Navegando por Palavras-chave "Dermatomyositis"
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- ItemAcesso aberto (Open Access)Association of anti-glomerular basement membrane antibody disease with dermatomyositis and psoriasis: case report(Associação Paulista de Medicina - APM, 2010-01-01) Machado, Natália Pereira [UNIFESP]; Camargo, Cintia Zumstein [UNIFESP]; Oliveira, Ana Cecília Diniz [UNIFESP]; Buosi, Ana Letícia Pirozzi [UNIFESP]; Pucinelli, Mário Luiz Cardoso [UNIFESP]; Souza, Alexandre Wagner Silva de [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)CONTEXT: Anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: A 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.
- ItemAcesso aberto (Open Access)Avaliação antropométrica, dietética e dos biomarcadores do metabolismo lipídico de pais de crianças e adolescentes com doenças reumáticas crônicas(Universidade Federal de São Paulo (UNIFESP), 2019-11-28) Pereira, Lucila [UNIFESP]; Terreri, Maria Teresa De Sande E Lemos Ramos Ascensao [UNIFESP]; Sarni, Roseli Oselka Saccardo [UNIFESP]; http://lattes.cnpq.br/2661280959330284; http://lattes.cnpq.br/2661280959330284; http://lattes.cnpq.br/2587439606675653; Universidade Federal de São Paulo (UNIFESP)Introduction: Chronic rheumatic diseases in children and adolescents increase short- and long-term cardiovascular risk. Thus, knowing the eating habits, biomarkers of lipid and glycidic metabolism and the lifestyle of patients and their parents will enable the development of strategies aimed at reducing this risk. Objectives: To describe the classification of body mass index, dietary intake, physical activity and lipid metabolism biomarkers of parents of children and adolescents with chronic rheumatic diseases and compare them with those of their children. Methods: Cross-sectional study with 91 parents and their respective children diagnosed with JIA (n = 30 / 33.0%), JSLE (n = 41 / 45.0%) and JDM (n = 20 / 22.0%). Anthropometry, dietary intake, physical activity, total cholesterol and fractions, triglycerides, and apolipoproteins A-I and B were evaluated. Data werw statistically treated using the SPSS statistical program, considering a significance level of 5% (p<0.05). To compare the categorical variables, the chi-square test or Fisher’s exact test was uded. Pearson’s correlation coefficient was tested for correlation between parent and child variables and the Cramer’s V test was used to verify association. Results: Most parents were female, as were their children (89.8 and 83.5% respectively). The average age of the parents was 40.5 ± 7.4 years. The disease follow up time was 4.5 ± 3.4 years and active disease was observed in 56% of children / adolescents. 80% of overweight children / adolescents also had parents with the same nutritional diagnosis. There was a moderate association to total lipid intake (Cramer's V test = 0.254; p = 0.037) and weak to saturated fat (Cramer's V test = 0.219; p = 0.050) and cholesterol (Cramer's V test = 0.234; p = 0.025) between parents and their children. When the daily protein intake in grams of kilo weight was evaluated, there was a positive linear correlation (corr=0.2239; p=0.033) between parents and children. The prevalence of dyslipidemia (TC, LDL-c, HDL-c, non-HDL-c and TG) of the parents’ children / adolescents was 83.5% and 82.4% respectively. Both parents and children / adolescents mostly had low HDL-c (52.8 and 64.8% respectively). Conclusion: The present study draws attention to the high prevalence of overweight, dyslipidemia, physical inactivity and central adiposity in the parents of children and adolescents with chronic rheumatic diseases. There was an association between inadequate fat consumption by parents and their children; this did not occur for BMI classification, lipid profile and for physical activity. The findings point to the importance of intervention strategies with parental involvement.
- ItemAcesso aberto (Open Access)Bone mineral density of Brazilian girls with juvenile dermatomyositis(Associação Brasileira de Divulgação Científica, 2005-02-01) Castro, Tania Caroline Monteiro de [UNIFESP]; Terreri, Maria Teresa Ramos Ascensão [UNIFESP]; Szejnfeld, Vera Lucia [UNIFESP]; Len, Claudio Arnaldo [UNIFESP]; Fonseca, A.s.m. da; Hilário, Maria Odete Esteves [UNIFESP]; Universidade Federal de São Paulo (UNIFESP); Universidade Federal do Rio Grande do Norte Departamento de PediatriaWe measured bone mineral density (BMD) in girls with juvenile dermatomyositis (JDM) considering multiple factors in order to determine if it could be used as a predictor of reduction in bone mass. A cross-sectional study of lumbar spine BMD (L2-L4) was conducted on 10 girls aged 7-16 years with JDM. A group of 20 age-matched healthy girls was used as control. Lumbar spine BMD was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in all patients and controls. Duration of disease and mean daily and cumulative steroid doses were calculated for all patients on the basis of their medical charts. JDM activity was determined on the basis of the presence of muscle weakness, cutaneous vasculitis and/or elevation of serum concentration of one or more skeletal muscle enzymes. Seven patients demonstrated osteopenia or osteoporosis. Lumbar BMD was significantly lower in the JDM patients than the age-matched healthy control girls (0.712 vs 0.878, respectively; Student t-test, P = 0.041). No significant correlation between BMD and age, height, Tanner stage, disease duration, corticosteroid use, or disease activity was observed in JDM girls, but a correlation was observed between BMD and weight (Pearson's correlation coefficient, r = 0.802). Patients with JDM may be at risk for a significant reduction in BMD that might contribute to further skeletal fragility. Our results suggest that reduced bone mass in JDM may be related to other intrinsic mechanisms in addition to steroid treatment and some aspects of the disease itself may contribute to this condition.
