Rheumatology Advance Access originally published online on March 27, 2006
Rheumatology 2006 45(10):1255-1260; doi:10.1093/rheumatology/kel099
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The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)—clinical characteristics of children recruited within the first 5 yr
1Royal Liverpool NHS Trust, Alder Hey, Liverpool, 2Rheumatology Unit, Institute of Child Health, University College London, and Great Ormond Street Hospital NHS Trust, London, UK, 3Royal Hospital for Sick Children, Glasgow, UK and 4Princess Margaret Hospital, Perth, Western Australia.
Correspondence to: Dr C. A. Pilkington, Rheumatology Unit, Institute of Child Health, 30 Guilford Street, London, WC1N 1EH, UK. E-mail: c.pilkington{at}ich.ucl.ac.uk
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Abstract |
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Objectives. To identify epidemiological, clinical and laboratory characteristics of juvenile dermatomyositis (JDM) in a national multi-centre cohort of patients, and to review recent changes in the understanding of management and prognosis in the light of these data.
Methods. All children with idiopathic inflammatory myositis recruited to the Juvenile Dermatomyositis National Registry and Repository (UK and Ireland) were included. Features at presentation, and later in disease, were assessed and evaluated. A total of 63 out of 175 children with a new diagnosis of myositis were recruited at the time of diagnosis and followed prospectively. Out of the 175 children, 122 diagnosed prior to 2000 were recruited retrospectively, with subsequent data collected prospectively.
Results. One patient died (0.7%), which is equivalent to one death per 465 patient years. Data were available at the time of analysis on 151 registered patients. The most common presenting features were characteristic rash, weakness, tiredness, Gottron's patches and myalgia. Muscle biopsy, magnetic resonance imaging and muscle enzymes were frequently, but not always, abnormal. Muscle enzymes and erythrocyte sedimentation rate were not useful markers of disease activity.
Conclusions. The JDM National Registry and Repository captures data on a significant cohort of children with inflammatory myositis. The current study reports the largest European cohort of children with dermatomyositis to date. This powerful resource will help improve our understanding of this rare disease. Prospective data collection will allow a fuller analysis of poor prognostic features, impact of therapy, and variable outcome of childhood myositis.
KEY WORDS: Juvenile, Dermatomyositis, Myositis, Inflammatory, Weakness.
Submitted 19 October 2005;
revised version accepted 21 February 2006.
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