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Id: biblio-1026207
Autor: Corrêa, Alana Bazán; Oliveira, Marianne Schrader; Peres, Alessandra.
Título: A evolução do diagnóstico da doença mista do tecido conjuntivo / Evolution of the diagnosis of mixed connective tissue disease
Fonte: Clin. biomed. res;39(1):89-96, 2019.
Idioma: pt.
Resumo: A Doença Mista do Tecido Conjuntivo (DMTC) é uma doença autoimune crônica composta por um misto de quatro doenças: Lúpus Eritematoso Sistêmico, Esclerose Sistêmica, Dermatomiosite/Polimiosite e Artrite Reumatoide. Por se tratar de uma combinação de doenças autoimunes o diagnóstico é bastante complexo. Atualmente existem quatro combinações sugeridas por diferentes autores para a realização de um diagnóstico preciso, são eles: Kasukawa, Alarcón-Segovia e Villareal, Kahn e Appeboom e Sharp. Desde a sua descoberta em 1972 por Sharp, passaram-se 46 anos e desta forma o objetivo desta revisão foi verificar a evolução do diagnóstico da DMTC desde a sua descoberta até a atualidade. Para isso utilizou-se sites de busca PUBMED e SCIELO. Por se tratar de uma doença autoimune que leva ao desenvolvimento de um quadro inflamatório crônico utilizou-se a ferramenta STRING que permite a análise da interação de proteínas. Até a presente data, não existe um consenso de qual critério deve ser usado para o diagnóstico correto e eficiente desta doença. A baixa relação de interações observadas a partir da ferramenta STRING demonstra que ainda não existem dados suficientes na literatura para que a ligação entre proteínas marcadoras e a DTMC possa ser estabelecida. (AU)

Mixed connective tissue disease (MCTD) is a chronic autoimmune disorder consisting of a mixture of four diseases: systemic lupus erythematosus, systemic sclerosis, dermatomyositis/polymyositis, and rheumatoid arthritis. Because it is a combination of different autoimmune disorders its diagnosis is quite complex. Currently there are four combinations suggested by the following authors to establish an accurate diagnosis: Kasukawa, Alarcón-Segovia & Villareal, Kahn, and Appeboom & Sharp. It has been 46 years since Sharp reported the disease in 1972 and thus the purpose of this review was to investigate the evolution of the diagnosis of MCTD since then. PubMed and SciELO databases were used for this investigation. Because MCTD is an autoimmune disease that leads to the development of a chronic inflammatory condition, the STRING tool was used to allow the analysis of protein interaction. To date, there is no consensus as to what criterion should be used for a correct and efficient diagnosis of this disease. The low ratio of interactions observed from the STRING tool demonstrates that there is not yet enough data in the literature for establishing the binding between marker proteins and MCTD. (AU)
Descritores: Doença Mista do Tecido Conjuntivo/diagnóstico
Doença Mista do Tecido Conjuntivo/genética
-Anticorpos Antinucleares/genética
Anticorpos Antinucleares/sangue
Biologia Computacional/métodos
Limites: Seres Humanos
Masculino
Feminino
Tipo de Publ: Revisão
Responsável: BR18.1 - Biblioteca FAMED/HCPA


