What is the best approach to manage eosinophilic esophagitis?

Management of Eosinophilic Esophagitis: From Diagnosis to Treatment

Topical corticosteroids are the first-line pharmacological treatment for eosinophilic esophagitis (EoE), with high-quality evidence supporting their effectiveness in achieving histological remission and improving symptoms. 1

Diagnosis

Clinical Presentation

• Adults and adolescents: Dysphagia, food impaction
• Children: Failure to thrive, feeding problems, vomiting, heartburn, abdominal discomfort

Diagnostic Criteria

• Symptoms of esophageal dysfunction
• ≥15 eosinophils per high-power field (eos/hpf) on esophageal biopsy
• Exclusion of other causes of esophageal eosinophilia

Endoscopic Findings

• Rings
• Linear furrows
• White plaques/exudates
• Edema or decreased vascularity
• Strictures or luminal narrowing

Treatment Algorithm

First-Line Treatments

1. Topical Corticosteroids

   • High-quality evidence supports their effectiveness in inducing histological remission 1
   • Leads to 63% higher histological improvement compared to placebo 2
   • Recommended for both induction and maintenance therapy 1
   • Monitor for candida infection, which can be managed with topical antifungals while continuing steroids 1

2. Proton Pump Inhibitors (PPIs)

   • Consider as initial therapy or in combination with other treatments
   • If PPI causes unwanted side effects (diarrhea, GI infections, magnesium deficiency), switch to diet or topical steroids 1
   • Very low certainty evidence for effectiveness 1

3. Dietary Therapy

   • Options include:
     • Elemental diet (moderate certainty evidence) 1
     • Empiric elimination diets (2-food, 4-food, or 6-food elimination) 1, 3
     • Allergy testing-directed elimination (less effective, especially in adults) 3
   • Step-up approach recommended: start with less restrictive diets (e.g., milk elimination or 2-food elimination) before progressing to more restrictive options 3
   • Discuss psychological impact of dietary therapy with patients 1

Management of Complications

1. Strictures and Narrow Esophagus

   • Endoscopists often underestimate frequency of strictures 1
   • For patients with dysphagia from strictures:
     • Endoscopic dilation is effective and safe using either balloon or bougie dilators 1
     • Best outcomes achieved when dilation is combined with anti-inflammatory therapy 1
     • Note: Dilation does not address underlying inflammation 1

2. Esophageal Perforation

   • EoE is the most common cause of spontaneous esophageal perforation 1
   • If perforation occurs:
     • Perform CT contrast study to assess extravasation 1
     • For limited extravasation, manage conservatively with multidisciplinary input 1

Refractory Disease

• For patients with refractory disease or significant concomitant atopic disease:

  • Joint management by gastroenterologist and allergist 1
  • Consider repeating endoscopy if symptoms recur while on treatment 1

• Not recommended based on current evidence:

  • Immunomodulators (azathioprine, 6-mercaptopurine) 1
  • Anti-TNF therapies 1
  • Sodium cromoglycate, montelukast, antihistamines 1

Emerging Therapies

• Novel biologics (anti-IL-5, anti-IL-13, anti-IL-4 receptor α) show promise but currently recommended only in clinical trials 1, 4
• Biologics result in better histological improvement (55% higher) compared to placebo 2

Monitoring and Follow-up

• Repeat endoscopy to assess treatment response
• For maintenance therapy, continue topical steroids after histological remission is achieved 1
• Monitor for potential side effects:
  • Candida infection (treat with antifungals while continuing steroids) 1
  • In children and adolescents: monitor bone mineral density and adrenal suppression 1

Important Considerations

• EoE is a chronic disease requiring long-term management 2
• Treatment goals include:
  • Symptom improvement
  • Resolution of esophageal eosinophilia
  • Endoscopic improvement
  • Prevention of disease progression and complications 2
• Untreated inflammation can lead to esophageal remodeling and stricture formation 4
• Anxiety and depression in EoE patients due to persistent symptoms and social restrictions can be alleviated with effective therapy 1

Treatment Selection Pitfalls

1. Relying solely on symptoms: Symptoms may not correlate with histological activity
2. Underestimating strictures: Endoscopists often underestimate the frequency of strictures 1
3. Discontinuing treatment prematurely: High relapse rate after withdrawal of topical steroid treatment 1
4. Overreliance on allergy testing: Allergy testing-directed food restrictions are less effective, especially in adults 3
5. Treating with dilation alone: Dilation addresses mechanical obstruction but not underlying inflammation 1

By following this evidence-based approach to EoE management, clinicians can effectively control symptoms, reduce inflammation, and prevent disease progression and complications.