Longitudinal assessment of disease activity and muscle strength in juvenile dermatomyositis: a multicentre registry study

Objective To define disease activity measures, muscle strength and functional assessments in new-onset juvenile dermatomyositis (JDM) patients, at disease onset and follow up. Methods A registry was set up in 18 hospitals, enrolling patients over 3-years (2015-2018). Clinical assessments were perfor...

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Bibliographic Details
Authors: Antonio, D. Hortelan [UNESP], Carneiro, B. O.L., Fernandes, T. A.P. [UNESP], Elias, A. M., de Moraes, A.J. Pantoja, Vecchi, A. P., Cavalcanti, A., Rabelo, C. N., Magalhaes, C. M., Sztajnbok, F. R., Carvalho, L. M., Marques, L. Paim, Terreri, M. T., Fraga, M. M., de Oliveira, S. K.F., Sacchetti, S. B., Appenzeller, S., Robazzi, T., Ferriani, V. P.L., Len, C. A., Silva, C. A.A., Saad-Magalhaes, C. [UNESP]
Format: article
Status:Published version
Publication Date:2025
Country:Brasil
Institution:Universidade Estadual Paulista (UNESP)
Repository:Repositório Institucional da UNESP
Language:English
OAI Identifier:oai:repositorio.unesp.br:11449/300173
Online Access:http://dx.doi.org/10.55563/clinexprheumatol/yrb7m2
https://hdl.handle.net/11449/300173
Access Level:Open access
Keyword:disability
juvenile dermatomyositis
muscle strength
myositis
Description
Summary:Objective To define disease activity measures, muscle strength and functional assessments in new-onset juvenile dermatomyositis (JDM) patients, at disease onset and follow up. Methods A registry was set up in 18 hospitals, enrolling patients over 3-years (2015-2018). Clinical assessments were performed at baseline, and at 6, 12, 18 and 24 months after diagnosis. Disease Activity Score (DAS20), skin and musculoskeletal DAS sub-scales; Manual Muscle Test (MMT8); Childhood Myositis Assessment Scale (CMAS); Childhood Health Assessment Questionnaire disability index (CHAQ_DI 0-3) and 10 cm Visual Analogue Scale (VAS) for overall well-being scores were compared by Poisson Model and Wald post-test for repeated measures. Results Ninety-six cases, being 61 (64%) females, median age 10 years had JDM diagnosis and 12 (13%) onset calcinosis. Mean ±SD scores at diagnosis and 6 months intervals for DAS20 (0-20) were 7.8±5, 6.3 ±4.8, 5±4, 4.9 ±5 and 0.5 ±2.3; with significant difference from baseline (p<0.01). Skin DAS subscales were 2.8±3.3, 1.8±2.9, 1,1±2.2, 0.6±1.8, 0.4±1.5. MMT (0-80) 62.6±20.4, 70.2±13.5, 73.3±11, 75.7±7.9 and 74.8±7.8, with significant difference from baseline up to 6 months (p=0.016); CMAS (0-53) 29.5±11.4, 33.1±8.3, 34.2±5.8, 34±6 and 33.3±5.4. CHAQ-DI (0-3) 1±0.9, 0.6±0.7, 0.8±0.8, 1±0.8 and 1±0.3; parents VAS 4.1±2.5, 2±2.1; 1.3±2.8, 4.1±3.1, 1.7±2.2. There was no significant difference for CMAS, CHAQ-DI and parents VAS from baseline up to 24-month assessment. Conclusion DAS20 scores improved gradually during follow up, MMT8 improved significantly during the first 6 months and CMAS, CHAQ-DI and parents VAS scores had no significant improvement with persistent functional impairment over 2-years.

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