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Id: biblio-842896
Autor: Quadrelli, Silvia; Otaola, María; Tabaj, Gabriela; Aguirre, Raquel; Bosio, Martín; Chertcoff, Julio.
Título: Supervivencia en pacientes con neumonía intersticial usual en contexto de fibrosis pulmonar idiopática y enfermedad del tejido conectivo / Survival in Patients with Usual Interstitial Pneumonia Secondary to Idiopathic Pulmonary Fibrosis and Connective Tissue Disease
Fonte: Rev. am. med. respir;15(1):36-50, mar. 2015. graf, tab.
Idioma: es.
Resumo: Introducción: La neumonía intersticial usual (NIU) es un patrón histológico que conlleva mal pronóstico. Sin embargo, en los últimos años se ha sugerido que la NIU asociada a las enfermedades del tejido conectivo (NIU-ETC) puede tener un comportamiento diferente a la asociada a la fibrosis pulmonar idiopática (FPI). Objetivos: Conocer si existen diferencias en la severidad y supervivencia entre los pacientes con NIU asociada a FPI y los pacientes con NIU en contexto de ETC, incluyendo esclerosis sistémica, artritis reumatoidea, polidermatomiositis y enfermedad mixta del tejido conectivo. Materiales y métodos: Fueron evaluadas las características clínicas y la supervivencia de 102 pacientes (81 con FPI y 21 con NIU-ETC) diagnosticados en base a biopsia quirúrgica o una tomografía computada de alta resolución (TCAR) con NIU definida. Resultados: La media de seguimiento fue de 24 meses (0 a 146 meses). Cuarenta y cuatro pacientes murieron durante el seguimiento, una proporción significativamente mayor entre los pacientes con FPI que entre los pacientes con NIU-ETC (49.4 vs 19.0%, p = 0.014) y la supervivencia a 3 y 5 años fue mayor en pacientes con NIU asociada a ETC que en pacientes con FPI. Los pacientes con NIU-ETC tuvieron una tasa de mortalidad a los 3 y 5 años de 19.5% y 20.0%, respectivamente, comparado con pacientes con FPI que tuvieron una tasa de mortalidad a 3 y 5 años de 35.0%, y 65.9% respectivamente (p = 0,014). Los pacientes con FPI fueron mayores que los pacientes con NIU-ETC (edad 67.95 ± 9.4 vs 57.78 ± 14.5, p = 0.021), con una proporción mayor de pacientes de sexo masculino (67.9% vs 33.3%, p = 0.006). No hubo diferencias significativas en la función pulmonar basal, la cantidad de pacientes con disnea en el momento del diagnóstico, el tiempo de inicio de síntomas al diagnóstico o en número de pacientes biopsiados entre ambos grupos. En el análisis multivariado, la DLCO y el diagnóstico de FPI fueron los únicos factores pronósticos independientes. Conclusiones: Nuestro estudio sugiere que los pacientes con NIU-ETC se asocian con una mejor supervivencia que aquellos pacientes con FPI, a pesar de presentar la misma severidad de enfermedad al momento del diagnóstico.

Background: Usual interstitial pneumonia (UIP) is a histologic pattern that implies poor prognosis. However, some studies have suggested that UIP associated to connective tissue diseases (CTD-UIP) may have a different outcome than that associated with idiopathic pulmonary fibrosis (IPF). Objectives: To compare disease severity and survival between IPF and UIP associated to connective tissue diseases including scleroderma, rheumatoid arthritis, polymyositis and mixed CTD. Methods: The study included the analysis of clinical features and survival of 102 patients (81 with IPF and 21with CTD-UIP) diagnosed through surgical biopsy or high resolution computed tomography (HRCT) in patients with definitive UIP. Results: Median follow-up was 24 months (0 to 146 months). Forty-four patients died during the follow-up; the proportion of deaths was significantly higher amongst patients with IPF than amongst patients with CTD-UIP (49.4 vs 19.0%, p = 0.014). The 3 and 5 year survival was higher in patients with UIP secondary to CTD than in patients with IPF. Patients with CTD-UIP showed 3 and 5-year case fatality rate of 19.5% and 20.0% respectively, compared to 3 and 5-year case fatality rate of 35.0%, and 65.9% respectively in patients with IPF (p = 0.014). Patients with IPF were older than patients with CTD-UIP (age 67.95 ±9.4 vs 57.78 ±14.5, p = 0.021) and were more likely to be male (67.9% vs 33.3%, p = 0.006). There were no significant differences among baseline lung function, time between onset of symptoms and diagnosis, number of patients biopsied and the proportion of patients with dyspnea at the time of diagnosis between IPF and CTD-UIP patients. By multivariate analysis, the diffusing capacity of the lung for carbon monoxide (DLCO) and the presence of IPF were independent prognostic factors. Conclusions: Our data suggest that patients with UIP associated to CTD have a better survival than patients with IPF related UIP despite similar disease severity at the time of the diagnosis.
Descritores: Pneumonia
Fibrose Pulmonar
Doenças Pulmonares Intersticiais
Doença Mista do Tecido Conjuntivo
Responsável: AR423.1 - Biblioteca


