Hama Kiwa, Miwa Hideto, Nishino Ichizo, Nonaka Ikuya, Kondo Tomoyoshi
Department of Neurology, Wakayama Medical University, 811-1 Kimiidera, Wakayama 641-8510, Japan.
No To Shinkei. 2004 Jun;56(6):503-7.
A 67-year-old woman was admitted to our hospital, because of wasting of the thigh muscles. Muscular atrophy was confined to the thigh muscles, suggesting that she had quadriceps myopathy. Muscle biopsy from quadriceps muscle revealed characteristic findings, such as invasion of the endomysium and muscle fibers by inflammatory cells, the presence of rimmed vacuoles and ragged red fibers; thereby, she was diagnosed as having inclusion body myositis (IBM). Based on elevated titers of autoantibody as well as biopsy findings from salivary gland and liver, she was also diagnosed as having chronic thyroiditis, Sjögren's syndrome and autoimmune cholangitis. Currently, the pathogenic mechanism underlying IBM is not clarified, however, various factors have been suggested to contribute to it, such as viral infection or mitochondrial insufficiency. Although the pathogenic backgrounds underlying IBM are likely heterogeneous, an autoimmune-mediated mechanism may be related to the pathogenesis of IBM in the present patient.
一名67岁女性因大腿肌肉萎缩入住我院。肌肉萎缩仅限于大腿肌肉，提示她患有股四头肌肌病。股四头肌肌肉活检显示出特征性表现，如炎性细胞浸润肌内膜和肌纤维、存在镶边空泡和破碎红纤维；因此，她被诊断为包涵体肌炎（IBM）。基于自身抗体滴度升高以及唾液腺和肝脏的活检结果，她还被诊断为慢性甲状腺炎、干燥综合征和自身免疫性胆管炎。目前，IBM的致病机制尚不清楚，然而，已提出多种因素与之相关，如病毒感染或线粒体功能不全。尽管IBM的致病背景可能具有异质性，但自身免疫介导的机制可能与该患者IBM的发病机制有关。
