Research Article| Volume 334, ISSUE 1-2, P123-125, November 15, 2013

The effect of anakinra, an IL1 receptor antagonist, in patients with sporadic inclusion body myositis (sIBM): A small pilot study

Published:September 03, 2013DOI:


      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.


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        • Needham M.
        • Mastaglia F.L.
        Inclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches.
        Lancet Neurol. 2007; 6: 620-631
        • Schmidt J.
        • Dalakas M.C.
        Inclusion-body myositis in the elderly: an update.
        Aging Health. 2010; 6: 687-694
        • Dalakas M.C.
        Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies.
        Nat Rev Neurol. 2006; 2: 437-447
        • Dalakas M.C.
        An update on inflammatory and autoimmune myopathies.
        Neuropathol Appl Neurobiol. 2011; 37: 226-242
        • Askanas V.
        • Engel W.K.
        • Nogalska A.
        Inclusion body myositis: a degenerative muscle disease associated with intra-muscle fiber multi-protein aggregates, proteasome inhibition, endoplasmic reticulum stress and decreased lysosomal degradation.
        Brain Pathol. 2009; 19: 493-506
        • Schmidt J.
        • Barthel K.
        • Wrede A.
        • Salajegheh M.
        • Bähr M.
        • Dalakas M.C.
        Interrelation of inflammation and APP in sIBM: IL-1 beta induces accumulation of beta-amyloid in skeletal muscle.
        Brain. 2008; 131: 1228-1240
        • Schmidt J.
        • Barthel K.
        • Zschüntzsch J.
        • Muth I.E.
        • Swindle E.J.
        • Hombach A.
        • et al.
        Nitric oxide stress in sporadic inclusion body myositis muscle fibres: inhibition of inducible nitric oxide synthase prevents interleukin-1beta-induced accumulation of beta-amyloid and cell death.
        Brain. 2012; 135: 1102-1114
        • Dalakas M.C.
        Polymyositis, dermatomyositis and inclusion-body myositis.
        N Engl J Med. 1991; 325: 1487-1498
        • Griggs R.C.
        • Askanas V.
        • DiMauro S.
        • Engel A.
        • Karpati G.
        • Mendell J.R.
        • et al.
        Inclusion body myositis and myopathies.
        Ann Neurol. 1995; 38: 705-713