Measures of adult and juvenile dermatomyositis, polymyositis, and inclusion body myositis: physician and patient/parent global activity, manual muscle testing (mmt) …

LG Rider, VP Werth, AM Huber… - Arthritis care & …, 2011 - Wiley Online Library
LG Rider, VP Werth, AM Huber, H Alexanderson, AP Rao, N Ruperto, L Herbelin, R Barohn
Arthritis care & research, 2011Wiley Online Library
The idiopathic inflammatory myopathies, including adult and juvenile dermatomyositis (DM),
polymyositis (PM), and inclusion body myositis (IBM), are rare systemic autoimmune
diseases that are characterized by chronic proximal muscle inflammation and weakness. In
previous decades, there were few commonly used outcome measures in myositis, and those
outcome measures were not validated. Therefore, in the past the assessment of outcomes in
therapeutic trials was focused on nonstandardized measurement of muscle strength and …
The idiopathic inflammatory myopathies, including adult and juvenile dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM), are rare systemic autoimmune diseases that are characterized by chronic proximal muscle inflammation and weakness. In previous decades, there were few commonly used outcome measures in myositis, and those outcome measures were not validated. Therefore, in the past the assessment of outcomes in therapeutic trials was focused on nonstandardized measurement of muscle strength and function only. Over the last decade, however, 2 international collaborative groups, the International Myositis Assessment and Clinical Studies Group (IMACS) and the Paediatric Rheumatology International Trials Organisation (PRINTO), have defined consensus core set measures to assess myositis disease activity and damage in adults and children and have begun to validate and standardize these measures (1, 2). IMACS and PRINTO have also developed preliminary definitions of improvement that can be used as outcomes for therapeutic trials. These response criteria combine the core set activity measures to determine clinically meaningful improvement (3, 4). Our section on myositis assessment focuses first on these core set measures of disease activity, quality of life (which is part of the PRINTO core set of activity, but a separate assessment domain for IMACS), and disease damage. To date, most of the validation data available for these core set measures are in patients with juvenile DM, with more limited validation in adult patients with DM or PM. Despite these efforts, there are still important gaps in the validation of these core set measures, and no validation studies have yet been performed in patients with IBM, although they are now being used frequently in myositis therapeutic trials. We end this article with tools that have been used primarily in research studies and a few therapeutic trials that
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