Hortelan Antonio Darcisio, Carneiro Beatriz O L, Fernandes Taciana A P, Elias Adriana M, Pantoja de Moraes Ana Julia, Vecchi Ana Paula, Cavalcanti Andre, Rabelo Carlos N, Magalhaes Cristina M, Sztajnbok Flavio R, Carvalho Luciana M, Paim Marques Luciana, Terreri Maria Teresa, Fraga Melissa M, de Oliveira Sheila K F, Sacchetti Silvana B, Appenzeller Simone, Robazzi Teresa, Ferriani Virginia P L, Len Claudio A, Silva Clovis A A, Saad-Magalhaes Claudia
Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (UNESP), Botucatu, Brazil.
Instituto da Criança e Adolescente, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, Brazil.
Clin Exp Rheumatol. 2025 Feb;43(2):379-386. doi: 10.55563/clinexprheumatol/yrb7m2. Epub 2024 Sep 24.
To define disease activity measures, muscle strength and functional assessments in new-onset juvenile dermatomyositis (JDM) patients, at disease onset and follow up.
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.
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.
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.
确定新发青少年皮肌炎（JDM）患者在疾病发作时和随访期间的疾病活动度测量指标、肌肉力量和功能评估。
在18家医院建立了一个登记处，纳入3岁以上（2015 - 2018年）的患者。在基线时以及诊断后6、12、18和24个月进行临床评估。通过泊松模型和重复测量的Wald事后检验比较疾病活动评分（DAS20）、皮肤和肌肉骨骼DAS子量表；徒手肌力测试（MMT8）；儿童肌炎评估量表（CMAS）；儿童健康评估问卷残疾指数（CHAQ_DI 0 - 3）以及用于总体健康评分的10厘米视觉模拟量表（VAS）。
96例患者被诊断为JDM，其中61例（64%）为女性，中位年龄10岁，12例（13%）出现钙质沉着。诊断时以及每隔6个月的DAS20（0 - 20）平均±标准差分数分别为7.8±5、6.3±4.8、5±4、4.9±5和0.5±2.3；与基线相比有显著差异（p<0.01）。皮肤DAS子量表分数分别为2.8±3.3、1.8±2.9、1.1±2.2、0.6±1.8、0.4±1.5。MMT（0 - 80）分数分别为62.6±20.4、70.2±13.5、73.3±11、75.7±7.9和74.8±7.8，在6个月内与基线相比有显著差异（p = 0.016）；CMAS（0 - 53）分数分别为29.5±11.4、33.1±8.3、34.
