Inclusion body myositis (IBM) remains an enigmatic and complex muscle disorder where a deeper understanding of disease pathomechanisms and the identification of potential genetic contributors represent an unmet need. The absence of effective treatments has spurred endeavours to reassess the interplay between degeneration, including autophagy, mitochondrial dysfunction and proteasomal dysregulation, and autoimmunity. IBM is unique among the other idiopathic inflammatory myopathies owing to its molecular signature involving highly differentiated cytotoxic T cells that evade immune regulation. This has led to a resurgence of interest in the development of immunomodulatory therapy. This review discusses the potential role of cellular immunosenescence in sustaining inflammation and/or fibrotic remodelling observed in IBM and appraises the rationale for some potential therapeutic approaches to mitigate disease progression.

中文翻译：

---

包涵体肌炎和免疫衰老：当前证据和未来前景


包涵体肌炎 （IBM） 仍然是一种神秘而复杂的肌肉疾病，其中对疾病病理机制的更深入理解和对潜在遗传因素的识别代表了未满足的需求。缺乏有效的治疗方法促使人们努力重新评估退化（包括自噬、线粒体功能障碍和蛋白酶体失调）与自身免疫之间的相互作用。IBM 在其他特发性炎症性肌病中是独一无二的，因为它的分子特征涉及逃避免疫调节的高度分化的细胞毒性 T 细胞。这导致人们对免疫调节疗法的发展重新产生兴趣。本综述讨论了细胞免疫衰老在维持 IBM 中观察到的炎症和/或纤维化重塑中的潜在作用，并评估了一些缓解疾病进展的潜在治疗方法的基本原理。