PR_146: Dysphagia in Patients With Inclusion Body Myositis: Management and Clinical Outcome

Abstract

Objective: To assess the clinical features, treatment strategies, and clinical outcome of patients with inclusion body myositis (IBM) and dysphagia. Design: Retrospective review. Setting: Multidisciplinary group practice. Participants: 26 consecutive patients (mean age, 72.2y) with IBM-associated dysphagia seen in 1997 to 2001. Interventions: Not applicable. Main Outcome Measures: Presence of dysphagia, and response to rehabilitation and interventional treatment. Results: Dysphagia in patients with IBM was more common in women (20:6). 11 patients (42.3%) presented with dysphagia as an initial presenting symptom. The common dysphagia-related symptoms included the sensation of food sticking, coughing while eating, and difficulty eating solid and dry foods. Cricopharyngeal muscle dysfunction was noted in all patients who had barium swallow studies. Swallowing compensation techniques and exercises were of unclear benefit, but the Mendelsohn maneuver as an exercise appears to help in maintaining oral intake. 18 (69%) patients underwent interventional procedures: 10, cricopharyngeal myotomy; 6, pharyngoesophageal dilation; 6, percutaneous endoscopic gastrostomy; and 2, botulinum injection. The mean follow-up duration was 55.5 months. Dysphagia tended to worsen with time, with improvement noted only in those who were treated interventionally: 5 with cricopharyngeal myotomy and 1 with pharyngoesophageal dilation. 13 (50%) patients died, with the mean age at death of 80.8 years and the mean interval of 110.4 months after the diagnosis of IBM. When the cause of death was identified in 7 patients, it was respiratory complications of aspiration in all 7. Conclusions: Dysphagia may precede weakness of the extremities and be a presenting symptom of IBM. Treatment involves both conservative and interventional measures. Despite interventional procedures in 69% of the patients, dysphagia in IBM was generally progressive. Selective patients may benefit from cricopharyngeal myotomy. Aspiration was the main cause of death in this population.