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 – topical corticosteroid

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ACUTE
|
at initial diagnosis
1st line – 

topical corticosteroid

Pharmacological therapy can be used as a first-line treatment.​​​[2][3][27][105]​​​​​​​ Topical corticosteroids (e.g., budesonide, fluticasone) are one suitable option.

There are various topical corticosteroid formulations available, and availability depends on geographic location. Budesonide is commercially available for this indication as an oral suspension, or as an orodispersible tablet. An oral viscous formulation may also be prepared. There is no commercially available formulation of fluticasone for this indication and existing asthma formulations must be used.

Current guidelines recommend treating patients with topical corticosteroids for 8-12 weeks, and then repeating the oesophagogastroduodenoscopy (OGD) and biopsy to assess endoscopic and histological response.​​​[2][3][105]

Budesonide as an orodispersible tablet is specifically approved for use in adults with eosinophilic oesophagitis (EoO), although it may not be universally available. The National Institute for Health and Care Excellence (NICE) in the UK recommends orodispersible budesonide for inducing remission of eosinophilic oesophagitis in adults.[119]​ Orodispersible budesonide may be beneficial for adolescents but may not be licensed for use in this age group.[2]​ 

Budesonide oral suspension is approved in the US for patients aged ≥11 years with EoO, based on results from clinical trials.[120][121][122]​ Treatment up to 12 weeks is recommended. 

Budesonide has also been used in the oral viscous form.[131][132][133]​​ Oral viscous budesonide is prepared by mixing the budesonide aqueous inhalation solution (nebules) into a slurry with sucralose before being swallowed. This is an option for children who cannot receive the commercially available formulations.

Topical administration of corticosteroids may also be accomplished by modifying existing asthma formulations. 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 histological 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 OGD performed to confirm ongoing response.

Local irritation from drug deposition and oesophageal candidiasis are commonly reported adverse effects of topical corticosteroid therapy, but candidiasis resolves on treatment and does not require topical corticosteroids to be stopped.​ These effects are seen in up to 15% to 20% of patients.[27]​​

Appropriate administration facilitates optimal exposure of the oesophageal mucosa to the active substance. Patients should not eat or drink for 30-60 minutes after the dose to prevent washing the drugs out of the oesophagus.

Systemic corticosteroids are not routinely used in the management of EoO. However, they may be considered as the initial treatment for oesophageal strictures before oesophageal dilation and 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.[79]​ 

Primary options

budesonide: adults: 1 mg orally (orodispersible tablet) twice daily; place on tip of tongue, gently press against the roof of the mouth, allow to dissolve, and swallow saliva as tablet disintegrates

OR

budesonide: children ≥11 years of age and adults: 2 mg orally (suspension) twice daily for 12 weeks

OR

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

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 oesophageal dilation

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ACUTE
|
at initial diagnosis
Consider – 

endoscopic oesophageal dilation

Additional treatment recommended for SOME patients in selected patient group

An important adjunct treatment in patients who have signs of oesophageal remodelling (e.g., oesophageal strictures or narrowing).[27]​​[150]​ It is recommended initially in patients with severely symptomatic oesophageal stenosis.​​[2][3]​​​​[105]​​​ Patients may have a rapid symptomatic improvement with this procedure.

All three modalities (i.e., wire-guided bougie, non-wire-guided bougie, through-the-scope balloon) have been reported to be safe and effective.​​[2][27]​​

The key principle is to start low and go slow; the endoscopist should carefully gauge the lumen of the oesophagus 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 eosinophilic oesophagitis (EoO), this makes sense. If a balloon is used, direct visualisation and measurement of the oesophageal calibre 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 therapy in combination with endoscopic oesophageal dilation.[2]

Up to three-quarters of patients will have chest discomfort for several days post-dilation and an analgesic can be prescribed for this.​[153] Risk of oesophageal perforation is approximately 0.4% to 0.9% in patients with EoO.[163] There is also a low risk of bleeding.​​​​[163]

1st line – proton-pump inhibitor (PPI)

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ACUTE
|
at initial diagnosis
1st line – 

proton-pump inhibitor (PPI)

Pharmacological therapy can be used as a first-line treatment.​[2][3][27][105]​ PPIs are one suitable option.

