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The relevance of polyautoimmunity, defined as the presence of two or more autoimmune diseases in the same individual, is one of the issues not yet elucidated in medical practice. The coexistence of myasthenia gravis (MG) and systemic lupus erythematosus (SLE) is a clinical challenge due to the possible differential diagnoses of muscle involvement in patients with SLE. We present the case of a patient who came to the emergency room of Hospital Universitario San Ignacio in Bogota, Colombia, with a previous diagnosis of SLE, who developed acute weakness in the context of a systemic infection, with a clinical and electrophysiological diagnosis of MG.

RESUMEN El síndrome de Sjögren es una enfermedad autoinmunitaria sistémica con un alto impacto individual y social. El compromiso pulmonar presenta múltiples manifestaciones, con impacto en calidad de vida y riesgo de mortalidad. El abordaje dinámico integrado mediante un grupo de diagnóstico multidisciplinario que incluya expertos en neumología, reumatología, radiología y patología tiene el potencial de impactar en la identificación, las estrategias de manejo y los desenlaces. Aunque es necesario reconocer tempranamente a los pacientes con mayor riesgo, en la actualidad no se cuenta con biomarcadores confiables. Las estrategias de manejo farmacológico se basan en la inmunomodulación, pero la evidencia para su uso es de baja calidad. Promover el entrenamiento y la sensibilización del personal de salud podría reducir los retrasos en el acceso a una evaluación especializada. ABSTRACT Sjögren’s syndrome is a systemic autoimmune disease with a high burden for the individual, as well as society. Pulmonary compromise presents with a myriad of manifestations that influence patient quality of life and mortality risk. An integrated dynamic approach by a multidisciplinary diagnostic discussion team that includes experts in chest diseases, rheumatology, radiology, and pathology has the potential to improve the identification, management strategies, and outcomes. Although early recognition of patients at high risk is essential, there is currently a lack of reliable biomarkers. Pharmacological therapies are based on immunomodulation, although the evidence to support their use is of low quality. Increasing awareness and training among healthcare professionals may reduce a delayed access to specialized assessment.

Las inmunoglobulinas son proteínas heterodiméricas compuestas de 2 cadenas pesadas y 2 cadenas ligeras. La inmunoglobulina G humana es un derivado del plasma y contiene más de 95 % de IgG. La composición de las subclases de IgG es similar a la del plasma humano normal. El tratamiento con inmunoglobulina comenzó hace más de 50 años y su uso se ha descrito en numerosas enfermedades. En Colombia, la importancia de este recurso inmunomodulador condujo a la necesidad de contar con una guía de práctica clínica para su uso, para lo cual se reunió un grupo multidisciplinario de expertos, quienes se distribuyeron en mesas de trabajo, por especialidad, para redactar un texto base. Se llevaron a cabo búsquedas bibliográficas sistemáticas; las evidencias identificadas se valoraron y clasificaron para sustentar un texto preliminar que fue discutido, analizado y corregido. Se emitieron recomendaciones de uso de la inmunoglobulina intravenosa en patologías que abarcan inmunodeficiencias primarias y secundarias, enfermedades autoinmunes, alteraciones neurológicas, infecciones, trasplantes y enfermedades misceláneas; se asignaron calificaciones según el sistema GRADE para cada una. El resultado final se tradujo en las recomendaciones que se presentan con la finalidad de informar, orientar y apoyar en el uso óptimo de dicho recurso inmunomodulador.

Resumen Introduccion Los instrumentos de tamizaje de artritis psoriasica son una herramienta util en la consulta dermatologica, para optimizar la remision al reumatologo. La herramienta Toronto Psoriatic Arthritis Screening Questionnaire (ToPAS) ha sido desarrollada y validada en Canada. Objetivos Traducir y aplicar algunas pruebas de validacion de la escala ToPAS para ser utilizada en la consulta dermatologica en Colombia. Metodos Estudio de traduccion y validacion de escalas. Resultados Se llevo a cabo la traduccion y validacion del ToPAS con una metodologia estandarizada, obteniendo un documento en espanol con el cual se realizo una prueba piloto del documento final en 20 pacientes con diagnostico de psoriasis y artritis psoriasica, con el fin de evaluar los items en cuanto a aspectos como comprension, ambiguedad y tiempo de respuesta. Posteriormente, el instrumento se aplico a 108 pacientes, 65 (60,2%) eran hombres, 36 (33,3%) tenian diagnostico de artritis psoriasica y 72 (66,6%) solo psoriasis. Se considero como punto de corte un puntaje > 8 puntos, encontrando una sensibilidad del 75% y una especificidad del 92%, valor predictivo positivo del 82% y valor predictivo negativo del 88%. Para evaluar la confiabilidad test-retest se seleccionaron los ultimos 25 participantes, calculandose el coeficiente de correlacion de Pearson para evaluar la correlacion entre la primera y la segunda aplicacion del ToPAS; el valor de p fue de 0,94, valor que representa un alto nivel de correlacion entre la primera y la segunda aplicacion. Conclusiones Se realizo la traduccion, validacion y evaluacion de caracteristicas operativas de la encuesta ToPAS para deteccion de artritis psoriasica, constituyendose en una herramienta practica para uso en la consulta dermatologica.

