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-1 beta 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 slBM 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. 2013 Published by Elsevier B.V.
机构:
UCL, Queen Sq Inst Neurol, London, England
King Abdullah Med City, Clin Lab, Mol Diagnost Unit, Mecca, Saudi ArabiaUCL, Queen Sq Inst Neurol, London, England
机构:
Univ Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, AustraliaUniv Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, Australia
Rojana-Udomsart, A.
Bundell, C. S.
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Queen Elizabeth II Med Ctr, PathW Lab Med, Perth, WA, AustraliaUniv Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, Australia
Bundell, C. S.
Hollingsworth, P. N.
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Queen Elizabeth II Med Ctr, PathW Lab Med, Perth, WA, AustraliaUniv Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, Australia
Hollingsworth, P. N.
Fabian, V.
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Royal Perth Hosp, Sect Neuropathol, Dept Anat Pathol, Perth, WA, AustraliaUniv Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, Australia
Fabian, V.
Mastaglia, F. L.
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Univ Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, AustraliaUniv Western Australia, Ctr Neuromuscular & Neurol Disorders, Perth, WA 6009, Australia
机构:
Univ Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, GreeceUniv Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, Greece
Kosmidis, Michalis L.
Pikazis, Dimitris
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Univ Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, GreeceUniv Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, Greece
Pikazis, Dimitris
Vlachoyiannopoulos, Panayotis
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Univ Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, GreeceUniv Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, Greece
Vlachoyiannopoulos, Panayotis
Tzioufas, Athanasios G.
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Univ Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, GreeceUniv Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, Greece
Tzioufas, Athanasios G.
Dalakas, Marinos C.
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Univ Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, Greece
Thomas Jefferson Univ, Philadelphia, PA 19107 USAUniv Athens, Dept Pathophysiol, Neuroimmunol Unit, Med Sch, Athens, Greece