vascular disease, and peripheral arteries atherosclerotic disease). The event classified for the analysis was that after the diagnosis of SLE. SLE diagnosis was previous to CVD. Two groups matched by age and sex, 1:2 were formed. Results: a total of 1515 patients older than 18 years participated in the registry. Eighty patients with CVD (5.3%) were described in the registry. Two-hundred and forty patients were included, according to two groups. The mean age was 47.8 (SD 14.4) and 47.6 (SD 14.2) in patients with and without CVD, respectively. Patients with CVD had a longer duration of SLE in months, a higher Charlson index, a higher SLICC, increased frequency of neurological manifestations, antiphospholipid syndrome, hospitalizations, and use of cyclophosphamide. The associated variables in the multivariate were the Charlson Index (p=0.004) and the SLICC (p<0.001). Conclusions: CVDs have a significant influence on our patients, being associated with a greater possibility of damage and comorbidities.

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency.

OBJECTIVE: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. METHODS: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. RESULTS: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. CONCLUSIONS: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.

Behçet disease in Latin American countries: A systematic literature review of demographic and clinical features, and HLA-B*51 allele frequency / Enfermedad de Behçet en países de Latinoamérica: Revisión sistemática de la literatura sobre datos demográficos, hallazgos clínicos y frecuencia del alelo HLA-B*51

Objective: To describe the demographic and clinical features, as well as the frequency of the HLA-B*51 allele in Behçet disease (BD) patients in Latin American countries. Methods: A systematic literature review of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines was conducted without performing a meta-analysis. We included observational studies (cross-sectional or cohort) of BD patients fulfilling the International Study Group for BD classification criteria and reported the demographic, clinical, and laboratory features of the disease in adult patients. Results: Twelve studies were included in the SLR. Information from 532 patients across 5 Latin American countries was included for the analysis. Mean age at disease diagnosis was 33 years, 58.3% were female and 41.7% male; most patients were non-Caucasian. The most common clinical manifestations were recurrent oral ulcers and genital ulcers, followed by skin, eye, joint, neurological, gastrointestinal, vascular, and cardiac involvement. The prevalence of BD was described in 2 studies, 1 conducted in Brazil that reported a prevalence of .3/100,000 inhabitants, and another in Colombia with a prevalence of 1.1/100,000 inhabitants. The frequency of HLA-B*51 allele in BD patients was 38%, 30.1%, and 9% in Argentina, Brazil, and Mexico, respectively. Conclusions: The prevalence of BD in the Latin American countries seems to be low, as well as the frequency of HLA-B*51 allele. However, the strength of association between HLA-B*51 and BD remains high in our population. The key clinical features of BD are like those reported in countries/regions where BD is endemic.(AU)

Objetivo: Describir las características demográficas, clínicas y la frecuencia del alelo HLA-B*51 en pacientes con enfermedad de Behçet (EB) en países de América Latina. Métodos: Se llevó a cabo una revisión sistemática de la literatura (RSL) según la guía PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) sin realizar un metaanálisis final. Se incluyeron estudios observacionales (transversales o de cohortes) de pacientes con EB que cumplieron con los criterios de clasificación del Grupo Internacional de Estudio de la EB e informaron las características demográficas, clínicas y de laboratorio en pacientes adultos con EB. Resultados: Doce estudios fueron incluidos para la RSL. La información de 532 pacientes provenientes de 5 países de América Latina se incluyó para el análisis. La edad media al diagnóstico fue de 33 años, el 58,3% fueron mujeres y el 41,7% hombres; la mayoría de los pacientes fueron no caucásicos. Las manifestaciones clínicas más comunes fueron las úlceras orales y genitales recurrentes, seguidas del compromiso cutáneo, ocular, articular, neurológico, gastrointestinal, vascular y cardíaco. La prevalencia de la EB fue descrita en 2 estudios, uno realizado en Brasil que reportó una prevalencia de 0,3/100.000 habitantes, y otro en Colombia con una prevalencia de 1,1/100.000 habitantes. La frecuencia del HLA-B*51 en pacientes con EB fue del 38%, 30,1% y 9% en Argentina, Brasil y México, respectivamente. Conclusiones: La prevalencia de la EB en los países latinoamericanos parece ser baja, así como la frecuencia del alelo HLA-B*51. Sin embargo, la fuerza de asociación entre el HLA-B*51 y la EB sigue siendo alta en nuestra población. Las características clínicas claves de la EB son similares a las reportadas en países/regiones donde es endémica.(AU)

Significance of HLA-B[*]51 allele expression in Crohn's disease: a case-control study.

