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Review
. 2018;5(2):109-129.
doi: 10.3233/JND-180308.

Current Classification and Management of Inflammatory Myopathies

Jens Schmidt  1
Affiliations
Review

Current Classification and Management of Inflammatory Myopathies

Jens Schmidt. J Neuromuscul Dis. 2018.

Abstract

Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyography (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis.

Keywords: Skeletal muscle; autoimmunity; immunosuppression; muscle inflammation; myositis; neuroinflammation.

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Conflict of interest statement

JS has received honoraria, research grants or reimbursements for travel from Bayer, Biogen, BioMarin, Biotest, CSL Behring, Grifols, Hormosan, Novartis, and Octapharma.

Figures

Fig.1
Fig.1
Overview of the main items required for appropriate care for myositis.
Fig.2
Fig.2
Overview of the basic and escalating treatment modalities in myositis (modified from [1]).
See this image and copyright information in PMC

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