Resumen Introducción: Los instrumentos de tamizaje de artritis psoriásica son una herramienta útil en la consulta dermatológica, para optimizar la remisión al reumatólogo. La herramienta Toronto Psoriatic Arthritis Screening Questionnaire (ToPAS) ha sido desarrollada y validada en Canadá. Objetivos: Traducir y aplicar algunas pruebas de validación de la escala ToPAS para ser utilizada en la consulta dermatológica en Colombia. Métodos: Estudio de traducción y validación de escalas. Resultados: Se llevó a cabo la traducción y validación del ToPAS con una metodología estandarizada, obteniendo un documento en español con el cual se realizó una prueba piloto del documento final en 20 pacientes con diagnóstico de psoriasis y artritis psoriásica, con el fin de evaluar los ítems en cuanto a aspectos como comprensión, ambigüedad y tiempo de respuesta. Posteriormente, el instrumento se aplicó a 108 pacientes, 65 (60,2%) eran hombres, 36 (33,3%) tenían diagnóstico de artritis psoriásica y 72 (66,6%) solo psoriasis. Se consideró como punto de corte un puntaje > 8 puntos, encontrando una sensibilidad del 75% y una especificidad del 92%, valor predictivo positivo del 82% y valor predictivo negativo del 88%. Para evaluar la confiabilidad test-retest se seleccionaron los últimos 25 participantes, calculándose el coeficiente de correlación de Pearson para evaluar la correlación entre la primera y la segunda aplicación del ToPAS; el valor de p fue de 0,94, valor que representa un alto nivel de correlación entre la primera y la segunda aplicación. Conclusiones: Se realizó la traducción, validación y evaluación de características operativas de la encuesta ToPAS para detección de artritis psoriásica, constituyéndose en una herramienta práctica para uso en la consulta dermatológica. Abstract Introduction: Psoriatic arthritis screening tools are useful for the rheumatologist in optimising referrals to the dermatology clinic. The Toronto Psoriatic Arthritis Screening Questionnaire (ToPAS) is a tool that has been developed and validated in Canada. Objectives: To translate, and perform some validation tests on TOPAS for use in dermatology clinics in Colombia. Methods: Translation and validation of scales. Results: ToPAS was translated using standardised methodology. A pilot test was conducted on 20 patients diagnosed with psoriasis and psoriatic arthritis using the final version of the document translated into Spanish, in order to evaluate items of aspects such as understanding, ambiguity, and response time. The tool was then used on 108 patients, including 65 (60.2%) males, with 36 (33.3%) cases with a diagnosis of psoriatic arthritis, and 72 (64.7%) with psoriasis only. Using a cut-off score > 8 points, it showed a sensitivity of 75% and specificity 92%, positive predictive value 82% and negative predictive value of 88%. To assess the reliability test re-test, 25 participants were selected and Pearson correlation coefficient was calculated to evaluate the correlation between the first and second application of ToPAS, which gave a value of P = .94, which represents a high level of correlation between the first and second application. Conclusions: A translation, validation and evaluation of operational characteristics of the ToPAS questionnaire was conducted, being a practical tool for use in the dermatology clinic.

Resumen La terapia antifactor de necrosis tumoral se ha convertido en los ultimos anos en uno de los pilares fundamentales para el tratamiento de la artritis reumatoide. El adalimumab es un anticuerpo monoclonal humanizado empleado en el tratamiento de la artritis reumatoide. Se describe un caso de lupus cutaneo inducido por adalimumab.

Tumor necrosis factor inhibitors have become one of the most important treatments of rheumatoid arthritis. Adalimumab is a monoclonal antibody used for the treatment of this condition. A case is described of adalimumab induced cutaneous lupus.

