Abstract
Dysferlinopathies are often accompanied by inflammatory infiltrates and may even mimic idiopathic inflammatory myopathies. In the present study, the inflammatory reaction profile with respect to the cellular composition of the infiltrates and the expression of major cytokines was characterized by immunofluorescence in dysferlinopathies and compared to idiopathic inflammatory myopathies. Infiltrates consisted mainly of CD4+ CD25- T cells and macrophages. The prominent expression of interferon-gamma may underlie the marked upregulation of MHC class I antigen on the vast majority of muscle fibres. Remarkably, these data indicate that membrane attack complex was deposited on intact and necrotic muscle fibres. Collectively, these data indicate that membrane attack complex deposits and a pro-inflammatory milieu without interleukin-10 expression may contribute to pathology of dysferlinopathies.
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