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Pathogenesis of idiopathic inflammatory myopathies

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Abstract

The idiopathic inflammatory myopathies, myositis, are characterized by a chronic course with decreased muscle endurance and by infiltrates of T lymphocytes and macrophages in muscle tissue. Treatment with immunosuppressives rarely leads to recovery of muscle function, despite abolishment of inflammatory cell infiltrates in muscle tissue. Therefore, other mechanisms than immunemediated muscle fiber damage are likely to contribute to the pathogenesis. One such non-immune-mediated muscle dysfunction could be caused by a disturbed microcirculation due to capillary loss or to phenotypically changed endothelial cells in the capillaries. These aberrations may affect the micro-environment of muscle tissue and lead to local tissue hypoxia with development of a secondary metabolic myopathy. Another possible non-immune-mediated mechanism leading to muscle dysfunction is the newly identified endoplasmatic reticulum (ER) stress response in myositis. The ER stress response is thought to be a consequence of the up-regulation of major histocompatibility complex class I in muscle fibers. These newly identified molecular pathways could play a major role in the pathogenesis of myositis and could be important targets in the development of new therapies.

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Authors and Affiliations

  1. Rheumatology Unit, Department of Medicine, Karolinska University Hospital, Solna, SE-171 76, Stockholm, Sweden

    Ingrid E. Lundberg MD, PhD

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  1. Cecilia Grundtman MSc
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  2. Ingrid E. Lundberg MD, PhD
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Correspondence to Ingrid E. Lundberg MD, PhD.

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Grundtman, C., Lundberg, I.E. Pathogenesis of idiopathic inflammatory myopathies. Curr Rheumatol Rep 8, 188–195 (2006). https://doi.org/10.1007/s11926-996-0024-4

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