Resumen Objetivo Describir la prevalencia de enfermedades reumaticas en una cohorte de pacientes con infeccion por VIH y sus caracteristicas demograficas y clinicas. Pacientes y metodos Estudio descriptivo de corte transversal de una cohorte de 1.712 pacientes con infeccion por VIH, en un hospital universitario, seguidos desde enero del 2005 hasta septiembre del 2013. Resultados Se encontro una prevalencia de enfermedades reumaticas del 5,2% (n = 89 casos), el 24% de sexo femenino y el 76% de sexo masculino. Edad promedio ± desviacion estandar 45 ± 11 anos. Las enfermedades encontradas fueron: artritis reactiva 15% (n = 14), artrosis 15% (n = 14), purpura trombocitopenica inmune 11% (n = 10), otras 59% (n = 53). El tiempo promedio entre el diagnostico de VIH y la condicion reumatica fue de 73 ± 66 meses. Dentro de las comorbilidades se encontraron: dislipidemia 39% (n = 43), hepatitis B 17% (n = 19), lipodistrofia 11% (n = 12), herpes zoster 10% (n = 11) e hipotiroidismo 9% (n = 10). Conclusiones Se presenta informacion respecto a las comorbilidades reumaticas en una cohorte de pacientes con infeccion por VIH. Como se ha documentado en otras series, la artritis reactiva fue la enfermedad reumatica mas frecuente junto con osteoartrosis. Nuestro estudio evidencia una tendencia hacia el envejecimiento de los pacientes con VIH gracias a un mejor control de la enfermedad, con el uso de terapia antirretroviral, lo cual aumenta la prevalencia de osteoartrosis.

Resumen La amiloidosis secundaria es una complicacion de enfermedades inflamatorias cronicas, sin embargo, no es frecuente su asociacion con lupus eritematoso sistemico, siendo pocos casos los reportados en la literatura. Se describe un caso de una paciente de 50 anos con lupus eritematoso sistemico, quien presenta linfadenopatias cervicales con compresion extrinseca traqueal, en las que se documenta amiloidosis secundaria.

a b s t r a c t Serositis is one of the clinical findings in patients with systemic lupus erythematosus. When is present generally causes chronic pericardial effusions and not generate hemodynamic instability in the patient. We present two cases of women with lupus with pericardial effusion leading to cardiac tamponade, unusual presentation of this clinical manifestation in systemic lupus erythematosus

Resumen Objetivo: Describir un caso clínico Metodología: reporte de caso Resultados: Mujer de 36 años de edad quien consulta por cuadro clínico de ocho meses de evolución consistente en dolor abdominal, asociado a diarrea y emesis, siendo manejada inicialmente como cuadro de colon irritable. Ante la persistencia del dolor solicitan estudios imagenológicos con hallazgos sugestivos de masa retroperitoneal por lo cual remiten para valoración por cirugía. Al examen físico se encuentra dolor a la palpación abdominal generalizada sin adenopatías o masas. Paraclínicos con resonancia abdominal evidenciando masa sólida retro peritoneal de localización para aórtica izquierda y espacio inter aorto-cavo, englobando de forma circunferencial la aorta abdominal a la emergencia de arterias renales comprometiendo tejidos adyacentes, con disminución del tamaño del riñón izquierdo, compensando con hipertrofia del riñón derecho. Marcadores tumorales negativos, colonoscopia sin evidencia de masas. Es llevada a biopsia guiada por TAC con patología conclusiva de fibrosis retroperitoneal idiopática. Conclusión: Se presenta un caso de fibrosis retrooperitoneal idiopática, enfermedad de muy baja frecuencia de presentación en la práctica clínica diaria Abstract Objective: To describe a clinical case Methodology: Case report Results: 36-year-old female who consulted for clinical history of eight-month of abdominal pain, diarrhea and emesis, was threated initially as irritable bowel disease. The continuing pain request imaging with findings suggestive of retroperitoneal mass, therefore referred for evaluation for surgery. On physical examination as positive findings abdominal tenderness without generalized lymphadenopathy or masses. MRI shows abdominal retroperitoneal solid mass localized to left aortic aorto-cava intercostal space, encompassing a circumferential abdominal aorta to the emergence of compromising renal arteries adjacent tissues, decreasing the size of the left kidney hypertrophy compensating right kidney. Negative tumor markers, colonoscopy without evidence of masses. It is carried by CT-guided biopsy pathology idiopathic retroperitoneal fibrosis conclusive. Conclusion: We present a case of idiophatic fibrosis retroperitoneal, disease of low frequency in daily practice

