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01-09-2006 | Original Paper

The inflammatory reaction pattern distinguishes primary dysferlinopathies from idiopathic inflammatory myopathies: an important role for the membrane attack complex

Authors: Anna Brunn, Rolf Schröder, Martina Deckert

Published in: Acta Neuropathologica | Issue 3/2006

Abstract

Degenerative muscle changes in dysferlinopathies are often accompanied by inflammatory infiltrates and may even mimic primary idiopathic inflammatory myopathies. In the present study, the inflammatory reaction pattern with respect to the cellular composition of the infiltrates and the expression of potent cytokines was characterized in dysferlinopathies and in idiopathic inflammatory myopathies. Cellular infiltrates in dysferlinopathies mainly consisted of CD4+CD25- T cells and macrophages. We noted a prominent expression of interferon-γ which may contribute to the marked upregulation of MHC class I antigen observed on the vast majority of muscle fibres. Furthermore, membrane attack complex positive deposits were found on intact as well as necrotic muscle fibres. Collectively, our study indicates that the inflammatory reaction pattern in dysferlinopathies is distinct from the one in idiopathic inflammatory myopathies. In particular, membrane attack complex deposits and a pro-inflammatory milieu in the absence of interleukin-10 expression may contribute to progressive muscle damage in dysferlinopathies.

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Title: The inflammatory reaction pattern distinguishes primary dysferlinopathies from idiopathic inflammatory myopathies: an important role for the membrane attack complex
Authors: Anna Brunn
Rolf Schröder
Martina Deckert
Publication date: 01-09-2006
Publisher: Springer-Verlag
Published in: Acta Neuropathologica / Issue 3/2006
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI: https://doi.org/10.1007/s00401-006-0113-5

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The inflammatory reaction pattern distinguishes primary dysferlinopathies from idiopathic inflammatory myopathies: an important role for the membrane attack complex

Abstract

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Abstract

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