Ann Rheum Dis ：PRINTO标准或可确定不活动性JDM

　　来自欧美的一项多国研究表明，儿童风湿病国际试验组织（PRINTO）制定了数据导向标准，以识别那些在临床上表现为不活动性疾病的青少年型皮肌炎（JDM）患者。这些标准可用于临床试验、研究以及临床实践。该论文发表在2013年第5期《风湿病学年鉴》[Ann Rheum Dis 2013;72:686-693]。
　　儿童风湿病国际试验组织（The Paediatric Rheumatology International Trials Organisation ，简称PRINTO）数据库包含275例活动性青少年型皮肌炎（JDM）患者，评估预期为24个月。24个月时，38例患者脱离治疗，确定为临床不活动性JDM，并归入参照组。这些患者与包含76例研究基线有活动性疾病患者的随机样本进行比较。研究者评估个体指标如肌肉力量/耐力、肌肉酶类、医师和患者的整体疾病活动性/损伤评估，以及基于文献和其他专门标准的不活动性疾病标准的敏感性、特异性和科恩卡帕协议（Cohen's κ agreement）。
　　表现最佳特征性不活动性疾病（敏感性和特异性＞0.8，Cohen's κ >0.8）的个体指标是徒手肌力检查（MMT）≥78，医师整体评估肌肉活动度=0，医师整体评估总疾病活动性（PhyGloVAS）≤0.2，儿童肌炎评估量表（CMAS）≥48，疾病活动评分≤3以及肌炎疾病活动性评估视觉模拟评分≤0.2。患者归类为接受或不接受治疗的不活动性疾病状态的最佳联合指标至少有以下四个标准中的三个：肌酸激酶≤150，CMAS≥48，MMT≥78和PhyGloVAS≤0.2。24个月后，96.8%（30/31）患者有不活动性疾病，脱离治疗；47.6%（69/145）患者有不活动性疾病，仍接受治疗。
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The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis
OBJECTIVES
To develop data-driven criteria for clinically inactive disease on and off therapy for juvenile dermatomyositis (JDM).
METHODS
The Paediatric Rheumatology International Trials Organisation (PRINTO) database contains 275 patients with active JDM evaluated prospectively up to 24 months. Thirty-eight patients off therapy at 24 months were defined as clinically inactive and included in the reference group. These were compared with a random sample of 76 patients who had active disease at study baseline. Individual measures of muscle strength/endurance, muscle enzymes, physician's and parent's global disease activity/damage evaluations, inactive disease criteria derived from the literature and other ad hoc criteria were evaluated for sensitivity, specificity and Cohen's κ agreement.
RESULTS
The individual measures that best characterised inactive disease (sensitivity and specificity >0.8 and Cohen's κ >0.8) were manual muscle testing (MMT) ≥78, physician global assessment of muscle activity=0, physician global assessment of overall disease activity (PhyGloVAS) ≤0.2, Childhood Myositis Assessment Scale (CMAS) ≥48, Disease Activity Score ≤3 and Myositis Disease Activity Assessment Visual Analogue Scale ≤0.2. The best combination of variables to classify a patient as being in a state of inactive disease on or off therapy is at least three of four of the following criteria: creatine kinase ≤150, CMAS ≥48, MMT ≥78 and PhyGloVAS ≤0.2. After 24 months, 30/31 patients (96.8%) were inactive off therapy and 69/145 (47.6%) were inactive on therapy.
CONCLUSION
PRINTO established data-driven criteria with clearly evidence-based cut-off values to identify JDM patients with clinically inactive disease. These criteria can be used in clinical trials, in research and in clinical practice.