Navegando por Palavras-chave "Myositis"
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- ItemAcesso aberto (Open Access)Análise da concentração sérica de miostatina e folistatina em pacientes com Dermatomiosite e Polimiosite(Universidade Federal de São Paulo (UNIFESP), 2020-06-26) Maso, Cintia [UNIFESP]; Sato, Emilia Inoue [UNIFESP]; Universidade Federal de São PauloBackground: dermatomyositis (DM) and polymyositis (PM) are systemic autoimmune myopathies (SAM) characterized by chronic inflammation of the skeletal muscle. Myostatin is a protein of the TGF- ß family that negatively regulates muscle mass and the follistatin is an antagonist of myostatin. Objective: to evaluate the serum concentration of myostatin and follistatin in patients with SAM and to evaluate the correlation of these protein with muscle strength, fatigue, functional capacity, damage index, serum creatine kinase and aldolase and body composition index. Methods: cross-sectional study with 50 patients (34 DM and 16 PM) and 52 age-and gender-matched controls. Myostatin and follistatin were measured by ELISA. The patients were evaluated according to the International consensus guidelines for trials of therapies in the idiopathic inflammatory myopathies (IMACS). Fatigue was evaluated through the fatigue severity scale (FSS), quality of life through the Short-Form Health Survey-36 (SF-36), physical activity level trough the International Physical Activity Questionnaire (IPAQ) and body composition was performed by a dual emission densitometer with an X-ray source (Lunar Radiation Corporation, model DPX, Madison, WI, USA). Results: we found no difference in myostatin (14.15±9.65 vs 10.97±6.77; p=0.131) and follistatin (0.53 ±0.71 vs 0.49±0.60; p=0.968) serum concentration between patients with SAM and controls, respectively. When DM, PM and control groups were analyzed separately, we observed higher serum levels of myostatin in the PM group compared to the control group (16.92 ± 12.07 vs. 10.97 ± 6.77 ng / mL; p=0.036). There was no difference in serum myostatin [10.99 (5.94-37.48) vs. 12.48 (1.51 - 35.44); p=0.861] and follistatin levels [0.40 (0.0-0.89) vs. (0.30 (0.0-2.28); p=0.886] among patients with and without low muscle mass by body composition, respectively. Patients without corticosteroids had higher serum levels of myostatin than controls. There was no correlation between myostatin levels with muscle strength, fatigue, functional capacity, damage index, serum creatine kinase and aldolase. There was a weak negative correlation of follistatin with muscle strength, functional capacity (SF-36), lean mass index (LMI) and lean appendicular mass (LAM) and weak positive correlation with HAQ, with no correlation with other studied variables. Conclusion: myostatin and follistatin serum concentration was not different between patients and controls, with higher myostatin serum level in those patients without corticosteroids. There was a weak negative correlation between follistatin and muscle strength, functional capacity (SF-36), lean mass index and a weak positive correlation with HAQ. The dosage of serum myostatin and follistatin did not seem useful to asses disease activity or as markers of treatment response in patients with MAS.