Resumen Se presenta el caso clinico de una mujer de 35 anos con infeccion por VIH, desde hace 6 anos, en manejo antirretroviral, sin antecedentes de enfermedad reumatica previa, quien presenta cuadro de poliartralgias de predominio en manos, asociado a sinovitis, con elevacion de reactantes de fase aguda, factor reumatoide y anti CCP positivos, configurandose diagnostico de artritis reumatoide. Esta enfermedad tiene una baja incidencia en el contexto de infeccion por retrovirus y se considera asociada al sindrome de reconstitucion inmune posterior a terapia antirretroviral. Se inicia manejo con farmacos modificadores con lo cual se obtiene control de la enfermedad autoinmune.

La dermatomiositis es una miopatía inflamatoria con afectación cutánea que se ha encontrado asociada con procesos neoplásicos. El artículo presenta el caso de una paciente de 74 años de edad, quien posterior al diagnóstico de dermatomiositis consultó por síntomas de disfagia, asociada con una significativa pérdida de peso. Dadas descripciones previas de la relación de dermatomiositis con cáncer, se decidió iniciar búsqueda de malignidad asociada y se encontró un proceso neoplásico avanzado, con causa primaria desconocida, que requirió manejo paliativo. Por la mayor incidencia de malignidad en pacientes con dermatomiositis, comparada con la población general, es de vital importancia atender a los signos descritos como predictores de la asociación y no pensar en estas como entidades aisladas.

We report the case of a 70-year-old male with chronic abdominal pain, who presented with increased intensity of the pain, and was initially diagnosed and treated for acute biliary pancreatitis. However, the symptoms persisted after hospital discharge, and he was subsequently studied with cholangio-MRI, abdominal CT scan, and ERCP, which revealed dilation of the biliary tract and a mass in the head of the pancreas. An excisional biopsy of the lesion showed chronic inflammation with severe sclerosing fibrosis and a lymphoplasmacytic infiltrate. A diagnosis of autoimmune pancreatitis was made, and the patient was initiated on azathioprine with good clinical response.

Chronic urticaria (CU) has a widespread spectrum on causal or exacerbating factors, clinical manifestations, therapeutic response and quality of life affectation. Registries are useful tools in several real-life diagnosis and management approach.We aimed to evaluate the characteristics of CU patients living in Latin America through an original cross-sectional registry with data entered by regional allergologists. Results: Three hundred patients were included, being 72% female, with median age of 36 years (1–85) and 20 months of CU median evolution time. The cause of CU was reported as unknown in 72% of them.Thirty-nine percent of suspected cases presented positive serology for Mycoplasma, positive autologous serum skin test (ASST) was reported in 47%, and occasional presence of thyroid or antinuclear autoantibodies and parasites. The impact of pruritus in their quality of life was moderate to severe in 60% of patients, with almost 3 out of four patients having partial or lack of urticaria control with anti-histamines. Conclusions: Our registry provides retrospective data on the real-life assistance of a large number of patients from the region. Continuous search for associated conditions and better treatment possibilities are needed, in order to control the significant impact on quality of life and the length of disease. Keywords: Chronic urticaria, Registry, Latin America, Evaluations, Management

Resumen Presentamos el caso clinico de una mujer de 50 anos con cuadro de dermatomiositis, relacionado cronologicamente con la picadura de avispas. La dermatomiositis es una miopatia inflamatoria sistemica, de etiologia idiopatica, relacionada con debilidad muscular proximal prominentemente y asociada con lesiones cutaneas caracteristicas. El tratamiento radica en el control de la actividad inflamatoria y, en general, el uso de esteroides en las fases tempranas; de acuerdo con la severidad y el compromiso, se requiere el uso de otros inmunomoduladores. Se presenta el segundo caso reportado en la literatura de dermatomiositis posterior a picadura de himenoptero.

Resumen Colombia esta viviendo 2 fenomenos importantes, las transiciones demografica y epidemiologica, los cuales se ven reflejados en un proceso acelerado de enveje cimiento poblacional que, a su vez, aumenta la prevalencia de enfermedades cronicas, entre ellas la artritis reumatoide (AR). De acuerdo con la edad de aparicion, la AR se clasifica en 2 subgrupos: la enfermedad reumatoide de aparicion temprana y la AR de aparicion tardia (en pacientes > 65 anos). Anteriormente, se pensaba que la diferencia mas importante entre los 2 subgrupos era que la AR de aparicion tardia tenia un mejor pronostico que la enfermedad reumatoide de aparicion temprana. En la actualidad, se han descrito las diferencias que existen entre estas 2 formas de presentacion de la AR. A traves de este articulo se describiran las principales diferencias que existen en cuanto a la epidemiologia, patogenia, manifestaciones clinicas, tratamiento, pronostico y funcionalidad de los pacientes con AR de presentacion temprana y tardia.