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Id: biblio-842921
Autor: Varela, Brenda; Tabaj, Gabriela; Enghelmayer, Juan Ignacio; Quadrelli, Silvia; Papucci, Tulio; Pino, Andrea; Fernández Casares, Marcelo.
Título: Manejo de las enfermedades intersticiales en Argentina: Una encuesta a neumonólogos / Management of Interstitial Lung Diseases in Argentina: a Survey to Pulmonologists
Fonte: Rev. am. med. respir;15(3):171-189, set. 2015. graf, tab.
Idioma: es.
Resumo: Introducción: Las enfermedades pulmonares intersticiales difusas (EPID) son un grupo de enfermedades raras que, si bien comparten ciertas características clínicas, tienen un pronóstico muy diferente. La fibrosis pulmonar idiopática (FPI) es la más prevalente en muchos países y su diagnóstico puede ser dificultoso. Luego de los resultados expuestos en el consenso sobre diagnóstico y manejo de la FPI, y la llegada de nuevas drogas como la pirfenidona, se ha modifcado el enfoque de esta enfermedad. Se realizó una encuesta a neumonólogos argentinos, con el fin de evaluar la aceptabilidad e implementación de estas guías en Argentina. Materiales y métodos: Se diseñó una encuesta con 24 preguntas. Entre los datos que se recolectaron en el cuestionario estaban demografía de los encuestados, lugar de trabajo (instituciones públicas, privadas, grandes o pequeños centros o instituciones), frecuencia con la que evaluaban pacientes con FPI, disponibilidad de pruebas diagnósticas y estrategias diagnósticas empleadas para pacientes con EPID. Por último, la encuesta se focalizó en las recomendaciones terapéuticas en los pacientes diagnosticados como FPI. Dicha encuesta fue completada durante el Congreso Argentino de Medicina Respiratoria que se realizó en el 2013 en la ciudad de Mendoza. La misma metodología y cuestionario fueron utilizados previamente en el Congreso Argentino de Medicina Respiratoria del 2011. Resultados: Un total de 252 médicos respondieron la encuesta en el 2013, lo que representó alrededor del 20% de los concurrentes al congreso. El método complementario de mayor disponibilidad fue la prueba de marcha de 6 minutos (PM6M). El método complementario más utilizado fue la tomografía computada de tórax (86.9% de los encuestados la realizaban ante la sospecha de EPID) y solo el 44.4% de los encuestados realizaban difusión de monóxido de carbono (DLCO) en todos sus pacientes. Cerca del 50% de los encuestados consultaban a centros de referencia en menos del 30% de sus casos con sospecha de EPID. Menos del 20% de los respondedores consideraban que llegaban a un diagnóstico defnitivo de EPID en más del 60% de sus pacientes. La distribución final de los diagnósticos fue heterogénea. Notablemente, casi el 50% de los encuestados consideraba que la FPI había sido el diagnóstico final en menos de 30% de sus pacientes. Solo el 30% de los encuestados prescribieron pirfenidona como tratamiento de elección en la FPI y más del 60% todavía continuaban prescribiendo tratamientos que incluían diferentes combinaciones de corticoides e inmunosupresores. Conclusiones: Nuestra encuesta sugiere que existen dificultades en el abordaje diagnóstico de estas entidades, que existe un bajo porcentaje de pacientes que son evaluados en centros de referencia y que hay una baja proporción de estos que reciben tratamiento específico.

