1794 Esophageal Adenocarcinoma in a Patient With Inclusion Body Myositis

Abstract

INTRODUCTION: Inflammatory myopathies are rare autoimmune diseases characterized by chronic muscle inflammation and weakness. Dysphagia is often an early onset symptom in inflammatory myopathies. Here we present a case of esophageal adenocarcinoma diagnosed in a patient with inclusion body myositis (IBM) and chronic progressive dysphagia. CASE DESCRIPTION/METHODS: A 59 year old woman with an 8 year history of gradually progressive IBM presented to the ER with worsening dysphagia; she has had progressive dysphagia over the preceding 10 days to the point she is unable to tolerate solids. Despite longstanding, progressive symptoms, referral to a gastroenterologist was never made as it was assumed her symptoms were from her underlying IBM. On exam, she is a functional quadriplegic from her underlying IBM and is largely bedbound as a result. Her labs were notable for a macrocytic anemia with negative inflammatory myopathy antibody panel. Barium esophagram revealed a large filling defect in the mid to distal esophagus measuring approximately 3.6 × 2.4 cm and subsequent upper endoscopy revealed an 8cm nearly obstructive distal esophageal mass which appeared to arise in the setting of suspected Barrett’s esophagus. Biopsies demonstrated esophageal adenocarcinoma with signet cell features. Subsequent staging was consistent with cT3N0 disease though she was deemed not to be a surgical candidate due to her quadriplegia and severe deconditioning. An endoscopic esophageal bare metal stent was placed for symptom palliation and she was referred to hospice. DISCUSSION: The inflammatory myopathies are a group of autoimmune diseases characterized by chronic muscle inflammation accompanied by weakness. IBM, in contrast to polymyositis and dermatomyositis, has normal or minimally elevated creatine kinase, insidious progression, and is poorly responsive to immunosuppressive therapy. While IBM is generally not thought to be a paraneoplastic syndrome, there are case reports of them being paraneoplastic with a negative antibody panel (as seen in this case). Bulbar symptoms are common in IBM, and >50% of patients will develop oropharyngeal dysphagia usually due to cricoarytenoid weakness. In this case, the patient’s dysphagia was prematurely attributed to IBM, precluding a timely diagnosis of esophageal adenocarcinoma. In patients with inflammatory myopathy and dysphagia, endoscopy is still of utmost importance and symptoms should not be solely attributed to underlying IBM.