Resumen El sindrome de Felty es la triada de artritis, esplenomegalia y neutropenia. Adicionalmente, se pueden encontrar en este sindrome multiples factores propios de la artritis reumatoide, entre ellos la presencia de nodulos pulmonares. Presentamos el caso de un paciente con hallazgos clinicos y paraclinicos que evidencian la presencia del sindrome de Felty, presentando, ademas, nodulos pulmonares en los estudios radiologicos e histologicos que documentaron granulomas cronicos, bronquiolitis obliterante y neumonia organizante.

Resumen La educacion superior en Colombia ha sufrido una importante transformacion desde diciembre de 1992, cuando entra en vigencia la ley 30. Los programas de educacion en ciencias de la salud y en especial los de pregrado en medicina no fueron ajenos a estos cambios y se presento una proliferacion de facultades y escuelas de medicina a lo largo y ancho del pais. Las criticas desde diversos sectores no se han hecho esperar y los entes gubernamentales han buscado diversos mecanismos para garantizar la calidad de los procesos de formacion y por ende de los futuros medicos. En el presente estudio se analiza la evolucion de la oferta de programas de pregrado en Colombia durante los ultimos 30 anos.

Resumen Objetivo Describir las caracteristicas clinicas, paraclinicas y manejo de la enfermedad de Still del adulto (E.S.A.). Metodos Analisis retrospectivo de una cohorte de 24 pacientes. Resultados Las caracteristicas clinicas fueron: fiebre (100%), rash (79.1%) y artritis (66.66%). Leucocitosis (75%) fue la alteracion paraclinica mas frecuente, seguida por un incremento de la PCR, VSG y ferritina en 70.8%, 66.65% y 62.5%, respectivamente. El 50% de los pacientes presentaron un curso monociclico; el 33%, policiclico sistemico y el 17%, policiclico articular. No se presentaron casos de monociclico poliarticular. Se evidenciaron manifestaciones inusuales de enfermedad tales como: urticaria en 7 pacientes (29%); enfermedad pulmonar intersticial, en 6 pacientes (25%); meningitis aseptica, 4 pacientes (16.6%) y SDRA, en 4 pacientes (16.6%). Por su parte, todos los pacientes recibieron esteroides y 9 de ellos (37.5%) necesitaron tratamiento con pulsos de metilprednisolona. Dentro de las drogas modificadoras, el metotrexate fue el medicamento mas usado en 15 casos (62.5%), seguido por cloroquina sola o combinada en 11 pacientes (45.8%). Ademas, 3 pacientes (12.5%), requirieron tratamiento con terapia anti TNF por pobre respuesta a la terapia convencional. Fallecieron 2 pacientes (8.4%) asociados a cuadros de SDRA. Conclusiones La ESA tiene un amplio y dificil diagnostico diferencial a pesar de manifestaciones clasicas. La presencia de expresiones clinicas poco usuales puede retardar el diagnostico y tratamiento de ESA. La ESA no es una enfermedad benigna y puede cursar con alta mortalidad cuando se asocia a manifestaciones pulmonares severas.

Resumen Se presenta el caso de una paciente de 67 anos, natural y procedente de Santa Ana (Boyaca), con dermatopolimiositis, en la ciudad de Tunja (Boyaca, Colombia), con inmunosupresores, Prednisolona, Metotrexate. A los tres meses del diagnostico presenta un cuadro infeccioso pulmonar catalogado como neumonia necrotizante de lobulo superior derecho, la cual progresivamente lleva a la paciente a falla ventilatoria hipercapnica y sepsis de origen pulmonar. Se realiza lavado bronco-alveolar demostrando la presencia de Histoplasma capsulatum; de manera posterior la paciente desarrolla shock cardiogenico y fallece .

a b s t r a c t Gout is a disease characterized by acute episodes of pain, which occurs as the result of monosodic urate crystal deposit in the joint and periarticular tissue. In some cases, gout behaves as a severe inflammatory arthopathy that is difficult to manage, generating structural joint damage and functional impairment. We report the case of a 44 years old man with gouty arthritis for 12 years, not responding to NSAIDs, alopurinol, colchicine or corticosteroids. Tocilizumab was started with favorable clinical and laboratory results after treatment.