OBJECTIVES: Crohn's disease (CD) and Behçet's disease (BD) are two autoinflammatory diseases that share clinical and pathogenic features. Furthermore, when BD involves the gastrointestinal tract, it is extremely difficult to distinguish endoscopic lesions from CD lesions. HLA-B*51 allele expression is highly associated with BD diagnosis. In this study we analysed HLA-B*51 status in 70 Argentine patients with confirmed CD diagnosis and compared it to our previous Argentine BD cohort, with the aim of finding similarities or differences between these two diseases regarding HLA-B*51 status. METHODS: This is a multi-centre case-control study, including 70 patients with confirmed CD diagnosis, who underwent HLA-B*51 allele status testing; the results were compared to our previous BD cohort of 34 patients. RESULTS: Among patients with CD, 12.85% were positive for the HLA-B*51 allele, compared with 38.24% patients with BD (OR=0.238; 95% CI=0.089-0.637; p=0.004). CONCLUSIONS: Our finding suggests that determination of HLA-B*51 allele status may contribute to the differential diagnosis between CD and BD.

Recomendaciones de la Sociedad Argentina de Reumatología en el manejo de la arteritis de células gigantes / Recommendations of the Argentine Society of Rheumatology in the management of giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Giant cell arteritis (GCA) is a systemic vasculitis affecting adult patients and involving large and medium vessels. Potential serious complications as blindness may occur and it is considered a medical emergency. The objective of elaborating this guideline was to develop first Argentinian GCA treatment recommendations using GRADE methodology. An expert panel generated clinically meaningful questions addressing aspects of the treatment of GCA in the Population, Intervention, Comparator and Outcome (PICO) format and then a group of methodology experts reviewed and extracted data from literature summarizing available evidence. A patient's focus group discussion took place gathering information on their preferences and experiences. Finally, the vasculitis expert panel, with all the information obtained, voted recommendations here presented.

Guías Argentinas de Vasculitis Suplemento. Tablas arteritis de células gigantes / Argentine Vasculitis Guidelines Supplement. Giant cell arteritis tables

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Multicenter lupus register from Argentina, the RELESSAR database: Influence of ethnicity on disease phenotype.

OBJECTIVE: The objective is to describe the main characteristics of patients with systemic lupus erythematosus (SLE) in Argentina and to examine the influence of ethnicity on the expression of the disease. PATIENTS AND METHODS: RELESSAR is a multicentre register carried out by 106 researchers from 67 rheumatologic Argentine centres. It is a cross-sectional study of SLE (1982/1997 ACR) patients. RELESSAR electronic database includes demographic, cumulative SLE manifestations, SELENA-SLEDAI, SLICC-SDI, Katz's severity and Charlson's comorbidity indexes and treatment patterns. RESULTS: We included 1,610 patients, 91.7% were female with a median age at diagnosis of 28.1 ± 12.8; 96.2% met ≥4 ACR 1982/97 criteria. Frequent manifestations were arthritis (83.5%), malar rash (79.5%), photosensitivity (75.3%), haematological (63.8%) and renal disease (47.4%), antinuclear antibodies (96%), anti-dsDNA (66.5%) and anti-Smith antibodies (29%). The mean Selena-SLEDAI score at last visit was 3.18 (SD 4.3) and mean SDI was 1 (SD 1.3). The accumulated treatments most frequently used were antimalarials (90.4%), corticosteroids (90%), azathioprine (31.8%), intravenous cyclophosphamide (30.2%), mycophenolate mofetil or mycophenolic acid (24.5%), methotrexate (19.3%), belimumab 5.3% and rituximab 5.1%. Refractory lupus was diagnosed in 9.3% of the cases. The main causes of death were lupus activity (25.0%), activity and concomitant infections (25.0%), infections (18.2%), vascular disease (13.6%) and cancer (4.5%). Mortality was associated with higher SLEDAI, Katz, damage indexes and comorbidities. Of the 1610 patients included, 44.6% were Caucasian, 44.5% Mestizo, 8.1% Amerindian and 1.2% Afro-Latin American. Mestizo patients had higher male representation, low socioeconomic status, more inadequate medical coverage, fewer formal years of education and shorter disease duration. Polyadenopathies and Raynaud's phenomenon were more frequent in Caucasians. In the logistic regression analysis higher damage index (OR 1.28, CI 95% 1.02-1.61, p = 0.03) remained associated to mestizo ethnicity. CONCLUSIONS: This study represents the largest number of adult patients with SLE studied in Argentina. Caucasian patients were differentiated by having Raynaud's phenomenon and polyadenopathy more frequently, while patients of Mestizo origin had higher damage indexes.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

