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Paediatric Rheumatology

 

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

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22

 

  1. Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, Brazil.
  2. Instituto da Criança e Adolescente, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, Brazil.
  3. Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, Brazil.
  4. Instituto da Criança e Adolescente, Hospital das Clínicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo (USP), Sao Paulo, Brazil.
  5. Universidade Federal do Para (UFPA), Belem, Brazil.
  6. Hospital Materno-Infantil de Goiania, Brazil.
  7. Universidade Federal de Pernambuco (UFPE), Recife, Brazil.
  8. Hospital Infantil Albert Sabin, Fortaleza, Brazil.
  9. Hospital Jose de Alencar, Brasilia, Brazil.
  10. Universidade Estadual do Rio de Janeiro (UERJ), Brazil.
  11. Faculdade de Medicina de Ribeirao Preto, Universidade de Sao Paulo (USP), Ribeirao Preto, Brazil.
  12. Hospital Infantil Albert Sabin, Fortaleza, Brazil; and Paediatrics Department of Texas Tech University Health Science Center (TTUHSC), El Paso, USA.
  13. Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, Brazil.
  14. Hospital Infantil Darcy Vargas, São Paulo, Brazil.
  15. Instituto de Puericultura e Pediatria Martagao Gesteira, Universidade Federal do Rio de Janeiro (IPPMG-UFRJ), Rio de Janeiro, Brazil.
  16. Faculdade de Medicina da Santa Casa de Sao Paulo, Brazil.
  17. Universidade Estadual de Campinas (UNICAMP), Campinas, Brazil.
  18. Universidade Federal da Bahia (UFBA), Salvador, Brazil.
  19. Faculdade de Medicina de Ribeirao Preto, Universidade de Sao Paulo (USP), Ribeirao Preto, Brazil.
  20. Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, Brazil.
  21. Instituto da Criança e Adolescente, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, Brazil.
  22. Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, Brazil. claudia.saad@unesp.br

CER17663
2025 Vol.43, N°2
PI 0379, PF 0386
Paediatric Rheumatology

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PMID: 39360369 [PubMed]

Received: 09/03/2024
Accepted : 18/07/2024
In Press: 24/09/2024
Published: 26/02/2025

Abstract

OBJECTIVES:
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 Analog Scale (VAS) for overall wellbeing 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.
CONCLUSIONS:
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.

DOI: https://doi.org/10.55563/clinexprheumatol/yrb7m2

Rheumatology Article