Selected aspects of the current management of myositis

Abstract

The idiopathic inflammatory myopathies (IIM) are a rare and heterogeneous group of acquired autoimmune muscle disorders, often referred to as 'myositis'. Clinical assessment, together with muscle biopsy findings and autoantibody status are key factors to consider when making a diagnosis of IIM, and in stratification of the 'IIM spectrum' into disease subgroups. Treatment stratified according to serotype (and in the future, likely also genotype) is increasingly being used to take account of the heterogeneity within the IIM spectrum. Subgroup classification is also important in terms of monitoring for complications, such as malignancy and interstitial lung disease. Disease monitoring should include the use of standardized tools such as the IMACS disease activity outcome measures. Other tools such as muscle MRI can be useful in identifying areas of active muscle inflammation. Treatment outcomes in IIM remain unsatisfactory. The evidence base to guide treatment decisions is remarkably limited. In addition to muscle inflammation, a number of noninflammatory cell-mediated mechanisms may contribute to weakness and disability, and for which no specific treatments are currently available.

Keywords: Myositis; myopathy; myositis damage; myositis disease activity; myositis specific antibodies; myositis subgroups; outcome assessment tools.