Clinical implication of denervation in sporadic inclusion body myositis
Clinical implication of denervation in sporadic inclusion body myositis
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DOI：
10.1016/j.jns.2022.120317
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发表时间：
2022-06-13
影响因子：
4.4
通讯作者：
Katsuno, Masahisa
中科院分区：
文献类型：
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作者：
Noda, Seiya;Murakami, Ayuka;Katsuno, Masahisa
简介：散发性包涵体肌炎（sIBM）常伴有肌电图（EMG）提示去神经支配的体征，类似神经源性疾病。因此，本研究旨在评估sIBM去神经支配后的再神经支配及其临床意义。方法：我们回顾性检查了从2001年至2018年转诊到我们机构进行诊断性肌肉活检的109例sIBM患者中收集的连续肌肉活检标本。通过肌肉活检和EMG评估sIBM患者去神经后的神经再支配。乙酰胆碱受体γ亚基（Chrng）和肌肉特异性激酶（MuSK）mRNA的水平，这是去神经支配的标志物，采用实时聚合酶链反应进行了检查。对治疗的反应被定义为增加1级或更高的两个或更多的肌肉群使用医学研究理事会scale.Results评估：在总数中，93（85.3%）的109 sIBM患者有神经支配后，去神经支配的组织学检查和/或肌电图。去神经支配后再神经支配的患者活检前的平均病程明显长于未再神经支配的患者（p < 0.00001）。失神经支配患者的Chrng和MuSK mRNA水平显著高于未失神经支配患者。去神经组对免疫抑制治疗有反应的患者比例低于无神经组（p < 0.05）。然而，两组之间从发病到使用助行器的时间没有显著差异。讨论：去神经支配后的神经再支配与sIBM患者的病程和短期治疗反应有关。
Introduction: Sporadic inclusion body myositis (sIBM) is often accompanied by signs suggestive of denervation on electromyography (EMG), which mimics neurogenic disorders. Hence, the current study aimed to assess reinnervation after denervation in sIBM and its clinical impllcation.Methods: We retrospectively examined consecutive muscle biopsy specimens collected from 109 sIBM patients who were referred to our institution for diagnostic muscle biopsy from 2001 to 2018. Reinnervation after denervation in sIBM patients was assessed via muscle biopsy and EMG. The levels of acetylcholine receptor subunit gamma (Chrng) and muscle-specific kinase (MuSK) mRNA, which are markers of denervation, were examined using real-time polymerase chain reaction. Response to treatment was defined as an increase of grade 1 or higher in two or more muscle groups as assessed using the Medical Research Council scale.Results: In total, 93 (85.3%) of 109 sIBM patients had reinnervation after denervation on histological examination and/or EMG. The mean disease duration before biopsy was significantly longer in patients with reinnervation after denervation than in those without (p < 0.00001). Patients with denervation had significantly higher levels of Chrng and MuSK mRNA than those without. The proportion of patients who responded to immunosuppressive therapies was smaller in the patients with denervation than those without (p < 0.05). However, there was no significant difference regarding time from onset to using a walking aid between the two groups. Discussion: Reinnervation after denervation is associated with disease duration and short-term response to therapy in individuals with sIBM.