Lupus en Argentina. Pacientes no respondedores al tratamiento estándar y belimumab como posible opción. Datos del registro RELESSAR / Lupus in Argentina. Non-responders to standard treatment and belimumab as a possible option. RELESSAR record data

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.

Introduction: lupus is a complex disease and often difficult to approach. Achieving remission is one of the objectives, incorporating therapeutic options. Objectives: to describe the characteristics of the patients and the use of belimumab, according to the status of the disease. Materials and methods: cross-sectional study. Patients of the RELESSAR registry. Stratification: Remission: SLEDAI=0 and without corticosteroids. Low disease activity SLEDAI> 0 and ≤4 and without corticosteroids and non-optimal control: SLEDAI> 4 and any dose of corticosteroids. Results: a total of 1,277 patients were included, 23.4% in remission, 12.6% in low disease activity and 63.8% in non-optimal control. The last group was younger and had a shorter duration of the disease. They had higher activity and chronicity indices and greater use of immunosuppressants. Only 22.3% of the patients with potential criteria for the use of belimumab (activity disease despite standard treatment) were receiving it. The variables associated with hospitalizations were: corticosteroids, cyclophosphamide and higher SLICC. Those associated with severe infection: mycophenolate mofetil, azathioprine, corticosteroids, and higher SLICC. Conclusions: the complexity of the management of these patients is reflected, visualizing structural aspects such as inequality. The use of belimumab could be beneficial in selected patients.

The HLA-B*51 allele is strongly associated with Behçet Disease in an Argentinean population / El alelo HLA-B*51 se asoció fuertemente a la Enfermedad de Behçet en la población argentina

OBJECTIVE: To assess the association between the HLA-B*51 allele and Behçet Disease (BD) in Argentinean patients. METHODS: We enrolled 34 consecutive Argentinean patients with definitive diagnosis of BD between October 2016 and March 2017. None of the patients had the HLA-B*51 allele determined at study entry. Unrelated controls (n=240) were randomly obtained from the national cadaveric donor database. Demographic and clinical features of the patients were recorded by attending physicians through a questionnaire. RESULTS: Mean age of cases was 42 years old. Nineteen (55.8%) were male, and the mean age at diagnosis was 35 years old; twenty (58.8%) were Mestizos, 8 (23.5%) were Caucasian, and 6 (17.6%) were Amerindians. Thirteen (38.2%) of 34 cases were HLA-B*51 allele positive; 11 were heterozygous and 2 homozygous for the allele. Thirty-four (14.2%) of 240 controls were positive for the HLA-B*51 allele. The association between BD and HLA-B*51 allele was greater than that of control group (OR=3.75; p = 0.0012). CONCLUSIONS: The HLA-B*51 allele is strongly associated with BD in Argentinean patients. Our finding is consistent with previous studies indicating that the HLA-B*51 allele is an important susceptibility gene in BD regardless the geographical region and ethnicity

