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1.
Article in English | WPRIM | ID: wpr-879957

ABSTRACT

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.


Subject(s)
Autoimmune Diseases/complications , Hashimoto Disease , Humans , Lichen Planus, Oral/complications , Mouth Mucosa , Th17 Cells
2.
Braz. j. med. biol. res ; 54(10): e11355, 2021. graf
Article in English | LILACS | ID: biblio-1285647

ABSTRACT

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.


Subject(s)
Humans , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Subacute Combined Degeneration/complications , Subacute Combined Degeneration/diagnosis , Subacute Combined Degeneration/pathology , Spinal Cord , Vitamin B 12 , Magnetic Resonance Imaging
3.
Rev. bras. oftalmol ; 79(6): 403-405, nov.-dez. 2020. graf
Article in Portuguese | LILACS | ID: biblio-1156169

ABSTRACT

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.


Subject(s)
Humans , Male , Adult , Retinal Diseases/diagnostic imaging , Autoimmune Diseases/complications , Angiography/methods , Crohn Disease/diagnosis , Tomography, Optical Coherence/methods
4.
Rev. cuba. reumatol ; 22(3): e852, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1149925

ABSTRACT

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)


Subject(s)
Humans , Male , Female , Arthritis, Rheumatoid/complications , Autoimmune Diseases/complications , Adaptation, Psychological , Coronavirus Infections/complications , Lupus Erythematosus, Systemic/complications , Immunosuppressive Agents/adverse effects , Immunosuppressive Agents/therapeutic use
5.
Rev. Assoc. Med. Bras. (1992) ; 66(1): 55-60, Jan. 2020. tab, graf
Article in English | LILACS | ID: biblio-1091898

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Aged , Young Adult , Autoimmune Diseases/epidemiology , Celiac Disease/epidemiology , Anemia/epidemiology , Autoimmune Diseases/complications , Thyroid Diseases/complications , Thyroid Diseases/epidemiology , Turkey/epidemiology , Celiac Disease/complications , Prevalence , Retrospective Studies , Age Distribution , Statistics, Nonparametric , Tertiary Care Centers/statistics & numerical data , Transaminases/blood , Anemia/complications , Middle Aged
6.
Autops. Case Rep ; 10(4): e2020238, 2020. graf
Article in English | LILACS | ID: biblio-1131842

ABSTRACT

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.


Subject(s)
Humans , Male , Adult , Sarcoidosis/complications , Giant Cells/pathology , Myocarditis/complications , Autoimmune Diseases/complications , Autopsy , Death, Sudden, Cardiac , Rare Diseases , Diagnosis, Differential
8.
Rev. bras. enferm ; 72(4): 994-1000, Jul.-Aug. 2019. graf
Article in English | LILACS, BDENF | ID: biblio-1020533

ABSTRACT

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.


Subject(s)
Humans , Autoimmune Diseases/therapy , Problem-Based Learning/methods , Hematopoietic Stem Cell Transplantation/instrumentation , Hematopoietic Stem Cell Transplantation/methods , Virtual Reality , Autoimmune Diseases/complications , Problem-Based Learning/standards , France
10.
Rev. cuba. pediatr ; 90(3): 1-14, jul.-set. 2018. tab
Article in Spanish | LILACS, CUMED | ID: biblio-978451

ABSTRACT

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)


Subject(s)
Humans , Male , Female , Autoimmune Diseases/complications , Thyroid Diseases/complications , Thyroid Diseases/immunology , Thyroiditis, Autoimmune/epidemiology , Thyroid Diseases/physiopathology , Epidemiology, Descriptive , Cross-Sectional Studies , Prospective Studies
12.
Mem. Inst. Oswaldo Cruz ; 113(4): e170383, 2018. tab
Article in English | LILACS | ID: biblio-894911

ABSTRACT

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.


Subject(s)
Autoimmune Diseases/complications , Chagas Cardiomyopathy/complications , Switzerland/epidemiology , Emigrants and Immigrants
13.
Rev. chil. reumatol ; 34(1): 9-15, 2018. tab
Article in Spanish | LILACS | ID: biblio-1254081

ABSTRACT

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.


