Amicrobial pustulosis of the folds: report of two cases / Pustulosis amicrobiana de los pliegues. Informe de dos casos

ABSTRACT Amicrobial Pustulosis of the Folds is a relapsing, chronic and rare neutrophilic dermatosis, characterized by papulopustular, eczematous and aseptic lesions on skin folds. This disorder usually occurs predominantly in females (30 years of age average) with a history of an autoimmune disorder, especially systemic lupus erythematosus. There is no standard therapy, but systemic corticosteroids, alone or in combination with other immunosuppressive drugs, are usually the first-line therapy. We report two females aged 37 and 20 years with the disease but without associated autoimmune diseases. They were successfully treated with non-steroidal treatments.

La pustulosis amicrobiana de los pliegues es una dermatosis neutrofílica crónica, recurrente y poco común. Se caracteriza por lesiones pápulo-pustulosas, eczematosas y asépticas de los pliegues cutáneos. Este cuadro se presenta predominantemente en mujeres de alrededor de 30 años con enfermedades autoinmunes, especialmente lupus eritematoso sistémico. No existe un tratamiento estándar pero los corticoides solos o con inmunosupresores se usan de primera línea. Informamos dos mujeres de 27 y 20 años sin patología autoinmune, con la enfermedad. Ellas fueron tratadas exitosamente sin usar esteroides.

COVID-19, aterosclerosis y la respuesta inflamatoria en la enfermedad reumática / COVID - 19, atherosclerosis and the inflammatory response in rheumatic disease

Introducción: La enfermedad por el coronavirus 2019 (COVID-19) es causada por el síndrome respiratorio agudo severo coronavirus-2 (SARS-CoV-2). Además de la invasión tisular directa, el SARS-CoV-2 también puede provocar una respuesta inmune exagerada del huésped, que con frecuencia conduce a una tormenta de citocinas, que contribuye, significativamente, a la disfunción multiorgánica. Las enfermedades autoinmunes se caracterizan por un estado proinflamatorio, aterosclerosis acelerada e incremento de la incidencia de complicaciones vasculares por lo que en estos pacientes existe un alto riesgo de complicaciones ante la presencia del SARS-CoV-2. La alteración en la regulación del sistema inmunológico, el aumento de la demanda metabólica y la actividad procoagulante probablemente explican parte del mayor riesgo de resultados adversos en las personas con enfermedad aterosclerótica relacionada con la COVID-19. Objetivo: Actualizar los conocimientos del personal de salud en el manejo de estas enfermedades. Método: Se realizó una búsqueda de artículos en PubMed, SciELO y Google Scholar con estrategia de vocabulario controlado, de diciembre 2019 a noviembre 2020. Se seleccionaron un total de 50 artículos. Desarrollo: La elevada producción de autoanticuerpos ya sea secundaria a la enfermedad reumática o por una infección intercurrente, puede estar implicada en el desarrollo de la disfunción endotelial, la progresión y la ruptura de la placa aterosclerótica. Conclusiones: Se ha demostrado que estas enfermedades crónicas autoinmunitarias se asocian a una elevada morbimortalidad cardiovascular por lo que el médico de estos tiempos tiene que ser capaz de reconocer estos pacientes(AU)

Introduction: Coronavirus disease 2019 (Covid-19) is caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). In addition to direct tissue invasion, SARS-CoV-2 can also elicit an exaggerated host immune response, often leading to a cytokine storm, significantly contributing to multi-organ dysfunction. Autoimmune diseases are characterized by a pro-inflammatory state, accelerated atherosclerosis and an increased incidence of vascular complications, which is why there is a high risk of complications in the presence of SARS-CoV-2 in these patients. Altered immune system regulation, increased metabolic demand, and procoagulant activity likely explain part of the increased risk of adverse outcomes in people with Covid-19-related atherosclerotic disease. Objective: To update the knowledge of health personnel in the management of these diseases. Method: We reviewed articles in PubMed, SciELO and Google Scholar with a controlled vocabulary strategy, from December 2019 to November 2020. We selected a total of 50 articles. Findings: The high production of autoantibodies, whether secondary to rheumatic disease or due to intercurrent infection, may be involved in the development of endothelial dysfunction, the progression and rupture of the atherosclerotic plaque. Discussion: It has been shown that these autoimmune chronic diseases are associated with high cardiovascular morbidity and mortality, so the doctor of these times must be able to recognize these patients(AU)

Subacute combined degeneration of the spinal cord with concomitant autoimmune disease: report of 2 cases

