Rheumatology Advance Access published online on March 27, 2006
Rheumatology, doi:10.1093/rheumatology/kel099
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 Royal Liverpool NHS Trust, Alder Hey, Liverpool, UK
* To whom correspondence should be addressed. 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.
Received October 19, 2005
Accepted February 21, 2006
Original Papers
The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr
L. J. McCann 1,
A. D. Juggins 2,
S. M. Maillard 2,
L. R. Wedderburn 2,
J. E. Davidson 3,
K. J. Murray 4,
C. A. Pilkington 2 *,
and
on behalf of the Juvenile Dermatomyositis Research Group
2 Rheumatology Unit, Institute of Child Health, University College London, and Great Ormond Street Hospital NHS Trust, London, UK
3 Royal Hospital for Sick Children, Glasgow
4 Princess Margaret Hospital, Perth, Western Australia
C. A. Pilkington, E-mail: c.pilkington{at}ich.ucl.ac.uk
Abstract 
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  H. Chinoy, D. Payne, K. V. Poulton, N. Fertig, Z. Betteridge, H. Gunawardena, J. E. Davidson, C. V. Oddis, N. J. McHugh, L. R. Wedderburn, et al. HLA-DPB1 associations differ between DRB1*03 positive anti-Jo-1 and anti-PM-Scl antibody positive idiopathic inflammatory myopathy Rheumatology, October 1, 2009; 48(10): 1213 - 1217. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Sanner, J.-T. Gran, I. Sjaastad, and B. Flato Cumulative organ damage and prognostic factors in juvenile dermatomyositis: a cross-sectional study median 16.8 years after symptom onset Rheumatology, September 23, 2009; (2009) kep302v1. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. K.-c. Li, P. Knopp, H. Moncrieffe, B. Singh, S. Shah, K. Nagaraju, H. Varsani, B. Gao, and L. R. Wedderburn Overexpression of MHC Class I Heavy Chain Protein in Young Skeletal Muscle Leads to Severe Myositis: Implications for Juvenile Myositis Am. J. Pathol., September 1, 2009; 175(3): 1030 - 1040. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Gunawardena, Z. E. Betteridge, and N. J. McHugh Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression Rheumatology, June 1, 2009; 48(6): 607 - 612. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Marhaug, V. Shah, R. Shroff, H. Varsani, L. R. Wedderburn, C. A. Pilkington, and P. A. Brogan Age-dependent inhibition of ectopic calcification: a possible role for fetuin-A and osteopontin in patients with juvenile dermatomyositis with calcinosis Rheumatology, July 1, 2008; 47(7): 1031 - 1037. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. Manlhiot, L. Liang, D. Tran, A. Bitnun, P. N. Tyrrell, and B. M. Feldman Assessment of an infectious disease history preceding juvenile dermatomyositis symptom onset Rheumatology, April 1, 2008; 47(4): 526 - 529. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Gunawardena, L. R. Wedderburn, J. North, Z. Betteridge, J. Dunphy, H. Chinoy, J. E. Davidson, R. G. Cooper, N. J. McHugh, and for the Juvenile Dermatomyositis Research Group UK Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis Rheumatology, March 1, 2008; 47(3): 324 - 328. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. R. Wedderburn, N. J. McHugh, H. Chinoy, R. G. Cooper, F. Salway, W. E. R. Ollier, L. J. McCann, H. Varsani, J. Dunphy, J. North, et al. HLA class II haplotype and autoantibody associations in children with juvenile dermatomyositis and juvenile dermatomyositis scleroderma overlap Rheumatology, December 1, 2007; 46(12): 1786 - 1791. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
L. J. McCann, S. M. Garay, M. M. Ryan, R. Harris, P. Riley, and C. A. Pilkington Oropharyngeal dysphagia in juvenile dermatomyositis (JDM): an evaluation of videofluoroscopy swallow study (VFSS) changes in relation to clinical symptoms and objective muscle scores Rheumatology, August 1, 2007; 46(8): 1363 - 1366. [Abstract] [Full Text] [PDF]
|
|