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2015 ; 129
(5
): 611-24
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English Wikipedia
Amyloid deposits and inflammatory infiltrates in sporadic inclusion body
myositis: the inflammatory egg comes before the degenerative chicken
#MMPMID25579751
Benveniste O
; Stenzel W
; Hilton-Jones D
; Sandri M
; Boyer O
; van Engelen BG
Acta Neuropathol
2015[May]; 129
(5
): 611-24
PMID25579751
show ga
Sporadic inclusion body myositis (sIBM) is the most frequently acquired myopathy
in patients over 50 years of age. It is imperative that neurologists and
rheumatologists recognize this disorder which may, through clinical and
pathological similarities, mimic other myopathies, especially polymyositis.
Whereas polymyositis responds to immunosuppressant drug therapy, sIBM responds
poorly, if at all. 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.