1143 Prevalence of Eosinophilic Esophagitis Among Patients With Cystic Fibrosis: A Web-Based Survey

Abstract

INTRODUCTION: Cystic Fibrosis (CF) is a multi-system disease including gastrointestinal (GI) manifestations such as growth failure, chronic/recurrent abdominal pain and gastroesophageal reflux disease. Eosinophilic Esophagitis (EoE) is an atopic condition characterized by infiltration of the esophageal mucosa by eosinophils and associated with symptoms of esophageal dysfunction such as dysphagia, vomiting and feeding difficulties. The prevalence of EoE in the general population is estimated at 0.5-1 in 2000. EoE prevalence in individuals with CF is not well determined. OBJECTIVE: To assess the prevalence of EoE in pediatric and adult CF patients, to explore the healthcare provider’s awareness of this disease, and to evaluate the availability of resources for reliable investigations and diagnosis. METHODS: We conducted a web-based survey of CF care centers in the United States. The survey was sent to 234 CF care center directors (CDs). Responses were gathered from May 7 to May 30, 2019. A combination of open- and closed-ended questions was used. RESULTS: The survey was completed by 8.1% (19/234) of the contacted CDs/designees (9 pediatric, 3 adult and 7 combined programs) (Table). 68% (13/19) of respondents had at least one suspected EoE case in the previous 24 months; a total of 18 confirmed EoE cases was reported out of total of 2395 patients with CF. All centers reported access to appropriate GI service with expertise in EoE. The prevalence of EoE among the CF patients was found to be 7/1000 (9/1295), 8.5/1000 (3/355), and 8/1000 (6/745) in the pediatric, adult and combined programs respectively. The total prevalence in all age groups was estimated at 7.5/1000. CONCLUSION: This study indicates high awareness of EoE among US CF care centers. Appropriate GI services with expertise in the diagnosis and management of EoE was available to all providers who responded to this survey. The prevalence of EoE among individuals with CF regardless of age is approximately 0.75% which is 7-14 times higher than that of the general population. There was no significant difference in the prevalence of EoE in pediatric versus adult CF populations. These results deserve more studies into the pathogenesis of EoE in CF, the reasons behind increased prevalence, and further characterization of CF patients with EoE.