OBJETIVO: Evaluar la asociación entre el alelo HLA-B*51 y la enfermedad de Behçet (EB) en pacientes argentinos. MÉTODOS: Incluimos en forma consecutiva 34 pacientes argentinos con diagnóstico definitivo de EB entre los meses de octubre de 2016 y marzo de 2017. Ninguno de los pacientes tenía el alelo HLA-B*51 determinado al inicio del estudio. Los controles no relacionados (n=240) se obtuvieron al azar de la base nacional de datos de donantes cadavéricos. Las características demográficas y clínicas de los pacientes fueron registradas por los médicos asistentes a través de un cuestionario. RESULTADOS: La edad promedio de los casos fue de 42 años. Diecinueve (55,8%) fueron varones, y la edad promedio en el momento del diagnóstico fue de 35 años; 20 (58,8%) fueron mestizos, 8 (23,5%) caucásicos y 6 (17,6%) amerindios. Trece (38,2%) de los 34 casos fueron positivos para el alelo HLA-B*51; 11 de ellos fueron heterocigotas y 2 homocigotas para dicho alelo. Treinta y cuatro (14,2%) de los 240 controles fueron positivos para el alelo HLA-B*51. La asociación entre la EB y el alelo HLA-B*51 fue mayor que en el grupo control (OR=3,75; p = 0,0012). CONCLUSIONES: El alelo HLA-B*51 está fuertemente asociado con la EB en pacientes argentinos. Nuestro hallazgo es consistente con estudios previos que indican que el alelo HLA-B*51 es un gen de susceptibilidad importante en la EB independientemente de la región geográfica y la etnia

The HLA-B*51 Allele is strongly associated with Behçet Disease in an Argentinean population.

OBJECTIVE: To assess the association between the HLA-B*51 allele and Behçet Disease (BD) in Argentinean patients. METHODS: We enrolled 34 consecutive Argentinean patients with definitive diagnosis of BD between October 2016 and March 2017. None of the patients had the HLA-B*51 allele determined at study entry. Unrelated controls (n=240) were randomly obtained from the national cadaveric donor database. Demographic and clinical features of the patients were recorded by attending physicians through a questionnaire. RESULTS: Mean age of cases was 42 years old. Nineteen (55.8%) were male, and the mean age at diagnosis was 35 years old; twenty (58.8%) were Mestizos, 8 (23.5%) were Caucasian, and 6 (17.6%) were Amerindians. Thirteen (38.2%) of 34 cases were HLA-B*51 allele positive; 11 were heterozygous and 2 homozygous for the allele. Thirty-four (14.2%) of 240 controls were positive for the HLA-B*51 allele. The association between BD and HLA-B*51 allele was greater than that of control group (OR=3.75; p=0.0012). CONCLUSIONS: The HLA-B*51 allele is strongly associated with BD in Argentinean patients. Our finding is consistent with previous studies indicating that the HLA-B*51 allele is an important susceptibility gene in BD regardless the geographical region and ethnicity.

Enfermedad por arañazo de gato en paciente con artritis reumatoidea tratado con etanercept / Cat scratch disease during etanercept therapy in a rheumatoid arthritis patient

La enfermedad por arañazo de gato (EAG) es una enfermedad infecciosa causada por la proteobacteria Bartonella henselae, caracterizada por fiebre y linfadenopatía granulomatosa. La inmunosupresión es un factor de riesgo para el desarrollo de formas atípicas de la enfermedad. Presentamos el caso de una mujer de 52 años de edad que presentó fiebre y adenomegalias inguinales bilaterales. No tenía contacto aparente con animales. La paciente estaba recibiendo tratamiento con etanercept por artritis reumatoidea. La biopsia del ganglio linfático informó de una adenopatía granulomatosa. Evolucionó favorablemente con la interrupción de etanercept y el tratamiento con minociclina. Presentó remisión clínica y la seroconversión típica. La infección por Bartonella debería ser considerada como un diagnóstico diferencial en pacientes con artritis reumatoidea, con linfadenopatía de origen desconocido

Cat scratch disease (CSD) is an infectious disorder caused by Bartonella henselae and characterized by fever and granulomatous lymphadenopathy. Immunosuppression is a risk factor for the development of atypical forms of the disease. We report the case of a 52-year-old woman who presented with fever and bilateral inguinal lymph node enlargement. She did not have apparent contact with animals. The patient was receiving etanercept therapy for rheumatoid arthritis. Lymph node biopsy demonstrated granulomatous lymphadenitis. She was successfully managed by discontinuing etanercept and by treatment with minocycline. She developed clinical remission and typical seroconversion. Infection with Bartonella should be considered in the differential diagnosis in rheumatoid arthritis patients with lymphadenopathy of unknown origin

Cat scratch disease during etanercept therapy in a rheumatoid arthritis patient. / Enfermedad por arañazo de gato en paciente con artritis reumatoidea tratado con etanercept.

