Machado Pedro M, Ahmed Mhoriam, Brady Stefen, Gang Qiang, Healy Estelle, Morrow Jasper M, Wallace Amanda C, Dewar Liz, Ramdharry Gita, Parton Matthew, Holton Janice L, Houlden Henry, Greensmith Linda, Hanna Michael G
MRC Centre for Neuromuscular Diseases, Institute of Neurology, University College London, Box 102, 8-11 Queen Square, London, WC1N 3BG, UK,
Curr Rheumatol Rep. 2014 Dec;16(12):477. doi: 10.1007/s11926-014-0477-9.
Sporadic inclusion body myositis (IBM) is an acquired muscle disorder associated with ageing, for which there is no effective treatment. Ongoing developments include: genetic studies that may provide insights regarding the pathogenesis of IBM, improved histopathological markers, the description of a new IBM autoantibody, scrutiny of the diagnostic utility of clinical features and biomarkers, the refinement of diagnostic criteria, the emerging use of MRI as a diagnostic and monitoring tool, and new pathogenic insights that have led to novel therapeutic approaches being trialled for IBM, including treatments with the objective of restoring protein homeostasis and myostatin blockers. The effect of exercise in IBM continues to be investigated. However, despite these ongoing developments, the aetiopathogenesis of IBM remains uncertain. A translational and multidisciplinary collaborative approach is critical to improve the diagnosis, treatment, and care of patients with IBM.
散发性包涵体肌炎(IBM)是一种与衰老相关的后天性肌肉疾病,目前尚无有效治疗方法。正在进行的进展包括:可能为IBM发病机制提供见解的基因研究、改进的组织病理学标志物、一种新的IBM自身抗体的描述、对临床特征和生物标志物诊断效用的审视、诊断标准的完善、MRI作为诊断和监测工具的新应用,以及新的致病见解促使针对IBM试验新的治疗方法,包括旨在恢复蛋白质稳态的治疗和肌肉生长抑制素阻滞剂。运动对IBM的影响仍在研究中。然而,尽管有这些不断的进展,IBM的病因发病机制仍不明确。采用转化医学和多学科协作方法对于改善IBM患者的诊断、治疗和护理至关重要。