Current guidelines recommend treating patients with a PPI for 8-12 weeks, and then repeating the oesophagogastroduodenoscopy and biopsy to assess endoscopic and histological response.​[2][3]​​​[105]

The most studied PPI is omeprazole, but the choice of PPI is probably unimportant. Although PPIs are not licensed for eosinophilic oesophagitis (EoO), current UK guidelines recommend giving omeprazole with a clear explanation of the indication (EoO rather than GORD) given to the primary care team.[2]

There are no special administration or monitoring requirements for PPIs, and serious adverse events are rare.

Primary options

omeprazole: children: 1 mg/kg orally twice daily, maximum 40 mg/day; adults: 20 mg orally twice daily

Consider – endoscopic oesophageal dilation

Back
ACUTE
|
at initial diagnosis
Consider – 

endoscopic oesophageal dilation

Additional treatment recommended for SOME patients in selected patient group

An important adjunct treatment in patients who have signs of oesophageal remodelling (e.g., oesophageal strictures or narrowing).[27][150]​ It is recommended initially in patients with severely symptomatic oesophageal stenosis.​[2][3]​​[105]​ Patients may have a rapid symptomatic improvement with this procedure.

All three modalities (i.e., wire-guided bougie, non-wire-guided bougie, through-the-scope balloon) have been reported to be safe and effective.[2][27]

The key principle is to start low and go slow; the endoscopist should carefully gauge the lumen of the oesophagus 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 eosinophilic oesophagitis (EoO), this makes sense. If a balloon is used, direct visualisation and measurement of the oesophageal calibre 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 therapy in combination with endoscopic oesophageal dilation.[2]

Up to three-quarters of patients will have chest discomfort for several days post-dilation and an analgesic can be prescribed for this.[153]​​​​ Risk of oesophageal perforation is approximately 0.4% to 0.9% in patients with EoO.[163]​ There is also a low risk of bleeding.[163]

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.​[3]​​[27]​​

Patients remain on the diet for 8-12 weeks and an oesophagogastroduodenoscopy (OGD) is then performed to assess histological and endoscopic response.[2]

Elemental formula diets, however, have been shown to have a more rapid treatment effect, and repeat OGD may be performed 4 weeks into treatment.[38][79]​​

The American College of Gastroenterology (ACG) suggests empirical elimination diet for the treatment of eosinophilic oesophagitis (EoO).[3]

All paediatric and most adult patients require the support of a multidisciplinary team, consisting of a gastroenterologist, an allergist, and a dietitian.[2]​ Education, support, and encouragement are all needed in motivated patients electing this treatment. 

Empirical 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.[2] 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.​[2]

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%.[30][38][140][147]​​​ 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.[2]

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

Consider – endoscopic oesophageal dilation

Back
ACUTE
|
at initial diagnosis
Consider – 

endoscopic oesophageal dilation

Additional treatment recommended for SOME patients in selected patient group

An important adjunct treatment in patients who have signs of oesophageal remodelling (e.g., oesophageal strictures or narrowing).[27]​​[150]​ It is recommended initially in patients with severely symptomatic oesophageal stenosis.​​[2][3]​​[105]​​​ Patients may have a rapid symptomatic improvement with this procedure.

All three modalities (i.e., wire-guided bougie, non-wire-guided bougie, through-the-scope balloon) have been reported to be safe and effective.​​[2][27]​​

The key principle is to start low and go slow; the endoscopist should carefully gauge the lumen of the oesophagus 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 eosinophilic oesophagitis (EoO), this makes sense. If a balloon is used, direct visualisation and measurement of the oesophageal calibre 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 therapy in combination with endoscopic oesophageal dilation.[2]

Up to three-quarters of patients will have chest discomfort for several days post-dilation and an analgesic can be prescribed for this.​[153]​ Risk of oesophageal perforation is approximately 0.4% to 0.9% in patients with EoO.[163] There is also a low risk of bleeding.​[163]

1st line – dupilumab

Back
ACUTE
|
at initial diagnosis
1st line – 

dupilumab

Pharmacological therapy can be used as a first-line treatment.​[2][3]​​​[27][105]​ Dupilumab, an interleukin (IL)-4 receptor antagonist monoclonal antibody, is a suitable option in select patients.