- ItemSomente MetadadadosCaracterísticas clínicas, resposta ao tratamento e sobrevida em uma coorte de doenças pulmonares intersticiais com achados de miosites(Universidade Federal de São Paulo (UNIFESP), 2021) Miranda, Gustavo Frazatto Medeiros De [UNIFESP]; Pereira, Carlos Alberto De Castro [UNIFESP]; Universidade Federal de São PauloIntroduction: Interstitial lung diseases (ILD) include a large number of conditions, which can sometimes be associated with myositis findings. Objectives: The aims of present study were to compare the clinical, functional, and tomographic characteristics, treatment response and survival in a Brazilian cohort of patients with ILD and myositis, classified into two groups: (1) With myositis-specific antibodies (MSA); and (2) myositis-associated antibodies (MAA) or clinical features of myositis. Methods: The diagnosis of myositis was characterized by: (1) presence of a mechanic's hands; or (2) elevation of muscle enzymes (creatine phosphokinase or aldolase) 2.5 times above the upper limit of normal associated with subjective muscle weakness or myalgia. All patients included in the study had pulmonary involvement on high-resolution chest tomography (HRCT). Data between the two groups were compared by t-test, median test, chi-square test, Fisher's exact test and survival curves were compared by Kaplan-Meier test. Significant functional decline was characterized by decrease in forced vital capacity (FVC) ≥ 5% of predicted value. Results: Mean age was 51 years and median follow-up was 50 months [IQ=25 - 98]. The mean FVC was 61.9 ± 16.6%. Among 77 patients analyzed, 43 (55.9%) were included in group 1, and 34 (44.1%) in group 2. The groups were similar, except that group 2 had more male subjects (61% versus 39%), more commonly audible pulmonary crackles (91% versus 72) and no SD pattern in capillaroscopy. There was no difference in types and duration of treatment between groups, neither in survival. Overall survival was greater than 80% at five years. Predictive factors for shorter survival were the presence honeycombing on HRCT (log-rank=34.65; p<0.001), and a decrease in FVC from 5% of predicted in longitudinal assessment (log-rank=16.50; p<0.001). Conclusion: Clinical, functional, and tomographic characteristics are similar between patients with ILD and presence of specific antisynthetase antibodies, and those with clinical findings of myositis, with or without associated antibodies. Presence of honeycombing on HRCT and a decrease in predicted FVC ≥ 5% are predictors of lower survival.
- ItemAcesso aberto (Open Access)Miosite de corpos de inclusão : correlação dos achados na ressonância magnética com parâmetros clínicos e funcionais(Universidade Federal de São Paulo (UNIFESP), 2017-08-17) Guimarães, Júlio Brandão [UNIFESP]; Fernandes, Artur da Rocha Correa [UNIFESP]; Zanoteli, Edmar [UNIFESP]; http://lattes.cnpq.br/0345383118760159; http://lattes.cnpq.br/5802740927050065; http://lattes.cnpq.br/8592264349028831; Universidade Federal de São Paulo (UNIFESP)Objetivo: O objetivo deste estudo foi avaliar os achados de ressonância magnética (RM) em pacientes com o diagnóstico definitivo de miosite de corpos de inclusão (MCI) e correlacionar com parâmetros clínicos e funcionais. Material e métodos: Este estudo incluiu 12 pacientes com MCI comprovada por biópsia. Todos os pacientes foram submetidos a estudo por ressonância magnética (RM) de ambos os membros superiores e inferiores. As imagens foram avaliadas para atrofia muscular, infiltração de gordura e padrão de edema. Os dados clínicos incluíram o início e a duração da doença; a força muscular foi medida usando a escala Medical Research Council (MRC) e o estado funcional foi avaliado utilizando a Escala de Rankin Modificada (mRS). As correlações entre os achados da RM e os diferentes parâmetros clínicos e funcionais foram calculados usando o Teste Spearman e correlação de Pearson. Resultados: Todos os pacientes apresentaram anormalidades no estudo por RM, mais graves nos membros inferiores e nos segmentos distais. O achado de RM mais prevalente foi a infiltração de gordura. Houve correlação estatisticamente significativa entre a duração da doença e o número de músculos infiltrados por gordura (r = 0,65, P = 0,04). O número de músculos com infiltração de gordura correlacionou-se com a soma dos escores de MRC (r = -0,60, p = 0,04) e com mRS (r = 0,48, p = 0,03) e o número de músculos com atrofia também correlacionou-se com a soma dos escores de MRC (r = -0,58, p = 0,04). Conclusão: Nossos resultados sugerem que a maioria dos pacientes com biópsia comprovada e diagnóstico definitivo de MCI apresentam um padrão típico de envolvimento muscular na ressonância magnética, mais extenso nos membros inferiores. Além disso, os achados de RM se correlacionaram fortemente com os parâmetros clínicos e funcionais, uma vez que quanto maior o envolvimento e gravidade do comprometimento muscular na RM, pior são os parâmetros clínicos e funcionais desses pacientes.