O ultrassom e a elastografia muscular seriam uma ferramenta promissora para avaliar atividade de doença nos pacientes com dermatomiosite juvenil?
Arquivos
Data
2022-07-28
Autores
Andrade, Renata Lopes Francisco de [UNIFESP]
Orientadores
Terreri, Maria Teresa de Sande e Lemos Ramos Ascensão [UNIFESP]
Tipo
Dissertação de mestrado
Título da Revista
ISSN da Revista
Título de Volume
Resumo
Introdução: A Dermatomiosite Juvenil (DMJ) é a miopatia inflamatória idiopática (MII) mais comum na faixa etária pediátrica. Para o diagnóstico, muitas vezes são necessários exames invasivos de difícil aceitação pela população pediátrica. Além disso, há dificuldade em definir se a doença se apresenta em atividade ou apenas com sequelas musculares, o que é importante na definição do tratamento. A introdução de novos exames de imagem tem permitido uma melhor avaliação do acometimento muscular da DMJ, sendo a Ultrassonografia (USG) um método promissor na faixa etária pediátrica. Em MII de pacientes adultos, a USG demonstrou boa capacidade em definir o sítio para biópsia muscular, boa sensibilidade para auxílio diagnóstico, além de especificidade diagnóstica semelhante à da Ressonância Magnética (RM) e utilidade na diferenciação entre as fases aguda e crônica das miopatias. Na DMJ, a USG mostrou-se útil no auxílio à definição do grau de atividade de doença e no seguimento dos pacientes, demonstrando gravidade e dano muscular associados à miopatia. Com relação à elastografia muscular, técnica mais recentemente incorporada à USG, os estudos são mais escassos e têm resultados controversos. Em MII, a elastografia parece evidenciar aumento da rigidez muscular compatível à encontrada na RM. Entretanto, na faixa etária pediátrica, a elastografia não foi satisfatória para detecção de miosite ativa, embora tenha apresentado correlação entre as anormalidades elastográficas encontradas e o aumento da ecogenicidade muscular encontrada na RM. Pela escassez de publicações no tema, decidimos realizar este estudo.
Objetivos: Os objetivos deste estudo foram estabelecer associação da avaliação clínica (através dos instrumentos validados), laboratorial e de capilaroscopia periungueal (CPU) com a avaliação ultrassonográfica por escala de cinza (qualitativa e semiquantitativa), por Power-Doppler (PD) e de elastografia muscular através da técnica de Strain-Elastography (SE - Elastografia por compressão), em pacientes diagnosticados com DMJ; e avaliar a sensibilidade, especificidade e valores preditivos da elastografia.
Metodologia: Foram avaliados 22 pacientes diagnosticados com DMJ e em seguimento ambulatorial regular, e 14 indivíduos controles saudáveis, entre 5 e 21 anos de idade, comparados por idade e sexo. Os pacientes foram submetidos à avaliação clínica através da aplicação de questionários de avaliação muscular (CMAS - Childhood Myositis Assessment Scale e MMT - Manual Muscle Testing), avaliação global da doença por aplicação de questionário (DAS - Disease Activity Score) e de pontuação em escala visual analógica (VAS - Visual analogic scale) do médico e dos pais e pacientes e avaliação da capacidade funcional através do questionário CHAQ (Childhood Health Assessment Questionnaire). Os pacientes também foram submetidos à CPU e realização de dosagem de enzimas musculares, conforme preconizado no seguimento ambulatorial. Todos os indivíduos do estudo foram submetidos à avaliação ultrassonográfica através de escala de cinza, aplicação do PD e aplicação da SE.
Resultados: Na avaliação pela USG pela escala de cinza qualitativa e semiquantitativa de pacientes e controles observamos maior frequência de alteração nos exames dos pacientes (p<0,001 para ambas). Na avaliação do PD houve maior frequência de positividade nos deltoides e tibiais anteriores de pacientes (p<0,001). Parâmetros de atividade de doença (dosagem de enzimas musculares, MMT, CMAS, DAS e VAS) correlacionaram-se com alterações ultrassonográficas em grupos musculares distintos. A presença de atividade de doença associou-se com alteração importante na escala de cinza semiquantitativa em deltoides (p=0,007), bíceps braquiais (p<0,001) e quadríceps femorais (p=0,005). A partir da escala de cinza, propusemos um ponto de corte de 54,36% de rigidez muscular patológica pela elastografia, com sensibilidade de 45,3%, especificidade de 64,4%, valor preditivo positivo de 18% e valor preditivo negativo (VPN) de 87,2% e acurácia de 61,6%.
