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. 2011 Sep;179(3):1347–1359. doi: 10.1016/j.ajpath.2011.05.055

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© 2011 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

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Scheme for changes in the molecular pattern of inflammatory signaling, antigen processing, and presentation in attacked myofibers. HLA-I molecules are up-regulated in all IBM myofibers. We show the pronounced effect of IFN-γ–induced CIITA on HLA-II by a continuous arrow and the relatively small effect of CIITA on HLA-I expression (Gobin et al, 1998) as a dotted arrow. However, the effect of CIITA cannot be held responsible for the initial IFN-γ–independent and probably ubiquitous HLA-I up-regulation. In A_IBM, we found up-regulation of IFN-γ pathway components IFNGR1 and IFNGR2. The response to IFN-γ explains the up-regulation of IFN-γ–inducible genes, such as STAT1, CIITA, PSMB8, and HLA-II, in A_IBM.