Antisynthetase antibody syndrome is a rare autoimmune disease that may present with variable systemic manifestations, mainly polymyositis, interstitial lung disease, skin lesions, and Raynaud’s phenomenon. This diagnosis should always come to mind in patients that present with signs of myositis, dermatomyositis, or polymyositis associated with interstitial lung disease. On the following paper, we report the case of a 52-year-old man who presented with a 2-month history of asymmetric polyarthralgia, myalgia, weight loss of 8 kg, and progressive muscle weakness associated with dyspnea, orthopnea, and dysphonia. Further tests revealed myositis, interstitial pneumonia, and elevation of anti-Jo-1 antibodies. A diagnosis of antisynthetase antibody syndrome was made and the patient showed good response to treatment with corticoids and methotrexate. Finally, we present a short review of the literature.

Gout is a disease characterized by acute episodes of pain, which occurs as the result of monosodic urate crystal deposit in the joint and periarticular tissue. In some cases, gout behaves as a severe inflammatory arthopathy that is difficult to manage, generating structural joint damage and functional impairment. We report the case of a 44 years old man with gouty arthritis for 12 years, not responding to NSAIDs, alopurinol, colchicine or corticosteroids. Tocilizumab was started with favorable clinical and laboratory results after treatment.

Throughout history and according to changes in society have noted that they have used different formulas for employers to ensure the sustainability of organizations. These formulas in many cases there are specific companies, but imitate those who have been successful. In other cases, models have emerged within organizations as a result of environmental monitoring. Currently we are facing a new environment where globalization and rapid change have become a constant.According to research conducted in 2008 by the GEM (Global Entrepreneurship Monitor) which measures the rate of entrepreneurial activity, Colombia ranks third after Bolivia and Peru. However, we also found that many companies end their activities in Colombia before his first five years (Global Entrepreneurship Monitor, 2009).Hence, it is vital for companies to find tools that give items to businessmen and entrepreneurs to have wider vision of a sector and thus make better decisions. Structural analysis of strategic sectors (AESE) meets financial and nonfinancial information to achieve the vision mentioned (Roatta Acevedo, 2006).Ready desserts sector in Colombia has not been investigated and many entrepreneurs choose to opt for this option as a business informally, with few companies that remain in time.

Presentamos el caso de una paciente quien ingresa por la presencia de lesiones petequiales, artralgias y una úlcera en miembro inferior. La presencia de azoados en ascenso progresivo se documenta durante su estancia. Se instaura manejo con corticoide IV sin respuesta y se obtienen mejoría con plasmaféresis. Posteriormente se observa la presencia de deterioro del patrón respiratorio, disnea sumado a la presencia de compromiso pulmonar en parches por medio de tomografía de tórax y con evidencia de hemorragia alveolar. Se descartan causas autoinmunes e infecciosas, así como neoplasias hematológicas. Se concluye que la presencia de crioglobulinemia es la única causa de dicha entidad.

Background The Human Leukocyte Antigen HLA B 27; is a class I antigen from MHC that is strongly associated with ankylosing spondylitis (AS) and other spondyloarthritis (SPA), with positivities for HLA-B27 in the population from 80 to 95% worldwide. The prevalence of HLA-B27 in the general population varies by geographic area, with ratios ranging between 2-4% in African/African American compared to 8% in Caucasian populations 1 Objectives To determine the proportion of positive HLA B 27 in the population of transplanted and donors treated at the San Ignacio University Hospital; their clinical characteristics and relationship to the presence of spondyloarthritis. Methods It was performed a descriptive cross-sectional study of 465 individuals typed for HLA of a cohort of transplants in a university hospital in Colombia including donors and receivers. Designing a database in Excel to include data relevant to the study. The search was performed in hospital records and telephone contact for family not treated at the hospital to extract information. Results 465 individuals with an age range between 10 and 80 years with a mean of 44.9 years (SD: 14.04); finding 21 positive cases for HLA B 27 indicating a prevalence of 4.5% positivity for HLA-B27 in this population. At evaluating the behavior by gender, a proportion of HLA B 27 of 6,5% was found in the group of men (n=261; 56.1%) compared with 2.0% in women. Of the 21 patients positive for HLA B 27 it was found that 4 of them (19%) had joint pain of small and large joints so they were referred to evaluation by the Rheumatology service at Hospital where the presence of spondyloarthritis or other autoinmune disease was discarted. Conclusions It has been described in the literature the prevalence of HLA B 27 in ranges from 2% to 8%, according to the region evaluated; it was found in our study a ratio of 4.5% of the total population and comparing by gender a higher proportion in males (6.5% compared with 2% in women). Spondylitis or other autoimmune disease were not detected in any patient of the series evaluated in our study; although 19% of the populatinon with positive HLA B 27 was referred to rheumatology for articular symptoms discarding the presence of it. San Ignacio Universitary Hospital is a reference center of the city to perform transplants, so it is important to emphasize the prevalence found in our study that fits within international parameters reported. Disclosure of Interest None declared