The etiology of subacute combined degeneration (SCD) of the spinal cord is closely associated with vitamin B12 (VitB12) deficiency. The clinical manifestations of SCD are complex and vary substantially. Due to some SCD patients with atypical manifestations and concomitant autoimmune disorders, the probability of misdiagnosis and missed diagnosis is still relatively high in the early stage. We report the cases of two patients who were missed or misdiagnosed at another hospital because of the normal initial VitB12 level and partial overlap of clinical manifestations, finally diagnosed as SCD with atypical manifestations and concomitant autoimmune disorders, pharyngeal-cervical-brachial Guillain-Barre syndrome in Case 1 and SCD with autoimmune thyroiditis in Case 2. After undergoing corresponding treatment, death was reported in Case 1 and improvement in Case 2. Analysis of the clinical manifestations and investigation of the underlying pathogenesis in such patients could help improve the rate of early diagnosis and allow timely treatment of SCD, thereby preventing disease progression and poor clinical outcomes.

Immune mechanisms involved in the coexistence of oral lichen planus and autoimmune thyroid diseases / 浙江大学学报·医学版

Oral lichen planus (OLP) is a chronic inflammatory oral mucosal disease with unclear etiology. Autoimmune thyroid diseases (AITD) is a type of autoimmune disease characterized by increased thyroid-specific antibodies. In recent years, more and more studies have found that the incidence of AITD is increased in OLP patients. The occurrence and development of OLP and AITD may be related to the expression of thyroid autoantigen in oral keratinocytes, the imbalance of thyroid hormone （Th）1/Th2 and Th17/Treg cell subsets, the abnormal quantity and function of follicular helper T cells and chemokines and the specific killing ability of CD8 T cells to target cells. In this article, the possible immune mechanisms involved in the coexistence of OLP and AITD are reviewed to provide insights for the diagnosis, treatment and prevention of these two diseases from the perspective of immunology.

Estrategias en el manejo y actualización de las enfermedades autoinmunes y reumáticas en la epidemia por SARSCoV-2 y la COVID-19 Sociedad Cubana de Reumatología y su Grupo Nacional / Strategies in its Management and Updating of the Autoimmune-rheumatic Diseases in the SARSCoV-2 Pandemic and COVID-19 Cuban Rheumatology Society and its National Group

Al igual que en las enfermedades autoinmunes y reumáticas, las infecciones por virus pueden ser disparadores de mecanismos inmunopatogénicos. El SARSCoV-2 puede causar la liberación de citocinas y provocar un daño tisular grave, sobre todo pulmonar, con peligro para la vida. Se suma a ello el riesgo del uso de medicamentos inmunosupresores, empleados en los protocolos de tratamiento de las afecciones autoinmunes y también contra la infección viral de COVID-19. Hoy no contamos con conocimientos y evidencias científicas suficientemente sólidas para el enfrentamiento a la COVID-19, y cómo puede impactar entre los pacientes inmunodeprimidos por afecciones reumáticas. El Grupo Nacional de Atención y Enfrentamiento a la COVID-19 de la Sociedad Cubana de Reumatología se propuso elaborar un documento científico actualizado con las bases teórico-prácticas que permita acceder al conocimiento acerca de la infección por SARSCoV-2 y la COVID-19, y su repercusión sobre los pacientes con enfermedades autoinmunes y reumáticas para esbozar una estrategia de trabajo y ofrecer recomendaciones para los reumatólogos y pacientes cubanos. Se realizó un estudio de revisión y actualización acerca de la asociación entre las enfermedades reumáticas y autoinmunes y la COVID-19. Se emplearon las palabras clave enfermedades reumáticas, lupus eritematoso sistémico, artritis reumatoide, virus SARSCoV-2 y COVID-19. Se realizó una amplia búsqueda en MEDLINE y LILACS, y se revisaron más de 150 artículos, boletines de actualización de los sitios Web, páginas de salud de Cuba, resúmenes seleccionados por su calidad metodológica, revisiones y metaanálisis sobre el tema. A partir de la información recogida, se estableció una discusión y análisis considerando las principales experiencias internacionales, criterios de expertos, experiencias previas con otros virus en el Sistema de Salud de Cuba, con la participación de su red de científicos liderados por la Sociedad Cubana de Reumatología y su Grupo Nacional y el apoyo de la comunidad de reumatólogos(AU)

