Strictures in Eosinophilic Esophagitis
Background & Aims: Development of strictures is a major concern for patients with eosinophilic esophagitis (EoE). At diagnosis, EoE can present with an inflammatory phenotype (characterized by whitish exudates, furrows, and edema), a stricturing phenotype (characterized by rings and stenosis), or a combination of these. Little is known about progression of stricture formation; we evaluated stricture development over time in the absence of treatment and investigated risk factors for stricture formation.
Methods: We performed a retrospective study using the Swiss EoE Database, collecting data on 200 patients with symptomatic EoE (153 men; mean age at diagnosis, 39 ± 15 years old). Stricture severity was graded based on the degree of difficulty associated with passing of the standard adult endoscope.
Results: The median delay in diagnosis of EoE was 6 years (interquartile range, 2–12 years). With increasing duration of delay in diagnosis, the prevalence of fibrotic features of EoE, based on endoscopy, increased from 46.5% (diagnostic delay, 0–2 years) to 87.5% (diagnostic delay, >20 years; P = .020). Similarly, the prevalence of esophageal strictures increased with duration of diagnostic delay, from 17.2% (diagnostic delay, 0–2 years) to 70.8% (diagnostic delay, >20 years; P < .001). Diagnostic delay was the only risk factor for strictures at the time of EoE diagnosis (odds ratio = 1.08; 95% confidence interval: 1.040–1.122; P < .001).
Conclusions: The prevalence of esophageal strictures correlates with the duration of untreated disease. These findings indicate the need to minimize delay in diagnosis of EoE.
Eosinophilic esophagitis (EoE) has recently been defined by an expert panel as "a chronic, immune/antigen-mediated, esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation."
There is evidence that EoE incidence increased over the last decades with current prevalence rates in the United States and Europe of about 1 affected individual among 2000 inhabitants in the pediatric as well as in the adult population. Adult EoE patients primarily suffer from dysphagia, often culminating in food impaction necessitating endoscopic bolus removal.
The endoscopic presentation of EoE is quite variable. Recently, the endoscopic features of EoE have been graded by Hirano et al. For the purposes of this study, characteristic features of EoE were classified into the following categories: the inflammatory and fibrotic group of EoE features. According to this classification, whitish exudates, edema, and linear furrows represent the items of acute inflammation. Normal esophageal diameter is another hallmark characteristic associated with the inflammatory group of endoscopic features. The fibrotic features of EoE are rings, strictures, and crêpe-paper esophagus. Most patients present with a mix of these inflammatory and fibrotic features at the time of EoE diagnosis.
There is a lack of data evaluating stricture development over time in EoE. We know from natural history studies in Crohn's disease that patients initially present with an inflammatory phenotype and that complications (strictures and/or fistulas) develop over time. It is currently unknown whether, similar to Crohn's disease progression, EoE is initially characterized by the occurrence of inflammatory features and, as inflammation persists, fibrotic features, including strictures, develop over time. The lack of data on stricture formation in EoE might be related to the fact that EoE is diagnosed with a longer diagnostic delay (time period from appearance of first symptoms to diagnosis, median 5 years in EoE) when compared with Crohn's disease (median, 0.75 years). During this long diagnostic delay, unbridled eosinophil-predominant inflammation is allowed to persist, such that patients can present with strictures at the time of diagnosis. Given the imminent risk of food bolus impactions, purely observational studies of untreated EoE patients are rare for reasons related to ethical standards. Therefore, we examined the relationship between the duration of untreated disease, which corresponds to the diagnostic delay (time period from symptom onset to EoE diagnosis), and the appearance of endoscopic alterations at time of EoE diagnosis.
We aimed to examine whether the length of diagnostic delay positively correlates with the frequency of encountering strictures in EoE patients at the time of diagnosis. An additional aim of the present study was to determine the kinetics of the appearance of inflammatory and fibrotic endoscopic features over time. We also aimed to identify risk factors for stricture development.
