Article

Sporadic inclusion-body myositis (sIBM) is the most frequent myopathy after 50
years of age. As the clinical presentation may often be typical, pathological
confirmation by muscle biopsy appears necessary, but sometimes difficult. Further
delineation of the framework of this particular disease, especially during its
early-onset stage, appears to be challenging. New classification of diagnostic
criteria as well as the identification of new diagnostic hallmarks appear to be
the two main tools towards to achieve this purpose. sIBM pathophysiology has long
been discussed and remains yet controversial. Since its initial description,
there have been two major pathogenic hypotheses: inflammatory and degenerative.
To date, the debate is still ongoing, as recent works support both
pathophysiological mechanisms, although the inflammatory process seems to be
slightly more preeminent in the recent literature. Treatment remains the most
disappointing aspect of the disease as, despite various therapeutic attempts, no
significant efficacy has been reported thus far. Nevertheless, advances in our
pathophysiological understanding of the disease are paving the way for further
therapeutic perspectives that might arise in the years to come. The objective of
the present work was to summarize the most significant data published on sIBM
during the past 2 years.
CI - Copyright (c) 2016. Published by Elsevier Masson SAS.

Langue : ANGLAIS