Eosinophilic Esophagitis in Adults

Background

• Eosinophilic esophagitis (EoE) is a chronic immune/antigen-mediated disease characterized by clinical symptoms of esophageal dysfunction and eosinophil-predominant inflammation.
• Proton pump inhibitor-responsive eosinophilic esophagitis (PPI-REE) shares genetic, histologic, and clinical features of eosinophilic esophagitis, and is now considered part of eosinophilic esophagitis disease spectrum, rather than a differential diagnosis.
• Gastroesophageal reflux disorder (GERD) may be associated with mucosal eosinophilia, and is related to acid-mediated mechanisms. This is considered proton pump inhibitor-responsive mucosal eosinophilia.
• EoE may be seen in all age groups. It is more commonly seen in men than women and in White individuals than from other ethnic groups.
• Risk factors for EoE include:
  • anaphylactic food allergy
  • eczema
  • rhinitis
  • asthma
  • dermatitis
  • allergic wasting syndrome
  • personal history of atopy
  • family history of EoE
  • PTEN hamartoma tumor syndromes (PHTS)
  • autosomal dominant hyperimmunoglobulin E (hyper-IgE) syndrome
• About 1% of EoE is associated with connective tissue hypermobility syndromes such as Loeys-Dietz syndrome, Marfan syndrome type II, and Ehlers-Danlos syndrome.
• Patients may report gastroesophageal reflux symptoms that are refractory to medical management.

Evaluation

• Chronic or relapsing symptoms of dysphagia, food impaction, and heartburn are the most common symptoms of eosinophilic esophagitis (EoE) on presentation.
• EoE is usually diagnosed by endoscopic evaluation of digestive symptoms.
• Diagnostic criteria for eosinophilic esophagitis requires the combination of (Strong recommendation):
  • symptoms of esophageal dysfunction such as dysphagia, food impaction, heartburn, upper abdominal pain, chest pain
  • mucosal eosinophilia (on esophageal biopsy, peak value ≥ 15 eosinophils/high power field)
    • eosinophilia is isolated to the esophagus
    • upper gastrointestinal contrast study may reveal esophageal strictures difficult to discern by endoscopy and may be useful in patients with dysphagia to assess for stricture
• EoE endoscopic findings may be normal or may have typical findings which include "EREFS":
  • Edema of the mucosa
  • esophageal Rings
  • eosinophilic Exudates as white specks
  • linear Furrows
• The EoE endoscopic reference score (EREFS) may help diagnose and evaluate treatment response of patients with eosinophilic esophagitis.
• Exclude other conditions associated with esophageal eosinophilia, such as:
  • gastroesophageal reflux disease (GERD) - intraesophageal pH monitoring may be useful for excluding or for determining the contribution of gastroesophageal reflux if the diagnosis of EoE is not apparent after endoscopy and biopsy
  • achalasia
  • allergic vasculitis
  • connective tissue disease (collagen vascular disease)
  • celiac disease
  • Crohn disease of the esophagus
  • drug hypersensitivity response
  • esophageal leiomyomatosis
  • eosinophilic gastroenteritis
  • graft-versus-host disease (GVHD)
  • hypereosinophilic syndrome (HES)
  • infectious esophagitis (such as herpes, Candida esophagitis)
  • parasitic infection
  • bullous pemphigoid

Management

• Complete symptom resolution may not be achieved and symptom reduction is often goal of therapy.
• Offer any of the following treatments as initial therapy.
  • Proton pump inhibitor 1 mg/kg orally twice daily for > 8-12 weeks (Strong recommendation).
  • A 6-food elimination diet (milk, wheat, soy, egg, nuts, seafood) or an elemental diet may induce histologic remission in patients with eosinophilic esophagitis. An elimination diet may be:
    • initial therapy in children and adults for treatment of EoE (Weak recommendation)
    • assessed by clinical response to dietary treatment (removal or reintroduction of food antigens), and endoscopic examination and esophageal biopsy (Weak recommendation)
  • Swallowed topical steroids with either fluticasone or budesonide (Strong recommendation).
    • budesonide 2 mg/day, usually in divided dose of 1 mg/2mL budesonide mixed with 5 g sucralose orally
    • fluticasone 880-1,760 mcg/day orally in 2-4 divided daily doses for 6-8 weeks
• Systemic corticosteroids are not recommended but have been used for rapid improvement in EoE symptoms and inflammatory eosinophilia.
• Esophageal dilation therapy benefits patients symptomatic with dysphagia and should be considered in patients who have failed first-line therapies (Strong recommendation).
• A team approach to therapy may include a gastroenterologist, a nutritionist, and an allergist.