Department of Neurology and Hope Center for Neurological Diseases, Washington University School of Medicine, Saint Louis, MO, USA.
Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Expression, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.
Neuropathol Appl Neurobiol. 2017 Feb;43(1):82-91. doi: 10.1111/nan.12384.
Sporadic inclusion body myositis (sIBM) is an insidious late-onset progressive myopathy that typically affects patients over the age of 50. Clinically, patients develop a characteristic pattern of weakness that affects the forearm flexors and knee extensors. Muscle biopsy, often utilized in the diagnosis, demonstrates a chronic myopathy with mixed pathologies harbouring intramyofiber protein inclusions and endomysial inflammation. The co-existence of these pathologic features (that is, inflammation and protein aggregation) has divided the field of sIBM research into two opposing (albeit slowly unifying) camps regarding disease pathogenesis. The present review explores the recent evidence supporting these distinct pathogenic mechanisms. Future therapies that are designed to target both aspects of sIBM pathologies will likely be necessary to treat sIBM.
散发性包涵体肌炎(sIBM)是一种隐匿性的迟发性进行性肌病,通常影响 50 岁以上的患者。临床上,患者会出现一种特征性的无力模式,影响前臂屈肌和伸膝肌。肌肉活检常用于诊断,显示出一种慢性肌病,具有混合病理学特征,包含肌纤维内蛋白包涵体和内膜炎症。这些病理特征(即炎症和蛋白聚集)的共存,将 sIBM 研究领域分为两个对立的(尽管正在缓慢统一)阵营,涉及疾病发病机制。本综述探讨了支持这些不同发病机制的最新证据。未来旨在针对 sIBM 病理学的两方面进行治疗的疗法,可能对治疗 sIBM 是必要的。