Background: Diffuse interstitial (or parenchymal) lung diseases (ILDs) are a very large group of diseases that although they share certain clinical features, have a very different prognosis. Idiopathic pulmonary fibrosis (IPF) is the most prevalent in many countries and its diagnosis can be difficult. After the results shown in the consensus on diagnosis and management of IPF, and the arrival of new drugs such as pirfenidone, the approach to this disease have changed. A survey was performed to argentine pulmonologists in order to evaluate the acceptability and implementation of these guidelines in Argentina. Material and Methods: A survey of 24 questions was designed. Among the data collected in the questionnaire were demographics of respondents, workplace (public or private healthcare facilities, referral center, large or small healthcare centers or institutions), frequency at which IPF patients were examined, availability of diagnostic tests, and diagnostic strategies used with ILD patients. Finally, the survey focused on therapeutic recommendations for patients diagnosed with IPF. The survey was completed during the Argentine Congress of Respiratory Medicine held in 2013 in the city of Mendoza. The same methodology and questionnaire were previously used in the Argentine Congress of Respiratory Medicine in 2011. Results: In 2013, a total of 252 physicians completed the survey, which represented approximately 20% of Congress attendees. The complementary test of higher availability was the the six minutes walk test (6MWT). The most widely used supplementary method was thoracic computed tomography (CT) as 86.9% of the responders used it if they suspected ILD, and only 44.4% of the responders used diffusing capacity of the lungs for carbon monoxide (DLCO) with all their patients. Almost 50% of the responders consulted referral centers for less than 30% of patients with suspected ILD. Less than 20% of the responders considered that they reached a final diagnosis of ILD in over 60% of their patients. Final distribution of diagnosis was heterogeneous. Interestingly, almost 50% of the responders considered IPF as the fnal diagnosis in less than 30% of their patients. Approximately 50% of the responders answered that less than 20% of their IPF patients received specifc treatment for the disease. Conclusions: Our survey suggests that there are difficulties in the diagnostic approach of ILDs, there is a low percentage of patients that are evaluated in referral centers and there is a low proportion of IPF patients receiving specific treatment
Descritores: Fibrose Pulmonar
Pneumopatias
Doença Mista do Tecido Conjuntivo
Responsável: AR423.1 - Biblioteca


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Id: biblio-842994
Autor: Petti, Marcos; Malamud, Patricia; Morandi, Valeria; Gutiérrez, Gabriela; Rivas, Juan; Angiono, Lucas; Nasep, Agustín; Romero, Vanesa.
Título: Aplicación de un algoritmo para la detección de hipertensión arterial pulmonar en pacientes con enfermedad del tejido conectivo / Application of an Algorithm for the Detection of Pulmonary Arterial Hyertension in Patients with Connective Tissue Disease
Fonte: Rev. am. med. respir;16(3):221-228, set. 2016. ilus, graf, tab.
Idioma: es.
Resumo: Introducción: La hipertensión pulmonar (HP) es una complicación grave que se puede presentar en las enfermedades del tejido conectivo (ETC), múltiples causas pueden ser el origen de la misma. Por lo tanto, es imprescindible establecer un diagnóstico preciso para determinar la causa de la HP. Los algoritmos de diagnóstico precoz están dirigidos a la detección temprana de la HP en pacientes con ETC Objetivos: 1) detectar la presencia de HP en una población con diagnóstico de ETC mediante un algoritmo de detección precoz y 2) diagnosticar HAP asociada a ETC. Materiales y Métodos: Estudio descriptivo, prospectivo. Se incluyeron pacientes adultos en control y seguimiento reumatológico, con diagnóstico de ETC sintomática o esclerosis sistémica/enfermedad mixta del tejido conectivo (EMTC) sintomática como asintomática. A aquellos pacientes con alta sospecha clínico-ecocardiográfica de HP se les realizó cateterismo cardíaco derecho (CCD). Resultados: Se incluyeron 90 pacientes, 82 mujeres (91%) y 8 hombres (9%), con una edad media de 52 años. Presentaban esclerosis sistémica 54 (60%), artritis reumatoidea 18 (20%), EMTC 8 (9%), lupus eritematoso sistémico 8 (9%), Sjögren 1 (1%), polimiositis 1 (1%). La presencia de disnea fue referida en 60 pacientes (67%); un total de 12 pacientes (13%) presentaron alta presunción de HP y se les realizó un CCD, confirmándose el diagnóstico de HP en 9 (10%), siendo 7 del grupo I HAP y 2 del grupo II. Los pacientes con HP del grupo I se encontraban distribuidos según Clase funcional (CF), de la siguiente manera: uno en CF III, cinco en CF II y uno en CF I. Conclusiones: EI algoritmo utilizado permite diagnosticar HAP en pacientes con ETC en etapas termpranas.

