Inclusion body myositis – pathomechanism and lessons from genetics

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 a...

Full description

Bibliographic Details
Main Authors: Murnyák Balázs, Bodoki Levente, Vincze Melinda, Griger Zoltán, Csonka Tamás, Szepesi Rita, Kurucz Andrea, Dankó Katalin, Hortobágyi Tibor
Format: Article
Language:English
Published: De Gruyter 2015-02-01
Series:Open Medicine
Subjects:
GNE
DES
VCP
Online Access:http://www.degruyter.com/view/j/med.2015.10.issue-1/med-2015-0030/med-2015-0030.xml?format=INT
Description
Summary: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.
ISSN:2391-5463