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Serological Findings

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The Inflammatory Myopathies

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Abstract

Patients with polymyositis and dermatomyositis (DM) have a high frequency of autoantibodies to cytoplasmic and nuclear cellular antigens. No single autoantibody is present in the majority of patients, but a group of these autoantibodies is relatively specific for myositis (myositis-specific autoantibodies [MSAs]). The MSAs tend to be mutually exclusive and have distinctive clinical associations, resulting in autoantibody-defined disease subgroups. Anti-Jo-1 and other ant synthetases are associated with several extramuscular features as well as myositis, including interstitial lung disease (ILD), arthritis, and other features that together constitute an “antisynthetase syndrome.” Anti-signal recognition particle (anti-SRP) is associated with a clinically and histologically distinctive myopathy that is often severe and sometimes rapidly progressive and relatively refractory, while anti-Mi-2 is associated with DM. Several recently identified autoantibodies also have relative myositis specificity and are associated predominantly with DM. Anti-p155/140 and anti-MJ are associated with juvenile DM, which has a low frequency of established MSAs. Anti-p155/140 has been increased in myositis with malignancy in adults. Anti-caDM-140 has been associated with clinically amyopathic DM (anti-caDM-140), often with ILD. These and additional new autoantibodies are increasing the proportion of myositis patients for whom specific autoantibodies can be identified. The MSAs can be helpful for diagnosis in patients with myositis and for patient characterization and classification. They also continue to provide insights into disease mechanisms. The new autoantibodies will improve the utility of the autoantibodies for these purposes.

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  1. Department of Veterans Affairs Medical Center, Oklahoma City; Department of Medicine, University of Oklahoma Health Sciences Center; and Oklahoma Medical Research Foundation, Oklahoma City, OK, USA

    Ira N. Targoff

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    © 2009 Humana Press, a part of Springer Science+Business Media, LLC

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    Targoff, I.N. (2009). Serological Findings. In: Kagen, L. (eds) The Inflammatory Myopathies. Humana Press. https://doi.org/10.1007/978-1-60327-827-0_10

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    • DOI: https://doi.org/10.1007/978-1-60327-827-0_10

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