Introduction: Pulmonary hypertension (PH) is a serious complication in connective tissue disease (CTD), although multiple causes can be the source of it. Therefore, it is essential to establish an accurate diagnosis to determine the cause of HP. Early detection algorithms are aimed to diagnose PH in patients with CTD in less advanced disease. Objectives: 1) To detect the presence of pulmonary hypertension (PH) in a population diagnosed with CTD by an early detection algorithm. 2) To diagnose Pulmonary arterial hypertension (PAH) associated to CTD. Materials and Methods: It's a descriptive, prospective study. Adult patients diagnosed with symptomatic CTD or symptomatic and asymptomatic systemic sclerosis, mixed connective tissue disease (MCTD) in the setting of rheumatologic control were included. Those patients with high clinical and echocardiographic suspicion of PH were catheterized (CCD). Results: 90 patients, 82 women (91%) were included, with a median age of 52 years. Fifty-flour (60%) corresponding to Systemic sclerosis, 18 (20%) to rheumatoid arthritis, 8 (9%) to MCTD, 8 (9%) to systemic lupus erythematosus, 1 (1%) to Sjögren and 1(1%) to polymyositis. Dyspnea was referred in 50 patients (67%); 12 patients (13%) had high presumption of HP and underwent a CCD, confirming the diagnosis of HP in 9 (10%). Sever belonged to PH Group I and 2 to group II. PH group I patients were distributed according to functional class (FC), as follows: 1 FC III, 5 FC II and 1 FC I. Conclusions: The used algorithm allows early PAH diagnosis in CTD.
Descritores: Hipertensão
Hipertensão Pulmonar
Doença Mista do Tecido Conjuntivo
Responsável: AR423.1 - Biblioteca


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Id: lil-633345
Autor: Barimboim, Enrique.
Título: Hipertensión pulmonar en las enfermedades del tejido conectivo: Nuevas perspectivas ante un problema de difícil resolución / Pulmonary hypertension in connective tissue diseases. New perspectives in the face of a difficult problem
Fonte: Insuf. card;4(3):101-106, jul.-sep. 2009. ilus, tab.
Idioma: es.
Descritores: Hipertensão Pulmonar
Doença Mista do Tecido Conjuntivo
Responsável: AR1.2 - Instituto de Investigaciónes Epidemiológicas


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Id: biblio-970663
Autor: Silva, Ana Cristina Diniz.
Título: Indicação de oxigenioterapia hiperbárica como auxiliar na cicatrização de úlceras de membro inferiores / Indication of hyperbaric oxygen therapy as an aid in healing lower limb wounds
Fonte: Rev. méd. Minas Gerais;28:[1-4], jan.-dez. 2018.
Idioma: pt.
Resumo: Esse artigo refere-se a um caso de perícia médica solicitada por motivo de ação judicial movida por paciente do sexo masculino de 49 anos de idade contra o município de Ipatinga, MG. O periciando é sabidamente portador de Doença Mista do Tecido Conjuntivo e Síndrome de Anticorpo Antifosfolípide, tendo desenvolvido, como consequência dessa última patologia, úlcera venosa crônica em membro inferior, não cicatrizada mesmo após duas cirurgias de enxertia. O paciente requer perante o juízo, por indicação de sua médica reumatologista, que o município arque com o procedimento de oxigenioterapia hiperbárica, considerando-se sua eficácia potencial na cicatrização de feridas. Deixando as considerações jurídicas do caso à parte, como médica perita do banco de peritos do Tribunal de Justiça de Minas Gerais, depois de avaliar clinicamente o paciente, realizei revisão da literatura médicocientífica no que diz respeito aos benefícios e indicações dessa forma de terapia para o mesmo. O objetivo desse artigo é descrever acerca desse procedimento médico, bem como esclarecer suas indicações e limitações, especialmente no que tange ao caso concreto apresentado. (AU)

