An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM)
An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM)
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DOI：
10.1093/rheumatology/kel025
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发表时间：
2006-08-01
期刊：
影响因子：
5.5
通讯作者：
Davidson, J. E.
中科院分区：
文献类型：
--
作者：
Brown, V. E.;Pilkington, C. A.;Davidson, J. E.
Objective.采用国际公认的诊断标准，修订儿童皮肌炎（JDM）的诊断标准。初步调查分发给JDM网络和儿科流变学国际试验组织（PRINTO）的成员。每个人都被要求确定那些被认为对诊断经典JDM最有帮助的标准。第二次调查来自这些结果，并用于排名这些建议的标准，以他们的重要性和实用性在临床实践中。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。所有应答者常规使用近端肌无力和特征性皮疹诊断JDM，而86.8%使用肌酶升高。肌肉活检、磁共振成像（MRI）和肌电图（EMG）改变被认为是重要的诊断标准。35.3%的受访者使用了其他标准，包括肌炎特异性或相关抗体、甲襞毛细血管镜检查、第VIII因子相关抗原、肌肉超声、钙质沉着和新蝶呤。第一次调查的78名受访者（66%）对第二次调查做出了回应。典型的MRI和肌肉活检变化被所有人评为最有用的临床相关的诊断标准后，近端肌无力，特征性皮疹和肌酶升高。其次是肌电图、钙质沉着、发音困难和甲襞毛细血管镜检查的肌病改变，排名相同。该过程确定了临床医生认为对JDM诊断有帮助或重要的9项标准。需要进一步的完善和验证过程，以商定一套国际上可接受的、临床上可用的诊断标准。
Objective. To develop revised criteria for the diagnosis of juvenile dermatomyositis (JDM) using an international consensus process.Methods. 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.Results. 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.Conclusion. 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.