幼年特发性关节炎疾病活动度和预后因素：应用儿科风湿病国际临床试验组织的疾病不活动标准和肌炎疾病活动评估工具进行的长期随访研究。
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.
机构信息
Section of Rheumatology, Norwegian Competence Center of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Institute for Experimental Medical Research, Department of Cardiology, Oslo University Hospital-Ullevål and Institute for Clinical Medicine, Medical Faculty, University of Oslo, Norway.Section of Rheumatology, Norwegian Competence Center of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Institute for Experimental Medical Research, Department of Cardiology, Oslo University Hospital-Ullevål and Institute for Clinical Medicine, Medical Faculty, University of Oslo, Norway.
Section of Rheumatology, Norwegian Competence Center of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Institute for Experimental Medical Research, Department of Cardiology, Oslo University Hospital-Ullevål and Institute for Clinical Medicine, Medical Faculty, University of Oslo, Norway.Section of Rheumatology, Norwegian Competence Center of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Institute for Experimental Medical Research, Department of Cardiology, Oslo University Hospital-Ullevål and Institute for Clinical Medicine, Medical Faculty, University of Oslo, Norway.Section of Rheumatology, Norwegian Competence Center of Pediatric and Adolescent Rheumatology, Oslo University Hospital-Rikshospitalet, Institute for Experimental Medical Research, Department of Cardiology, Oslo University Hospital-Ullevål and Institute for Clinical Medicine, Medical Faculty, University of Oslo, Norway.
出版信息
Rheumatology (Oxford). 2014 Sep;53(9):1578-85. doi: 10.1093/rheumatology/keu146. Epub 2014 Mar 31.
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, P < 0.05) except for muscle enzymes. Active patients had higher disease activity than inactive patients measured by MDAAT (P < 0.001) 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, age<9 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.
目的
本研究旨在通过儿科风湿病国际临床试验组织（PRINTO）的疾病不活动标准和肌炎疾病活动评估工具（MDAAT）检查 JDM 患者长期随访后的疾病活动，并确定这些结果的预测因素。
方法
在一项回顾性的起始队列研究中，对 59 例 JDM 患者进行了临床检查，这些患者在症状出现后中位数为 16.8 年（范围 2.0-38.1）。根据 PRINTO 标准，患者分为临床不活动和活动疾病。还通过 MDAAT 和其他经过验证的工具测量疾病活动。回顾病历以评估早期疾病变量和药物治疗。
结果
根据 PRINTO 标准，59 例患者中有 31 例（51%）为活动期，29 例（49%）为不活动期。根据 MDAAT，59 例患者中有 43 例（73%）有可测量的疾病活动，最常见的是皮肤（59%）和骨骼（27%）系统。MDAAT 与其他疾病活动测量指标（rsp 0.39-0.87，P < 0.05）具有中度至强相关性，除肌肉酶外。与 MDAAT （P < 0.001）和其他疾病特征（均 P ≤ 0.002）相比，活动期患者的疾病活动度高于不活动期患者，除患者对疾病活动的总体评估外。在校正性别和随访时间后，病程中的钙沉积预测 MDAAT 升高，诊断时年龄<9 岁预测为活动期疾病，6-12 个月后诊断时存在器官损伤预测这两种结果。
结论
16.8 年后，51-73%的 JDM 患者有活动期疾病。PRINTO 标准和 MDAAT 的疾病活动与大多数其他疾病特征具有中度至高度相关性，并可预测早期损伤。
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