Avaliação longitudinal das medidas de atividade, força muscular e capacidade funcional na Dermatomiosite Juvenil
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Data
2023-03-23
Autores
Antonio, Darcisio Hortelan
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Universidade Estadual Paulista (Unesp)
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Introdução: As miopatias inflamatórias idiopáticas são raras na faixa etária pediátrica; afetando a pele, os músculos esqueléticos, e múltiplos órgãos e sistemas,, sendo a Dermatomiosite Juvenil (DMJ) a mais frequente. A fraqueza muscular proximal em cinturas pélvica e escapular são os indicadores de atividade da DMJ , sendo a recuperação da força muscular um dos parâmetros de resposta ao tratamento, desfecho e prognóstico. Objetivo: Explorar os parâmetros clínicos, força e capacidade funcional na apresentação e no acompanhamento longitudinal (0, 6, 12, 18 e 24 meses) em uma coorte no registro nacional, incluindo 19 serviços de Reumatologia Pediátrica. Método: Análise longitudinal de DMJ, do diagnóstico aos 24 meses, das variáveis do perfil clínico demográfico, de casos iniciais de DMJ, registrados de 2015 a 2017. Foram realizados os testes padronizados, DAS, MMT, CMAS, CHAQ e pelo médico assistente em cada um dos serviços participantes, durante as visitas de rotina. Foi realizada a estatística descritiva e comparação de parâmetros clínicos e métricas funcionais por meio de um modelo de medidas repetidas de Poisson, sendo a diferença entre as médias dos testes em cada visita pelo teste de Wald, estabelecendo-se significância em 5% ou p<0,05. Resultados: Foram incluídos 96 casos, com idades entre 4 a 18 anos, 61 do sexo feminino. A pontuação dos instrumentos foi descrita pela média±DP. Entre os dois instrumentos de avaliação muscular a MMT obteve diferença significante entre as visitas (0) (37±34,6) , (6m) (59,1±28,7), (12m) (65,2±25,4), (18m), (71±21), (24m) (76,1±5,5) p=0,016. A pontuação do CMAS não mostrou variação significante (0) (28,6±12,7), (6m) (32,8±11), (12m) ( 33,7±6,8), (18m) (33,5±7,2) (24m) (34,2±4,3) p = 0,06. A avaliação de atividade por meio do DAS, indicou diferença significante entre todas as visitas e com melhora progressiva dos 0 a 24 meses (0) (10,7±4,2), (6m) (7,7±5), (12m) (2,5±4,1), (18m) (1,5±3,1) e (24m) (0,8±2,5) (p <0,0001). As medidas relatadas pelos pais, CHAQ-DI (0) (1±0,8), (6m) (0,5±0,7), (12m) (0,8±0,7), (18m) ( 0,7±0,7) (24m) (0,9±0,3) p=0,734. A VAS para bem estar global apresentou (0) (41,3±25,4), (6m) (20,1±21,3), (12m) (30,1± 27,7), (18m) (40±31) e (24m) (17,1±22,1) p = 0,284, e VAS – Dor (0) (43±25,7), (6m) (11,8±19,6), (12m) (15,9±19,6), (18m) (22,1±24,8) e (24m) (12,1±21,1) p = 0,284 também não apresentaram diferença significante em todas as visitas registradas. Conclusão: Observou-se a exequibilidade e responsividade dos parâmetros quantitativos de avaliação de força muscular, funcionalidade e atividade da miosite, tendo o MMT e o DAS os melhores desempenhos.
