Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence.
INTRODUCTION — Esophageal eosinophilia has been described in association with eosinophilic gastroenteritis, an uncommon condition that can cause a range of symptoms, including malabsorption, dysmotility, and ascites, depending upon the layer of the intestinal tract that is involved [1,2]. When the gastrointestinal eosinophilia is limited to the esophagus and is accompanied by characteristic symptoms, it is termed eosinophilic esophagitis. Eosinophilic esophagitis is an increasingly recognized cause of dysphagia and possibly heartburn that is unresponsive to antireflux measures.
The management of eosinophilic esophagitis includes dietary, pharmacologic, and endoscopic interventions . The approach to patients with eosinophilic esophagitis is based primarily on clinical experience, case series, and small controlled trials [4-6]. In addition, it is uncertain whether treatment of symptoms alone is sufficient or if resolution of the eosinophilic inflammation is required.
Commonly used treatments include:
●Elimination and elemental diets to decrease allergen exposure
●Acid suppression to treat gastroesophageal reflux disease, which may mimic or contribute to eosinophilic esophagitis
●Topical glucocorticoids to decrease esophageal inflammation
●Esophageal dilation to treat strictures
Other treatments that have been studied include systemic glucocorticoids, antihistamines, immunosuppressants, and immunomodulators.
This topic will review the treatment of eosinophilic esophagitis. The approaches outlined are consistent with a 2011 consensus statement and the American College of Gastroenterology clinical guidelines [7,8]. Other guidelines have also been published . The pathogenesis, clinical manifestations, and diagnosis of eosinophilic esophagitis in adults and children are discussed separately. (See "Eosinophilic esophagitis (EoE): Genetics and immunopathogenesis" and "Clinical manifestations and diagnosis of eosinophilic esophagitis".)
NATURAL HISTORY — While there are limited data regarding the natural history of eosinophilic esophagitis, patients should be counseled that the disease is chronic and that there is a high likelihood of symptom recurrence after discontinuing treatment . The available data suggest that dysphagia persists in patients who do not receive treatment. In one study of 30 untreated patients followed for an average of 7.2 years, dysphagia persisted in 29 (97 percent) . During follow-up, symptoms increased in 23 percent, were stable in 37 percent, and decreased in 37 percent. Attacks of dysphagia occurred more frequently in patients with blood eosinophilia or with pronounced findings on endoscopy. All patients maintained adequate caloric intake and body weight. Eleven patients were treated with esophageal dilation (seven had a single dilation and four had repeat dilations), which was at least partially successful in 10 (at least a 50 percent reduction in dysphagia).
DIETARY THERAPY — Dietary therapy is an effective first-line treatment for eosinophilic esophagitis in children and adults [11-19]. Dietary therapy is based upon the observation that patients with eosinophilic esophagitis have high rates of food allergies, and that those allergies may contribute to the development of eosinophilic esophagitis. (See "Eosinophilic esophagitis (EoE): Genetics and immunopathogenesis", section on 'Role of the immune system and environmental factors'.)
The appeal of the dietary approach is that it potentially offers an effective non-pharmacologic treatment. On the other hand, allergen avoidance with elimination and elemental diets poses a risk of nutritional deprivation, can be difficult for patients and families (particularly if nasogastric feedings are required), can lead to psychological problems, and may lead to unnecessary food aversion [20,21]. In addition, relapse upon discontinuation of the diet is common . When used, elemental and elimination diets should be administered under the supervision of a registered dietician .
We refer both adults and children to an allergist with expertise in the evaluation of food allergies to guide dietary therapy, assist in the treatment of eosinophilic esophagitis, and identify and treat extraesophageal atopic conditions . We suggest avoidance of known allergens (both food and environmental) for patients in whom specific allergies can be identified after discussion of the pros and cons of the dietary approach. Dietary management of eosinophilic esophagitis is discussed in detail, separately. (See "Diagnostic evaluation of food allergy" and "Dietary management of eosinophilic esophagitis".)