The outbreak of the infection by the new coronavirus SARSCoV-2, COVID-19, in December in Wuhan Province of China, has become a pandemic and health emergency given the deficiency of antiviral therapy for the acute respiratory syndrome that generates danger to life. The debut of the epidemic was in China, then the epicenter developed in Europe, northern Italy that suffered a severe blow. Worldwide, more than 10 million people are infected with the virus that has impacted on health systems until it practically collapsed, resulting in thousands of deaths. Today the epicenter of the pandemic has shifted to the Americas. Alarming figures highlight the United States of North America with some 2,737,600 infected and more than 128,471 deaths, followed by the South American giant Brazil with 1.3 million infections and 57,659 deaths. The Caribbean has a better setting. In Cuba, by the end of June, 2,340 cases of patients infected with deaths from COVID-19 were reported. We conducted a review, analysis and evaluation study of more than 150 articles from international journals, update bulletins of the WEB sites, health pages of the MINSAP of Cuba, and summaries selected for their methodological quality, and reviews, on the subject COVID-19 and autoimmune-rheumatic diseases by MEDLINE: database prepared by the National Library of Medicine of The USA contains bibliographic references and abstracts from more than 4,000 biomedical journals published in the United States and in 70 other countries, We also use Latin American and Caribbean Center for Information on Health Sciences: System, in Latin America and the Caribbean, since 1982. Our objective and results achieved have been to develop the theoretical-practical bases in an updated scientific document that allow access in an essential and summarized way to current knowledge about the infection by SACOV-2, COVID-19, and its repercussion and impact on patients suffering from rheumatic autoimmune diseases, and thus outline a coping and action strategy with recommendations for the Cuban rheumatologists in their health care work, and for patients as a guideline, given their well-founded concerns and fears given their underlying condition and the immunosuppressive drugs prescribed in an unfavorable context of a pandemic. The information is based on international experiences with the most published scientific evidence and those treasured national experiences in the face of similar situations of epidemics, faced by the vast health system and achievements of Cuban science(AU)

Síndrome antifosfolipídico, lupus eritematoso sistémico y tromboembolismo pulmonar / Antiphospholipid syndrome, systemic lupus erythematosus and pulmonary thromboembolism. Case presentation

El síndrome antifosfolipídico o de Hughes, como también se le conoce, puede aparecer de manera aislada o asociado a otras enfermedades autoinmunes como el lupus eritematoso sistémico. La asociación de ambas entidades puede causar varias complicaciones, como el tromboembolismo pulmonar. Se presenta el caso de una paciente de 28 años de edad, con antecedentes de abortos a repetición y óbito fetal, ingresada en esta ocasión, debido a una trombosis venosa profunda del miembro superior derecho, confirmada mediante ecografía Doppler. Se comprobó el diagnóstico de síndrome antifosfolipídico secundario a lupus eritematoso sistémico, sustentado por los elementos clínicos e inmunitarios presentes. La paciente evolucionó satisfactoriamente, con el protocolo terapéutico empleado en fase aguda: heparina de bajo peso molecular del tipo clexane (enoxaparina) 1 mg/kg cada 12 h y dicumarínicos del tipo warfarina 5 mg con una razón normalizada internacional (INR) de 3. Se mantiene actualmente con una dosis de 10 mg/día e hidroxicloroquina 200 mg diarios. Conclusiones: Se resalta la importancia de diagnosticar el síndrome antifosfolipídico, ante toda paciente con abortos espontáneos o muertes perinatales inexplicables. El tratamiento debe ser multidisciplinario y se debe realizar una búsqueda sistemática de afecciones secundarias (particularmente enfermedades difusas del tejido conectivo) antes de calificar al síndrome como primario(AU)

The antiphospholipid or Hughes syndrome, as it is also known, can appear in isolation or in association with other autoimmune diseases such as systemic lupus erythematosus. The association of both entities can cause various complications, such as pulmonary thromboembolism. We present the case of a 28-year-old patient, with a history of repeated abortions and stillbirth, admitted on this occasion due to deep vein thrombosis of the right upper limb, confirmed by Doppler ultrasound. The diagnosis of antiphospholipid syndrome secondary to systemic lupus erythematosus was confirmed, supported by the clinical and immune elements present. The patient evolved satisfactorily, with the therapeutic protocol used in the acute phase, where she initially received treatment with low molecular weight heparin of the type clexane (enoxaparin) 1 mg x kg every 12 hours, and discoumarin drugs of the warfarin type, which she currently maintains at a 5mg dose with an INR of 3. Initially prednisone was placed at a dose of 1mg x kg with good therapeutic response, currently maintaining a 10mg dose. He is also currently on hydroxychloroquine 200 mg daily. Conclusions: The importance of diagnosing the antiphospholipid syndrome is highlighted in all patients with spontaneous abortions or unexplained perinatal deaths. Treatment should be multidisciplinary and a systematic search for secondary conditions (particularly diffuse connective tissue diseases) should be conducted before qualifying the syndrome as primary(AU)

