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

Q: What was the study objective regarding juvenile dermatomyositis?

To define disease activity measures, muscle strength, and functional assessments in new-onset juvenile dermatomyositis patients at onset and during follow-up.

Q: Which assessment tools were used in the registry?

Disease activity was measured with DAS20 and its skin and musculoskeletal subscales; muscle strength with MMT8; function with CMAS; disability with CHAQ_DI; and overall well-being using a 10 cm VAS.

Q: What were the main findings over the 24-month follow-up?

DAS20 improved gradually over time, MMT8 improved significantly during the first 6 months, while CMAS, CHAQ_DI and parents’ VAS showed no significant improvement up to 24 months, with persistent functional impairment.

Oct 2, 2026

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A multicentre registry study evaluates longitudinal disease activity and functional outcomes in patients with new-onset juvenile dermatomyositis. Patients were assessed at baseline and at 6, 12, 18 and 24 months using DAS20 with skin and musculoskeletal subscales, MMT8, CMAS, CHAQ_DI and a 10 cm VAS for overall well-being. Ninety-six cases were followed over the 2015–2018 enrollment period.

LanguageEnglish

CategoryHealthcare

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Pages8

Reading Time20 min

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Abstract

Objective

Methods

Results

Conclusion

Key words

Introduction

Paediatric rheumatology | Longitudinal assessment of disease activity and muscle strength in juvenile dermatomyositis: a multicentre registry study
D. Hortelan Antonio 1 , B.O.L. Carneiro2 , T.A.P. Fernandes 1 , A.M. Elias2 ,
A.J. Pantoja de Moraes3 , A.P. Vecchi4 , A. Cavalcanti5 , C.N. Rabelo Jr6 ,
C.M. Magalhaes7 , F.R. Sztajnbok8 , L.M. Carvalho9 , L. Paim Marques 10 ,
M.T. Terreri 11 , M.M. Fraga 12 , S.K.F. de Oliveira 13 , S.B. Sacchetti 14 , S. Appenzeller15 ,
T. Robazzi 16 , V.P.L. Ferriani 17 , C.A. Len 11 , C.A.A. Silva2 , C. Saad-Magalhaes 1 | | --- | | 1Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu; 2Instituto da Criança e Adolescente, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo; 3 Universidade Federal do Para (UFPA) , Belem; 4Hospital Materno-Infantil de Goiania, Goiania; 5 Universidade Federal de Pernambuco (UFPE), Recife; 6Hospital Infantil Albert Sabin, Fortaleza; 7Hospital Jose de Alencar, Brasilia: 8 Universidade Estadual do Rio de Janeiro (UERJ); 9Faculdade de Medicina de Ribeirao Preto, Universidade de Sao Paulo (USP), Ribeirao Preto; 10Hospital Infantil Albert Sabin, Fortaleza, Brazil; and Paediatrics Department of Texas Tech University Health Science Center (TTUHSC), El Paso, USA; 11 Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo; 12Hospital Infantil Darcy Vargas, São Paulo; 13Instituto de Puericultura e Pediatria Martagao Gesteira, Universidade Federal do Rio de Janeiro (IPPMG-UFRJ), Rio de Janeiro;
14Faculdade de Medicina da Santa Casa de Sao Paulo; 15 Universidade Estadual de Campinas (UNICAMP); 16 Universidade Federal da Bahia (UFBA), Salvador; 17Faculdade de Medicina de Ribeirao Preto, Universidade de Sao Paulo (USP), Ribeirao Preto, Brazil. | | Abstract
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. | | Key words
disability, juvenile dermatomyositis, muscle strength, myositis | Clinical and Experimental Rheumatology 2025; 43: 379-386. Muscle strength in juvenile dermatomyositis / D. Hortelan Antonio et al. Darcisio Hortelan Antonio, BPt, PhD Beatriz O.L. Carneiro, MD TacianaA.P. Fernandes, MD, PhD Adriana M. Elias, MD, PhD A.J. Pantoja de Moraes, MD, PhD A.P. Vecchi, MD, PhD

FAQs

What was the study objective regarding juvenile dermatomyositis?

To define disease activity measures, muscle strength, and functional assessments in new-onset juvenile dermatomyositis patients at onset and during follow-up.

Which assessment tools were used in the registry?

Disease activity was measured with DAS20 and its skin and musculoskeletal subscales; muscle strength with MMT8; function with CMAS; disability with CHAQ_DI; and overall well-being using a 10 cm VAS.

What were the main findings over the 24-month follow-up?

DAS20 improved gradually over time, MMT8 improved significantly during the first 6 months, while CMAS, CHAQ_DI and parents’ VAS showed no significant improvement up to 24 months, with persistent functional impairment.

Oct 2, 2026

Uploaded by Ophelia