Abstract
The idiopathic inflammatory myopathies: polymyositis (PM) and dermatomyositis (DM) 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 and internal organ involvement. Over the last few years, it has become increasingly apparent that using a clinico-serological approach, both DM and PM can be defined into more homogeneous subsets. A large number of antibodies are directed against cytoplasmic or nuclear components involved in key regulatory intra-cellular processes including protein synthesis, translocation and gene transcription within this disease spectrum. In addition, these autoantibodies are found in patients with clinical features other than myositis, in particular ‘idiopathic’ interstitial pneumonia emphasizing that these patients may in fact be a formes-frustes of autoimmune connective tissue disease. Other important findings are the identification of specific autoantibodies in both cancer-associated dermatomyositis, clinically amyopathic dermatomyositis and juvenile dermatomyositis, which previously were classically described as antibody-negative clinical subsets. Finally, work has highlighted how target autoantigens identified in the myositis-connective tissue disease overlap share common cellular mechanisms, which provides us with further insights into disease pathogenesis.
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Acknowledgments
We would like to thank Juliet Dunphy (Bath Institute for Rheumatic Diseases, Bath, UK) for providing the images for Fig. 1 (ANA immunofluorescence patterns). We also thank and acknowledge Dr. Zoe Betteridge and Professor Neil McHugh for their continued support.
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Tansley, S., Gunawardena, H. The Evolving Spectrum of Polymyositis and Dermatomyositis—Moving Towards Clinicoserological Syndromes: A Critical Review. Clinic Rev Allerg Immunol 47, 264–273 (2014). https://doi.org/10.1007/s12016-013-8387-6
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DOI: https://doi.org/10.1007/s12016-013-8387-6