Conclusão: A USG foi capaz, através da avaliação da escala de cinza qualitativa e semiquantitativa, de diferenciar pacientes com DMJ de pacientes saudáveis, enquanto o PD foi capaz de realizar tal diferenciação apenas para deltoides e tibiais anteriores. A escala de cinza semiquantitativa foi capaz de inferir atividade de doença nos músculos proximais. Entretanto, apenas alguns grupos musculares apresentaram correlação entre os achados ultrassonográficos, de escala de cinza, PD e SE, com os marcadores de atividade de doença. A SE apresentou baixa sensibilidade e especificidade, porém seu elevado VPN evidenciou boa capacidade da técnica em detectar ausência de lesão muscular inflamatória.
Background: Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy (IIM) in the pediatric age group. For diagnosis, we often need invasive tests that are difficult to be accepted by children. In addition, it is hard to define whether the disease is active or only sequelae lesions are present, which is important in determining the therapy. The introduction of new imaging exams has allowed a better assessment of muscle involvement in JDM, with ultrasound (US) being a promising method in the pediatric age group. In adults IIM, US demonstrated a good ability to determine the site for muscle biopsy, good sensitivity to help in diagnosis (similar to electroneuromyography - ENMG - and creatine kinase - CK measurement) and diagnostic specificity similar to Magnetic Resonance Imaging (MRI). Also, US is useful in differentiating between the acute and chronic phases of myopathies. In JDM, US proved to be useful in helping to define the degree of disease activity and also in patient follow-up, demonstrating the severity and tissue damage associated with the myopathy. Regarding muscle elastography, that is a technique more recently incorporated into US, studies are scarce and have controversial results. In IIM, elastography seems to show an increase in muscle stiffness compatible with that found in MRI. More particularly in the pediatric age group, elastography was not satisfactory for detecting active myositis, although it showed a correlation between the elastographic abnormalities with the increase in muscle echogenicity found in MRI. Due to the scarcity of publications, we decide to carry out this study. Objectives: The objectives of this study were to establish an association of clinical assessment (using validated instruments), laboratory and nailfold capillaroscopy (NC) assessment with gray scale (qualitative and semi-quantitative), Power-Doppler (PD) and muscle elastography ultrasound assessment. through the Strain-Elastography (SE) technique, in patients diagnosed with JDM; and to evaluate the sensitivity, specificity and predictive values of elastography. Methods: Twenty-two JDM-patients and fourteen healthy controls, aged between 5 and 21 years, matched for age and sex were enrolled. Patients underwent clinical evaluation through the application of muscle assessment questionnaires (Childhood Myositis Assessment Scale - CMAS and Manual Muscle Testing - MMT), global assessment of the disease through the application of a questionnaire (Disease Activity Score - DAS) and visual analogue scale (Visual analogic scale - VAS) scores by the physician and parents and patients and assessment of functional capacity through the 8 CHAQ (Childhood Health Assessment Questionnaire). Patients were also submitted to NC and measurement of muscle enzymes, as recommended in follow-up. All subjects in the study underwent US assessment using gray scale, application of PD and application of SE. Results: In the qualitative and semi-quantitative gray scale US evaluation of patients and controls, we observed a higher frequency of alterations in the patients' exams (p<0.001 for both). In the PD assessment, there was a higher frequency of positivity in the patients' anterior deltoids and tibialis (p<0.001). Disease activity parameters (muscle enzyme dosage, MMT, CMAS, DAS and VAS) correlated with sonographic changes in different muscle groups. The presence of disease activity was associated with an important change in the semi-quantitative gray scale in deltoids (p:0.007), biceps brachii (p<0.001) and quadriceps femoris (p:0.005.). From the gray scale, we proposed a cut-off point for elastography, from which we can consider the muscle with pathological stiffness. This cut-off point was 54.36%, with sensitivity of 45.3%, specificity of 64.4%, positive predictive value (PPV) of 18% and negative predictive value (NPV) of 87.2% and accuracy of 61.6%. Conclusion: US was able, through qualitative and semi-quantitative grayscale evaluation, to differentiate patients with JDM from healthy patients, while PD was able to perform such differentiation only for deltoids and tibialis anterior. The semiquantitative gray scale was able to infer disease activity in the proximal muscles. However, only a few muscle groups showed a correlation between ultrasound, grayscale, PD and SE findings with disease activity markers. SE showed low sensitivity and specificity, however, its high NPV evidences the technique's good ability to detect the absence of inflammatory muscle injury.
