Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

at initial diagnosis

1st line – corticosteroid or proton-pump inhibitor

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ACUTE
|
at initial diagnosis
1st line – 

corticosteroid or proton-pump inhibitor

Pharmacologic therapy can be used as a first-line treatment.[1][3][4][28]​​​[101]​​​​

Current guidelines recommend treating patients with topical corticosteroids or a proton-pump inhibitor (PPI) for 8-12 weeks, and then repeating the esophagogastroduodenoscopy (EGD) and biopsy to assess endoscopic and histologic response.[1][3][4][101]

Budesonide as an orodispersible tablet is specifically approved for use in adults with EoE, although it may not be universally available. Orodispersible budesonide may be beneficial for adolescents but may not be licensed for use in this age group.[4] Patients should not eat or drink for 30 minutes after the dose. Appropriate administration facilitates optimal exposure of the esophageal mucosa to the active substance. One short-term double-blind trial reported histologic remission in >90% of EoE patients randomized to orodispersible budesonide (compared with 0% in placebo recipients).​[103]

One double blind, double-dummy RCT found that swallowed fluticasone (from a multidose inhaler) and oral viscous budesonide significantly decreased esophageal eosinophil counts and improved dysphagia during initial treatment of EoE.[117]​ There was no significant difference in the change in peak eosinophil count from baseline between swallowed fluticasone and oral viscous budesonide; either appears to be is an acceptable treatment for EoE.

Network meta-analysis and retrospective data suggest that treatment with oral viscous budesonide improves endoscopic and histologic outcomes compared with topical/swallowed fluticasone.[114][115] Longer mucosal contact time associated with use of oral viscous budesonide is believed to contribute to improve histologic outcomes compared with topical/swallowed corticosteroids.[83][116]

Oral viscous budesonide is prepared by mixing the budesonide aqueous inhalation solution (nebules) into a slurry with sucralose before being swallowed. Patients should not eat or drink for 30-60 minutes after the dose.

Metered dose inhalers (MDIs; e.g., fluticasone) are puffed into the mouth during end-expiration and swallowed rather than inhaled.

For patients who initially have a good response (i.e., symptom improvement, endoscopic improvement, and histologic improvement with the eosinophil count decreasing at least to <15 eosinophils per high-power microscopy field [<15 eosinophils per 0.3 mm²]), the dose can be halved and a repeat EGD performed to confirm ongoing response.

Local irritation from medication deposition and esophageal candidiasis are commonly reported adverse effects of corticosteroid therapy, but candidiasis resolves on treatment and does not require topical corticosteroids to be stopped.[118]​ These effects are seen in up to 15% to 20% of patients.[28]​​

Systemic corticosteroids are not routinely used in the management of EoE. However, they may be considered in selected patients where rapid relief is required for severe symptoms such as dysphagia (which limits adequate nutrition or hydration), dehydration, or weight loss, or where other treatments have failed.[100] 

The most studied PPI is omeprazole, but the choice of PPI is probably unimportant. Although PPI therapy is not licensed for EoE, current UK guidelines recommend giving omeprazole with a clear explanation of the indication (EoE rather than GERD) given to the primary care team.[4] Histologic remission and symptom response mean that PPI treatment is considered effective in patients with EoE (GRADE of evidence = moderate; level of recommendation = strong; level of agreement = 100%).[4] The response rates reported are 60% to 70% (clinical) and 50% (histologic).​​[120][121]​ Most studies assessed response after 8 weeks' treatment. There are no randomized controlled trials to define the maintenance treatment strategy for PPIs, although PPI maintenance is considered an appropriate long-term treatment for EoE patients in clinical and histologic remission.​​[4] There are no special administration or monitoring requirements for PPIs, and serious adverse events are rare.​

Primary options

budesonide inhaled: (oral viscous budesonide is prepared by mixing budesonide aqueous inhalation solution/nebules into a slurry with sucralose before being swallowed) children: 0.5 mg twice daily; adults: 1 mg twice daily

OR

omeprazole: adults: 20 mg orally twice daily

Secondary options

fluticasone propionate inhaled: (MDI is puffed into the mouth during end-expiration and swallowed rather than inhaled) children: 88-440 micrograms two to four times daily; adults: 440-880 micrograms twice daily

Consider – endoscopic esophageal dilation

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ACUTE
|
at initial diagnosis
Consider – 

endoscopic esophageal dilation

Treatment recommended for SOME patients in selected patient group

An important adjunct treatment in patients who have signs of esophageal remodeling (e.g., esophageal strictures or narrowing).[28]​​[146]​ It is recommended initially in patients with severely symptomatic esophageal stenosis.[3][4][101]​​​ Patients may have a rapid symptomatic improvement with this procedure.

All three modalities (i.e., wire-guided bougie, nonwire-guided bougie, through-the-scope balloon) have been reported to be safe and effective.​​[4][28]​​

The key principle is to start low and go slow; the endoscopist should carefully gauge the lumen of the esophagus to choose an initial dilator size.

For bougies, a protocol where there is relook endoscopy after each dilator size is passed has been shown to be safe, and given the known mucosal fragility in EoE, this makes sense. If a balloon is used, direct visualization and measurement of the esophageal caliber is possible.

While the extent of dilation effect has not been studied, the result of an adequate dilation is often a 5-10 mm wide rent with a length throughout the narrowed or strictured area. Serial dilation is often required in these patients to achieve a goal diameter of >15 mm.

