封入体筋炎(inclusion body myositis:IBM)は,核内あるいは細胞質内に好酸性の封入体様構造物を認める炎症性筋疾患である.比較的高齡者に多く,治療抵抗性で緩徐に進行する.筋力低下は初期から遠位筋も罹患することもあり,血清CK上昇は軽度である.筋病理像は,炎症細胞浸澗を認めるが軽度であり,縁取り空胞(rimmed vacuoles)をもつ変性筋線維を認めるのが特徴である.IBMは炎症性変化と同時に筋の変性を認めるため,他の炎症性筋疾患とは異なる病態をもつと考えられている.
1) Yunis EJ, Samaha FJ:Inclusion body myositis. Lab Invest 1971;25(3):240-8 2) Askanas V, Serratrice GS, Engel WK:Inclusionbody myositis and myopathies, 1998. Cambridge Univ Press, Cambridge 3) Nonaka I, Sunohara N, Ishiura S, Satoyoshi E:Familial distal myopathy with rimmed vacuole and lamellar (myeloid) body formation. J Neurol Sci 1981;51(1):141-55 4) Askanas V, Engel WK, Alvarez RB:Enhanced detection of congo-red-positive amyloid deposits in muscle fibers of inclusion body myositis and brain of Alzheimer's disease using fluorescence technique. Neurology 1993;43(6):1265-7 5) Dalakas MC:Inflammatory disorders of muscle:progress in polymyositis, dermatomyositis and inclusion body myositis. Curr Opin Neurol 2004;17:561-7 6) Dalakas MC, Hohlfeld R:Polymyositis and dermatomyositis. Lancet 2003;362:971-82 7) Mastaglia FL, Garlepp MJ, Phillips BA, Zilko PJ:Inflammatory myopathies:clinical, diagnostic and therapeutic aspects. Muscle Nerve 2003;27:407-25 8) Engel AG, Hohlfeld R:The polymyositis and dermatomyositis syndrome, Engel AG, et al (eds), mcGraw-Hill, New York, 2004, pp.1321-66 9) Dau PC:Immunomodulation during treatment of polymyositis with plasmapheresis and immunosuppressive drugs. J Clin Apher 1994;9(1):21-5 10) Kiprov DD, Miller RG:Polymyositis associated with monoclonal gammopathy. Lancet 1984;2(8413):1183-6