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Amyloid deposits and inflammatory infiltrates in sporadic inclusion body myositis: the inflammatory egg comes before the degenerative chicken.
Acta Neuropathol. 2015 May;129(5):611-24. doi: 10.1007/s00401-015-1384-5
Benveniste O(), Stenzel W, Hilton-Jones D, Sandri M, Boyer O, van Engelen BG
Abstract:
.....Controversy reigns as to whether sIBM is primarily an inflammatory or a degenerative myopathy, the distinction being vitally important in terms of directing research for effective specific therapies. We review here the pros and the cons for the respective hypotheses. A possible scenario, which our experience leads us to favour, is that sIBM may start with inflammation within muscle. The rush of leukocytes attracted by chemokines and cytokines may induce fibre injury and HLA-I overexpression. If the protein degradation systems are overloaded (possibly due to genetic predisposition, particular HLA-I subtypes or ageing), amyloid and other protein deposits may appear within muscle fibres, reinforcing the myopathic process in a vicious circle.
PMID: 25579751
Free Full-Text: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4405277/