Abstract
In sIBM, an inflammatory process mediated by cytotoxic T cells and cytokines in conjunction
with a degenerative process, deposits of beta amyloid and misfolded proteins appear
to be the main culprits in disease pathogenesis. IL-1β may play a key role because
it is upregulated in sIBM myofibers, co-localizes with Amyloid Precursor Protein (APP)
and promotes the production of APP and amyloid deposits. We performed a small, pilot
study to examine whether anakinra, an IL1 receptor antagonist could benefit sIBM patients.
Four patients with biopsy-proven sIBM received anakinra for a mean period of 7.7 months. No improvement in muscle strength or stabilization was noted in any of the
patients based on grip strength and MRC measurements. The treatment failure may be
due to insufficiency of anakinra to suppress the intramuscular IL1, the short study
period, or the irrelevance of IL1 in the disease process.
Keywords
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Article info
Publication history
Published online: September 03, 2013
Accepted:
August 6,
2013
Received in revised form:
August 3,
2013
Received:
July 8,
2013
Identification
Copyright
© 2013 Published by Elsevier Inc.