Background Rheumatoid Arthritis (RA) is a systemic inflammatory disease where its physiopathology is based on immunological activity. It has been determined that an early therapy with Disease-Modifying Antirheumatic Drugs (DMARDs) stops the progression of this disease. One of the main limitations to achieve this objective is guarantee compliance to DMARDs. Several methods have been designed to measure compliance such as CQR that provides quick and concise information about medication compliance. Objectives To develop an initial linguistic and psychometric validation of the CQR questionnaire in patients with RA. Methods A validation study was conducted at San Ignacio University Hospital. Steps suggested by ISPOR Task Force for Translation and Cultural Adaptation were followed, and a final Spanish version for Colombia was created with semantic and content equivalence to the original version. Data collection was performed in four outpatient rheumatology wards. The data collection form completed by the patients included demographic data, CQR19, analog scale of compliance, adverse effects associated with the medication; while doctors registered DAS-28, rheumatoid factor, anti-citrulline antibodies and current treatment. Psychometric validation was evaluated from a hypothesis of correlation between compliance (CQR score) and the level of activity of the disease (DAS-28). The association between compliance, the presence of adverse events, and the amount of medication was also evaluated. Results CQR19 was completed by 233 patients with RA diagnosis (Female 75.1%) taking some type of pharmaceutical treatment (single or combined), with a mean of 11.3 years (SD 9.6) into the disease. Correlation between CQR19 score and DAS-28 activity categories was -0.64 (95% CI: -0.71to-0.56). Despite this result, correlation between CQR19 categories [satisfactory compliance (>80%) and unsatisfactory compliance (≤80%)] and DAS-28 was -0.16 (95% CI: -0.28 to -0.03). 97.8% of the patients were classified as satisfactory compliance. All analyses were conducted with Spearman9s rank correlation coefficient based on Fisher transformation. Exploratory analysis showed that association between CQR19 score and adverse events was -0.05 (95% CI: -0.18to0.08), while the association between CQR19 score and the amount of medication was -0.34 (95% CI: -0.45to-0.22). Conclusions CQR19 score relates to DAS-28 categories activity indicating that patients with less activity have higher compliance scores. However, this relationship was not evident when applying the compliance categories established by the CQR19 authors (compliance limit of 80%). Validations of CQR19 scores as a measure of compliance do not allow using this instrument in our local clinical practice in a regular basis. Further studies are necessary to evaluate the ability of CQR19 scores to indicate compliance to the treatment beyond just taking the medication in patients with RA. We suggest exploring other sources of evidence to validate this questionnaire. References De.Klerk E, et al. The compliance-questionnaire-rheumatology compared with electronic medication event monitoring: a validation study. J. Rheumatol. 30, 2469–2475 (2003). Disclosure of Interest None declared

Eighteen pediatric liver transplant recipients were converted from cyclosporine-based immunosuppression to tacrolimus for refractory rejection episodes affecting 21 grafts. Before conversion, steroid boluses were applied to all episodes followed by OKT3 monoclonal antibodies in 3 of them. Baseline biopsy showed cellular rejection in 18 patients and ductopenia in 3 cases. Thirteen episodes initiated within the first 2 postoperative weeks, and 8 occurred beyond the 21st day. A previous steroid-responsive episode of rejection was noted in 4 patients. Tacrolimus was administered by the oral route to obtain trough blood levels in the range 6–15 ng/ml. Reversal of rejection was obtained in 15 patients (71.4 %). Complete normalization of liver function tests was achieved in 10 out of 12 patients who were followed for more than 6 months. A refractory evolution affected 6 patients (28.5 %). Significant factors predictive for tacrolimus-resistant rejection were identified as ductopenia on baseline biopsy, previous episodes of acute rejection, late onset rejection (beyond 21st posttransplant (day), and a longer time of evolution of rejection prior to conversion.