- ItemSomente MetadadadosDetection of multifocal osteonecrosis in an adolescent with dermatomyositis using whole-body MRI(Springer, 2010-09-01) Castro, Tania C. M. [UNIFESP]; Lederman, Henrique [UNIFESP]; Terreri, Maria Teresa A. [UNIFESP]; Kaste, Sue C.; Hilario, Maria Odete E. [UNIFESP]; Universidade Federal de São Paulo (UNIFESP); St Jude Childrens HospOsteonecrosis is a well-recognized complication of corticosteroid use resulting in significant morbidity, often requiring surgical intervention. Whole-body MRI is a promising method that allows imaging of the whole patient in a reasonable time without the use of ionizing radiation. This technique has the potential for evaluating nonmalignant multifocal skeletal disease like osteonecrosis. This case highlights the value of whole-body MR in an adolescent with dermatomyositis who developed multifocal osteonecrosis.
- ItemAcesso aberto (Open Access)Estudo prospectivo de capilaroscopia periungueal em crianças e adolescentes com dermatomiosite e fenômeno de Raynaud(Universidade Federal de São Paulo (UNIFESP), 2010-06-30) Piotto, Daniela Gerent Petry [UNIFESP]; Hilário, Maria Odete Esteves [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)Objective: To evaluate prospectively the clinical features and nailfold capillaroscopy findings of a cohort of children and adolescents who presented Raynaud´s phenomenon without criteria for auto-immune rheumatic diseases. Methods: We included 40 children and adolescents with Raynaud’s phenomenon. Each patient was clinically evaluated and laboratory exams were performed, including antinuclear antibodies. Concomitantly we also performed a nailfold capillaroscopy by using a optical microscope with a magnification of 10 and 16X. Results: Thirty (75%) out of 40 patients were girls with a mean age of 14.6 years and mean follow-up time of 4.2 years. The mean age at disease onset was 10.4 years and the mean time until diagnosis 1.4 years. Thirteen out of 40 patients (32,5%) presented antinuclear antibodies positivity. Five (12.5%) patients had altered nailfold capillaroscopy: 4 inespecific microangiopathy and 1 scleroderma pattern. All patients were prospectively evaluated within a mean interval time of 1.6 years and 3 (7.5%) patients presented nailfold capillaroscopy alterations. Two patients that showed scleroderma pattern at the nailfold capillaroscopy presented during the follow-up mixed connective tissue disease and hypothyroidism, respectively. In a patient with normal nailfold capillaroscopy and auto-antibodies positivity systemic lupus erythematosus was diagnosed after 1 year of initial evaluation. Conclusions: Raynaud´s phenomenon remains primary in most cases, however nailfold capillaroscopy is useful to help in exclusion of auto-immune rheumatic diseases or other auto-immune diseases.
