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Eosinophilic esophagitis

Background

Overview

Definition
EoE is chronic, immune/antigen-mediated esophageal disease characterized by eosinophilic infiltration of the esophageal epithelium.
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Pathophysiology
In patients with EoE, environmental exposures to food antigens and aeroallergens lead to recruitment of eosinophils to the esophagus. Genetic pleomorphisms (encoding thymic stromal lymphopoietin, eotaxin-3, and calpain-14 genes) are associated with an increased risk.
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Epidemiology
The incidence of EoE is estimated at 0.35-12.8 cases per 100,000 person-years, while its prevalence is estimated at 7.3-90.7 persons per 100,000 population.
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Disease course
Eosinophil-mediated inflammation and fibrosis lead to esophageal dysmotility and formation of esophageal strictures that causes clinical manifestations of dysphagia, and food impaction.
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Prognosis and risk of recurrence
In some patients, symptomatic remission occurs spontaneously or following esophageal dilation, without the need for further dietary modification or medical therapy; however, in most patients, the symptoms recur after discontinuation of treatment.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of eosinophilic esophagitis are prepared by our editorial team based on guidelines from the American College of Gastroenterology (ACG 2025), the American Gastroenterological Association (AGA 2024,2022,2020), the British Society of Gastroenterology (BSG/BSPGHAN 2022), the European Society of Gastrointestinal Endoscopy (ESGE 2021), the European Society of Eosinophilic Oesophagitis (EUREOS/EAACI/UEG/ESPGHAN 2017), the American ...
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Screening and diagnosis

Clinical presentation: as per BSG/BSPGHAN 2022 guidelines, recognize that:
EoE is characterized by symptoms of dysphagia and/or food impaction in adult patients with esophageal histology showing a peak eosinophil count of ≥ 15 eosinophils/hpf, or ≥ 15 eosinophils/0.3 mm² or > 60 eosinophils/mm² in the absence of other causes of esophageal eosinophilia
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EoE responding clinically and histologically to a PPI is the same disease as EoE not responding to a PPI
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EoE and GERD are not mutually exclusive and can coexist in the same patient
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EoE is increasing in prevalence in both adults and children
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EoE is more common in males than females and in people of white ethnic origin compared with other ethnic groups
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having an affected first-degree relative increases the risk of EoE
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the incidence rises during adolescence and peaks in early adulthood
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there is seasonal variation in the symptoms of EoE in many patients, which seems to be associated with higher pollen counts
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food bolus obstruction and dysphagia are strongly associated with a diagnosis of EoE in adult patients
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EoE is the most common cause of spontaneous perforation of the esophagus and this can occur at any age.
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  • Diagnostic criteria

  • Differential diagnosis

Classification and risk stratification

Assessment of severity: as per ACG 2025 guidelines, consider using the Index of Severity for EoE to assess the baseline severity of EoE.
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Index of Severity for Eosinophilic Esophagitis (I-SEE)
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When to use
Symptoms
None
Weekly
Daily
Multiple times per day or disrupting social functioning
Food impaction
None
Food impaction with emergency department visit or endoscopy aged ≥ 18 years)
Food impaction with emergency department visit or endoscopy aged < 18 years)
Hospitalization due to eosinophilic esophagitis
No
Yes
Esophageal perforation
No
Yes
Malnutrition
No
Malnutrition with body mass < 5th percentile or decreased growth trajectory
Inflammation
No
Persistent inflammation requiring elemental formula, or systemic corticosteroid, or immunomodulatory treatments including biologics, azathioprine/6-mercaptopurine, or other immune-targeted treatments)
Endoscopic edema, furrows, and/or exudates
None
Localized
Diffuse
Eosinophil count on esophageal biopsy
Endoscopic rings, strictures
None
Present, but endoscope passes easily
Present, but requires dilation or a snug fit when passing a standard endoscope seen with a 10-14 mm esophagus; esophageal diameter can be measured by balloon-sizing, dilation effect, barium esophagram)
Cannot pass standard upper endoscope; repeated dilations in an adult aged ≥ 18 years); or any dilation in a child aged < 18 years)
Basal zone hyperplasia or laminate propria fibrosis on histology, or dyskeratotic epithelial cell/surface epithelial alteration if no laminate propria
No
Yes
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Diagnostic investigations

History and physical examination
As per ACG 2025 guidelines:
Elicit a careful history of symptoms of esophageal dysfunction, including dietary avoidance and modification behaviors.
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Consider assessing for features that may increase the risk of EoE, including multiple atopic disease and family history.
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  • Allergy tests

  • Esophageal physiologic testing

Diagnostic procedures

Esophageal biopsy: as per ACG 2025 guidelines, use a systematic endoscopic scoring system, such as the EoE Endoscopic Reference Score, to characterize endoscopic findings of EoE at every endoscopy.
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Eosinophilic Esophagitis Endoscopic Reference Score (EREFS)
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When to use
Edema
Grade 0: Absent distinct vascularity)
Grade 1: Present loss of clarity or absence of vascular markings)
Rings
Grade 0: None
Grade 1: Mild subtle circumferential ridges)
Grade 2: Moderate distinct rings not impairing passage of a standard diagnostic endoscope)
Grade 3: Severe distinct rings not permitting passage of a diagnostic endoscope)
Exudate (with plaques)
Grade 0: None
Grade 1: Mild ≤ 10% esophageal surface area)
Grade 2: Severe > 10% esophageal surface area)
Furrows (vertical lines, longitudinal furrows)
Grade 0: None
Grade 1: Mild without visible depth)
Grade 2: Severe with mucosal depth)
Stricture
Grade 0: Absent
Grade 1: Present
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Medical management

General principles: as per BSG/BSPGHAN 2022 guidelines, attempt early diagnosis and treatment of EoE effectively to prevent long-term complications of fibrosis and strictures requiring subsequent endoscopic intervention.
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  • Swallowed corticosteroids

  • Systemic corticosteroids

  • PPIs

  • Biologic agents

  • Anti-IgE therapy

  • Other pharmacologic therapies

  • Management of anxiety/depression

  • Maintenance therapy (general principles)

  • Maintenance therapy (corticosteroids)

  • Maintenance therapy (PPIs)

Nonpharmacologic interventions

Dietary modifications: as per ACG 2025 guidelines, consider offering an empiric food elimination diet as a treatment for EoE.
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Therapeutic procedures

Esophageal dilation: as per ACG 2025 guidelines, suspect esophageal stricture and narrowing in EoE in patients with dysphagia or dietary avoidance/modification behaviors.
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Specific circumstances

Pediatric patients, diagnosis: as per BSG/BSPGHAN 2022 guidelines, recognize that symptoms of EoE in pediatric patients may be nonspecific and vary with the age of the patient.
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  • Pediatric patients (evaluation)

  • Pediatric patients (management)

  • Patients with esophageal perforation

  • Patients with Candida infection

Patient education

General counseling: as per ACG 2025 guidelines, counsel patients that because EoE is a chronic condition and disease activity almost universally recurs when treatment is stopped.
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Follow-up and surveillance

Indications for referral: as per BSG/BSPGHAN 2022 guidelines, manage patients with EoE refractory to treatment and/or with significant concomitant atopic disease jointly by a gastroenterologist and specialist allergist to optimize treatment.
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  • Assessment of treatment response