Azoor: Retinopatia externa oculta zonal aguda associada com doença autoimune / Azoor: Acute Zonal Occute Outer retinopathy associated with autoimmune disease

Resumo Retinopatia externa oculta zonal aguda (AZOOR) foi descrita pela primeira vez por Gass em 1993 como uma síndrome com perda rápida de uma ou mais zonas extensas dos segmentos externos da retina. Paciente masculino, 35 anos, portador de doença de Crohn, queixando-se de dor ocular eventual e nictalopia em olho direito desde infância. Em uso regular de azatioprina e mesalazina. melhor acuidade visual 20/20 AO. À fundoscopia, lesões hiperpigmentadas em arcada temporal inferior de olho direito, em treliça, acompanhando vasculatura local. Após exclusão de diagnósticos diferenciais chegou-se ao diagnóstico de Azoor. Azoor é uma síndrome idiopática caracterizada por um quadro agudo início de fotopsia, escotoma ou ambos e é tipicamente associado a uma perda persistente de função visual que envolve uma ou mais zonas da retina externa. Apesar dos sintomas clássicos de fotopsia, o paciente em questão teve uma apresentação clínica atípica. Descrevemos um caso ocorrido em indivíduo do sexo masculino em região periférica apresentando afinamento coroidiano e com doença autoimune associada. Dessa forma, acreditamos que é necessária maior investigação para verificar a etiologia da alteração coroideana e da associação com a doença específica.

Abstract Acute zonal occult external retinopathy (AZOOR) was first described by Gass in 1993 as a syndrome with rapid loss of one or more large areas of the external retinal segments. Male, 35 years, with Crohn's disease complaining of occasional eye pain and right eye nictalopia since childhood. In regular use of azathioprine and mesalazine. better visual acuity 20/20 OU. At fundoscopy, hyperpigmented lesions in the right temporal arcade of the right eye, in trellis, accompanying local vasculature. After exclusion of differential diagnoses, Azoor's diagnosis was reached. Azoor is an idiopathic syndrome characterized by an acute onset of photopsy, scotoma or both and is typically associated with a persistent loss of visual function involving one or more areas of the external retina. Despite classic photopsy symptoms, the patient in question had an atypical clinical presentation. We describe a case of a peripheral male with choroidal thinning and associated autoimmune disease. Thus, we believe that further investigation is necessary to verify the etiology of choroidal alteration and its association with the specific disease.

Síndrome de Wilkie secundario a hipertiroidismo exógeno en una paciente con hipotiroidismo primario autoinmune: reporte de un caso y revisión de la literatura / Wilkie's syndrome caused by exogenous hyperthyroidism in a patient with primary autoimmune hypothyroidism: a case report and literature review

RESUMEN El síndrome de Wilkie o de arteria mesentérica superior es una causa poco común de obstrucción intestinal proximal, relacionada a pérdida de peso reciente. Reportamos el caso de una mujer de 19 años que se presenta a la clínica con pérdida de peso, dolor abdominal, nausea y vomito. Los exámenes de laboratorio reportaron anemia, hipoalbuminemia, hipomagnesemia y una hormona estimulante de la tiroides suprimida secundario al uso con levotiroxina. Se realizó una serie esofagogastroduodenal con datos compatibles con dilatación gástrica severa, gastroparesia y una tomografía axial computada reveló un ángulo aortomesentérico de 11,7°. Se inicio manejo conservador a base de nutrición enteral y parenteral total, siendo este el tratamiento de elección. En casos refractarios, la cirugía es una opción segura y efectiva.

ABSTRACT Wilkie's syndrome or superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction, primarily attributed to recent weight loss. We report the case of a 19-year-old woman comes to our clinic and reports weight loss, abdominal pain, nausea, and vomiting. Laboratory tests revealed anemia, hypoalbuminemia, hypomagnesemia, and a suppressed thyroid stimulating hormone secondary to levothyroxine. A barium swallow test showed gastric dilatation, delayed gastric emptying and an axial computed tomography revealed an aortomesenteric angle of 11.7°. Conservative management with total parenteral and enteral nutrition was initiated, being the first-line treatment. In refractory cases surgery is a safe and effective option.

