What is the management approach for eosinophilic esophagitis?

Management of Eosinophilic Esophagitis

Topical corticosteroids are the recommended first-line therapy for eosinophilic esophagitis, with treatment duration of 8-12 weeks before evaluating histological response. 1

Initial Treatment Algorithm

First-Line Options (Choose One)

Topical Corticosteroids (Preferred)

• Topical corticosteroids effectively reduce esophageal eosinophil counts to <15 per high-power field over 4-12 weeks with moderate certainty of evidence 1
• These agents have no documented systemic side effects during long-term treatment, though monitoring of bone mineral density and adrenal suppression is recommended in children and adolescents 1
• Candida infection may occur in a small proportion of patients and should be managed with topical antifungals while continuing topical steroids 1

Proton Pump Inhibitors (Alternative)

• PPIs should be started with twice-daily dosing for 8-12 weeks before assessing histological response 2
• Double doses of PPI induce remission in approximately half of EoE patients, irrespective of the drug used or patient age 3
• The anti-inflammatory effects of PPIs in EoE are independent from gastric acid secretion inhibition 3
• PPIs are commonly prescribed first-line due to their accessibility, low cost, and safety profile 3

Empiric Elimination Diets (Alternative)

• Consider elimination diets after inadequate response to medication or as an alternative first-line approach 2
• A step-up approach starting with 2-food elimination diet (milk +/- wheat or egg) is recommended over more restrictive diets initially 1, 4
• This approach reduces the need for endoscopic procedures, shortens diagnostic processing time, and avoids unnecessary restrictions 3, 4
• Dietary elimination must only be conducted under supervision of an experienced dietitian due to risk of nutritional deficiencies and potential development of de novo IgE-mediated food allergy upon reintroduction 1

Dietary Therapy Hierarchy (If Chosen)

Step-Up Strategy (Recommended Approach)

1. Two-food elimination diet (TFED): Remove milk +/- wheat or egg for 8-12 weeks 1, 4
2. Four-food elimination diet: If TFED fails, escalate to removing milk, wheat, egg, and soy/legumes 1, 4
3. Six-food elimination diet: Remove milk, wheat, egg, soy, nuts, and seafood if 4-food diet fails 1
4. Elemental diet: Reserved for patients refractory to other treatments due to high efficacy but impracticality 2, 3

Important Caveat: Allergy testing-directed elimination has very low certainty evidence with higher failure rates compared to empiric elimination and should not be used 1, 4

Maintenance Therapy

For patients achieving remission with any initial therapy, continuation of maintenance therapy is mandatory rather than discontinuation to prevent recurrent dysphagia, food impaction, and esophageal stricture formation 1

• Continue topical corticosteroids, possibly at lower maintenance dose, for patients who responded to this approach 2
• Continue PPI therapy, possibly at reduced dose, for patients who achieved initial response 2
• Continue dietary elimination for patients who responded to this approach 2
• Medical treatment with topical steroids likely reduces stricture development with moderate evidence 1

Management of Fibrostenotic Disease

In adult patients with dysphagia from EoE-associated strictures, endoscopic dilation in addition to anti-inflammatory therapy is required 1

• Endoscopic dilation is safe and effective for improving symptoms with high certainty evidence for safety and moderate for efficacy 1
• The pooled perforation rate is approximately 0.4%, with most complications occurring before 2009 2
• Critical pitfall: Dilation does not address esophageal inflammation and must be combined with effective anti-inflammatory therapy for optimal outcomes 1
• Best outcomes are achieved when endoscopic dilation is combined with topical steroids 2

Monitoring Requirements

Endoscopy with biopsy is required while on treatment to evaluate histological response, as symptoms do not always correlate with histological activity 1

• Treatment duration should be at least 8-12 weeks before evaluating histological response 1, 5
• Endoscopic and histological assessment should be performed between 8-12 weeks after each dietary change 5
• If symptoms recur during treatment, repeat endoscopy for evaluation and obtain more histology 2
• Inflammatory rather than stricturing EoE phenotype and treatment duration up to 12 weeks increase chances of achieving remission 3

Refractory Disease

Patients with EoE refractory to treatment and/or significant concomitant atopic disease should be jointly managed by a gastroenterologist and specialist allergist 1, 2

• Novel biologics (dupilumab, cendakimab, benralizumab) show promise but are currently recommended only for patients with coexisting allergic diseases 2
• Anti-IL-13 monoclonal antibody cendakimab has demonstrated significant reduction in eosinophil count and dysphagia scores in phase 2 trials 2

Psychosocial Considerations

The psychological impact of dietary therapy should be discussed with patients, including anxiety and depression related to persistent symptoms and social restrictions, which are alleviated by effective therapy 1, 5