包涵体肌炎吞咽功能及吞咽困难发生机制的影像学研究
Imaging swallowing function and the mechanisms driving dysphagia in inclusion body myositis.
作者信息
Salam Sharfaraz, Allen Jodi, Dimachkie Mazen M, Hanna Michael G, Machado Pedro M
机构信息
Department of Neuromuscular Diseases, UCL Queen Square Institute of Neurology and The National Hospital for Neurology and Neurosurgery, London, UK.
Department of Therapy and Rehabilitation, The National Hospital for Neurosurgery, London, UK.
出版信息
Clin Exp Rheumatol. 2024 Feb;42(2):425-435. doi: 10.55563/clinexprheumatol/t1x3qa. Epub 2024 Feb 16.
Sporadic inclusion body myositis (IBM) is a progressive condition which commonly affects patients aged above 40. IBM does not respond to immunosuppression and no proven treatments are available. Up to 80% of patients develop some degree of swallowing impairment during the disease course. Dysphagia is a source of marked morbidity in IBM and predisposes patients to life-threatening complications such as aspiration pneumonia. The pathophysiology behind dysphagia in IBM is not fully understood. Evidence from imaging demonstrates that impaired swallowing is predominantly underpinned by oropharyngeal deficits. Changes in cricopharyngeal physiology is thought to be an important factor influencing dysphagia in IBM. However, it is unclear whether this is secondary to structural changes within the cricopharyngeus itself or driven by impairment of the muscles promoting pharyngeal clearance. The approach to dysphagia in IBM patients is limited by a lack of validated instruments to reliably assess swallowing function and an absence of effective therapeutic interventions derived from controlled trials targeting dysphagia. Imaging modalities such as the video fluoroscopic swallowing study (VFSS) are commonly used to evaluate dysphagia in IBM. Whilst VFSS is a commonly used technique in clinical practice; cumulative radiation exposure with repeated testing can be a limitation. Alternative imaging techniques could be developed further as outcome measures for assessing swallowing.In this review, we provide an overview of imaging techniques used to assess swallowing and the insight provided from such investigations into the mechanisms behind dysphagia in IBM. We suggest future directions for evaluation and outcome measurement of dysphagia in this population.
散发性包涵体肌炎(IBM)是一种进行性疾病,通常影响40岁以上的患者。IBM对免疫抑制无反应,且尚无经证实有效的治疗方法。在疾病过程中,高达80%的患者会出现一定程度的吞咽功能障碍。吞咽困难是IBM患者发病的一个重要原因,且易使患者发生吸入性肺炎等危及生命的并发症。IBM吞咽困难背后的病理生理学尚未完全明确。影像学证据表明,吞咽功能受损主要是由口咽功能缺陷所致。环咽肌生理功能的改变被认为是影响IBM患者吞咽困难的一个重要因素。然而,目前尚不清楚这是继发于环咽肌本身的结构变化还是由促进咽部清除的肌肉功能受损所驱动。由于缺乏可靠评估吞咽功能的有效工具以及针对吞咽困难的对照试验所衍生的有效治疗干预措施,IBM患者吞咽困难的治疗方法受到限制。视频荧光吞咽造影检查(VFSS)等影像学检查方法常用于评估IBM患者的吞咽困难。虽然VFSS是临床实践中常用的技术,但反复检查所累积的辐射暴露可能是一个限制因素。可以进一步开发替代影像学技术作为评估吞咽的结果指标。在本综述中,我们概述了用于评估吞咽的影像学技术以及此类检查对IBM吞咽困难机制的深入了解。我们提出了该人群吞咽困难评估和结果测量的未来方向。