Immune and myodegenerative pathomechanisms in inclusion body myositis.
Immune and myodegenerative pathomechanisms in inclusion body myositis.
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DOI：
10.1002/acn3.419
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发表时间：
2017-06
影响因子：
5.3
通讯作者：
Lünemann JD
中科院分区：
文献类型：
--
作者：
Keller CW;Schmidt J;Lünemann JD
包涵体肌炎(IBM)是一种在50岁以上患者中相对常见的获得性炎症性肌病。IBM的病理特征是肌纤维内蛋白包涵体和肌内炎症，表明肌肉退行性变和炎症机制都参与了其发病机制。骨骼肌纤维中调节先天和获得性免疫反应的自噬机制导致的蛋白质降解受损，最近被认为是IBM潜在的关键病理机制。免疫疗法被成功用于治疗其他炎症性肌病，但在IBM缺乏疗效，到目前为止还没有有效的治疗方法。因此，更好地了解IBM进行性肌肉无力和萎缩背后的机制路径，对于确定新的有希望的治疗干预靶点至关重要。在这里，我们讨论对IBM期间相互依赖的炎症和退行性事件的病理机制网络的最新见解。
Inclusion Body Myositis (IBM) is a relatively common acquired inflammatory myopathy in patients above 50 years of age. Pathological hallmarks of IBM are intramyofiber protein inclusions and endomysial inflammation, indicating that both myodegenerative and inflammatory mechanisms contribute to its pathogenesis. Impaired protein degradation by the autophagic machinery, which regulates innate and adaptive immune responses, in skeletal muscle fibers has recently been identified as a potential key pathomechanism in IBM. Immunotherapies, which are successfully used for treating other inflammatory myopathies lack efficacy in IBM and so far no effective treatment is available. Thus, a better understanding of the mechanistic pathways underlying progressive muscle weakness and atrophy in IBM is crucial in identifying novel promising targets for therapeutic intervention. Here, we discuss recent insights into the pathomechanistic network of mutually dependent inflammatory and degenerative events during IBM.