This paper refers to a medical inquiry related to a lawsuit against Ipatinga County (MG), in which a male patient aged 49 years old requests the right to receive 60 sessions of hyberbaric oxygen therapy (HBOT), as an aid to healing wounds. The patient has been previously diagnosed with Mixed Disease of the Connective Tissue and Antiphospholipid Syndrome, having developed, as result of the latter disease, a chronic venous ulcer in the lower limb, not healed even after two grafting surgeries. Towards the Court, the patient requires the delivery of HBOT, considering its potential effectiveness in wound healing. Leaving apart the legal considerations of the case, as a medical expert of the Justice Court of Minas Gerais (TJMG), after a detailed clinical evaluation of the patient, I reviewed the medical literature regarding the benefits and indications of this form of therapy, correlating them to this concrete case. The aim of this article is to describe this relatively new medical procedure, and to clarify its indications and limitations, particularly with regard to the present case. (AU)
Descritores: Úlcera
Oxigenação Hiperbárica
Cicatrização
-Úlcera/tratamento farmacológico
Úlcera/terapia
Oxigênio/uso terapêutico
Ferimentos e Lesões/terapia
Doença Mista do Tecido Conjuntivo
Síndrome Antifosfolipídica
Trombofilia
SETIMMEDIATE$0.SOIL MONITORINGINOSITOL PHOSPHATES0ALBANIA0ABORTIFACIENT AGENTS, NONSTEROIDAL$ACANTHOCYTES
Limites: Seres Humanos
Masculino
Meia-Idade
Tipo de Publ: Guia de Prática Clínica
Responsável: BR1561.1 - Biblioteca Virtual AMMG


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Id: lil-735735
Autor: Lage, Renan; Biccigo, Danilo Guerreiro Zeolo; Santos, Felipe Borba Calixto; Chimara, Erica; Pereira, Elisangela Samartin Pegas; Costa, Adilson da.
Título: Mycobacterium chelonae cutaneous infection in a patient with mixed connective tissue disease
Fonte: An. bras. dermatol;90(1):104-107, Jan-Feb/2015. graf.
Idioma: en.
Resumo: Around 50 mycobacteria species cause human disease. Immunosuppressive states predispose to non-tuberculous mycobaterium infection, such as Mycobacterium chelonae: AFB, non-tuberculous, fast growth of low virulence and uncommon as a human pathogen. It may compromise the skin and soft tissues, lungs, lymph nodes and there is also a disseminated presentation. The diagnosis involves AFB identification and culture on Agar and Lowenstein-Jensen medium base. A 41-year-old female with MCTD (LES predominance) is reported, presenting painless nodules in the right forearm. She denied local trauma. Immunosuppressed with prednisone and cyclophosphamide for 24 months. Lesion biopsy has demonstrated positive bacilloscopy (Ziehl-Neelsen stain) and M.chelonae in culture (Lowenstein-Jensen medium base), therefore clarithromycin treatment has been started (best therapy choice in the literature).
Descritores: Doença Mista do Tecido Conjuntivo/tratamento farmacológico
Infecções por Micobactéria não Tuberculosa/imunologia
Infecções por Micobactéria não Tuberculosa/patologia
Mycobacterium chelonae/isolamento & purificação
-Antibacterianos/uso terapêutico
Claritromicina/uso terapêutico
Imunocompetência/imunologia
Imunossupressores/efeitos adversos
Doença Mista do Tecido Conjuntivo/complicações
Infecções por Micobactéria não Tuberculosa/tratamento farmacológico
Limites: Adulto
Feminino
Seres Humanos
Tipo de Publ: Relatos de Casos
Responsável: BR1.1 - BIREME