Acid suppression — The relationship between gastroesophageal reflux disease (GERD) and eosinophilic esophagitis is unclear. GERD may be a mimic of eosinophilic esophagitis, coexist with it , or contribute to it. Conversely, eosinophilic esophagitis may contribute to GERD . The diagnosis of eosinophilic esophagitis should generally include demonstration of persistent esophageal eosinophilia after a two-month course of proton pump inhibitor (PPI) (or with a normal pH study) . PPIs may benefit patients with esophageal eosinophilia either by reducing acid production in patients with co-existent GERD, or by other yet undefined anti-inflammatory mechanisms [25,26]. (See "Clinical manifestations and diagnosis of eosinophilic esophagitis", section on 'Distinction from GERD' and "Clinical manifestations and diagnosis of eosinophilic esophagitis", section on 'PPI-responsive esophageal eosinophilia'.)
Acid-suppressing therapy may also be helpful in patients with established eosinophilic esophagitis, since the already inflamed esophagus may be predisposed to injury and more sensitive to physiologic acid exposure [27,28]. Approximately one-third of patients with suspected eosinophilic esophagitis have a good clinical and histologic response to PPIs alone, suggesting that GERD, or a PPI-responsive form of esophageal eosinophilia, may be responsible [29-31]. In a randomized trial, 42 patients with newly diagnosed eosinophilic esophagitis were randomly assigned to treatment with aerosolized swallowed fluticasone (440 mcg twice daily) or esomeprazole (40 mg daily) for eight weeks followed by an upper endoscopy with biopsies . In patients without coexisting GERD, there was no significant difference in resolution of esophageal eosinophilia between the esomeprazole and fluticasone treatment arms (18 versus 24 percent). In contrast, among patients with GERD, those treated with esomeprazole were significantly more likely to have resolution of esophageal eosinophilia as compared with fluticasone (100 versus 0 percent). However, regardless of the presence of GERD, treatment with esomeprazole, but not fluticasone, was associated with a significant improvement in symptoms of dysphagia. (See "Clinical manifestations and diagnosis of eosinophilic esophagitis", section on 'PPI-responsive esophageal eosinophilia'.)
Approximately two-thirds of patients with a PPI-responsive esophageal eosinophilia remain in remission on PPIs. In a retrospective cohort study of 75 patients with PPI-responsive esophageal eosinophilia, during a mean follow-up of 26 months, 55 patients (73 percent) had a sustained histologic remission on low-dose PPI therapy . CYP2C19 rapid metabolizer genotype and rhinoconjunctivitis, possibly due to exposure to airborne allergens, were independent predictors of a loss of response to PPI therapy (odds ratio [OR] 12.5 and 8.6, respectively). Eosinophilia was limited to the distal esophagus in 14 of 20 (70 percent) relapsing patients. However, an increase in PPI dose resulted in histologic remission in 9 of 10 relapsing patients with distal eosinophilia, all of whom were CYP2C19 rapid metabolizers.
Topical glucocorticoids — Most patients with eosinophilic esophagitis respond to topical glucocorticoids as demonstrated by a decrease in eosinophil counts [23,33,34]. Fluticasone, budesonide, and ciclesonide have been studied. No formulation of topical glucocorticoids has been approved specifically for eosinophilic esophagitis. We treat most adult patients with swallowed fluticasone [23,33,35-40]. Symptoms often recur when steroids are discontinued . (See "Major side effects of inhaled glucocorticoids" and "Major side effects of systemic glucocorticoids".)
Randomized controlled trials have not consistently demonstrated an improvement in dysphagia with topical glucocorticoids in adults [34,42]. It is unclear if this is due to differences in patient selection, definitions of symptom response, steroid formulations and duration of treatment, or dietary modification that may have resulted in a higher placebo response rate .
Predictors of response to glucocorticoids are poorly understood. In a retrospective cohort study that included 221 patients with eosinophilic esophagitis who received topical glucocorticoids, endoscopic, symptomatic, and histologic improvement were noted in 71, 79, and 57 percent of patients who underwent a repeat endoscopy approximately eight weeks after treatment . On multivariate logistic regression analysis, esophageal narrowing that required dilation at baseline endoscopy was an independent predictor of a lack of response to topical glucocorticoid therapy and abdominal pain was a predictor of response to therapy (OR nonresponse 2.9, 95% CI 1.4-6.3 and 0.3, 95% CI 0.1-0.8, respectively). Higher baseline levels of tryptase and eotaxin-3, but not major basic protein, were associated with a response to topical glucocorticoids.
Fluticasone propionate — Fluticasone is administered using a metered dose inhaler without a spacer. The medication is sprayed into the patient’s mouth and then swallowed. Patients should not inhale when the medication is being delivered and they should not eat or drink for 30 minutes following administration.