Cat scratch disease (CSD) is an infectious disorder caused by Bartonella henselae and characterized by fever and granulomatous lymphadenopathy. Immunosuppression is a risk factor for the development of atypical forms of the disease. We report the case of a 52-year-old woman who presented with fever and bilateral inguinal lymph node enlargement. She did not have apparent contact with animals. The patient was receiving etanercept therapy for rheumatoid arthritis. Lymph node biopsy demonstrated granulomatous lymphadenitis. She was successfully managed by discontinuing etanercept and by treatment with minocycline. She developed clinical remission and typical seroconversion. Infection with Bartonella should be considered in the differential diagnosis in rheumatoid arthritis patients with lymphadenopathy of unknown origin.

La hiperostosis esquelética idiopática difusa (DISH) como expresión de un proceso sistémico / Diffuse idiopathic skeletal hiperostosis (DISH) as an expression of a systemic process

La Hiperostosis Esquelética Idiopática Difusa es una condición caracterizada por la calcificación y/u osificación de los tejidos blandos, principalmente entesis, ligamentos y cápsulas articulares. En 1950, Forestier y Rotés-Querol publicaron una serie denominándola "hiperostosis anquilosante vertebral senil" y la distinguieron como entidad nosológica separada de la espondiloartrosis y la espondilitis anquilosante

Rituximab en el tratamiento de la granulomatosis eosinofílica con poliangitis / Rituximab in the treatment of eosinophilic granulomatosis with polyangiitis

Antecedentes: Algunos pacientes con granulomatosis eosinofílica con poliangitis (EGPA) y factores de mal pronóstico son refractarios o presentan efectos adversos al tratamiento de inducción (glucocorticoides [GC] y ciclofosfamida [CF]), o recaen durante el mantenimiento (GC y azatioprina), haciendo necesaria la búsqueda de alternativas terapéuticas. En ensayos clínicos, el rituximab (RTX) demostró ser eficaz para el tratamiento de las vasculitis asociadas al ANCA; sin embargo, los pacientes con EGPA no fueron incluidos. Objetivo: Revisar y analizar la bibliografía sobre la uso de RTX para el tratamiento de la EGPA. Métodos: La búsqueda se realizó en MEDLINE y LILACS (1965 y 1986, respectivamente, hasta febrero del 2014). Resultados: Se incluyó a 27 pacientes. La indicación de RTX fue por enfermedad refractaria (n = 20), recaída (n = 5) y nuevo diagnóstico (n = 2). Los órganos afectados fueron los pulmones, el sistema nervioso periférico, el riñón y los ojos. Se observó remisión en 16 y respuesta en 8 pacientes. Conclusiones: El RTX fue eficaz y bien tolerado para el tratamiento de la EGPA (AU)

Background: The general consensus is that for patients with EGPA with poor prognosis, intensive therapy with both GC and CF is indicated. The maintenance of remission is made with GC and AZA. A considerable number of patients with EGPA are refractory to first line therapy, experience dose-limiting side effects or relapse. In clinical trials, RTX was effective for the treatment of ANCA-associated vasculitis. However, patients with a diagnosis of EGPA were not included. Objective: to review and analyze the published literature regarding the use of RTX in the treatment of EGPA. Methods: The literature search was performed in MEDLINE and LILACS from 1965 and 1986 respectively until february 2014. Results: 27 patients were included. RTX treatment was due to refractory disease (n = 20), relapse (n = 5) and with newly diagnosed (n = 2). The affected organs were the lungs, peripheral nervous system, kidney and the eyes. Sixteen patients had clinical remission and 8 patients had clinical response. Conclusions: RTX was effective and well tolerated for the treatment of EGPA (AU)

Rituximab in the treatment of eosinophilic granulomatosis with polyangiitis.