Up to 25% of babies with biliary atresia (BA) bear associated malformations that most often cluster in the polysplenia syndrome (PS). This article examines the impact of associated PS on the final outcome of the authors' BA patients and the necessary modifications of surgical technique during orthotopic liver transplantation (OLT) in these patients. The authors studied 88 consecutive infants with biliary atresia operated upon during the 10-year period between 1984 and 1993. Biliary drainage was provided by a Roux-en-Y hepaticojejunostomy. OLT was performed in 38. Eleven infants (12%) had three or more components of PS: malrotation (11), preduodenal portal vein (11), polysplenia (10), situs inversus (4), absent inferior vena cava (3), cardiac defects (2), and anomalous hepatic artery supply (2). Bile drainage was achieved in 7/11 infants (63% versus 48% for regular BA patients) 5 of whom (45% versus 31%) are jaundice-free with a mean follow-up of 105 months. Three patients received transplants (2 from living-related donor) and are alive and well with normal liver function after a mean follow-up of 35 months. Overall survival was 72% in the BA plus PS and 66% in the BA group (P = not significant [NS]). Results show that long-term bile drainage in these PS patients can be achieved at least as often as in other BA patients (45% versus 31%) (P = NS) and indicate that Kasai operation remains the best initial choice. When OLT becomes necessary, attention to vascular anatomy is required to determine the feasibility of reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Background The systemic lupus erythematosus is an autoimmune chronic disease that affects most of the body systems including the hematologic system, being a prognostic marker in terms of morbidity and mortality. However, little is known about the specific repercussion of the disease in each one of the cell lines, and its physiopathological mechanisms or the aspects that can be used as predictors of the damage remain unclear. Objectives To analyze the Flow cytometry findings and Bone Marrow in adult patients with hematological manifestations and systemic lupus erythematosus (SLE). Methods A descriptive retrospective study was performed, evaluating the bone marrow findings (aspiration, biopsy and flow cytometry) of 15 adult patients that had anemia or leukopenia in monthly hemograms in a six-month period that had diagnostic criteria for SLE. Results The average age was 39 years with a standard deviation of 12.9 years. The minor age was 21 years and the oldest age 64 years. 93% of the subjects were women, 93% of them had positive ANAS, 53% had positive AntiDNA, arthralgia and photosensitivity were present in 93% of the patients, sores in 100%, renal compromise evidenced by hematuria, proteinuria; nitrate elevation in 46%, serositis in 12%, antiphospholipid antibodies were present in 20% of the subjects, anti Smith were not evidence in any one of the cases, Anti Ro were positive in 66% of the cases, anti RNP in 6%. 20% of the subjects were in treatment with steroids, 26% with hydroxicloroquine and 6% were in therapy with rituximab. The average number of leucocytes was 2972, lymphocytes 1660, neutrophils 1237, platelets 167606, average hemoglobin concentration was 10,8. Megaloblastic changes were present in 6%, erythroid hyperplasia in 20%, immature cells were found in 33% of the cases, chromatin accumulation in 33%, augmentation of reticular cell concentration in 12%, hypocellularity in 33%, inversion of myeloid erythroid rate in 26% of the subjects, immature myeloid lines in 13%, megakariocyte augmentation in 6%. CD8 T lymphocytes were present in 93% of the cases; average number of promyelocytes (CD13++/CD11b-) was 4,86; myelocytes (weak CD13+/CD11b-) 25,6; metamyelocyte bands (CD13+/CD11b+) 37,5; mature neutrophils (CD13++/CD11b+) 33,2; monocytes 3,13; eosinophils 1, T lymphocytes 9,53; CD4 2,86; CD8 6,13; CD4-CD8- 0,73; NK cells 0,06; CD34+ myeloid cells 0,93. Conclusions Bone marrow findings in 15 patients were studied. It is important to note that between ENAs, there was a high frequency of positive Anti Ro antibodies (66%). In the flow cytometry findings there was a strong depletion in B-lymphocytes with a mild augmentation of T-lymphocytes, especially CD34, in comparison to the data found in healthy subjects. Bone marrow aspiration findings didn’t evidence any relevant alterations, but bone marrow biopsy findings showed frequent alteration of nuclear chromatin, positive Prussian blue staining, and augmentation of reticular cell concentration, but the meaning of these findings has not been elucidated. Other relevant results were the hypocelularity and immature B-cells. References Nossent JC, Swaak AJ. Prevalence and significance of hematologic abnormalities in patients with systemic lupus erythematosus. 1991. Q. J. Med. 80, 605. Disclosure of Interest None Declared