Evaluation of adult celiac disease from a tertiary reference center: a retrospective analysis

SUMMARY OBJECTIVE It has been observed that celiac disease (CD) is not restricted to a single type characterized by diarrhea but also has atypical, asymptomatic (silent), and latent forms. The prevalence of this autoimmune disease, which affects approximately 1% of the world, is estimated to be around 3%, including atypical and asymptomatic cases. In our study, we aimed to evaluate adult celiac patients. METHODS Between December 2008-2015, patients diagnosed with CD over the age of 18 years old were included in the study. Patients' symptoms at admission, frequency and type of anemia, transaminase levels, and celiac antibody positivity, and autoimmune diseases diagnosed at follow up were evaluated retrospectively. RESULTS Of 195 patients, 151 (77.4%) were female. The mean age of the patients was 35.73 ± 12.19 years (range, 18-71 years). A hundred patients (51.3%) had gastrointestinal symptoms. At the time of admission, 118 patients (60.5%) had anemia, and 52 (26.7%) had hypertransaminasemia. During the mean follow-up period of 58 months (36-120 months), 84 (43.1%) of the patients presented at least one autoimmune disease, and this rate was 96.6% in individuals diagnosed above the age of 50 years. CONCLUSION In adult CD, resistant anemia, dyspepsia, and hypertransaminasemia are very common findings at the time of diagnosis, and the association with other autoimmune diseases, especially Hashimoto's thyroiditis, is high.

RESUMO OBJETIVOS Observou-se que a doença celíaca (DC) não se restringe a um único tipo caracterizado por diarreia, mas também tem formas atípicas, assintomáticas (silenciosas) e latentes. Estima-se que a prevalência desta doença autoimune, que afeta aproximadamente 1% da população do mundo, seja em torno de 3%, incluindo casos atípicos e assintomáticos. Em nosso estudo, objetivou-se avaliar pacientes celíacos adultos. MÉTODOS Entre dezembro de 2008 e 2015, pacientes diagnosticados como DC com idade acima de 18 anos foram incluídos no estudo. Os sintomas dos pacientes na admissão, frequência e tipo de anemia, níveis de transaminases e positividade de anticorpos celíacos e doenças autoimunes diagnosticadas no seguimento foram avaliados retrospectivamente. RESULTADOS Dos 195 pacientes, 151 (77,4%) eram do sexo feminino. A média de idade dos pacientes foi de 35,73±12,19 anos (variação de 18 a 71 anos). Cem pacientes (51,3%) foram encaminhados com sintomas gastrointestinais. No momento da internação, 118 pacientes (60,5%) apresentavam anemia e 52 (26,7%) apresentavam hipertransaminemia. Durante o período médio de acompanhamento de 58 meses (36-120 meses), 84 (43,1%) pacientes estavam acompanhados por pelo menos uma doença autoimune, e essa taxa foi de 96,6% em indivíduos diagnosticados acima dos 50 anos de idade. CONCLUSÃO No adulto DC, anemia resistente, dispepsia e hipertransaminasemia são achados muito comuns no momento do diagnóstico e a associação com outras doenças autoimunes, especialmente tireoidite de Hashimoto, é alta.

Giant cell myocarditis causing sudden death in a patient with sarcoidosis

Giant cell myocarditis (GCM) is a rare and rapidly fatal cardiovascular condition most often seen in young adults. It is characterized microscopically by myocardial necrosis with multinucleated giant cells in the absence of well-defined granulomas. This disorder has typically been attributed to manifest as heart failure, but in some individuals, GCM may present as sudden cardiac death. Herein, we present a fatal case of GCM in a 36-year-old male with a history of autoimmune disorders. The decedent presented to the emergency room due to vomiting and was treated for nausea due to suspected dehydration. He was discharged that night and found dead on his bathroom floor the following day. Postmortem examination revealed psoriasis and granulomatous lesions in the lungs consistent with sarcoidosis, further supporting circumstantial evidence existing between GCM and autoimmune disorders. Additionally, this case provides an opportunity to distinguish GCM from the distinct clinical entity of cardiac sarcoidosis (CS), especially in the setting of systemic sarcoidosis. We hope to raise awareness of this rare disease process and its potential to cause sudden cardiac death so that it may be considered in a differential diagnosis as immunosuppression and early cardiac transplantation largely determine the prognosis.

Virtual learning object in hematopoietic stem cell transplantation for autoimmune diseases / Objeto virtual de aprendizaje en el trasplante de células madre hematopoyéticas para enfermedades autoinmunes / Objeto virtual de aprendizagem no transplante de células-tronco hematopoéticas para doenças autoimunes

ABSTRACT Objective: describe the development of a virtual learning object to provide information about autologous transplantation of hematopoietic stem cells to autoimmune diseases. Methods: methodological study of a website development, using the instructional design model that includes Analysis, Design, Development and Implementation. Results: the virtual object, available at http://www.transplantardai.com.br, was developed in a web platform, in the Hypertext Markup Language, using the software WebAcappella - Responsive Website Creator (Intuisphere, France 2016). The content was structured in the modules: History, Transplant, Autoimmune Diseases, Links, Guidelines, Speech Team and Doubts. The icons and menus were created in order to attract the user, facilitating the search for information and allowing maximum use of the resources available on the website. Conclusion: the methodology used allowed the development of the virtual learning object, which can be used as a tool to guide and disseminate knowledge about this treatment.

