Inclusion body myositis and dysphagia. Presentation, intervention and outcome at a swallowing clinic

Abstract

Objective This study reviewed patients with inclusion body myositis who were referred for assessment of dysphagia at a tertiary swallow clinic. It describes symptoms at presentation, imaging and management strategies. Method A retrospective review of electronic patient records was performed between 2016 and 2020. Results Twenty-four patients were included, with a mean age of 72 years. Baseline modified Sydney Swallow Questionnaires identified problems with hard or dry food, food sticking, and repeated swallowing. Twenty-two patients had a Reflux Symptom Index score that could indicate significant reflux. Video swallow identified specific problems, including tongue base retraction (96 per cent) and residual pharyngeal pooling (92 per cent). Seven patients (30 per cent) had features of aspiration on imaging despite a median penetration-aspiration scale score of 2. Four patients received balloon dilatation, and two patients underwent cricopharyngeal myotomy. Conclusion This study helped to profile features of dysphagia in patients with inclusion body myositis. More evidence is needed to determine the most effective management pathway for these patients.

Idiopathic inflammatory myopathies: one year in review 2022

“…A similar immunophenotype, with some differences at the cellular level, has been found in DM-like skin lesions in antisynthetase syndrome (101). Dysphagia is a common symptom in patients with IBM; these patients should also be investigated for the presence of gastroesophageal reflux since, according to what was reported by selfadministered questionnaires in a UK third-level centre, they often also have symptoms secondary to this condition (102). Investigated by videofluoroscopy (VFS), most common features of IBM patients with dysphagia were impairment of tongue base retraction, residual pharyngeal pooling, pharyngeal constrictor impairment and cricopharyngeal hypertrophy (102).…”

“…Dysphagia is a common symptom in patients with IBM; these patients should also be investigated for the presence of gastroesophageal reflux since, according to what was reported by selfadministered questionnaires in a UK third-level centre, they often also have symptoms secondary to this condition (102). Investigated by videofluoroscopy (VFS), most common features of IBM patients with dysphagia were impairment of tongue base retraction, residual pharyngeal pooling, pharyngeal constrictor impairment and cricopharyngeal hypertrophy (102). The presence of a cricopharyngeal bar on VFS is associated with a higher risk of aspiration and subsequent pneumonia (103); moreover, the cricopharyngeal bar appears to be related to an underlying endomysial fibrosis in this muscle (104).…”

An Unusual Disease With a Common Presentation: Cricopharyngeal Dysfunction in Inclusion Body Myositis

“… 12 , 13 Endoscopic dilation is a simple and minimally invasive option; however, less than one-third of patients undergoing dilation have symptomatic improvement. 14 CP myotomy seems to be the most effective and durable treatment option to improve dysphagia symptoms. This procedure involves an incision of the CP muscle to permanently reduce resistance to bolus transit and is typically performed by otolaryngologists or gastroenterologists.…”

Imaging swallowing function and the mechanisms driving dysphagia in inclusion body myositis.

“…Secondly, contraction of the suprahyoid and thyrohyoid muscles allowing hyolaryngeal elevation, the forward movement of which mechanically opens the UES (32 (26,29). Despite the classic histopathological features of IBM observed within the CP (as described above), many reports describe hypertrophy of CP macroscopically (33)(34)(35). This contradicts the atrophy noted in the limb musculature of IBM patients.…”

“…However, some evidence indicates that CP relaxation is not overtly impaired in IBM (3,16). It has been suggested that weak suprahyoid muscle contraction and subsequent inadequate hyolaryngeal elevation contributes to reduced UES opening (3,16,33). The prevalence of impaired laryngeal elevation has been reported as high as 40-50% in some studies (16,33).…”