Introduction: Idiopathic inflammatory myopathies are rare pediatric rheumatic diseases and Juvenile Dermatomyositis is the most frequent; it affects the skin, skeletal muscles, and multiple organs and systems. Proximal muscle weakness is the hallmark of JDM activity, being the recovery of muscle strength one of the parameters of response to treatment, outcome and prognosis. Objective: Explore clinical presentation and followup data at 0, 6, 12, 18, and 24 months in an case series from a national registry, recruited from 19 Pediatric Rheumatology services, with standardized quantitative measures of muscle strength, functionality, and activity indices. Method: Descriptive analysis, from the diagnosis up to 24 months, of the clinical-demographic variables of incident JDM cases from 2015 to 2017. Standardized tests, DAS, MMT, CMAS, CHAQ were carried out during the visits made by the attending physician in each of the participating services. Clinical parameters and functional metrics comparison was by Poisson repeated measures model, and the difference between the means of each test during each visit was compared by Wald test, with significance at 5% or p <0,05. Results: A total of 96 cases were included, aged between 4 and 20 years, 61 female and 35 male. The scores were described by means±SD. MMT scoring had a significant difference between visits p=0,016, and being the baseline to 6m significant (0) (37±34,6) , (6m) (59,1±28,7), (12m) (65,2±25,4), (18m), (71±21), (24m) (76,1±5,5). The CMAS at (0) (28,6±12,7), (6m) (32,8±11), (12m) ( 33,7±6,8), (18m) (33,5±7,2) (24m) (34,2±4,3) p = 0,06, with no significant difference among the visits from baseline. The activity assessment using the DAS indicated a significant difference and progressive improvement from 0 to 24 months (0) (10,7±4,2), (6m) (7,7±5), (12m) (2, 5±4.1), (18m) (1,5±3,1) and (24m) (0,8±2,5) (p <0,0001), discriminating all the visits. Measures reported by parents, CHAQ-DI (0) (1±0,8), (6m) (0,5±0,7), (12m) (0,8±0,7), (18m) ( 0,7±0,7) (24m) (0,9±0,3) p=0,734; the VAS for global well-being presented (0) (41,3±25,4), (6m) (20,1±21,3), (12m) (30,1± 27,7), (18m) ( 40±31) and (24m) (17,1±22,1) p = 0,284, and the VAS – Pain (0) (43±25,7), (6m) (11,8±19,6), ( 12m) (15,9±19,6), (18m) (22,1±24,8) and (24m) (12,1±21,1) p = 0,284 all showed no significant difference in all recorded visits. Conclusion: The feasibility and responsiveness of the quantitative parameters for assessing muscle strength, functionality and myositis activity were observed, with MMT and DAS having the best performances
Introduction: Idiopathic inflammatory myopathies are rare pediatric rheumatic diseases and Juvenile Dermatomyositis is the most frequent; it affects the skin, skeletal muscles, and multiple organs and systems. Proximal muscle weakness is the hallmark of JDM activity, being the recovery of muscle strength one of the parameters of response to treatment, outcome and prognosis. Objective: Explore clinical presentation and followup data at 0, 6, 12, 18, and 24 months in an case series from a national registry, recruited from 19 Pediatric Rheumatology services, with standardized quantitative measures of muscle strength, functionality, and activity indices. Method: Descriptive analysis, from the diagnosis up to 24 months, of the clinical-demographic variables of incident JDM cases from 2015 to 2017. Standardized tests, DAS, MMT, CMAS, CHAQ were carried out during the visits made by the attending physician in each of the participating services. Clinical parameters and functional metrics comparison was by Poisson repeated measures model, and the difference between the means of each test during each visit was compared by Wald test, with significance at 5% or p <0,05. Results: A total of 96 cases were included, aged between 4 and 20 years, 61 female and 35 male. The scores were described by means±SD. MMT scoring had a significant difference between visits p=0,016, and being the baseline to 6m significant (0) (37±34,6) , (6m) (59,1±28,7), (12m) (65,2±25,4), (18m), (71±21), (24m) (76,1±5,5). The CMAS at (0) (28,6±12,7), (6m) (32,8±11), (12m) ( 33,7±6,8), (18m) (33,5±7,2) (24m) (34,2±4,3) p = 0,06, with no significant difference among the visits from baseline. The activity assessment using the DAS indicated a significant difference and progressive improvement from 0 to 24 months (0) (10,7±4,2), (6m) (7,7±5), (12m) (2, 5±4.1), (18m) (1,5±3,1) and (24m) (0,8±2,5) (p <0,0001), discriminating all the visits. Measures reported by parents, CHAQ-DI (0) (1±0,8), (6m) (0,5±0,7), (12m) (0,8±0,7), (18m) ( 0,7±0,7) (24m) (0,9±0,3) p=0,734; the VAS for global well-being presented (0) (41,3±25,4), (6m) (20,1±21,3), (12m) (30,1± 27,7), (18m) ( 40±31) and (24m) (17,1±22,1) p = 0,284, and the VAS – Pain (0) (43±25,7), (6m) (11,8±19,6), ( 12m) (15,9±19,6), (18m) (22,1±24,8) and (24m) (12,1±21,1) p = 0,284 all showed no significant difference in all recorded visits. Conclusion: The feasibility and responsiveness of the quantitative parameters for assessing muscle strength, functionality and myositis activity were observed, with MMT and DAS having the best performances
Descrição
Palavras-chave
CMAS, Dermatomiosite, Força muscular, MMT, Miopatias inflamatórias idiopáticas, Registro, Músculos - Doenças inflamatórias., Artrite idiopática juvenil