Background: Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy (IIM) in the pediatric age group. For diagnosis, we often need invasive tests that are difficult to be accepted by children. In addition, it is hard to define whether the disease is active or only sequelae lesions are present, which is important in determining the therapy. The introduction of new imaging exams has allowed a better assessment of muscle involvement in JDM, with ultrasound (US) being a promising method in the pediatric age group. In adults IIM, US demonstrated a good ability to determine the site for muscle biopsy, good sensitivity to help in diagnosis (similar to electroneuromyography - ENMG - and creatine kinase - CK measurement) and diagnostic specificity similar to Magnetic Resonance Imaging (MRI). Also, US is useful in differentiating between the acute and chronic phases of myopathies. In JDM, US proved to be useful in helping to define the degree of disease activity and also in patient follow-up, demonstrating the severity and tissue damage associated with the myopathy. Regarding muscle elastography, that is a technique more recently incorporated into US, studies are scarce and have controversial results. In IIM, elastography seems to show an increase in muscle stiffness compatible with that found in MRI. More particularly in the pediatric age group, elastography was not satisfactory for detecting active myositis, although it showed a correlation between the elastographic abnormalities with the increase in muscle echogenicity found in MRI. Due to the scarcity of publications, we decide to carry out this study. Objectives: The objectives of this study were to establish an association of clinical assessment (using validated instruments), laboratory and nailfold capillaroscopy (NC) assessment with gray scale (qualitative and semi-quantitative), Power-Doppler (PD) and muscle elastography ultrasound assessment. through the Strain-Elastography (SE) technique, in patients diagnosed with JDM; and to evaluate the sensitivity, specificity and predictive values of elastography. Methods: Twenty-two JDM-patients and fourteen healthy controls, aged between 5 and 21 years, matched for age and sex were enrolled. Patients underwent clinical evaluation through the application of muscle assessment questionnaires (Childhood Myositis Assessment Scale - CMAS and Manual Muscle Testing - MMT), global assessment of the disease through the application of a questionnaire (Disease Activity Score - DAS) and visual analogue scale (Visual analogic scale - VAS) scores by the physician and parents and patients and assessment of functional capacity through the 8 CHAQ (Childhood Health Assessment Questionnaire). Patients were also submitted to NC and measurement of muscle enzymes, as recommended in follow-up. All subjects in the study underwent US assessment using gray scale, application of PD and application of SE. Results: In the qualitative and semi-quantitative gray scale US evaluation of patients and controls, we observed a higher frequency of alterations in the patients' exams (p<0.001 for both). In the PD assessment, there was a higher frequency of positivity in the patients' anterior deltoids and tibialis (p<0.001). Disease activity parameters (muscle enzyme dosage, MMT, CMAS, DAS and VAS) correlated with sonographic changes in different muscle groups. The presence of disease activity was associated with an important change in the semi-quantitative gray scale in deltoids (p:0.007), biceps brachii (p<0.001) and quadriceps femoris (p:0.005.). From the gray scale, we proposed a cut-off point for elastography, from which we can consider the muscle with pathological stiffness. This cut-off point was 54.36%, with sensitivity of 45.3%, specificity of 64.4%, positive predictive value (PPV) of 18% and negative predictive value (NPV) of 87.2% and accuracy of 61.6%. Conclusion: US was able, through qualitative and semi-quantitative grayscale evaluation, to differentiate patients with JDM from healthy patients, while PD was able to perform such differentiation only for deltoids and tibialis anterior. The semiquantitative gray scale was able to infer disease activity in the proximal muscles. However, only a few muscle groups showed a correlation between ultrasound, grayscale, PD and SE findings with disease activity markers. SE showed low sensitivity and specificity, however, its high NPV evidences the technique's good ability to detect the absence of inflammatory muscle injury.