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  • Review Article
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Technology Insight: tools for research, diagnosis and clinical assessment of treatment in idiopathic inflammatory myopathies

Nature Clinical Practice Rheumatology volume 3, pages 282–290 (2007)Cite this article

Abstract

Idiopathic inflammatory myopathies, known collectively as myositis, are chronic diseases that cause disability, mainly from muscle weakness, despite the use of immunosuppressive therapies. An improved outcome requires increased knowledge of the key molecular pathways that cause symptoms in muscles and other organs. Technological advances offer promise for improving our understanding of disease mechanisms, and some tools will be helpful in diagnosis and the assessment of therapeutic success. The application of new tools depends on their validation in longitudinal studies using clinical outcome measures combined with assessments of molecular events in affected organs. Clinical outcome measures and definitions of improvement have been developed and validated through the International Myositis Assessment and Clinical Studies collaboration. Some imaging techniques, such as MRI and magnetic resonance spectroscopy of muscles, and high-resolution CT of lungs, can assess changes in local inflammatory activity, among many other aspects of pathology. Changes in protein and gene expression patterns in repeated biopsies from affected organs (muscle, skin and lungs) provide molecular information and allow increasingly precise disease classifications and therapeutic evaluation, but are to date only research tools. This Review focuses on advances in diagnostic and outcome tools and their roles in clinical practice and clinical research in patients with polymyositis and dermatomyositis.

Key Points

  • A consensus has been reached within an international multidisciplinary collaborative network, the International Myositis Assessment and Clinical Studies collaboration (IMACS), to use a three-component clinical outcome measure in clinical trials with patients with polymyositis or dermatomyositis

  • A definition of improvement according to the myositis disease activity core set has been proposed, which should facilitate clinical trials

  • Myositis-specific autoantibodies are helpful in diagnosis and in identifying subsets of myositis

  • Imaging, including MRI or magnetic resonance spectroscopy, is a noninvasive technique for use as a diagnostic tool and an outcome measure that is available in many centres and useful in clinical practice

  • Muscle biopsy is a cornerstone for diagnosis and might also be a useful tool to assess molecular effects of interventions, but is so far limited to research projects

  • Gene expression analysis, combined with protein analysis, in muscle biopsy samples is a promising new technique for diagnostic evaluation by identifying patterns of molecular pathways, but requires validation and development and is limited to research

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Figure 1: The longitudinal course of symptoms in polymyositis and dermatomyositis
Figure 2: Muscle biopsy changes identified by immunohistochemistry in different phases of polymyositis and dermatomyositis in patients with impaired muscle endurance

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Acknowledgements

We thank Professor L Klareskog for his critical review of the manuscript and Associate Professor R van Vollenhoven for his valuable comments on the manuscript and for linguistic advice.

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Authors and Affiliations

  1. I Lundberg is a Professor and Consultant in the Rheumatology Unit in the Department of Medicine at the Karolinska Institutet, where H Alexanderson is a Postdoctoral Research Fellow. H Alexanderson is also a registered physical therapist in the Department of Physical Therapy, Stockholm, Sweden, Karolinska University Hospital.,

    Ingrid E Lundberg & Helene Alexanderson

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Correspondence to Ingrid E Lundberg.

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Competing interests

I.E.L. states the following conflict of interest disclosures: that she is the recipient of an unrestricted research grant from Schering–Plough Nordic Biotech for $20,000 and an unrestricted research grant from Roche for $20,000. H Alexanderson declared she has no competing interests.

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Lundberg, I., Alexanderson, H. Technology Insight: tools for research, diagnosis and clinical assessment of treatment in idiopathic inflammatory myopathies. Nat Rev Rheumatol 3, 282–290 (2007). https://doi.org/10.1038/ncprheum0479

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