(See also Overview of Esophageal and Swallowing Disorders Overview of Esophageal and Swallowing Disorders The swallowing apparatus consists of the pharynx, upper esophageal (cricopharyngeal) sphincter, the body of the esophagus, and the lower esophageal sphincter (LES). The upper third of the esophagus... read more .)
Eosinophilic esophagitis is an increasingly recognized disease that can begin at any time between infancy and young adulthood; it occasionally manifests in older adults. It is more common among males.
The cause of eosinophilic esophagitis is likely an immune response to dietary antigens in patients with genetic susceptibility; environmental allergens may also play a role. Untreated chronic esophageal inflammation ultimately can lead to esophageal narrowing and strictures Obstructive Disorders of the Esophagus Most esophageal obstruction develops slowly and is incomplete when patients first seek care, typically for difficulty swallowing solids. However, sometimes complete esophageal obstruction develops... read more .
Symptoms of Eosinophilic Esophagitis
Infants and children may present with food refusal, vomiting, weight loss, abdominal pain, and/or chest pain.
In adults, esophageal food impaction is sometimes the first manifestation, and most patients have dysphagia Dysphagia Dysphagia is difficulty swallowing. The condition results from impeded transport of liquids, solids, or both from the pharynx to the stomach. Dysphagia should not be confused with globus sensation... read more . Symptoms of gastroesophageal reflux disease Gastroesophageal Reflux Disease (GERD) Incompetence of the lower esophageal sphincter allows reflux of gastric contents into the esophagus, causing burning pain. Prolonged reflux may lead to esophagitis, stricture, and rarely metaplasia... read more (GERD), such as heartburn, may occur.
Patients often also have manifestations of other atopic disorders (eg, asthma, eczema, allergic rhinitis).
Diagnosis of Eosinophilic Esophagitis
Endoscopy with biopsy
Sometimes a barium swallow
The typical patient with eosinophilic esophagitis has dysphagia for solids and a history of atopy. The diagnosis of eosinophilic esophagitis is also considered when reflux symptoms fail to respond to acid-suppression therapy. It should also be considered in adults who present with esophageal food impaction or in adults who have noncardiac chest pain.
Diagnosis requires endoscopy with biopsy showing eosinophilic infiltration (≥ 15 eosinophils/high-powered field). Although visible abnormalities (eg, linear furrows, strictures, stacked circular rings, loss of vascular markings, white exudates) may be apparent on endoscopy, the appearance can be normal, so biopsies are essential. Because GERD can also cause eosinophilic infiltrates, patients who have mainly reflux symptoms should have biopsies; samples from the proximal and middle esophagus should be processed separately from samples from the distal esophagus.
A barium swallow may show stacked circular rings, longitudinal furrows, a narrow-caliber esophagus, or strictures.
Impedance planimetry Impedance Planimetry (See also Manometry.) Impedance planimetry provides real-time assessment of luminal distensibility and geometric changes in response to applied pressure in various parts of the gastrointestinal... read more is occasionally used in patients with significant symptoms to evaluate for subtle strictures.
Testing for food allergies Diagnosis Food allergy is an exaggerated immune response to dietary components, usually proteins. Manifestations vary widely and can include atopic dermatitis, gastrointestinal or respiratory symptoms... read more is often done to identify possible triggers but is of minimal benefit because eosinophilic esophagitis is not thought to be IgE-mediated.
Treatment of Eosinophilic Esophagitis
Proton pump inhibitors
Sometimes esophageal dilation
(See also the American Gastroenterological Association (AGA) and the Joint Task Force on Allergy-Immunology Practice Parameters' (JTF) 2020 clinical guidelines for the management of eosinophilic esophagitis.)
In adults, medical options include proton pump inhibitors (PPIs), topical corticosteroids, and the biologic dupilumab.
In children, PPIs are typically used if dietary changes are ineffective. PPIs are thought to work via the eotaxin-3 pathway.
If PPI therapy fails, topical corticosteroids are often given to treat eosinophilic esophagitis. Patients may use a multi-dose inhaler of fluticasone; they puff the medication into their mouth without inhaling and then swallow it. Alternatively, budesonide 1 to 2 mg oral viscus slurry can be taken 30 minutes after breakfast and 30 minutes after dinner. Fluticasone or budesonide is given for at least 8 weeks to determine efficacy. If the patient achieves remission with either of these therapies, they are often continued indefinitely. Maintenance doses of these medications are not well established.
Dupilumab is a biologic given by subcutaneous injection to patients ≥ 1 year of age who weigh at least 15 kg (33 lb). It is a human monoclonal antibody that is an interleukin (IL)-4 receptor alpha antagonist and inhibits IL-4 and IL-13 signaling. A phase 3 clinical trial showed patients with eosinophilic esophagitis who received weekly injections of dupilumab had improved histologic outcomes and symptoms (1 Treatment references Eosinophilic esophagitis is a chronic immune-mediated disease of the esophagus resulting in eosinophil-predominant inflammation of the esophagus; it can cause reflux-like symptoms, dysphagia... read more ).
Elimination diets can be effective for some patients in the management of eosinophilic esophagitis (2 Treatment references Eosinophilic esophagitis is a chronic immune-mediated disease of the esophagus resulting in eosinophil-predominant inflammation of the esophagus; it can cause reflux-like symptoms, dysphagia... read more ). The elemental diet may be successful in both adults and children but is often not practical in adults.
Patients who have significant strictures may need careful esophageal dilation using a balloon or bougie; multiple, careful, progressive dilations are done to help prevent esophageal perforation.
Injection and infusion therapies that target the eosinophil pathway in the body are being studied for eosinophilic esophagitis.
1. Dellon ES, Rothenberg ME, Collins MH, et al: Dupilumab in Adults and Adolescents with Eosinophilic Esophagitis. N Engl J Med 387(25):2317-2330, 2022. doi: 10.1056/NEJMoa2205982
2. Mayerhofer C, Kavallar AM, Aldrian D, et al: Efficacy of Elimination Diets in Eosinophilic Esophagitis: A Systematic Review and Meta-analysis. Clin Gastroenterol Hepatol 21(9):2197-2210.e3, 2023. doi: 10.1016/j.cgh.2023.01.019
The following English-language resources may be useful. Please note that THE MANUAL is not responsible for the content of these resources.
American College of Gastroenterology: Evidenced Based Approach to the Diagnosis and Management of Esophageal Eosinophilia and Eosinophilic Esophagitis (EoE) (2013)
American Gastroenterological Association and the Joint Task Force on Allergy-Immunology Practice Parameters: Clinical guidelines for the management of eosinophilic esophagitis (2020)