Aoki Masashi, Suzuki Naoki
Department of Neurology, Tohoku University School of Medicine.
Nihon Rinsho. 2012 May;70(5):895-906.
Sporadic inclusion body myositis (sIBM), the most common form of myopathy with inflammation in those over the age of 50 in Western countries, is an intractable and progressive skeletal muscle disease of unknown cause and without effective treatment. Muscle biopsy typically reveals endomysial inflammation, invasion of mononuclear cells into non-necrotic fibers and rimmed vacuoles, suggesting inflammation and degeneration are co-existed in the pathogenesis. We estimated the prevalence of sIBM in Japan is 9.83 per million in 2003 and an increase in the number of sIBM in Japan in the decade. In this review, we discuss the diagnostic criteria sIBM in Japan, and describe available treatments and promising new therapeutic strategies. We also provide an update on the current understanding of sIBM pathogenesis.
散发性包涵体肌炎(sIBM)是西方国家50岁以上人群中最常见的伴有炎症的肌病形式,是一种病因不明且无有效治疗方法的难治性进行性骨骼肌疾病。肌肉活检通常显示肌内膜炎症、单核细胞侵入非坏死纤维和镶边空泡,提示炎症和变性在发病机制中并存。我们估计2003年日本sIBM的患病率为每百万人口9.83例,且在过去十年中日本sIBM的病例数有所增加。在这篇综述中,我们讨论了日本sIBM的诊断标准,描述了现有的治疗方法和有前景的新治疗策略。我们还提供了关于sIBM发病机制当前认识的最新情况。