Up to three-quarters of patients will have chest discomfort for several days post-dilation and an analgesic can be prescribed for this.[3][149]

Risk of esophageal perforation is approximately 0.4% to 0.9% in patients with EoE.[159] There is also a low risk of bleeding.​​[159]​​

1st line – dietary elimination therapy

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ACUTE
|
at initial diagnosis
1st line – 

dietary elimination therapy

Dietary therapy can be used as a first-line treatment.[1][3][28]​​

Patients remain on the diet for 8-12 weeks and an esophagogastroduodenoscopy (EGD) is then performed to assess histologic and endoscopic response.[4]

Elemental formula diets, however, have been shown to have a more rapid treatment effect, and repeat EGD may be performed 4 weeks into treatment.[39][100]

All pediatric and most adult patients require the support of a multidisciplinary team, consisting of a gastroenterologist, an allergist, and a dietitian.[4]​ Education, support, and encouragement are all needed in motivated patients electing this treatment. 

Empiric elimination diet: UK guidelines recommend beginning with a 2-food elimination diet (TFED; eliminating milk plus wheat or egg) before stepping up to the 4-food elimination diet (FFED; eliminating milk, wheat, egg, and soya) and finally the 6-food elimination diet (SFED; eliminating milk, wheat, egg, soya, fish/shellfish, and tree nuts/peanuts) if remission is not achieved.[4] Each exclusion diet should be adhered to for at least 8-12 weeks and assessed endoscopically and histologically, with endoscopy being repeated after the reintroduction of individual foods.​[4]

Elemental formula diet: elemental formulas are hypoallergenic and contain only amino acids, simple carbohydrates, and medium-chain triglycerides. Response rate in children is from 90% to 95%.[31][39][136][143]​​​ Use is typically limited to infants, patients with severe disease complicated by malnutrition, and patients who are refractory to all other treatments. UK guidelines only recommend elemental diets for selected patients with disease refractory to conventional treatments and after careful consideration by a multidisciplinary team.[4]

Dietary changes should be maintained once symptoms resolve as there is a high rate of recurrence when treatment is stopped.[3]

Consider – endoscopic esophageal dilation

Back
ACUTE
|
at initial diagnosis
Consider – 

endoscopic esophageal dilation

Treatment recommended for SOME patients in selected patient group

An important adjunct treatment in patients who have signs of esophageal remodeling (e.g., esophageal strictures or narrowing).[28]​​[146]​ It is recommended initially in patients with severely symptomatic esophageal stenosis.[3][4][101]​​​ Patients may have a rapid symptomatic improvement with this procedure.

All three modalities (i.e., wire-guided bougie, nonwire-guided bougie, through-the-scope balloon) have been reported to be safe and effective.​​[4][28]​​

The key principle is to start low and go slow; the endoscopist should carefully gauge the lumen of the esophagus to choose an initial dilator size.

For bougies, a protocol where there is relook endoscopy after each dilator size is passed has been shown to be safe, and given the known mucosal fragility in EoE, this makes sense. If a balloon is used, direct visualization and measurement of the esophageal caliber is possible.

While the extent of dilation effect has not been studied, the result of an adequate dilation is often a 5-10 mm wide rent with a length throughout the narrowed or strictured area. Serial dilation is often required in these patients to achieve a goal diameter of >15 mm. UK guidelines recommend use of anti-inflammatory medication in combination with endoscopic esophageal dilation.[4]

Up to three-quarters of patients will have chest discomfort for several days post-dilation and an analgesic can be prescribed for this.[3][149]

Risk of esophageal perforation is approximately 0.4% to 0.9% in patients with EoE.[159] There is also a low risk of bleeding.​[159]

ONGOING

response to initial therapy

1st line – maintenance therapy

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ONGOING
|
response to initial therapy
1st line – 

maintenance therapy

When patients achieve a good response to treatment (i.e., resolution of esophageal eosinophilia or a decrease in eosinophils to <15 eosinophils per high-power microscopy field (<15 eosinophils per 0.3 mm²); resolution or improvement in symptoms; normalization or improvement in the endoscopic appearance), most will need ongoing maintenance therapy.[127][130][160]

One phase 3 trial reported a remission rate of 75% after 48 weeks' treatment with orodispersible budesonide, compared with a 4.4% remission rate with placebo.[118]

At a minimum, patients who have had food impactions, fibrostenotic remodeling of the esophagus with strictures or narrowing, or rapidly recurrent symptoms after stopping treatment, should be placed on maintenance therapy. However, guidelines recommend all patients with EoE should be considered for maintenance therapy, given the risk of possible progression to fibrostenosis with ongoing esophageal eosinophilia.[3][28]​​[101]

For patients treated with topical corticosteroids, the lowest dose that continues to provide the best clinical, endoscopic, and histologic response should be used for maintenance therapy.[28]​​

For patients treated with dietary elimination, long-term avoidance of the identified food triggers is recommended.

Primary options

omeprazole: adults: 20 mg orally twice daily

nonresponse or relapse

1st line – increase dose of corticosteroid or proton-pump inhibitor or switch to alternative therapy

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ONGOING
|
nonresponse or relapse
1st line – 

increase dose of corticosteroid or proton-pump inhibitor or switch to alternative therapy

For patients who do not respond or who relapse, there are various options. The corticosteroid or PPI dose can be increased, the corticosteroid can be swapped for a PPI (or vice versa), or combination pharmacologic treatment (corticosteroid plus a PPI) can be considered. Other treatment modalities can be tried, such as dietary elimination (most common), monoclonal antibodies, inclusion in clinical trials, or dilation.

Endoscopic esophageal dilation can be considered in symptomatic patients with strictures that persist in spite of medical or dietary therapy.[3][101]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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