RESUMEN Objetivo: describir el desarrollo de un objeto de aprendizaje virtual para proporcionar información sobre el trasplante autólogo de células madre hematopoyéticas en las enfermedades autoinmunes. Métodos: estudio metodológico del desarrollo del sitio web, utilizando el modelo de diseño instruccional (Análisis, Diseño, Desarrollo e Implementación). Resultados: el objeto virtual, disponible en http://www.transplantardai.com.br, fue desarrollado en una plataforma web, en el lenguaje de marcación Hypertext Markup Language, utilizando el software WebAcappella - Responsive Website Creator (Intuisphere, Francia 2016). El contenido se estructuró en los módulos: Historia, Trasplante, Enfermedades Autoinmunes, Links, Guías, Habla Equipo y Dudas. Los iconos y menús fueron creados para atraer al usuario, facilitando la búsqueda de información y permitiendo el máximo aprovechamiento de los recursos disponibles en el sitio web. Conclusión: la metodología utilizada permitió el desarrollo del objeto de aprendizaje virtual, que puede ser utilizado como una herramienta para guiar y difundir el conocimiento sobre este tratamiento.

RESUMO Objetivo: descrever o desenvolvimento de um objeto virtual de aprendizagem para disponibilização de informações sobre transplante autólogo de células-tronco hematopoéticas para doenças autoimunes. Métodos: estudo metodológico de desenvolvimento de um website, empregando o modelo de design instrucional que envolve Análise, Design, Desenvolvimento e Implementação. Resultados: o objeto virtual, disponível no endereço eletrônico http://www.transplantardai.com.br, foi desenvolvido em plataforma web, na linguagem de marcação Hypertext Markup Language, utilizando-se o software WebAcappella - Responsive Website Creator (Intuisphere, França 2016). O conteúdo foi estruturado nos seguintes módulos: História, Transplante, Doenças Autoimunes, Links, Orientações, Fala Equipe e Dúvidas. Os ícones e menus foram criados de modo a atrair o usuário, facilitando a busca de informações e permitindo máximo uso dos recursos disponíveis no website. Conclusão: a metodologia empregada permitiu o desenvolvimento do objeto virtual de aprendizagem, que poderá ser utilizado como ferramenta para orientar e disseminar o conhecimento sobre esse tratamento.

La función tiroidea en pacientes con enfermedades autoinmunes / Thyroid function in patients with autoimmune diseases

Introducción: la aparición de enfermedad tiroidea autoinmune presenta una gran prevalencia en pacientes con diagnósticos de enfermedades autoinmunes. Objetivo: describir las enfermedades tiroideas autoinmunes en pacientes con enfermedades autoinmunes, atendidos en el Hospital Pediátrico William Soler Ledea, del 1ro. de octubre de 2014 al 31 de enero de 2017. Métodos: estudio descriptivo, transversal y prospectivo en 42 pacientes con diagnóstico de enfermedad autoinmune. Se midieron variables demográficas, enfermedad autoinmune (lupus eritematoso sistémico, hepatitis autoinmune, artritis idiopática juvenil y esclerodermia), función tiroidea (eutiroidismo, hipotiroidismo e hipertiroidismo) y anticuerpos antitiroideos (antiperoxidasa y antitiroglobulina). Resultados: las enfermedades autoinmunes, con enfermedad tiroidea, se presentaron en 26,2 por ciento (n= 11): con hipotiroidismo 81,8 por ciento (n= 9) y con hipertiroidismo 18,2 por ciento (n= 2). El hipotiroidismo estuvo presente en adolescentes del sexo femenino (n= 7), el lupus eritematoso sistémico en el 88,9 por ciento (n= 8), seguido de esclerodermia en el 11,1 por ciento (n= 1). El hipertiroidismo solo se diagnosticó en el lupus eritematoso sistémico 100 por ciento (n= 2), ambas en adolescentes del sexo femenino. Solo 3 pacientes tuvieron anticuerpos antitiroideos positivos, solo 2 se correspondieron con enfermedad tiroidea autoinmune: hipotiroidismo con anticuerpos antitiroideos positivos 22,2 por ciento (n= 2, en este caso correspondiente al lupus eritematoso sistémico). Los pacientes sin enfermedad tiroidea (73,8 por ciento; n= 31), a su vez, predominaron en edades de 10-14 años (n= 15; 48,4 por ciento) y en el sexo femenino (n= 22; 71,0 por ciento). Conclusión: el hipotiroidismo es más frecuente en adolescentes femeninas, con diagnóstico de lupus eritematoso sistémico, y con evidencia de anticuerpos antitiroideos positivos(AU)

