Eosinophilic esophagitis (EoE) is a chronic autoimmune and digestive disorder in which large numbers of eosinophils, a type of white blood cell, are present in the esophagus. Eosinophils play a role in immune system regulation and fighting infections. Their accumulation is a hallmark of allergic diseases. 

EoE was once thought to be a component of gastroesophageal reflux disease (GERD). However, it is now known to be a separate entity as more is understood about the esophagus being an active immunogenic organ. 

EoE may also be called allergic esophagitis.

 

Marc Rothenberg, MD, of the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR), and Professor of Medicine at Cincinnati Children’s Hospital, gives an overview of eosinophilic esophagitis (EoE).

 

Etiology

The exact cause of EoE is unknown. There is a strong correlation between patients who develop EoE and those who report a history of allergic diseases such as seasonal allergies, asthma, and eczema. The accumulation of eosinophils that characterize the disease may be due to immune responses to environmental factors or food allergies. Most patients with EoE have higher expression of the eotaxin-3 gene. Contributions from the CAPN14 and TSLP genes have also been identified.

 

Signs and Symptoms

Clinically, EoE presents with various esophageal dysfunction. Pathologically, there is mucosal inflammation predominantly with eosinophils, which is confined to the esophagus only.  Common symptoms of EoE include:

  • Difficulty swallowing
  • Food getting stuck in throat
  • Nausea
  • Vomiting
  • Poor growth
  • Weight loss
  • Stomach or chest pain
  • Poor appetite
  • Malnutrition

 

Evan S. Dellon, MD, MPH, Gastroenterologist, and Professor of Medicine and Adjunct Professor of Epidemiology at the University of North Carolina Chapel Hill, gives an overview of the symptoms of eosinophilic esophagitis (EoE).

 

 

Diagnosis

EoE is diagnosed following an endoscopy procedure and biopsy to check the tissue for eosinophils. Diseases which can cause eosinophilia should be ruled out before diagnosing a patient with EoE.

 

 

Management Strategies and Treatment

The most common management strategy is change in diet to avoid foods that commonly cause allergic reactions such as milk, nuts, soy, wheat, eggs, and fish (six-food elimination diet). Steroids and proton pump inhibitor therapies are also commonly prescribed to reduce inflammation. However, none of these treatments address the underlying cause of EoE.

 

Marc Rothenberg, MD, of the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR), and Professor of Medicine at Cincinnati Children’s Hospital, gives an overview of the recent study showing removal of milk from the diet is as effective as stricter elimination diets for patients with eosinophilic esophagitis (EoE).

 

The following therapies have been approved by the U.S. Food and Drug Administration (FDA):

  • Dupilumab (Brand name: Dupixent)
  • Budesonide oral suspension (Brand name: Eohilia)

 

 

Clinical Trials and Studies

For a full list of clinical trials relating to eosinophilic esophagitis, click here.

 

 

Resources

Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR)

EOSNetwork

American Partnership for Eosinophilic Disorders

 

References

Roussel JM, Pandit S. Eosinophilic esophagitis. [Updated 2023]. In: StatPearls [Internet]. Available at https://www.ncbi.nlm.nih.gov/books/NBK459297/

Franciosi JP et al. Medical treatment of eosinophilic esophagitis. Cochrane Database Syst Rev. 2023;7:CD004065. doi: 10.1002/14651858.CD004065.pub4.

 

 

For more information on eosinophilic esophagitis and other rare autoimmune disorders, visit https://checkrare.com/diseases/autoimmune-auto-inflammatory-disorders/