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首页> 外文期刊>Rheumatology >Disease activity and prognostic factors in juvenile dermatomyositis: A long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool
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Disease activity and prognostic factors in juvenile dermatomyositis: A long-term follow-up study applying the Paediatric Rheumatology International Trials Organization criteria for inactive disease and the myositis disease activity assessment tool

机译：少年皮肌炎的疾病活动性和预后因素：一项长期随访研究，应用小儿风湿病国际试验组织关于非活动性疾病的标准和肌炎疾病活动性评估工具

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Objectives: The aims of this study were to examine disease activity by the Paediatric Rheumatology International Trials Organization (PRINTO) criteria for inactive disease and the Myositis Disease Activity Assessment Tool (MDAAT) in JDM patients after long-term follow-up and to identify predictors of these outcomes. Methods: A retrospective inception cohort of 59 patients diagnosed with JDM was clinically examined in a cross-sectional study a median of 16.8 years (range 2.0-38.1) after symptom onset. Patients were divided by the PRINTO criteria into clinically inactive and active disease. Disease activity was also measured by MDAAT and other validated tools. Medical records were reviewed for early disease variables and medication. Results: By the PRINTO criteria, 31/59 (51%) patients were active and 29/59 (49%) were inactive. By MDAAT, 43/59 (73%) of the patients had measurable disease activity, most commonly found in the skin (59%) and skeletal (27%) systems. MDAAT showed moderate to strong correlations with other disease activity measures (rsp 0.39-0.87, P0.05) except for muscle enzymes. Active patients had higher disease activity than inactive patients measured by MDAAT (P0.001) and other disease characteristics (all P≤0.002) except for patients' global assessment of disease activity. After controlling for gender and follow-up time, calcinosis during disease-course predicted high MDAAT, age9 years at diagnosis predicted active disease and organ damage present 6-12 months post diagnosis predicted both outcomes. Conclusion: After 16.8 years, 51-73% of JDM patients had active disease. Disease activity by the PRINTO criteria and MDAAT were moderately to highly associated with most other disease characteristics and was predicted by early damage.
机译：目的：本研究的目的是通过国际长期小儿风湿病国际临床试验组织（PRINTO）对非活动性疾病的标准和JDM患者的肌炎疾病活动性评估工具（MDAAT）进行检查，并确定预测因素这些结果。方法：在一项横断面研究中，对症状发作后中位数为16.8年（范围2.0-38.1）的临床回顾性队列研究，对59例诊断为JDM的患者进行了回顾性研究。根据PRINTO标准将患者分为临床上不活动和活动中的疾病。还通过MDAAT和其他经过验证的工具测量了疾病活动。回顾病历以了解早期疾病变量和药物治疗。结果：根据PRINTO标准，有31/59（51％）患者处于活动状态，有29/59（49％）患者处于非活动状态。通过MDAAT，有43/59（73％）的患者具有可测量的疾病活动，最常见于皮肤（59％）和骨骼（27％）系统。除肌肉酶外，MDAAT与其他疾病活动性指标（rsp 0.39-0.87，P <0.05）显示中等至强相关性。通过MDAAT（P <0.001）和其他疾病特征（所有P≤0.002）测量的活动患者比未活动患者具有更高的疾病活动性，但患者对疾病活动的总体评估除外。在控制了性别和随访时间之后，疾病过程中的钙化病预示着MDAAT较高，诊断时年龄<9岁预示了活动性疾病，诊断后6-12个月内器官损害预示了两种预后。结论：16.8年后，有51-73％的JDM患者患有活动性疾病。根据PRINTO标准和MDAAT进行的疾病活动与大多数其他疾病特征呈中度至高度相关，并且可以通过早期损害进行预测。

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