Diagnosis and Management of Esophageal Eosinophilia and Eosinophilic Esophagitis

Publication Date: May 1, 2013
Last Updated: March 14, 2022

Recommendations

DIAGNOSIS

Definition and causes of esophageal eosinophilia

Esophageal eosinophilia, the finding of eosinophils in the squamous epithelium of the esophagus, is abnormal and the underlying cause should be identified. (Strong  “We recommend”, Moderate)
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Definition of eosinophilic esophagitis (EoE) and diagnostic criteria

EoE is clinicopathologic disorder diagnosed by clinicians taking into consideration both clinical and pathologic information without either of these
parameters interpreted in isolation, and defined by the following criteria:
  • Symptoms related to esophageal dysfunction
  • Eosinophil-predominant inflammation on esophageal biopsy, characteristically consisting of a peak value of ≥15 eosinophils per high-power field (eos/hpf)
  • Mucosal eosinophilia is isolated to the esophagus and persists after a PPI trial
  • Secondary causes of esophageal eosinophilia excluded
  • A response to treatment (dietary elimination; topical corticosteroids) supports, but is not required for, diagnosis.
(Strong  “We recommend”, Low)
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Esophageal biopsies are required to diagnose EoE. 2 – 4 biopsies should be obtained from both the proximal and distal esophagus to maximize the likelihood of detecting esophageal eosinophilia in all patients in whom EoE is being considered. (Strong  “We recommend”, Low)
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At the time of initial diagnosis, biopsies should be obtained from the antrum and/or duodenum to rule out other causes of esophageal eosinophilia in all children and in adults with gastric or small intestinal symptoms or endoscopic abnormalities. (Strong  “We recommend”, Low)
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Diagnostic challenges: PPI-responsive esophageal eosinophilia and GERD

Proton-pump inhibitor esophageal eosinophilia (PPI-REE) should be diagnosed when patients have esophageal symptoms and histologic findings of esophageal eosinophilia but demonstrate symptomatic and histologic response to proton-pump inhibition. At this time, the entity is considered distinct from EoE, but not necessarily a manifestation of GERD. (Conditional (weak)  “We suggest”, Low)
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To exclude PPI-REE, patients with suspected EoE should be given a 2-month course of a PPI followed by endoscopy with biopsies. (Strong  “We recommend”, Low)
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A clinical, endoscopic and/or histologic response to a PPI does not establish gastroesophageal reflux as the cause of esophageal eosinophilia. To determine whether reflux is contributing to esophageal eosinophilia, additional evaluation for GERD, as per standard clinical practice, is recommended. This may include ambulatory pH testing in selected cases. (Conditional (weak)  “We suggest”, Low)
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TREATMENT

Endpoints of treatment in EoE

The endpoints of therapy of EoE include improvements in clinical symptoms and esophageal eosinophilic inflammation. While complete resolution of symptoms and pathology is an ideal endpoint, acceptance of a range of reductions in symptoms and histology is a more realistic and practical goal in clinical practice. (Conditional (weak)  “We suggest”, Low)
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Symptoms are an important parameter of response in EoE, but cannot be used alone as a reliable determinant of disease activity and response to therapy, given that compensatory dietary and lifestyle factors can mask symptoms. (Conditional (weak)  “We suggest”, Moderate)
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Pharmacologic treatments

Topical steroids (i.e., fluticasone or budesonide, swallowed rather than inhaled, for an initial duration of 8 weeks) are a first-line pharmacologic therapy for treatment of EoE. (Strong  “We recommend”, High)
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Prednisone may be useful to treat EoE if topical steroids are not effective or in patients who require rapid improvement in symptoms. (Conditional (weak)  “We suggest”, Low)
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Patients without symptomatic and histologic improvement after topical steroids might benefit from a longer course of topical steroids, higher doses of topical steroids, systemic steroids, elimination diet, or esophageal dilation. (Conditional (weak)  “We suggest”, Low)
There are few data to support the use of mast cell stabilizers or leukotriene inhibitors, and biologic therapies remain experimental at this time.
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Dietary treatments

Dietary elimination can be considered as an initial therapy in the treatment of EoE in both children and adults. (Strong  “We recommend”, Moderate)
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The decision to use a specific dietary approach (elemental, empiric, or targeted elimination diet) should be tailored to individual patient needs and available resources. (Conditional (weak)  “We suggest”, Moderate)
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Clinical improvement and endoscopy with esophageal biopsy should be used to assess response to dietary treatment when food antigens are either being withdrawn from or reintroduced to the patient. (Conditional (weak)  “We suggest”, Low)
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Gastroenterologists should consider consultation with an allergist to identify and treat extraesophageal atopic conditions, assist with treatment of EoE, and to help guide elemental and elimination diets. (Conditional (weak)  “We suggest”, Low)
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Endoscopic treatment

Esophageal dilation, approached conservatively, may be used as an effective therapy in symptomatic patients with strictures that persist in spite of medical or dietary therapy. (Conditional (weak)  “We suggest”, Moderate)
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Patients should be well informed of the risks of esophageal dilation in EoE including post-dilation chest pain, which occurs in up to 75 % of patients, bleeding, and esophageal perforation. (Conditional (weak)  “We suggest”, Moderate)
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OUTCOMES

Natural history of EoE

While knowledge of the natural history of EoE is limited, patients should be counseled about the high likelihood of symptom recurrence after discontinuing treatment due to the chronic nature of this disease. (Strong  “We recommend”, Moderate)
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Maintenance therapy

The overall goal of maintenance therapy is to minimize symptoms and prevent complications of EoE, preserve quality of life, with minimal long-term adverse effects of treatments. (Conditional (weak)  “We suggest”, Low)
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Maintenance therapy with topical steroids and / or dietary restriction should be considered for all patients, but particularly in those with severe dysphagia or food impaction, high-grade esophageal stricture and rapid symptomatic/histologic relapse following initial therapy. (Conditional (weak)  “We suggest”, Low)
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Recommendation Grading

Overview

Title

Diagnosis and Management of Esophageal Eosinophilia and Eosinophilic Esophagitis

Authoring Organization

Publication Month/Year

May 1, 2013

Last Updated Month/Year

January 9, 2024

Supplemental Implementation Tools

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

In this evidence-based review, recommendations developed by adult and pediatric gastroenterologists are provided for the evaluation and management of Esophageal eosinophilia and eosinophilic esophagitis patients

Target Patient Population

Patients with esophageal eosinophilia and eosinophilic esophagitis patients

Inclusion Criteria

Male, Female, Adolescent, Adult, Child, Older adult

Health Care Settings

Ambulatory, Hospital, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Assessment and screening, Treatment, Management

Diseases/Conditions (MeSH)

D004802 - Eosinophilia, D004941 - Esophagitis, D057765 - Eosinophilic Esophagitis

Keywords

eosinophilic esophagitis, eosinophilic gastroenteritis

Source Citation

American Journal of Gastroenterology: May 2013 - Volume 108 - Issue 5 - p 679-692
doi: 10.1038/ajg.2013.71

Supplemental Methodology Resources

Data Supplement