What is the treatment approach for a patient with eosinophilic esophagitis and eosinophilia?

Treatment Approach for Eosinophilic Esophagitis with Eosinophilia

The first-line treatment for eosinophilic esophagitis (EoE) with eosinophilia should be either proton pump inhibitors (PPIs) or topical swallowed corticosteroids, with dietary therapy as an effective alternative option. 1

Initial Treatment Options

Proton Pump Inhibitor (PPI) Therapy

• PPIs are effective in inducing histological and clinical remission in patients with EoE 1
• Recommended dosing:
  • Adults: 20-40 mg twice daily for 8-12 weeks 1, 2
  • Children: 1 mg/kg per dose, twice daily for 8-12 weeks (maximum adult dose) 1
• Approximately 23-42% of patients achieve histological remission with PPI therapy 3, 4
• PPIs should be given for at least 8-12 weeks before assessing histological response 1
• Maintenance PPI therapy is effective in sustaining remission in responders 1

Topical Corticosteroids

• Highly effective for inducing histological and clinical remission (strong recommendation, high-quality evidence) 1
• Options include:
  • Fluticasone: 880 mcg (four puffs) twice daily for adults 5
  • Budesonide: 1 mg twice daily as an orodispersible tablet or aqueous gel 1, 3
• Histological response rates are significantly higher than with placebo (64.9% vs 13.3%) 4
• Clinical and histological relapse is high after withdrawal, so maintenance treatment is recommended 1

Dietary Therapy

• Effective in achieving clinico-histological remission in both adults and children 1
• Three main approaches:
  1. Six-food elimination diet (highest histological remission rates but lower compliance) 1
  2. Four-food or two-food elimination diet (better compliance but lower success rates) 1
  3. Elemental diet (highest efficacy but lowest compliance - reserved for refractory cases) 1
• Support from an experienced dietitian is strongly recommended throughout both elimination and reintroduction phases 1
• Food triggers can only be identified by documenting disease remission after elimination followed by recurrence on reintroduction 1
• Allergy testing (skin prick, specific IgE, patch testing) is not recommended for choosing dietary restrictions 1

Treatment Algorithm

1. Initial Assessment and Diagnosis:

   • Endoscopy with at least 6 biopsies from different anatomical sites
   • Diagnosis: ≥15 eosinophils per 0.3 mm² in any biopsy specimen 1

2. First-line Treatment (choose one):

   • PPI therapy: twice daily for 8-12 weeks
   • Topical corticosteroids: daily for 8-12 weeks
   • Elimination diet with dietitian support 1

3. Assessment of Response:

   • Repeat endoscopy with biopsy while on treatment (symptoms may not correlate with histological activity) 1
   • Remission defined as <15 eosinophils/0.3 mm² 1

4. For Responders:

   • Continue maintenance therapy with the effective treatment 1
   • For PPI responders: consider maintenance therapy at same or reduced dose 1
   • For topical steroid responders: continue maintenance treatment 1
   • For diet responders: structured food reintroduction to identify specific triggers 1, 3

5. For Non-responders:

   • Switch to an alternative first-line treatment 1
   • Consider combining elimination diet with pharmacological treatment in cases of drug treatment failure 1
   • For patients with fibrostenotic disease: consider endoscopic dilation 1

6. Refractory Cases:

   • Patients with refractory disease and/or significant concomitant atopic disease should be jointly managed by a gastroenterologist and specialist allergist 1
   • Consider novel biologics used in other allergic conditions (e.g., dupilumab) 1

Important Considerations and Pitfalls

• Symptom-histology discordance: Symptoms may not always correlate with histological activity, making endoscopic assessment necessary 1
• Medication adherence: Long-term compliance with dietary therapy is challenging, with only 55% maintaining remission at 9 months 3
• Stricture development: Medical treatment with topical steroids likely reduces stricture development 1
• Perforation risk: EoE is the most common cause of spontaneous esophageal perforation 1
• Psychological impact: The psychological impact of dietary therapy should be discussed with patients 1
• Candida infection: May occur in a small proportion of patients on topical steroids 1
• Medication contraindications:
  • Immunomodulators (azathioprine, 6-mercaptopurine) are not recommended 1
  • Anti-TNF and anti-integrin therapies are not recommended 1
  • Sodium cromoglycate, montelukast, and antihistamines are not recommended for EoE management 1

By following this structured approach to treatment, clinicians can effectively manage eosinophilic esophagitis with eosinophilia, improving symptoms, reducing inflammation, and preventing complications such as stricture formation and perforation.