Inclusion body myositis – pathomechanism and lessons from genetics

Balázs Murnyák; Levente Bodoki; Melinda Vincze; Zoltán Griger; Tamás Csonka; Rita Szepesi; Andrea Kurucz; Katalin Dankó; Tibor Hortobágyi

doi:10.1515/med-2015-0030

Inclusion body myositis – pathomechanism and lessons from genetics

Balázs Murnyák, Levente Bodoki, Melinda Vincze, Zoltán Griger, Tamás Csonka, Rita Szepesi, Andrea Kurucz, Katalin Dankó, Tibor Hortobágyi

Source

Open Medicine > 2014 > 10 > 1

Abstract

Inclusion body myositis is a rare, late-onset myopathy. Both inflammatory and myodegenerative features play an important role in their pathogenesis. Overlapping clinicopathological entities are the familial inclusion body myopathies with or without dementia. These myopathies share several clinical and pathological features with the sporadic inflammatory disease. Therefore, better understanding of the genetic basis and pathomechanism of these rare familial cases may advance our knowledge and enable more effective treatment options in sporadic IBM, which is currently considered a relentlessly progressive incurable disease.