Open AccessImmune and myodegenerative pathomechanisms in inclusion body myositis
Author(s) -
Keller Christian W.,
Schmidt Jens,
Lünemann Jan D.
Publication year - 2017
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.419
Subject(s) - inclusion body myositis , medicine , myositis , inflammation , inflammatory myopathy , autophagy , immune system , acquired immune system , innate immune system , pathogenesis , immunology , ibm , muscle weakness , myopathy , bioinformatics , neuroscience , pathology , biology , genetics , apoptosis , materials science , nanotechnology
Inclusion Body Myositis ( IBM ) is a relatively common acquired inflammatory myopathy in patients above 50 years of age. Pathological hallmarks of IBM are intramyofiber protein inclusions and endomysial inflammation, indicating that both myodegenerative and inflammatory mechanisms contribute to its pathogenesis. Impaired protein degradation by the autophagic machinery, which regulates innate and adaptive immune responses, in skeletal muscle fibers has recently been identified as a potential key pathomechanism in IBM . Immunotherapies, which are successfully used for treating other inflammatory myopathies lack efficacy in IBM and so far no effective treatment is available. Thus, a better understanding of the mechanistic pathways underlying progressive muscle weakness and atrophy in IBM is crucial in identifying novel promising targets for therapeutic intervention. Here, we discuss recent insights into the pathomechanistic network of mutually dependent inflammatory and degenerative events during IBM .