Subject(s)
Humans , Autoimmune Diseases/complications , Connective Tissue Diseases/complications , Pulmonary Arterial Hypertension/complications , Scleroderma, Systemic , Survival , Connective Tissue Diseases/therapy , Pulmonary Arterial Hypertension/mortality
14.
Rev. chil. reumatol ; 34(1): 16-26, 2018. tab
Article in Spanish | LILACS | ID: biblio-1254082

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Autoimmune Diseases/complications , Connective Tissue Diseases/complications , Pulmonary Arterial Hypertension/epidemiology , Chile/epidemiology , Epidemiology, Descriptive , Lung Diseases, Interstitial/etiology , Early Diagnosis , Pulmonary Arterial Hypertension/diagnosis , Pulmonary Arterial Hypertension/mortality
15.
Rev. chil. reumatol ; 34(1): 28-34, 2018. tab
Article in Spanish | LILACS | ID: biblio-1254083

ABSTRACT

Las miopatías inflamatorias idiopáticas (MII) comprenden un grupo de enfermedades multisistémicas de baja prevalencia que afectan tanto adultos como a niños, con ma-nifestaciones clínicas variables como lo son: debilidad muscular de predominio proxi-mal, alteraciones cutáneas (pápulas de Gottron, signo del chal, ulceras cutáneas), artritis, enfermedad pulmonar intersticial difusa (EPD), calcinosis y malignidad; en-marcadas en diferentes subtipos clínicos. Se cree que la autoinmunidad tiene un pa-pel clave en la patogénesis de estas enfermedades y como tal se han identificado autoanticuerpos en más del 50% de los pacientes con MII (algunos específicos y otros relacionados a miositis), lo cual ha permito clasificar diferentes características fenotí-picas e histológicas de estas enfermedades al igual de reconocer diferentes patrones de respuesta a tratamiento y factores pronósticos.En esta revisión mencionaremos los autoanticuerpos mas conocidos en relación a las miopatías inflamatorias idiopáticas, incluida la identificación de anticuerpos asocia-dos con las miopatía necrotizantes autoinmunes (MNA), la miositis por cuerpos de inclusión (MCI) y la asociación miositis - cáncer.


The Idiopathic inflammatory myopathies (IIM) comprise a group of low prevalence multisitemic diseases that affect both adults and children, with variable clinical man-ifestations such as muscular weakness of predominantly proximal, skin alterations (Gottron papules, sign of the shawl, skin ulcers), arthritis, diffuse interstitial lung dis-ease (ILD), calcinosis and malignancy; framed in different clinical subtypes. It is be-lieved that autoimmunity plays a key role in the pathogenesis of these diseases and as such autoantibodies have been identified in more than 50% of patients with IIM (some specific and others related to myositis), which has allowed to classify different phenotypic characteristics and histological of these diseases as well as recognizing different patterns of response to treatment and prognostic factors.In this review we will mention the most known autoantibodies in relation to idio-pathic inflammatory myopathies, including the identification of antibodies associated with autoimmune necrotizing myopathies (ANM), inclusion body myositis (IBM) and the myositis - cancer association.


Subject(s)
Humans , Autoantibodies/analysis , Autoimmune Diseases/complications , Myositis/complications , Calcinosis , Dermatomyositis , Muscular Diseases , Myositis/immunology , Neoplasms
16.
Rev. Soc. Bras. Clín. Méd ; 15(4): 222-225, 20170000. tab, graf
Article in Portuguese | LILACS | ID: biblio-877062

