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.