Cellular immune mechanisms in inflammatory myopathies

Reinhard Hohlfeld, Andrew G. Engel, Norbert Goebels, Lüder Behrens

Research output: Contribution to journalReview article

72 Scopus citations

Abstract

The inflammatory myopathies include dermatomyositis, polymyositis, and inclusion body myositis. In dermatomyositis, muscle fiber injury is secondary to an antibody- or immune-complex-mediated immune response against a vascular-endothelial component. In polymyositis and inclusion body myositis, CD8+ T cells and macrophages invade and eventually destroy initially nonnecrotic muscle fibers. The autoaggressive T cells have the phenotype of activated (HLA-DR+) memory (CD45RO+) cells. T-cell receptor analyses indicate that the autoaggressive T cells are oligoclonal. In inflammatory lesions, muscle fibers express various cytoplasmic and surface molecules that are not detectable in normal fibers. These molecules, which include HLA class I antigens, heat-shock proteins, adhesion molecules, and Fas, are probably induced by locally secreted cytokines. The autoaggressive CD8+ T cells harbor granules containing perforin that aggregate near the contact zone with the target muscle fiber. This is consistent with a perforin- and secretion-dependent mechanism of muscle fiber injury. Many invaded muscle fibers also express the Fas 'death receptor,' but signs of apoptosis are absent.

Original languageEnglish (US)
Pages (from-to)520-526
Number of pages7
JournalCurrent opinion in rheumatology
Volume9
Issue number6
DOIs
StatePublished - Jan 1 1997

ASJC Scopus subject areas

  • Rheumatology

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