Epidemiology of Eosinophilic Esophagitis in Patients with Cystic Fibrosis: A Population-Based 5-Year Study

Abstract

Purpose The prevalence of eosinophilic esophagitis (EoE) has been on the rise since it was first described in the 1990s. Several diseases and exogenous factors have been associated with EoE. Our aim was to investigate the epidemiology of EoE in cystic fibrosis (CF) patients. Methods We identified individuals with CF from September 2014 to September 2019 within a database (IBM Explorys Solutions, Inc.). The prevalence of EoE in patients with CF was compared to the general population. Results The database included 36,111,860 patients during the 5-year study period: 12,950 with CF (0.036%) and 28,090 with EoE (0.078%). EoE prevalence was higher in CF patients than the general population (46 in 10,000 vs. 7.8 in 10,000, p <0.001). Patients with CF and EoE were more likely to be male (50% vs. 33.5%, p <0.008), children (33.3% vs. 16.5%, p <0.001), and non-Hispanic (100% vs. 88.7%, p <0.001) than CF patients without EoE. CF with EoE patients were more likely to be children than EoE only (33.3% vs. 10.5%, p <0.001). Allergic conditions were generally more prevalent in CF with EoE than CF only (83.3% vs. 68.3%, p =0.01) and EoE only (83.3% vs. 69.3%, p =0.014). Conclusion EoE is nearly 6-times more prevalent in CF patients. Those patients had higher incidence of other atopic conditions. EoE must be considered in the differential diagnosis of patients with CF presenting with dysphagia, refractory gastroesophageal reflux, vomiting, and other esophagus-related symptoms.

Cystic fibrosis and fat malabsorption: Pathophysiology of the cystic fibrosis gastrointestinal tract and the impact of highly effective CFTR modulator therapy

“…PwCF may experience an increased incidence of other esophageal diseases. Eosinophilic esophagitis, a chronic inflammatory disease of the esophagus often associated with food allergies, appears to be increased in pwCF compared with the general population 22 . The cause is not clear but may be due to a genetic effect or immune dysfunction.…”

“…Eosinophilic esophagitis, a chronic inflammatory disease of the esophagus often associated with food allergies, appears to be increased in pwCF compared with the general population. 22 The cause is not clear but may be due to a genetic effect or immune dysfunction. Eosinophilic esophagitis should be considered in pwCF who have associated feeding problems, chronic emesis, dysphagia/odynophagia, GERD-like symptoms not responsive to acid suppression therapy, and a history of food impaction.…”

“…Eosinophilic esophagitis should be considered in pwCF who have associated feeding problems, chronic emesis, dysphagia/odynophagia, GERD-like symptoms not responsive to acid suppression therapy, and a history of food impaction. [22][23][24] Although GERD may be a contributing factor of such disorders, it is unclear if PPI usage is protective in the setting of CF. 23,24 An increased incidence of Barrett's esophagus (metaplasia transformation of the esophageal squamous epithelium) and esophageal adenocarcinoma have been reported in pwCF.…”

“…The cause is not clear but may be due to a genetic effect or immune dysfunction. Eosinophilic esophagitis should be considered in pwCF who have associated feeding problems, chronic emesis, dysphagia/odynophagia, GERD‐like symptoms not responsive to acid suppression therapy, and a history of food impaction 22–24 . Although GERD may be a contributing factor of such disorders, it is unclear if PPI usage is protective in the setting of CF 23,24 …”

Gastrointestinal Pathologies in Pediatric Patients With Cystic Fibrosis Undergoing Endoscopy: A Single-Center Retrospective Review Over 15 Years

“…Small case series of pediatric patients with CF have described associated CD, eosinophilic esophagitis (EoE), and inflammatory bowel disease [11][12][13][14][15][16]. Furthermore, studies including both adults and children with CF have demonstrated a potential increased incidence of CD and EoE [5,17].…”