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Alves, Milton Ruiz
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Id: lil-703599
Autor: Usuba, Fany Solange; Lopes, Jaqueline Barros; Fuller, Ricardo; Yamamoto, Joyce Hisae; Alves, Milton Ruiz; Pasoto, Sandra Gofinet; Caleiro, Maria Teresa C..
Título: Sjogren's syndrome: An underdiagnosed condition in mixed connective tissue disease
Fonte: Clinics;69(3):158-162, 3/2014. tab.
Idioma: en.
Resumo: OBJECTIVE: To determine the prevalence of sicca symptoms, dry eye, and secondary Sjögren's syndrome and to evaluate the severity of dry eye in patients with mixed connective tissue disease. METHODS: In total, 44 consecutive patients with mixed connective tissue disease (Kasukawa's criteria) and 41 healthy controls underwent Schirmer's test, a tear film breakup time test, and ocular surface staining to investigate dry eye. In addition, the dry eye severity was graded. Ocular and oral symptoms were assessed using a structured questionnaire. Salivary gland scintigraphy was performed in all patients. Classification of secondary Sjögren's syndrome was assessed according to the American-European Consensus Group criteria. RESULTS: The patients and controls had comparable ages (44.7±12.4 vs. 47.2±12.2 years) and frequencies of female gender (93 vs. 95%) and Caucasian ethnicity (71.4 vs. 85%). Ocular symptoms (47.7 vs. 24.4%) and oral symptoms (52.3 vs. 9.7%) were significantly more frequent in patients than in controls. Fourteen (31.8%) patients fulfilled Sjögren's syndrome criteria, seven of whom (50%) did not have this diagnosis prior to study inclusion. A further comparison of patients with mixed connective tissue disease with or without Sjögren's syndrome revealed that the former presented significantly lower frequencies of polyarthritis and cutaneous involvement than did the patients without Sjögren's syndrome. Moderate to severe dry eye was found in 13 of 14 patients with mixed connective tissue disease and Sjögren's syndrome (92.8%). CONCLUSIONS: Sjögren's syndrome, particularly with moderate to severe dry eye, is frequent in patients with mixed connective tissue disease. These findings alert the physician regarding the importance of the appropriate diagnosis of this syndrome in such patients. .
Descritores: Doença Mista do Tecido Conjuntivo/diagnóstico
Síndrome de Sjogren/diagnóstico
-Brasil/epidemiologia
Métodos Epidemiológicos
Fluoresceína
Índice de Gravidade de Doença
Distribuição por Sexo
Sialografia
Síndrome de Sjogren/classificação
Síndrome de Sjogren/epidemiologia
Limites: Adulto
Idoso
Feminino
Seres Humanos
Masculino
Meia-Idade
Adulto Jovem
Tipo de Publ: Research Support, Non-U.S. Gov't
Responsável: BR1.1 - BIREME


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Id: lil-686504
Autor: Favarato, Maria Helena Sampaio; Miranda, Sofia Silveira de Castro; Caleiro, Maria Teresa Correia; Assad, Ana Paula Luppino; Halpern, Ilana; Fuller, Ricardo.
Título: Cutaneous mucinosis in mixed connective tissue disease* / Mucinose cutanea na doenca mista do tecido conjuntivo
Fonte: An. bras. dermatol;88(4):635-638, ago. 2013. graf.
Idioma: en.
Resumo: Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

A mucinose cutânea é um grupo de condições em que há um acúmulo de mucina ou glicosaminoglicanos na pele e seus anexos. É descrita em algumas doenças do tecido conjuntivo, porem nunca em associação com doença mista do tecido conjuntivo. Relatamos dois casos de mucinose cutânea em pacientes com doença mista do tecido conjuntivo em remissão, um apresentava-se sob a forma papular e outro sob a forma reticular eritematosa de mucinose. Estes são os primeiros casos de mucinose descritos na doença mista do tecido conjuntivo. Ambos os casos apresentaram o quadro cutâneo de modo isolado, sem nenhuma outra manifestação clínico-laboratorial, havendo resposta à azatioprina em um e à cloroquina associada a prednisona no outro.
Descritores: Doença Mista do Tecido Conjuntivo/patologia
Mucinoses/patologia
-Biópsia
Doença Mista do Tecido Conjuntivo/complicações
Mucinoses/complicações
Limites: Adulto
Feminino
Seres Humanos
Masculino
Tipo de Publ: Relatos de Casos
Responsável: BR1.1 - BIREME


