PMID: 15367860Sep 16, 2004Paper

Inflammatory disorders of muscle: progress in polymyositis, dermatomyositis and inclusion body myositis

Current Opinion in Neurology
Marinos C Dalakas

Abstract

To provide an update on the major advances in inflammatory myopathies. Polymyositis is an uncommon disorder that can be misdiagnosed when the old, and never validated, criteria of Bohan and Peter are used. New diagnostic criteria were recently introduced, in which the MHC/CD8 complex is considered a specific immunopathological marker because it distinguishes the antigen-driven inflammatory cells that characterize polymyositis and sporadic inclusion-body myositis from the non-specific, secondary inflammation seen in other disorders, such as dystrophies. In sporadic inclusion-body myositis the inflammatory cells invade non-vacuolated fibers, whereas the vacuolated fibers are not invaded by T cells, implying two independent processes, a primary immune process with antigen-driven T cells identical to polymyositis, and a degenerative process in which beta-amyloid and amyloid-related proteins participate in vacuolar degeneration. In polymyositis and sporadic inclusion-body myositis, antigen-specific and clonally expanded autoinvasive T cells persist for years, even in different muscles, as reconfirmed by proof-of-principle techniques involving CDR3 spectratyping combined with laser microdissected single-cell polymerase chain reaction...Continue Reading

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