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Rheumatology 2009 48(6):607-612; doi:10.1093/rheumatology/kep078
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org


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Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression

Harsha Gunawardena1,2,3, Zoe E. Betteridge2,3 and Neil J. McHugh1,2,3

1Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, 2Bath Institute for Rheumatic Diseases and 3School for Health, University of Bath, Bath, UK.

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


   Abstract

The idiopathic inflammatory myopathies (IIMs)—DM and PM—have been historically defined by broad clinical and pathological criteria. These conditions affect both adults and children with clinical features including muscle weakness, skin disease, internal organ involvement and an association with cancer in adults. Using a clinico-serological approach, DM and PM can be defined into more homogeneous subsets. Over the last few years, myositis-specific autoantibodies (MSAs) have been better characterized including autoantibodies directed against the aminoacyl tRNA-synthetase enzymes, the signal-recognition particle and the Mi-2 protein. In addition, clinically significant novel autoantibodies—anti-CADM-140, anti-SAE (small ubiquitin-like modifier activating enzyme), anti-p155/140 and anti-p140—have been described in the adult and juvenile disease spectrum. MSAs are directed against cytoplasmic or nuclear components involved in key regulatory intracellular processes including protein synthesis, translocation and gene transcription. The striking association between unique serological profiles and distinct clinical phenotypes suggests that target autoantigens may play a role in disease induction and propagation. In this review, we discuss the clinical utility and pathogenic significance of MSAs in disease expression.

KEY WORDS: Inflammatory myopathy, Dermatomyositis, Polymyositis, Myositis-specific autoantibodies

Submitted 3 January 2009; revised version accepted 12 March 2009.
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