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Abstract Details

Cricopharyngeal bar in inclusion body myositis: A red flag
Neuromuscular and Clinical Neurophysiology (EMG)
P16 - Poster Session 16 (5:30 PM-6:30 PM)
1-004

To show the predictive risk factors for aspiration pneumonia and prognostic importance of a cricopharyngeal bar (CPB) on videofluoroscopic examination of swallowing (VFS) in inclusion body myositis (IBM).

The major cause of death in patients with IBM is pneumonia, especially aspiration pneumonia. However, aspiration was rarely detected on videofluoroscopic examination of swallowing (VFS) in a large series of the patients. Further studies are required to investigate the risk factors for aspiration pneumonia in these patients.

In this longitudinal study, we examined a consecutive series of 37 patients with clinicopathologically defined IBM based on the European Neuromuscular Center diagnostic criteria for IBM from 2013. A standard VFS was performed at diagnosis. The primary outcome was aspiration pneumonia. Secondary outcomes included IBM Functional Rating Scale score, forced vital capacity (FVC), and body mass index (BMI). Data were analyzed using Cox proportional hazard regression models, Kaplan–Meier survival curves, and log-rank tests.

Aspiration pneumonia occurred in 10 of 37 IBM patients (27%). Based on univariate analysis, 4 factors increased aspiration pneumonia risk: BMI < 18.5 (n = 5; hazard ratio [HR], 10.7; 95% CI, 2.50–46.0; p = 0.001); aspiration (n = 7; HR, 7.57; 95% CI, 1.82–31.6; p = 0.005); insufficient opening of the upper esophageal sphincter (n = 11; HR, 4.53; 95% CI, 1.12–18.3; p = 0.03); and CPB presence (n = 15; HR, 11.6; 95% CI, 1.46–91.8; p = 0.02). Clinical features of IBM-CPB(+) were elderly onset, obstruction-related dysphagia, and mild decreases in FVC, resulting in aspiration pneumonia in 1.3 years (interquartile range, 0.9–5.2); 67% of IBM-CPB(+) patients underwent interventional procedures for dysphagia. IBM-CPB(+) patients had a lower FVC than IBM-CPB(-).

A CPB in IBM is a risk factor that predicts aspiration pneumonia and refractory dysphagia requiring aggressive therapy.

Authors/Disclosures
Kenichiro Taira, MD, PhD (Jikei University School of Medicine)
PRESENTER
Dr. Taira has nothing to disclose.
Toshiyuki Yamamoto (National Center Hospital, National Center of Neurology and Psychiatry) Dr. Yamamoto has nothing to disclose.
Madoka Mori-Yoshimura, MD, PhD (National Center Hospital, National Center of Neurology and Psychiatry) Dr. Mori-Yoshimura has nothing to disclose.
No disclosure on file
No disclosure on file
Ichizo Nishino, MD, PhD, FÂé¶¹´«Ã½Ó³»­ (National Institute of Neuroscience, NCNP) The institution of Dr. Nishino has received research support from AMED.
Yuji Takahashi, MD, PhD (National Center of Neurology and Psychiatry) The institution of Dr. Takahashi has received research support from Nihon Medi-Physics Co. Limit.. The institution of Dr. Takahashi has received research support from Takeda Pharmaceutical Company Limited.