Abstract and Introduction
Abstract
Background & Aims: Development of strictures is a major concern for patients with eosinophilic esophagitis (EoE). At diagnosis, EoE can present with an inflammatory phenotype (characterized by whitish exudates, furrows, and edema), a stricturing phenotype (characterized by rings and stenosis), or a combination of these. Little is known about progression of stricture formation; we evaluated stricture development over time in the absence of treatment and investigated risk factors for stricture formation.
Methods: We performed a retrospective study using the Swiss EoE Database, collecting data on 200 patients with symptomatic EoE (153 men; mean age at diagnosis, 39 ± 15 years old). Stricture severity was graded based on the degree of difficulty associated with passing of the standard adult endoscope.
Results: The median delay in diagnosis of EoE was 6 years (interquartile range, 2–12 years). With increasing duration of delay in diagnosis, the prevalence of fibrotic features of EoE, based on endoscopy, increased from 46.5% (diagnostic delay, 0–2 years) to 87.5% (diagnostic delay, >20 years; P = .020). Similarly, the prevalence of esophageal strictures increased with duration of diagnostic delay, from 17.2% (diagnostic delay, 0–2 years) to 70.8% (diagnostic delay, >20 years; P < .001). Diagnostic delay was the only risk factor for strictures at the time of EoE diagnosis (odds ratio = 1.08; 95% confidence interval: 1.040–1.122; P < .001).
Conclusions: The prevalence of esophageal strictures correlates with the duration of untreated disease. These findings indicate the need to minimize delay in diagnosis of EoE.
Introduction
Eosinophilic esophagitis (EoE) has recently been defined by an expert panel as "a chronic, immune/antigen-mediated, esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation."
There is evidence that EoE incidence increased over the last decades with current prevalence rates in the United States and Europe of about 1 affected individual among 2000 inhabitants in the pediatric as well as in the adult population. Adult EoE patients primarily suffer from dysphagia, often culminating in food impaction necessitating endoscopic bolus removal.
The endoscopic presentation of EoE is quite variable. Recently, the endoscopic features of EoE have been graded by Hirano et al. For the purposes of this study, characteristic features of EoE were classified into the following categories: the inflammatory and fibrotic group of EoE features. According to this classification, whitish exudates, edema, and linear furrows represent the items of acute inflammation. Normal esophageal diameter is another hallmark characteristic associated with the inflammatory group of endoscopic features. The fibrotic features of EoE are rings, strictures, and crêpe-paper esophagus. Most patients present with a mix of these inflammatory and fibrotic features at the time of EoE diagnosis.
There is a lack of data evaluating stricture development over time in EoE. We know from natural history studies in Crohn's disease that patients initially present with an inflammatory phenotype and that complications (strictures and/or fistulas) develop over time. It is currently unknown whether, similar to Crohn's disease progression, EoE is initially characterized by the occurrence of inflammatory features and, as inflammation persists, fibrotic features, including strictures, develop over time. The lack of data on stricture formation in EoE might be related to the fact that EoE is diagnosed with a longer diagnostic delay (time period from appearance of first symptoms to diagnosis, median 5 years in EoE) when compared with Crohn's disease (median, 0.75 years). During this long diagnostic delay, unbridled eosinophil-predominant inflammation is allowed to persist, such that patients can present with strictures at the time of diagnosis. Given the imminent risk of food bolus impactions, purely observational studies of untreated EoE patients are rare for reasons related to ethical standards. Therefore, we examined the relationship between the duration of untreated disease, which corresponds to the diagnostic delay (time period from symptom onset to EoE diagnosis), and the appearance of endoscopic alterations at time of EoE diagnosis.
We aimed to examine whether the length of diagnostic delay positively correlates with the frequency of encountering strictures in EoE patients at the time of diagnosis. An additional aim of the present study was to determine the kinetics of the appearance of inflammatory and fibrotic endoscopic features over time. We also aimed to identify risk factors for stricture development.
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