Centre for Molecular Medicine and Innovative Therapeutics, Murdoch University, Perth, WA, Australia.
Centre for Molecular Medicine and Innovative Therapeutics, Murdoch University, Perth, WA, Australia.
Best Pract Res Clin Rheumatol. 2022 Jun;36(2):101761. doi: 10.1016/j.berh.2022.101761. Epub 2022 Jun 25.
Inclusion body myositis (IBM) is a slowly progressive muscle disease affecting ageing individuals. IBM presents with a distinctive pattern of weakness involving the quadriceps and finger flexor muscles, although other muscles including pharyngeal muscles become affected over time. Pathological hallmarks of IBM include autoimmune features, including endomysial infiltration by highly differentiated T cells, as well as degenerative features marked by intramyofibre protein aggregates organised into inclusion bodies. Despite some progress in understanding the cellular pathways involved in IBM, it remains untreatable, and the progression of the disease leads to progressive weakness, disability, wheelchair dependency and loss of independence. Therefore, there is an urgent need to improve our understanding of the underlying mechanisms and pathways involved in this disease to identify new treatment targets. Here, we discuss the current understanding of aetiopathogenesis, the interrelationship between autoimmunity and degeneration, and how ageing is a major influencer of both these features.
包涵体肌炎（IBM）是一种影响老年人的进行性肌肉疾病。IBM 的表现为一种独特的无力模式，累及股四头肌和手指屈肌，但随着时间的推移，包括咽部肌肉在内的其他肌肉也会受到影响。IBM 的病理特征包括自身免疫特征，包括高分化 T 细胞对肌内膜的浸润，以及退行性特征，其特征是肌纤维内的蛋白聚集体形成包涵体。尽管在理解 IBM 涉及的细胞途径方面取得了一些进展，但它仍然无法治疗，疾病的进展导致进行性无力、残疾、轮椅依赖和丧失独立性。因此，迫切需要提高我们对这种疾病涉及的潜在机制和途径的理解，以确定新的治疗靶点。在这里，我们讨论了发病机制的当前理解、自身免疫和退行性变之间的相互关系，以及衰老如何成为这两个特征的主要影响因素。
