Brown V E, Pilkington C A, Feldman B M, Davidson J E
Juvenile Dermatomyositis Research Centre, Institute of Child Health, University College London, London, UK.
Rheumatology (Oxford). 2006 Aug;45(8):990-3. doi: 10.1093/rheumatology/kel025. Epub 2006 Feb 8.
To develop revised criteria for the diagnosis of juvenile dermatomyositis (JDM) using an international consensus process.
An initial survey was circulated to members of the Network for JDM and the Paediatric Rheumatology International Trials Organisation (PRINTO). Each individual was asked to identify those criteria that were felt to be most helpful in the diagnosis of classical JDM. A second survey was derived from these results and used to rank these proposed criteria in order of their importance and usefulness in clinical practice.
The first survey had a response rate of 49.8% (118 individuals) from 92 centres in 32 countries. All responders routinely used proximal muscle weakness and characteristic skin rash in the diagnosis of JDM, while 86.8% used elevated muscle enzymes. Muscle biopsy, magnetic resonance imaging (MRI) and changes on the electromyogram (EMG) were deemed important diagnostic criteria. Other criteria, including myositis-specific or -related antibodies, nailfold capillaroscopy, factor VIII-related antigen, muscle ultrasound, calcinosis and neopterin, were used by 35.3% of respondents. Seventy-eight respondents to the first survey (66%) responded to the second survey. Typical MRI and muscle biopsy changes were rated by all to be the most useful clinically relevant diagnostic criteria after proximal muscle weakness, characteristic skin rash and elevated muscle enzymes. These were followed by myopathic changes on EMG, calcinosis, dysphonia and nailfold capillaroscopy, which were ranked equally.
This process identified nine criteria that clinicians felt to be helpful or important in the diagnosis of JDM. A further process of refinement and validation is necessary to agree an internationally acceptable, clinically usable set of diagnostic criteria.
通过国际共识程序制定青少年皮肌炎（JDM）诊断的修订标准。
向JDM网络和儿科风湿病国际试验组织（PRINTO）的成员进行了初步调查。要求每个人确定那些被认为对经典JDM诊断最有帮助的标准。第二次调查基于这些结果进行，用于对这些提议的标准在临床实践中的重要性和实用性进行排序。
第一次调查的回复率为49.8%（118人），来自32个国家的92个中心。所有回复者在JDM诊断中常规使用近端肌无力和特征性皮疹，而86.8%的人使用肌酶升高。肌肉活检、磁共振成像（MRI）和肌电图（EMG）变化被视为重要的诊断标准。其他标准，包括肌炎特异性或相关抗体、甲襞毛细血管镜检查、因子VIII相关抗原、肌肉超声、钙质沉着和新蝶呤，35.3%的受访者使用。第一次调查的78名受访者（66%）回复了第二次调查。在近端肌无力、特征性皮疹和肌酶升高之后，典型的MRI和肌肉活检变化被所有人评为临床上最有用的相关诊断标准。其次是EMG上的肌病性变化、钙质沉着、声音嘶哑和甲襞毛细血管镜检查，它们的排名相同。
该程序确定了九条临床医生认为对JDM诊断有帮助或重要的标准。需要进一步的完善和验证程序，以达成一套国际认可、临床可用的诊断标准。