ABSTRACT

Objetivo: Discriminar as variáveis de sexo e idade nos pacientes com anemia perniciosa; estudar seu perfil hematimétrico; verificar a prevalência de outras doenças autoimunes e anemia perniciosa; analisar a incidência da pancitopenia e sua relação com alterações laboratoriais comum na doença; e avaliar a frequência dos autoanticorpos anticélulas parietais e antifator intrínseco. Métodos: Estudo transversal descritivo, de base clínica e laboratorial, de 33 prontuários de pacientes com anemia perniciosa, diagnosticados em um ambulatório de hospital terciário de atenção à saúde, no período de junho de 2009 a junho de 2014. Para analisar a relação da presença e da ausência de pancitopenia com os níveis da enzima lactato desidrogenase e vitamina B12, foi utilizado o teste qui quadrado. O programa utilizado foi o software Epi Info, versão 7. Resultados: Na amostra, 63,6% eram mulheres, e a idade média geral foi de 47,3 anos. Doenças autoimunes estavam associadas em 30,3% dos pacientes. A pancitopenia esteve presente em 39,4% dos pacientes. Houve significância estatística na relação da pancitopenia com os níveis de enzima lactato desidrogenase (p<0,05). A prevalência do antifator intrínseco foi de 69,7% e dos autoanticorpos anticélulas parietais foi de 72,7%. Conclusão: A pancitopenia mostrou-se um achado significante na população com anemia perniciosa, assim como níveis elevado de LDH, acrescentando a anemia perniciosa como um diagnóstico diferencial de tais alterações laboratoriais.(AU)


Objective: To discriminate the gender and age variables in patients with pernicious anemia; to study erythrocyte profile; to check the prevalence of other autoimmune diseases and pernicious anemia; to analyze the incidence of pancytopenia and its relationship with common laboratory abnormalities in the disease; to evaluate the frequency of anti-gastric parietal cell antibodies, and anti-intrinsic factor antibodies. Methods: Descriptive, cross-sectional study of clinical and laboratorybased medical records of 33 patients with pernicious anemia diagnosed in an outpatient's department of a tertiary healthcare center, in the period between June 2009 and June 2014. To analyze the relationship between the presence and absence of pancytopenia with levels of lactate dehydrogenase enzyme and levels of Vitamin B12 we used the chi-squared test. The software used was Epininfo version 7. Results: The sample showed 63.6% women and 36.4% men with a mean age of 47.3 years. Autoimmune diseases were associated in 30.3% of the patients. Pancytopenia was present in 39.4% of patients. There was statistically significant relationship of pancytopenia with lactate dehydrogenase enzyme levels (p <0.05). The frequency of anti-intrinsic factor antibodies was 69.7%, and the antigastric parietal cell antibodies was 72.7%. Conclusion: Pancytopenia proved to be a significant finding among the population with pernicious anemia and high levels of LDH, which includes pernicious anemia as a differential diagnosis of such laboratory alterations.(AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Pancytopenia/complications , Pancytopenia/diagnosis , Autoimmune Diseases/epidemiology , Vitamin B 12/blood , Anemia, Pernicious/complications , Anemia, Pernicious/diagnosis , L-Lactate Dehydrogenase , Autoimmune Diseases/complications , Diagnosis, Differential , Antibodies
17.
Rev. Soc. Bras. Clín. Méd ; 15(4): 235-239, 20170000. tab
Article in Portuguese | LILACS | ID: biblio-877079

ABSTRACT

Objetivo: Analisar a presença de autoanticorpos antitireoidianos (anti-TPO) no soro de pessoas acometidas por vitiligo. Métodos: Estudo do tipo caso-controle retrospectivo realizado em serviço de dermatologia de referência na Amazônia, com amostra constituída por dois grupos: Grupo Vitiligo (n=56), com diagnóstico clínico de vitiligo, e Grupo Controle (n=30), que se declarou sadio, não portador de vitiligo, de outra dermatose e/ou doença autoimune diagnosticada. O registro dos dados foi feito pelo preenchimento de protocolo específico usado em entrevista para ambos os grupos, além de coleta de sangue para dosagem de autoanticorpos anti-TPO para os dois grupos. O teste qui quadrado e a odds ratio (OR) foram utilizados para variáveis qualitativas; para as quantitativas, foi utilizado o teste t de Student, e o nível de significância foi de p≤5%. Resultados: A história pessoal de doença autoimune esteve presente em 7,14% dos portadores versus 0% dos controles. Não houve diferenças estatisticamente relevantes com relação aos antecedentes familiares entre os grupos (OR: 0,5704; p=0,4146). Quanto à positividade para os autoanticorpos anti-TPO (níveis superiores ao ponto de corte), não houve relevância estatística (qui quadrado 2,844; p=0,229). Entretanto, na comparação dos níveis séricos absolutos de autoanticorpos anti-TPO entre os grupos, foram obtidos 129,49±323,88 para o portador da doença e 35,85±13,16 para o controle, com t=2,1602 e p=0,0351. Conclusão: Embora não tenha sido relevante a diferença entre os Grupo Vitiligo e Controle quanto à positividade para o autoanticorpos anti-TPO, ao se considerar a comparação com os valores séricos absolutos do Grupo Vitiligo, estes foram maiores que os apresentados pelos controles, sendo esta diferença estatisticamente relevante.(AU)