BACKGROUND: The general consensus is that for patients with EGPA with poor prognosis, intensive therapy with both GC and CF is indicated. The maintenance of remission is made with GC and AZA. A considerable number of patients with EGPA are refractory to first line therapy, experience dose-limiting side effects or relapse. In clinical trials, RTX was effective for the treatment of ANCA-associated vasculitis. However, patients with a diagnosis of EGPA were not included. OBJECTIVE: to review and analyze the published literature regarding the use of RTX in the treatment of EGPA. METHODS: The literature search was performed in MEDLINE and LILACS from 1965 and 1986 respectively until february 2014. RESULTS: 27 patients were included. RTX treatment was due to refractory disease (n=20), relapse (n=5) and with newly diagnosed (n=2). The affected organs were the lungs, peripheral nervous system, kidney and the eyes. Sixteen patients had clinical remission and 8 patients had clinical response. CONCLUSIONS: RTX was effective and well tolerated for the treatment of EGPA.

Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis.

The principal objective of this investigation was to analyze the association between diffuse idiopathic skeletal hyperostosis (DISH) and the presence of aortic valve sclerosis (AVS). For this study we used results from 1000 consecutive outpatients (473 males), older than 50 years of age (average 67.6 years), that had been examined with Doppler echocardiogram and anterior and lateral chest radiographs. Overall, 195 patients (19.5%) were diagnosed with DISH and 283 (28.3%) with AVS. DISH was more prevalent than AVS in males (66.7% vs. 42.6%, p< 0.0001) and in older patients (73.6 ± 9 years vs. 66.1 ± 9 years, p < 0.0001). Furthermore, 55.4% of patients with dorsal DISH presented aortic sclerosis calcification vs. 21.7% of patients free of DISH (OR = 4.47; 95% CI = 3.22-6.21). The adjusted odds ratio (OR) was calculated by sex and age resulting in 3.04 (95% CI = 2.12-4.36; p < .0001). A statistically significant association was found between DISH and AVS in accordance to age and sex. The biological plausibility of this association is based on similar risk factors, pathogenic mechanisms and vascular complications.

Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis / Asociación de hiperostosis esquelética idiopática difusa y esclerosis valvular aórtica

The principal objective of this investigation was to analyze the association between diffuse idiopathic skeletal hyperostosis (DISH) and the presence of aortic valve sclerosis (AVS). For this study we used results from 1000 consecutive outpatients (473 males), older than 50 years of age (average 67.6 years), that had been examined with Doppler echocardiogram and anterior and lateral chest radiographs. Overall, 195 patients (19.5%) were diagnosed with DISH and 283 (28.3%) with AVS. DISH was more prevalent than AVS in males (66.7% vs. 42.6%, p< 0.0001) and in older patients (73.6 ± 9 years vs. 66.1 ± 9 years, p < 0.0001). Furthermore, 55.4% of patients with dorsal DISH presented aortic sclerosis calcification vs. 21.7% of patients free of DISH (OR = 4.47; 95% CI = 3.22-6.21). The adjusted odds ratio (OR) was calculated by sex and age resulting in 3.04 (95% CI = 2.12-4.36; p < .0001). A statistically significant association was found between DISH and AVS in accordance to age and sex. The biological plausibility of this association is based on similar risk factors, pathogenic mechanisms and vascular complications.

El objetivo principal fue analizar la asociación entre la hiperostosis esquelética idiopática difusa (DISH) y la presencia de esclerosis valvular aórtica (AVS). Se evaluaron los resultados de 1000 pacientes ambulatorios consecutivos (473 varones), mayores de 50 años (promedio, 67.6 años), que habían sido examinados con un ecocardiograma Doppler y radiología torácica anterior y lateral. Globalmente, 195 pacientes (19.5%) tuvieron diagnóstico de DISH y 283 (28.3%) de AVS. DISH fue más prevalente que AVS en varones (66.7% vs. 42.6%, p < 0.0001) y en pacientes de mayor edad (73.6 ± 9 años vs. 66.1 ± 9 años, p < 0.0001). Además, 55.4% de los pacientes con DISH dorsal presentaron AVS vs. 21.7% de los pacientes sin DISH (OR = 4.47; 95% CI = 3.22-6.21). El odds ratio (OR) ajustado por sexo y edad fue 3.04 (95% CI = 2.12-4.36; p < 0.0001). Se encontró una asociación estadísticamente significativa entre DISH y AVS, que se mantuvo después de ajustar por sexo y edad. La plausibilidad biológica de esta asociación se basa en los factores de riesgo, mecanismos patogénicos y complicaciones vasculares compartidos.

