A phenotypic analysis shows that eosinophilic esophagitis is a progressive fibrostenotic disease

Abstract

Background Phenotypes of eosinophilic esophagitis (EoE) are not well characterized. Objective To describe clinical features of EoE patients with predefined phenotypes, determine predictors of these phenotypes, and make inferences about the natural history of EoE. Design Retrospective study. Setting Tertiary care center. Patients Incident EoE cases from 2001–2011 who met consensus diagnostic guidelines. Interventions n/a Main outcome measurements Endoscopic phenotypes, including fibrostenotic, inflammatory, or mixed. Other groups of clinical characteristics examined included atopy, level of esophageal eosinophilia, and age of symptom onset. Multinominal logistic regression assessed predictors of phenotype status. Results Of 379 cases of EoE identified, there were no significant phenotypic differences by atopic status or level of eosinophilia. Those with the inflammatory phenotype were more likely to be younger than those with mixed or fibrostenotic (13 vs 29 vs 39 years, respectively; p<0.001), and less likely to have dysphagia, food impaction, and esophageal dilation (p<0.001 for all). The mean symptom length prior to diagnosis was shorter for inflammatory (5 vs 8 vs 8 years; p=0.02). After multivariate analysis, age and dysphagia independently predicted phenotype. The OR for fibrostenosis for each 10-year increase in age was 2.1 (1.7–2.7). The OR for dysphagia was 7.0 (2.6–18.6). Limitations Retrospective, single-center study. Conclusions In this large EoE cohort, the likelihood of fibrostenosing disease increased markedly with age. For every ten year increase in age, the odds of having a fibrostenotic EoE phenotype more than doubled. This association suggests that the natural history of EoE is a progression from an inflammatory to a fibrostenotic disease.

Eosinophilic Esophagitis–More than Two Decades of Progress

“…The study showed that for every 10 -year increase in age the odds of fibrostenotic disease more than doubled. This suggests that the natural history of EoE progresses from an inflammatory to a fibrostenotic disease [82]. Previous studies reported an increase risk of perforation in EoE patients undergoing endoscopy with or without dilation of the esophagus.…”

Diagnosis and treatment of eosinophilic esophagitis in clinical practice

“…In children, unspecific symptoms (e.g., heartburn, nausea, vomiting, abdominal pain, or failure to thrive) are presented in addition to dysphagia, while in adults, eating difficulties (e.g., repeated dysphagia or food impaction) are predominantly presented [54]. This difference appears to be associated with the time-dependent disease progression in which active eosinophilic inflammation is predominantly present in children and subsequent fibrostenotic changes of the esophageal wall are the main complications in adults [55,56]. Dysfunction of the esophageal proper muscle layer is also considered to participate in symptom generation [57,58].…”

“…4) [87,88]. Subepithelial fibrosis in the esophageal wall progresses with persisting long-term eosinophilic inflammation, resulting in an esophagus of narrow caliber or with an esophageal stricture [55,56]. In some cases, a traumatic tear occurs with the passage of the endoscope, namely crepe-paper esophagus, indicative of mucosal fragility (Fig.…”

“…Patients requiring dilation are primarily adults since esophageal remodeling (e.g., esophageal stricture or narrowing) develops progressively during long-term and persistent eosinophilic inflammation [55,56]. Three types of procedures have primarily been employed, (1) the simple bougie; (2) the wire-guided bougie; and (3) through-the-scope (TTS) balloon dilation [142].…”

Eosinophilic Oesophagitis (EoE): An update

“…Indeed the odds of EoE transforming from an inflammatory to fibrostenotic disease increase significantly with age [47] and the prevalence of strictures increases with disease duration [48] . formula diet being helpful in children who had oesophageal eosinophilia that was not responsive to standard treatment [64] .…”