- ItemSomente MetadadadosLições da pandemia do Covid-19: papel da relação médico-paciente na adesão ao tratamento de crianças e adolescentes com doenças reumáticas crônicas imunomediadas com diagnóstico recente(Universidade Federal de São Paulo (UNIFESP), 2021) Silva, Ilana Izidoro Da [UNIFESP]; Terreri, Maria Teresa De Sande E Lemos Ramos Ascensao [UNIFESP]; Universidade Federal de São PauloIntroduction: Autoimmune rheumatic diseases (ARDs) have long-term treatments in common, leading to a great impact on the lives of patients, families and the health system. Studies on treatment adherence in the pediatric age group and on satisfaction with the health service are of great value. It is important to verify the risk of poor treatment adherence, especially during the COVID-19 pandemic. Objectives: To identify early on in pediatric patients recently diagnosed with ARDs the presence and risk of poor adherence to the recommended treatment approaches, through specific questionnaires and to associate them with a prospective assessment of patient adherence and their family’s satisfaction with health service during the COVID-19 pandemic. Methods: Prospective observational study with 50 parents and patients (26 children and 24 adolescents), with a recent diagnosis of ARDs, followed at a pediatric rheumatology clinic. The study was divided into two stages: in the first, patients and their guardians answered questions about demographic and disease data and the Pediatric Rheumatology Adherence Questionnaire (PRAQ). In the second stage (after 6 months), the questionnaires Test of Morisky-Green (TMG), Brief Medication Questionnaire (BMQ), Compliance Questionnaire for Rheumatology (CQR-19) and Pediatric Quality of Life Inventory version 3.0 PedsQl-SSS were applied and the adherence to treatment (global and to medications) was verified in the patients' records related to the last 12 weeks. Results: The mean age of patients was 10.2 ± 5.2 years and disease onset 9.6 ± 5.1 years. In the first assessment, the mean number of medications prescribed was 4.8 ± 2.5 and in the second assessment it was 5.0 ± 2.7. The mean score for global adherence was 94.3% ± 10.0 and for medication adherence, 97.3% ± 9.3. There were no associations between demographic characteristics, diagnosis, number of medications and compliance, neither no agreement between the TMG scores, BMQ, CQR-19, PRAQ, PedsQL-SSS and global adherence. On the other hand, there was agreement between these questionnaires and medication adherence. Although it was not observed a statistically significant correlation (via Spearman’s correlation) between the PRAQ score and the values of global and drug adherence to treatment (p= 0.111 and p=0.932 respectively), the levels of global adherence to treatment (90%) and adherence to drug treatment (94%) in our patients were high. In univariate logistic regression, no variable was found to be a predictor of good adherence. Conclusion: Our patients had very good global and medication adherence scores despite the COVID-19 pandemic. To our knowledge to date, there is no fully effective tool for predicting indicators of good treatment adherence.
- ItemAcesso aberto (Open Access)As miopatias inflamatórias no contexto brasileiro: uma revisão de escopo(Universidade Federal de São Paulo, 2023-11-23) Ruffato, Gabriella de Almeida [UNIFESP]; Melo, Daniela Oliveira de [UNIFESP]; Komoda, Denis Satoshi; http://lattes.cnpq.br/3643334150791076; http://lattes.cnpq.br/5052823551616937Introdução: As miopatias inflamatórias são doenças raras que envolvem inflamação crônica nos músculos esqueléticos. Apresentam uma ampla gama de manifestações clínicas e prognósticos variados, com prevalência mundial estimada entre 5 e 22 por 100 mil pessoas. No contexto brasileiro, não são bem conhecidas as características epidemiológicas desse grupo de doenças e pouco se sabe sobre as pesquisas realizadas. Objetivos: Mapear as evidências científicas publicadas relacionadas às miopatias inflamatórias, que sejam relacionadas ao contexto brasileiro. Métodos: Esta revisão de escopo foi conduzida de acordo com a metodologia publicada pelo Instituto Joanna Briggs (JBI). Foram realizadas buscas nas bases de dados: Medline (via Pubmed), Embase (via Elsevier) e Lilacs (via BVS), utilizando as expressões associados à "miopatia inflamatória" e "Brasil". A seleção de estudos e a extração de dados foi realizada por dois pesquisadores de forma independente e conflitos resolvidos por consenso e/ou com ajuda de um terceiro revisor. Foram incluídos quaisquer tipos de estudo enquanto em população brasileira e relacionados às miopatias inflamatórias. Foram excluídas as publicações em outros idiomas que não português, inglês e espanhol. Os dados foram extraídos em planilha eletrônica e posteriormente tabelados e analisados. Resultados: Foram identificadas 262 publicações. Após triagem por título e resumo e seleção por leitura integral do texto, foram incluídos 25 estudos nesta revisão. Vinte e três (92%) das publicações eram artigos originais, um resumo de congresso e uma tese. Vinte e três dos estudos (92%) foram publicados após 2010. Em relação ao estado da instituição de pesquisa, São Paulo representou 92% das publicações, seguido do Distrito Federal e Paraná (uma publicação cada). Com exceção de 2 revisões sistemáticas, todos os estudos eram observacionais (um caso-controle, três relatos de caso, oito transversais e 11 coortes). Quanto às características populacionais, 16 eram de população adulta e seis de população pediátrica, três não identificáveis. O conjunto de estudos avaliou quatro tipos de miopatia inflamatória, sendo dermatomiosite (68%) e polimiosite (32%) os mais comuns. A análise dos tópicos abordados, indicou que foram analisados 11 tópicos diferentes, sendo aspectos clínicos e laboratoriais o mais comum, representando 32% do conjunto de pesquisa, com um total de 8 estudos. Discussão e conclusão: A revisão revelou que a diversidade metodológica e temática dos estudos ressaltam a necessidade de uma abordagem integrativa e abrangente para entender e tratar os diferentes tipos de miopatias. Devido à baixa prevalência das miopatias inflamatórias, as abordagens de pesquisa têm sido limitadas, sendo conduzida por pesquisadores vinculados à universidade públicas e muito centralizados no estado de São Paulo.