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Int J Rheum Dis. 2015 Nov;18(8):818-25. doi: 10.1111/1756-185X.12736. Epub 2015 Sep 19.

Idiopathic inflammatory myopathies: from immunopathogenesis to new therapeutic targets.

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BSM Medical University, Dhaka, Bangladesh.
Rheumatology department CHU Clermont-Ferrand and UMR 1019 INRA/ University of Auvergne, Clermont-Ferrand, France.


Pathogenesis of idiopathic inflammatory myositis (IIM) involves strong interactions between dendritic cells (DCs), activated Th1 and Th17 cells, B cells, muscle cells, genes and environment. Local maturation of DCs permit the activation and polarization of CD4+ T cells into T(H)1 and T(H)17 that play a key role in maintaining chronic muscle inflammation. T-cell mediated myocytotoxicity promotes the liberation of specific muscle autoantigens from regenerating muscle cells with production of myositis-specific autoantibodies. Type I interferon signature is a key characteristic of IIM. Type I IFN that can be induced by immune complexes containing myositis-specific autoantibodies is produced by scattered plasmacytoid DCs but also by muscle cells particularly regenerating muscle cells. These immature muscle precursors appear to be critical in the pathogenesis of IIM as they up-regulate muscle autoantigens, type I IFN, HLA class I antigens and TLR3-7, all together involved in maintaining chronic muscle inflammation. In addition to the role of immune and muscle cells, genome-wide association studies have confirmed the importance of several MHC and non-MHC genes in IIM. Environmental factors can contribute to the pathogenesis of IIM. In sIBM, distinct features suggest both degenerative and inflammatory processes. In addition to our better understanding of the pathogenesis, identify molecular pathway leads to consider new targeted therapies including cytokine inhibition, B-cell and T-cell costimulation blockade, type I IFN neutralization or inhibition of the ubiquitin proteasome pathway.


dermatomyositis; idiopathic inflammatory myositis; inclusion body myositis; pathogenesis; polymyositis; treatment

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