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Rheumatology Advance Access originally published online on February 8, 2006
Rheumatology 2006 45(8):990-993; doi:10.1093/rheumatology/kel025
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© The Author 2006. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

An international consensus survey of the diagnostic criteria for juvenile dermatomyositis (JDM)

V. E. Brown, C. A. Pilkington1, B. M. Feldman3, J. E. Davidson2 on behalf of the Network for Juvenile Dermatomyositis, a working party of the Paediatric Rheumatology European Society (PReS)

Juvenile Dermatomyositis Research Centre, Institute of Child Health, University College London, 1 Great Ormond Street Hospital for Children NHS Trust, London, 2 Royal Liverpool Children's NHS Trust, Alder Hey, Liverpool, UK and 3 Hospital for Sick Children, University of Toronto, Toronto, Canada.

Correspondence to: J. Davidson, Royal Liverpool Children's NHS Trust, Eaton Road, Liverpool L12 2AP, UK. E-mail: joyce.davidson{at}yorkhill.scot.nhs.uk

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.

KEY WORDS: Juvenile dermatomyositis, Inflammatory myopathies, Muscle weakness, Skin rash, Elevated muscle enzymes


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