Eosinophilic esophagitis: Current concepts in diagnosis and management

Abstract

Eosinophilic esophagitis is an antigen-mediated chronic inflammatory disorder that has risen in incidence and prevalence over the past 2 decades. The clinical presentation is variable and consists of mainly esophageal symptoms such as dysphagia, heartburn, food impaction, and vomiting. Current management relies on dietary elimination, proton-pump inhibitors, and topical corticosteroids with different response rates and relapses after treatment discontinuation. With a better understanding of the underlying pathophysiology, many molecules emerged recently as targeted treatment including dupilumab (IL4/IL13 blocker), as the first FDA-approved biological treatment, which has changed the management paradigm.

Figure 1

Number of publications of EoE between 1978 and 2022

Figure 2

EoE is a chronic inflammatory condition of the esophagus which is allergen-driven, and its manifestation varies with age and could be provoked by either ingested food allergens or inhaled locally deposited aeroallergens. Although there is a genetic predisposition, environmental factors appear to have a larger role. Impaired epithelial barrier function with increased proteas activity, decreased junctional proteins, increased cellular proliferation (basal cell hyperplasia), and decreased differentiation. There is also an immune dysregulation with an increase in eosinophils, basophils, mast cells, and Th2 lymphocytes and an increase in IL-4, IL-5, and IL-13. These factors, in addition to the enhanced expression of TGF-β, result in activation of fibroblasts in the lamina propria, collagen deposition, and tissue stiffness. CCL 26: encodes eotaxin-3, CD4: cluster of differentiation 4, IL: interleukin, TGF-β: transforming growth factor beta, Th2: T helper 2 cell, TSP1: thrombospondin-1, TSPAN12: tetraspanin 12. Created by using pictures from Servier Medical Art. Servier Medical Art by Servier is licensed under a Creative Commons Attribution 3.0 Unported License (https://creativecommons.org/licenses/by/3.0/) and the figure was adapted from Underwood et al.[31]

Figure 3

Clinical presentations of EoE according to the age

Figure 4

Natural history of EoE if untreated

Figure 5

Endoscopic findings in EoE: a: Esophageal rings persisting in a patient with EoE on treatment. b: exudates and edema. c: Exudates, rings, edema, furrows, and mild narrowing. d: Crepe-paper mucosa after passing the scope in a narrowed proximal esophagus

Figure 6

EREFS endoscopic grading system for EoE[72] (Wolters Kluwer Health, Inc. Permission Licence Number 5720730303645)

Figure 7

Physiomechanical model of EoE. FLIP panometry outputs from six patients (A-F) with EoE are displayed reflecting the physiomechanical states of EoE. In A–C, esophageal distensibility is normal (noted by normal diameters of the esophageal body). In A, a normal contractile response involving repetitive antegrade contractions (RACs) is triggered, whereas B is weak with some evidence of contractions (BCR/IDCR with low bag pressures). C is nonreactive without triggering of contractility and can be achalasia-like (ALP) if the EGJ-DI >3.0 and Weak-GERD-like if EGJ-DI is >3.0. In D–F, fibrostenotic obstruction is reflected by reduced distensibilty (low diameters/reduced compliance) of the esophageal body and EGJ. In D, there is evidence of RACs with a rate of 6 and this pattern is associated with higher pressures than normal and a low EGJ-DI mechanical obstruction. Panel E shows reactive-spastic contractility patterns and is associated with spastic-like responses (IDCR/SRCR) and no evidence of RACs. The pressures in this pattern are usually well above 80 mmHg. F is nonreactive with absent contractile response, and the pressure is usually high, but does not have severe fluctuations like D and E (Image courtesy of Professor John E. Pandolfino, Chicago, IL, USA)

Figure 8

Treatment algorithm in patients with EoE