3114 A Common Gastrointestinal Manifestation of an Uncommon Disease

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INTRODUCTION: Inclusion body myositis (IBM) is a chronic progressive myopathy characterized by dysphagia in 10% to 73% of patients with one-year mortality of 31% in patients with symptoms of dysphagia. We present a case of a patient with IBM esophagitis causing severe weight loss and complete loss of ability to swallow with a discussion of potential management modalities. CASE DESCRIPTION/METHODS: The patient was a 77-year-old male with a past medical history of inclusion body myositis diagnosed at age of 57 and muscular dystrophy who presented with progressive dysphagia for several years and a significant weight loss of 40 pounds over last 8 months. He initially was able to tolerate small bites, however, he was not able to tolerate solids and his diet was modified to a mechanical soft diet. Prior to the hospital admission, the patient had an outpatient modified barium study (MBS) which revealed penetration and aspiration with thin and nectar thick liquids. An endoscopy was attempted for percutaneous gastrostomy tube placement but was unsuccessful due to the inability to pass through the upper esophageal sphincter. He subsequently underwent a fluoroscopy-guided percutaneous gastrostomy tube placement by an interventional radiologist. DISCUSSION: IBM is a chronic progressive inflammatory myopathy. Associated dysphagia can result in nutritional deficits, weight loss, aspiration pneumonia and decreased quality of life. The underlying mechanism for dysphagia is related to inadequate pharyngeal contraction, poor relaxation of the cricopharyngeal muscle, and reduced hyolaryngeal elevation. The management of dysphagia in IBM is challenging due to its steroid resistance nature. Multiple treatment modalities are used to treat dysphagia secondary to IBM, including conservative measures, intravenous immunoglobulin therapy, and balloon. Surgical intervention with cricopharyngeal myotomy has been suggested when conservative therapies fail or when severe weight loss and malnutrition are apparent. The later appears to be effective especially when hypertonicity of the cricopharyngeal muscle and impaired relaxation are present. A retrospective study of 26 patients with IBM has shown higher mortality in patients who required PEG placement compared to the ones who underwent cricopharyngeal myotomy. Per-oral endoscopic myotomy (POEM) showed success for spastic esophageal disorders such as diffuse esophageal spasm. At the meantime, no studies have implied the procedure for IBM esophagitis giving a great room for future studies.