Objective: To analyze the presence of antithyroid autoantibodies (anti-TPO) in the serum of people affected by vitiligo. Methods: This is a study of retrospective casecontrol, performed in a reference dermatological center in Amazonia, with a sample consisting of two groups: Vitiligo group (n=56), with clinical diagnosis of vitiligo, and the Control Group (n=30), who was self-declared as healthy, nonvitiligo carrier, with no other dermatosis and/or diagnosed autoimmune disease. The data recording was made with specific protocol completion in an interview for both groups, and blood collection for antithyroid autoantibodies dosage for both groups. Chi-square and odds ratio (OR) tests were used for qualitative variables; for the quantitative ones, t-Student test, and significance level of p≤5%. Results: The personal history of autoimmune disease was present in 7.14% of patients compared to 0% of controls. There were no statistically significant differences in relation to family history between the groups (odds ratio: 0.5704; p=0.4146). As for the positivity for antithyroid autoantibodies (levels above the cutoff point), there was no statistical significance (chi-square=2.844, p=0.229). However, when comparing the absolute serum levels of antithyroid autoantibodies between the groups, 129.49±323.88 was obtained for the carrier of the disease, and 35.85±13:16 to controls, with t=2.1602, and p=0.0351. Conclusion: Although the difference between vitiligo and control groups were not significant regarding positivity for antithyroid autoantibodies, when the comparison with the absolute serum levels of the group with vitiligo was considered, they were higher than those presented by the controls, with this difference being statistically significant.(AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Autoantibodies/analysis , Autoimmune Diseases/complications , Thyroid Gland , Vitiligo/physiopathology , Case-Control Studies
18.
Rev. bras. reumatol ; 57(2): 174-181, Mar.-Apr. 2017. tab, graf
Article in English | LILACS | ID: biblio-844226

ABSTRACT

Abstract Juvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents.


Resumo As doenças reumáticas juvenis afetam o sistema musculoesquelético e se iniciam antes dos 18 anos. Apresentam etiologia variada, identificável ou desconhecida, porém as de natureza inflamatória autoimune têm sido associadas ao maior risco de desenvolvimento de neoplasias, independentemente do tratamento. Este artigo propõe avaliar, por meio de revisão sistemática da literatura de acordo com os critérios de qualidade Prisma (Preferred Reporting Items for Systematic Reviews and Meta- Analyses), o risco de câncer em pacientes com doenças reumáticas juvenis e sua associação com imunobiológicos. Os critérios descritos pela iniciativa Strengthening the Reporting of Observational Studies in Epidemiology foram usados para avaliar a qualidade metodológica individual dos artigos selecionados no presente estudo. Foram analisadas nove publicações, de 251 incialmente selecionadas. Houve aumento no risco de câncer na população com doença reumática juvenil comparada com a população em geral. A maioria dos cânceres especificados foi de natureza linfoproliferativa. Sete estudos não especificaram a terapêutica ou não definiram associação entre ela e o risco de câncer. Apenas um estudo sugeriu essa associação e observou maior risco em pacientes diagnosticados nos últimos 20 anos, período de advento de novas terapias. Um estudo constatou maior risco em uma população não tratada com imunobiológicos, sugeriu tratar-se da evolução natural da doença, e não do efeito adverso da terapêutica. Os estudos demonstram aumento no risco de malignidade associada a doenças reumáticas juvenis que pode estar relacionada à atividade da doença, e não especificamente ao tratamento com imunobiológicos.


