Page "Inclusion body myositis" Paragraph 23

As mentioned above, in the past, some researchers have suggested that it is the protein changes that are primary and that precede or trigger the abnormal immune response.

From an article by Askanas and Engel: " Two hypotheses predominate regarding the key pathogenic mechanisms involved in s-IBM: an amyloid-beta-related degenerative process and an immune dysregulation.

Ultimately, both may be considered important, and their possible interrelationship may be clarified.

An intriguing feature is the accumulation within s-IBM muscle fibers of amyloid-beta ( Ab ), phosphorylated tau protein, and at least 20 other proteins that are also accumulated in the brain of Alzheimer's disease patients.

In the s-IBM muscle fibers, there is evidence of misfolding of proteins, pathologic proteinaceous inclusions including aggresomes, abnormalities of the two protein-disposal systems involving the ubiquitin proteasome pathway and the lysosomes, mitochondrial dysfunctions, and oxidative stress.

The pronounced T-cell inflammation can be striking, and it is characterized by activated, antigen-driven, cytotoxic CD8 + T-cells.