Introduction: the appearance of autoimmune thyroid disease has high prevalence in patients diagnosed with autoimmune diseases. Objective: to describe the autoimmune thyroid diseases in patients with autoimmune diseases attended at William Soler Ledea Pediatric Hospital from October 1, 2014 to January 31, 2017. Methods: descriptive, cross-sectional and prospective study in 42 patients diagnosed with autoimmune disease. Demographic variables, autoimmune disease (systemic lupus erythematosus, autoimmune hepatitis, juvenile idiopathic arthritis and scleroderma), thyroid function (euthyroidism, hypothyroidism and hyperthyroidism) and antithyroid antibodies (antiperoxidase and antithyroglobulin) were measured. Results: autoimmune diseases with thyroid disease occurred in 26.2 percent (n= 11), with hypothyroidism 81.8 percent (n= 9), and with hyperthyroidism 18.2 percent (n= 2). Hypothyroidism was present in female adolescents (n= 7), systemic erythematosus lupus in 88.9 percent (n= 8) followed by scleroderma in 11.1 percent (n= 1). Hyperthyroidism was only diagnosed 100 percent in systemic erythematosus lupus (n= 2), both in female adolescents. Only 3 patients had positive antithyroid antibodies, only 2 corresponded to autoimmune thyroid disease: hypothyroidism with positive antithyroid antibodies with 22.2 percent (n= 2, in this case corresponding to systemic lupus erythematosus). Patients without thyroid disease (73.8 percent; n= 31) predominated at ages from 10 to 14 years old (n= 15; 48.4 percent) and in females (n= 22; 71.0 percent). Conclusion: hypothyroidism is more frequent in female adolescents, with a diagnosis of systemic erythematosus lupus, and with evidence of positive antithyroid antibodies(AU)

Colangitis biliar primaria. Parte 2. Actualización: diagnóstico, enfermedades asociadas, tratamiento y pronóstico / Primary Biliary Cholangitis. Part 2. State of the Art, Diagnosis, Associated Diseases, Treatment and Prognosis

La colangitis biliar primaria (CBP), es una colangiopatía crónica caracterizada por la destrucción selectiva de las células epiteliales biliares de conductos hepáticos de pequeño y mediano calibre, que afecta principalmente a mujeres. Los principales síntomas son la fatiga y el prurito, sin embargo, gran porcentaje de los pacientes pueden ser asintomáticos. El diagnóstico se basa en anticuerpos antimitocondriales (AMA) con títulos >1:40, fosfatasa alcalina >1,5 veces del límite superior normal por más de 24 semanas e histología hepática compatible con la patología. Se asocia con múltiples enfermedades principalmente de carácter autoinmune extra hepáticas, enfermedades tiroideas, óseas, entre otras. El tratamiento de primera línea es el ácido ursodesoxicólico (AUDC) que a pesar que no cura la enfermedad, mejora las pruebas del perfil hepático, así como el retraso en la progresión a cirrosis. Actualmente se encuentran en estudio nuevos tratamientos y terapias adyuvantes. El propósito de esta revisión es ofrecer una actualización de este tema que se presenta en los servicios de medicina interna y gastroenterología; para su realización se conformó un equipo interdisciplinar que desarrolló una búsqueda en la base Medline a través de PubMed con los tesauros correspondientes y se procedió a una lectura crítica y analítica de títulos, resúmenes y textos completos para el filtro, extracción y síntesis de la información encontrada

Primary biliary cholangitis (PBC) is a chronic autoimmune cholangiopathy characterized by a selective destruction of biliary epithelial cells of small and medium caliber hepatic ducts, which mainly affects women. The main symptoms are fatigue and pruritus, however, a large proportion of patients may be asymptomatic. The diagnosis is based on AMA titers >1:40, alkaline phosphatase >1.5 times the upper limit for more than 24 weeks and compatible liver histology. It is associated with multiple autoimmune diseases mainly extrahepatic, thyroid diseases, bone diseases, among others. The first line treatment is ursodeoxycholic acid (UDCA), that improves liver function tests and delay the progression to cirrhosis. Currently, there are new treatments and adjuvant therapies on study. The purpose of this review is to offer an update in this topic, which is very important in gastroenterology and internal medicine. We formed an interdisciplinary team to search in the data base Medline thorough PubMed with the keywords describe below, we made a critical lecture of the titles and abstracts of each article to write this paper