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Id: lil-668796
Autor: Miossi, Renata; Fuller, Ricardo; Moraes, Júlio C. B.; Ribeiro, Ana Cristina M.; Saad, Carla G. S.; Aikawa, Nadia E.; Miraglia, Joao L.; Ishida, Maria A.; Bonfa, Eloisa; Caleiro, M. Teresa C..
Título: Immunogenicity of influenza H1N1 vaccination in mixed connective tissue disease: effect of disease and therapy
Fonte: Clinics;68(2):129-134, 2013. tab.
Idioma: en.
Projeto: Fundação de Amparo a Pesquisa do Estado de São Paulo; . Conselho Nacional de Desenvolvimento Científico e Tecnológico; . Federico Foundation.
Resumo: OBJECTIVE: To assess the potential acute effects regarding the immunogenicity and safety of non-adjuvanted influenza A H1N1/2009 vaccine in patients with mixed connective tissue disease and healthy controls. METHODS: Sixty-nine mixed connective tissue disease patients that were confirmed by Kasukawa's classification criteria and 69 age- and gender-matched controls participated in the study; the participants were vaccinated with the non-adjuvanted influenza A/California/7/2009 (H1N1) virus-like strain. The percentages of seroprotec-tion, seroconversion, geometric mean titer and factor increase in the geometric mean titer were calculated. The patients were clinically evaluated, and blood samples were collected pre- and 21 days post-vaccination to evaluate C-reactive protein, muscle enzymes and autoantibodies. Anti-H1N1 titers were determined using an influenza hemagglutination inhibition assay. ClinicalTrials.gov: NCT01151644. RESULTS: Before vaccination, no difference was observed regarding the seroprotection rates (p = 1.0) and geometric mean titer (p = 0.83) between the patients and controls. After vaccination, seroprotection (75.4% vs. 71%, (p = 0.7), seroconversion (68.1% vs. 65.2%, (p = 1.00) and factor increase in the geometric mean titer (10.0 vs. 8.0, p = 0.40) were similar in the two groups. Further evaluation of seroconversion in patients with and without current or previous history of muscle disease (p = 0.20), skin ulcers (p = 0.48), lupus-like cutaneous disease (p = 0.74), secondary Sjogren syndrome (p = 0.78), scleroderma-pattern in the nailfold capillaroscopy (p = 1.0), lymphopenia #1000/mm³ on two or more occasions (p = 1.0), hypergammaglobulinemia $1.6 g/d (p = 0.60), pulmonary hypertension (p = 1.0) and pulmonary fibrosis (p = 0.80) revealed comparable rates. Seroconversion rates were also similar in patients with and without immunosuppressants. Disease parameters, such as C-reactive protein (p = 0.94), aldolase (p = 0.73), creatine phosphokinase (p = 0.40) and ribonucleoprotein antibody levels (p = 0.98), remained largely unchanged pre and post-vaccination. No severe side effects were reported. CONCLUSIONS: The non-adjuvanted influenza A/H1N1 vaccination immune response in mixed connective tissue disease patients is adequate and does not depend on the disease manifestations and therapy.
Descritores: Vírus da Influenza A Subtipo H1N1/imunologia
Vacinas contra Influenza/imunologia
Influenza Humana/prevenção & controle
Doença Mista do Tecido Conjuntivo/imunologia
-Estudos de Casos e Controles
Vacinas contra Influenza/efeitos adversos
Estatísticas não Paramétricas
Fatores de Tempo
Limites: Adulto
Feminino
Seres Humanos
Masculino
Meia-Idade
Tipo de Publ: Research Support, Non-U.S. Gov't
Responsável: BR1.1 - BIREME



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