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. Author manuscript; available in PMC: 2012 Mar 19.
Published in final edited form as: Arthritis Rheum. 2011 Feb;63(2):479–487. doi: 10.1002/art.30113

Figure 6. Hypothesized role of HRS in the pathogenesis of idiopathic inflammatory myopathy.

Figure 6

Combining data from the literature as well as current findings, this schematic demonstrates the putative role of HRS in bridging innate and adaptive immune responses that ultimately result in myocytotoxicity. As shown in the upper left portion of this diagram, muscle damage leads to upregulated surface expression of MHC Class I as well as release of soluble HRS that can recruit immature dendritic cells (iDCs) and naive lymphocytes via CCR5 (innate immunity). Subsequent maturation of DCs that have incorporated HRS allows efficient presentation of HRS peptides in the context of MHC Class II and enhanced co-stimulation, promoting activation of HRS-specific CD4+ T cells (in situ or in draining/regional lymph nodes). This subset of HRS-specific CD4+ T cells can then provide help to autoantibody-producing B cells and CD8+ T cells mediating myocytotoxicity in the adaptive arm of HRS-targeted immune responses.