Chagas disease and systemic autoimmune diseases among Bolivian patients in Switzerland

BACKGROUND Chronic cardiomyopathy occurs in 20-40% of the patients with Chagas disease. Autoimmune mechanisms may contribute to its pathogenesis. We diagnosed several cases of systemic autoimmune diseases among Bolivian migrants in Geneva with a high prevalence of Chagas disease. OBJECTIVES We tested the hypothesis of a clinical association between systemic autoimmune diseases and Chagas disease, particularly with the development of cardiomyopathy. METHODS We retrospectively searched the medical records of all Bolivian patients visiting Geneva University Hospitals between 2012 and 2015 for diagnosis of Chagas disease or systemic autoimmune diseases. FINDINGS Of the 2,189 eligible patients, 28 [1.3%; 95% confidence interval (CI) = 0.9-1.9%] presented with systemic autoimmune disease. The Chagas status was known in 903 (41.3%) patient, of whom 244 (27.0%; 95% CI = 24.2-30.0%) were positive. Eight (28.6%; 95% CI = 15.3-47.1%) of the 28 cases of systemic autoimmune disease had Chagas disease. We found no association between both entities (p = 1.000) or with Chagasic cardiomyopathy (p = 0.729). Moreover, there was no evidence of a temporal relationship between antiparasitic chemotherapy and the development of systemic autoimmune diseases. CONCLUSIONS Our results do not support a clinical association between chronic Chagas disease and systemic autoimmune diseases. However, prospective studies in areas endemic for Chagas disease should better assess the prevalence of systemic autoimmune diseases and thus a possible relationship with this infection.

Hipertensión de la arteria pulmonar y enfermedades autoinmunes del tejido conectivo: revisión de la literatura / Pulmonary arterial hypertension and autoimmune connective tissue diseases: literature review

La hipertensión de la arteria pulmonar es una grave complicación que pueden presentar los pacientes con enfermedades autoinmunes del tejido conectivo Exis-ten distintas prevalencias reportadas según cada país. Por otro lado, la sobrevida de estos pacientes reportada al año y a los tres años, va desde 70-82% y 47-53% respectivamente dependiendo de cual es la enfermedad del tejido conectivo aso-ciada. En los últimos años se ha avanzado en la precocidad del diagnóstico y han aparecido nuevas terapias que han demostrado mejores resultados. Sin embargo, la respuesta al tratamiento sigue siendo mejor en pacientes con hipertensión pul-monar idiopática que en aquellas asociadas a enfermedad de tejido conectivo.

Pulmonary artery hypertension is a serious complication that may occur in pa-tients with autoimmune diseases of the connective tissue. There are different prevalence reported by country. On the other hand, the survival of these patients reported at one year and three years, going from 70-82% and 47-53% respec-tively, depending on which is the associated connective tissue disease. In recent years it has made progress in the precocity of diagnosis and new therapies have appearedthat have shown better results. However, the response to treatment is still better in patients with idiopathic pulmonary hypertension than in those associated with connective tissue disease.

Caracterización de pacientes con enfermedades autoinmunes del tejido conectivo e hipertensión de arteria pulmonar atendidos en Hospital San Juan de Dios / Patients characterization with autoimmune diseases of the connective tissue and high blood pressure in the pulmonary artery seen in hospital San Juan de Dios

La hipertensión de la arteria pulmonar (HAP) es una grave complicación que pue-den presentar los pacientes con enfermedades autoinmunes del tejido conectivo de prevalencia variable según cada país. Habitualmente su diagnóstico es tardío lo que influye negativamente en su pronóstico y respuesta terapéutica. En el pre-sente trabajo se ha iniciado una revisión en conjunto entre la Unidad de Hemodi-namia y de Reumatología del Hospital San Juan de Dios, con el propósito de tener una caracterización de los pacientes con HAP y enfermedades autoinmunes del tejido conectivo y poder así elaborar planes de manejo de acuerdo a las caracte-rísticas clínicas de estos pacientes e intentar obtener un diagnostico precoz, para mejorar la sobrevida de los portadores de estas enfermedades.

High blood pressure in the pulmonary artery (HAP) is a serious complication that may occur in patients with autoimmune diseases of the connective tissue of vari-able prevalence according to each country. Diagnosis is usually late which affects negatively their prognosis and therapeutic response. In the present work has been initiated a review in conjunction between the unit of Hemodynamic and Rheumatology of the Hospital San Juan de Dios, with the purpose of having a characterization of patients with PAH and autoimmune diseases of the connective tissue and can thus develop management plans according to the clinical features of these patients and try to get an early diagnosis, to improve the survival of the carriers of these diseases.