Association of diffuse idiopathic skeletal hyperostosis and aortic valve sclerosis / Asociación de hiperostosis esquelética idiopática difusa y esclerosis valvular aórtica

The principal objective of this investigation was to analyze the association between diffuse idiopathic skeletal hyperostosis (DISH) and the presence of aortic valve sclerosis (AVS). For this study we used results from 1000 consecutive outpatients (473 males), older than 50 years of age (average 67.6 years), that had been examined with Doppler echocardiogram and anterior and lateral chest radiographs. Overall, 195 patients (19.5%) were diagnosed with DISH and 283 (28.3%) with AVS. DISH was more prevalent than AVS in males (66.7% vs. 42.6%, p< 0.0001) and in older patients (73.6 ± 9 years vs. 66.1 ± 9 years, p < 0.0001). Furthermore, 55.4% of patients with dorsal DISH presented aortic sclerosis calcification vs. 21.7% of patients free of DISH (OR = 4.47; 95% CI = 3.22-6.21). The adjusted odds ratio (OR) was calculated by sex and age resulting in 3.04 (95% CI = 2.12-4.36; p < .0001). A statistically significant association was found between DISH and AVS in accordance to age and sex. The biological plausibility of this association is based on similar risk factors, pathogenic mechanisms and vascular complications.(AU)

El objetivo principal fue analizar la asociación entre la hiperostosis esquelética idiopática difusa (DISH) y la presencia de esclerosis valvular aórtica (AVS). Se evaluaron los resultados de 1000 pacientes ambulatorios consecutivos (473 varones), mayores de 50 años (promedio, 67.6 años), que habían sido examinados con un ecocardiograma Doppler y radiología torácica anterior y lateral. Globalmente, 195 pacientes (19.5%) tuvieron diagnóstico de DISH y 283 (28.3%) de AVS. DISH fue más prevalente que AVS en varones (66.7% vs. 42.6%, p < 0.0001) y en pacientes de mayor edad (73.6 ± 9 años vs. 66.1 ± 9 años, p < 0.0001). Además, 55.4% de los pacientes con DISH dorsal presentaron AVS vs. 21.7% de los pacientes sin DISH (OR = 4.47; 95% CI = 3.22-6.21). El odds ratio (OR) ajustado por sexo y edad fue 3.04 (95% CI = 2.12-4.36; p < 0.0001). Se encontró una asociación estadísticamente significativa entre DISH y AVS, que se mantuvo después de ajustar por sexo y edad. La plausibilidad biológica de esta asociación se basa en los factores de riesgo, mecanismos patogénicos y complicaciones vasculares compartidos.(AU)

Carta al Comité de Redacción / Letter to the Drafting Committee

He leído con atención el artículo del Dr. Alfredo Pinzón titulado: "Babinsky o Babinski" (1) publicado en su prestigiosa revista, al cual accedí a través de Internet. Coincido profundamente con los datos aportados por el Dr. Pinzón, siendo un tema de preocupación no sólo por la existencia de un simple error ortográfico, sino por otra serie de implicancias desarrolladas previamente. Nuestro trabajo "¿Qué Babinsky? Babinski" (2), dio origen a la respuesta del Dr. Fernando A. Navarro: "Ni Babinsky ni Babinski: ¡Babinski!" (3). El Dr. Navarro además, es autor del "Diccionario crítico de dudas inglés-español de medicina" (4), imprescindible para el manejo de la literatura médica de los profesionales hispanoparlantes, donde el tema de Babinski es nuevamente citado. Atendiendo al título de la sección, Lapsus Medicus, y siendo el lapsus (Del lat. lapsus, resbalón) una f.lta o equivocación cometida por descuido (Diccionario de la Real Academia Española), me parece correcto completar este aporte