Dupilumab is approved by the US Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the treatment of eosinophilic oesophagitis (EoO) in adults and children aged ≥1 year and weighing ≥15 kg body weight.[79][136][137][138]

Early use of dupilumab may be considered for patients with comorbid atopic conditions (e.g., asthma, atopic dermatitis, or chronic rhinosinusitis with nasal polyps), for patients with a strong preference to avoid dietary restriction or topical corticosteroids, or for patients with severe EoO.[139]​ Do not reduce or discontinue concurrent drug treatments for comorbid atopic conditions without consultant guidance.

Endoscopic assessment to monitor treatment response is recommended between 12 and 24 weeks based on clinical trial findings.[3]

Adverse effects of dupilumab treatment include possible injection site reactions and broad cytokine block, which might help with other allergies, but long-term safety is still unclear. Conjunctivitis has been observed with the use of dupilumab in other conditions, although it has not been reported yet in EoO.[139]

Primary options

dupilumab: children ≥1 year of age and 15-29 kg body weight: 200 mg subcutaneously every 2 weeks; children ≥1 year of age and 30-39 kg body weight: 300 mg subcutaneously every 2 weeks; children ≥1 year of age and ≥40 kg body weight and adults: 300 mg subcutaneously once weekly

Consider – endoscopic oesophageal dilation

Back
ACUTE
|
at initial diagnosis
Consider – 

endoscopic oesophageal dilation

Additional treatment recommended for SOME patients in selected patient group

An important adjunct treatment in patients who have signs of oesophageal remodelling (e.g., oesophageal strictures or narrowing).[27][150]​ It is recommended initially in patients with severely symptomatic oesophageal stenosis.​[2][3]​​[105]​ Patients may have a rapid symptomatic improvement with this procedure.

All three modalities (i.e., wire-guided bougie, non-wire-guided bougie, through-the-scope balloon) have been reported to be safe and effective.[2][27]

The key principle is to start low and go slow; the endoscopist should carefully gauge the lumen of the oesophagus 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 eosinophilic oesophagitis (EoO), this makes sense. If a balloon is used, direct visualisation and measurement of the oesophageal calibre 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 therapy in combination with endoscopic oesophageal dilation.[2]

Up to three-quarters of patients will have chest discomfort for several days post-dilation and an analgesic can be prescribed for this.[153]​ Risk of oesophageal perforation is approximately 0.4% to 0.9% in patients with EoO.[163]​ There is also a low risk of bleeding.[163]

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 oesophageal 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; normalisation or improvement in the endoscopic appearance), most will need ongoing maintenance therapy.[128][131][164]

At a minimum, patients who have had food impactions, fibrostenotic remodelling of the oesophagus with strictures or narrowing, or rapidly recurrent symptoms after stopping treatment, should be placed on maintenance therapy. However, guidelines recommend all patients with eosinophilic oesophagitis (EoO) should be considered for maintenance therapy, given the risk of possible progression to fibrostenosis with ongoing oesophageal eosinophilia.[3]​​[27]​​​[105]

For patients treated with topical corticosteroids or proton-pump inhibitors (PPIs), the lowest dose that continues to provide the best clinical, endoscopic, and histological response should be used for maintenance therapy.[27]​​ There are no randomised controlled trials to define the maintenance treatment strategy for PPIs, although PPI maintenance is considered an appropriate long-term treatment for patients with EoO in clinical and histological remission.​​[2]

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

For patients treated with dupilumab, it should be noted that although treatment has demonstrated efficacy for up to 1 year, the optimal long-term management strategy remains to be determined, specifically regarding whether treatment frequency can be safely reduced or if concomitant EoO therapies can be withdrawn.[139]

non-response or relapse

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

Back
ONGOING
|
non-response or relapse
1st line – 

increase dose of corticosteroid or proton-pump inhibitor (PPI) 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 pharmacological treatment (corticosteroid plus a PPI) can be considered. Other treatment modalities can be tried, such as dietary elimination, dupilumab, inclusion in clinical trials, or dilation.

Dupilumab may be considered for patients who are refractory to topical corticosteroids, PPIs, or dietary elimination therapy, which may be due to continued symptoms, persistent abnormal oesophageal inflammation, adverse effects, intolerance, or inability to adhere.[139]​ The American College of Gastroenterology (ACG) suggests dupilumab as a second-line or add-on therapy for patients with EoO who do not improve with PPI therapy.[3]

Endoscopic oesophageal dilation can be considered in symptomatic patients with strictures that persist in spite of medical or dietary therapy.[3]​​[105]

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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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