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Rheumatology Advance Access originally published online on January 30, 2008
Rheumatology 2008 47(3):324-328; doi:10.1093/rheumatology/kem359
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Clinical associations of autoantibodies to a p155/140 kDa doublet protein in juvenile dermatomyositis

H. Gunawardena1,2, L. R. Wedderburn3, J. North2, Z. Betteridge2, J. Dunphy1, H. Chinoy4,5, J. E. Davidson6, R. G. Cooper4, N. J. McHugh1,2 and for the Juvenile Dermatomyositis Research Group UK

1Royal National Hospital for Rheumatic Diseases, 2University of Bath, Bath, 3Rheumatology Unit, Institute for Child Health, University College London, 4Rheumatic Diseases Centre, Hope Hospital, Salford, 5Centre for Integrated Genomic Medical Research, University of Manchester and 6Royal Hospital for Sick Children, Glasgow, UK.

Correspondence to: N. J. McHugh, Royal National Hospital for Rheumatic Diseases, Upper Borough Walls, Bath BA1 1RL, UK. E-mail: neil.mchugh{at}rnhrd-tr.swest.nhs.uk


   Abstract

Objectives. Myositis-specific autoantibodies (MSAs) may define homogeneous clinical subsets of adult patients with dermatomyositis (DM). Recently, there have been descriptions of novel autoantibodies in DM. This study was conducted to establish the clinical significance of anti-p155/140 autoantibodies in juvenile DM (JDM).

Methods. The first 116 children recruited to the JDM National Registry and Repository (UK and Ireland) were studied. Comprehensive clinical features were recorded and sera screened for anti-p155/140 autoantibodies using radio-immunoprecipitation. Sera from adults with DM (n = 20), PM (n = 25), SSc (n = 150), SLE (n = 40) and healthy subjects (n = 50) were used for comparison. Immunodepletion experiments were used to establish whether the p155/140 kDa targets recognized by JDM sera were the same as adult DM sera.

Results. Twenty-seven out of 116 (23%) JDM cases were positive for anti-p155/140 in comparison with 6/20 (30%) adults with DM. Immunodepletion confirmed that the 155/140 kDa proteins recognized by JDM and adult DM sera were the same targets. All other adult control sera were negative for anti-p155/140 autoantibodies. There was a higher frequency of males in the anti-p155/140-positive JDM group (P = 0.02). JDM patients with anti-p155/140 autoantibodies had significantly more cutaneous involvement including Gottron's papules (P = 0.003), ulceration (P = 0.005) and oedema (P = 0.013). The distribution of skin lesions was more extensive particularly periorbitally (P = 0.014) and over the small (P < 0.001) and large joints (P = 0.003).

Conclusions. Anti-p155/140 autoantibodies are clinically significant in JDM and may define a clinical subset in terms of disease severity and outcome. The same autoantigen target is detected in adult DM patients.

KEY WORDS: Inflammatory myopathy, Dermatomyositis, Juvenile dermatomyositis, Autoantibodies

Submitted 16 July 2007; revised version accepted 6 December 2007.
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