Objective To determine whether histopathological, electromyographic and laboratory markers correlate with clinical measures in inclusion body myositis (IBM). Methods We reviewed our electronic medical records to identify patients with IBM according to European Neuromuscular Center (ENMC) 2011 criteria, seen between 2015 and 2020. We only included patients who had a muscle biopsy and needle electromyography (EMG) performed on the same muscle (opposite or same side). We used a detailed grading system [0 (normal) to 4 (severe)] to score histopathological and EMG findings. Clinical severity was assessed by the modified Rankin scale (mRS), muscle strength sum score (SSS), quadriceps strength and severity of dysphagia on swallow evaluation. Serum markers of interest were creatine kinase level and cN-1A antibodies. Results We included 50 IBM patients, with a median age of 69 years; 64% were males. Median disease duration at diagnosis was 51 months. On muscle biopsy, endomysial inflammation mainly correlated with dysphagia, and inversely correlated with mRS. Vacuoles and congophilic inclusions did not correlate with any of the clinical measures. On EMG, the shortness of motor un it potential (MUP) duration correlated with all clinical measures. Myotonic discharges, and not fibrillation potentials, correlated with the severity of inflammation. Serum markers did not have a statistically significant correlation with any of the clinical measures. Conclusions Dysphagia was the main clinical feature of IBM correlating with endomysial inflammation. Otherwise, inclusion body myositis clinical measures had limited correlation with histopathological features in this study. The shortness of MUP duration correlated with all clinical measures.

中文翻译：

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包涵体肌炎：临床结果与组织病理学、肌电图和实验室检查结果的相关性

目的确定组织病理学、肌电图和实验室标志物是否与包涵体肌炎 (IBM) 的临床测量相关。方法 我们审查了我们的电子病历，以根据欧洲神经肌肉中心 (ENMC) 2011 年标准（见于 2015 年至 2020 年）来识别 IBM 患者。我们只包括在同一肌肉上进行了肌肉活检和针状肌电图 (EMG) 的患者。对面或同侧）。我们使用详细的分级系统 [0（正常）到 4（严重）] 对组织病理学和 EMG 发现进行评分。通过改良 Rankin 量表 (mRS)、肌肉力量总分 (SSS)、股四头肌力量和吞咽困难的严重程度评估临床严重程度。感兴趣的血清标志物是肌酸激酶水平和 cN-1A 抗体。结果 我们纳入了 50 名 IBM 患者，中位年龄为 69 岁；64% 是男性。诊断时的中位病程为 51 个月。在肌肉活检中，肌内膜炎症主要与吞咽困难相关，与 mRS 呈负相关。液泡和嗜刚果红包涵体与任何临床指标均不相关。在 EMG 上，运动单位电位 (MUP) 持续时间的缩短与所有临床测量值相关。强直性放电，而不是纤颤电位，与炎症的严重程度相关。血清标志物与任何临床测量均无统计学显着相关性。结论 吞咽困难是IBM与肌内膜炎症相关的主要临床特征。否则，包涵体肌炎临床测量与本研究中的组织病理学特征的相关性有限。