Subject(s)
Humans , Child , Autoimmune Diseases/complications , Autoimmune Diseases/drug therapy , Biological Therapy , Rheumatic Diseases/complications , Rheumatic Diseases/drug therapy , Lymphoproliferative Disorders/complications , Lymphoproliferative Disorders/pathology , Autoimmune Diseases/pathology , Rheumatic Diseases/pathology , Lymphoma/complications , Lymphoma/pathology , Lymphoma/drug therapy , Lymphoproliferative Disorders/drug therapy
19.
Rev. Assoc. Med. Bras. (1992) ; 63(3): 215-218, Mar. 2017. graf
Article in English | LILACS | ID: biblio-956439

ABSTRACT

Summary Ménétrier's disease is an extremely rare disease of unknown etiology causing gastric mucosal hypertrophy and protein-losing gastropathy. Rare cases of this condition have been reported in patients with autoimmune diseases. However, to the best of our knowledge, Ménétrier's disease associated with autoimmune pancreatitis (AIP) has never been reported. We described a case of severe hypoproteinemia as a harbinger of Ménétrier's disease associated with AIP. The patient was successfully treated with octreotide and high-protein diet, which led to symptomatic remission and significant improvement in serum levels of albumin and recovery of the nutritional status. Thus, in AIP patients presenting with severe and persistent hypoproteinemia without apparent cause, clinicians need to consider Ménétrier's disease in the differential diagnosis. In this setting, endoscopic evaluation with histological examination of gastric biopsy material, including a full-thickness mucosal biopsy of involved mucosa, may be helpful in promptly establishing the diagnosis and allowing appropriate and timely therapy.


Resumo A doença de Ménétrier é uma condição extremamente rara, de etiologia desconhecida, caracterizada por hipertrofia da mucosa gástrica e gastropatia perdedora de proteína. Casos raros dessa patologia têm sido relatados em pacientes com doenças autoimunes. Até o momento, desconhecemos qualquer relato dessa doença associada à pancreatite autoimune (PAI). Descrevemos um caso de hipoproteinemia grave como indicador de doença de Ménétrier associada à PAI. O paciente foi tratado de forma satisfatória com octreotide e dieta hiperproteica, alcançando remissão sintomática, melhora significativa das concentrações de albumina e recuperação do estado nutricional. Portanto, em pacientes com PAI e hipoproteinemia grave e persistente, deve-se considerar a doença de Ménétrier como um diagnóstico diferencial. Nesses casos, a avaliação endoscópica com biópsia gástrica, incluindo biópsia de toda a espessura da mucosa, pode ser útil no estabelecimento do diagnóstico e do pronto início da terapêutica.


Subject(s)
Humans , Male , Pancreatitis/complications , Autoimmune Diseases/complications , Gastritis, Hypertrophic/complications , Hypoproteinemia/etiology , Pancreatitis/pathology , Pancreatitis/blood , Autoimmune Diseases/pathology , Autoimmune Diseases/blood , Biopsy , Severity of Illness Index , Endoscopy, Gastrointestinal , Gastric Mucosa/pathology , Gastritis, Hypertrophic/pathology , Gastritis, Hypertrophic/blood , Hypoproteinemia/pathology , Middle Aged
20.
An. bras. dermatol ; 92(1): 72-80, Jan.-Feb. 2017. tab, graf
Article in English | LILACS | ID: biblio-838007

ABSTRACT

ABSTRACT Autoinflammatory disorders are immune-mediated diseases with increased production of inflammatory cytokines and absence of detectable autoantibodies. They course with recurrent episodes of systemic inflammation and fever is the most common symptom. Cutaneous manifestations are prevalent and important to diagnosis and early treatment of the syndromes. The purpose of this review is to emphasize to dermatologists the skin symptoms present in these syndromes in order to provide their early diagnosis.


Subject(s)
Humans , Skin Diseases/etiology , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Inflammation/complications , Inflammation/diagnosis , Skin Diseases/immunology , Inflammation/immunology
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