The optimal dose has not been established. Our approach is based upon the patient's age:
●Children from one to four years of age: 44 mcg inhaler, two sprays twice daily
●Children from 5 to 10 years of age: 110 mcg inhaler, two sprays twice daily
●Patients ≥11 years of age (including adults): 220 mcg inhaler, two sprays twice daily
●Clinical experience suggests that response rates may increase with a higher dose of fluticasone. A 2013 guideline issued by the American College of Gastroenterology (ACG) suggested that the dose in children can range from 88 to 440 mcg/day in divided doses, while the dose in adults can range from 880 to 1760 mcg/day in divided doses .
Treatment is generally well tolerated and patients who are destined to respond tend to do so quickly (within one week and often within one to two days). In patients who respond, we continue treatment for eight weeks. Patients frequently relapse when treatment is stopped, with reported relapse rates of 14 to 91 percent [23,41,45,46]. For patients who relapse, we treat and discuss maintenance topical glucocorticoids or a trial of a dietary approach. For patients who do not respond to fluticasone, options include a higher dose of fluticasone, a change to oral viscous budesonide, or a dietary approach.
Representative studies of fluticasone for eosinophilic esophagitis have shown the following:
●A series in adults included 21 patients who were treated with fluticasone propionate 220 mcg twice daily . All patients had relief of dysphagia that lasted a minimum of four months. Relief often occurred within a few days after beginning treatment. Three patients (14 percent) relapsed and required additional therapy. Histologic outcomes were not assessed. However, in a later series, clinical improvement was associated with a significant decrease in esophageal eosinophil counts . The only adverse effect noted was dry mouth.
●Similar benefits have been observed in children, although different doses and dosing intervals have been used [33,36,40,46]. The largest controlled trial included 36 children who were randomly assigned to swallowed fluticasone (880 mcg/day in two divided doses) or placebo for three months . Histologic remission was observed significantly more often in the fluticasone group (50 versus 9 percent).
Side effects that have been reported with the use of fluticasone for eosinophilic esophagitis include candidal esophagitis [36,46] and herpes esophagitis was noted in a case report . In addition, in diseases other than eosinophilic esophagitis, inhaled doses of fluticasone higher than 440 mcg/day have been associated with systemic side effects including cataracts, impaired growth in children, and adrenal suppression [48-50]. It is not known if the risk of these side effects is reduced when fluticasone is swallowed and undergoes first-pass metabolism in the liver.
Budesonide — Budesonide has been evaluated in case series and randomized trials and appears to be effective for treating eosinophilic esophagitis [42,51-57]. Budesonide can be administered using a nebulizer and patients are then instructed to swallow the accumulated liquid or as an oral viscous slurry (1 mg daily for children under the age of 10 years, and 2 mg daily for older children and adults). Viscous budesonide can be compounded by mixing two or four 0.5 mg/2 mL Pulmicort Respules with sucralose (Splenda; 10 1-gram packets per 1 mg of budesonide, creating a volume of approximately 8 mL) . Patients should not eat or drink for 30 minutes after taking the budesonide suspension.
In a randomized trial, 22 patients with eosinophilic esophagitis were treated with budesonide either nebulized and then swallowed or as an oral viscous slurry . Although patients who received the viscous slurry had a higher duration of mucosal contact with budesonide and lower eosinophil count, there was no significant difference in dysphagia symptom scores which improved in both groups.
Studies of budesonide for eosinophilic esophagitis have shown the following:
●In a randomized trial with 24 children, the response rate, defined as an eosinophilic count ≤6/hpf was significantly higher with oral viscous budesonide (87 versus 0 percent with placebo) . Symptoms, endoscopic findings, and histologic features improved after treatment. Patients in both groups were also treated with a PPI. Since there were no responders in the placebo group, monotherapy with a PPI had no benefit.
●Similar benefits have been described in studies in adults [53,54,57]. In a randomized trial, 36 adults and adolescents with active eosinophilic esophagitis were randomized to budesonide 1 mg twice daily or placebo for 15 days . The budesonide was administered using a nebulizer and patients were instructed to continuously swallow the accumulated liquid. Patients who received budesonide were more likely to have significant improvements in dysphagia compared with those who received placebo (72 versus 22 percent). In addition, patients treated with budesonide showed histologic improvement, whereas those treated with placebo did not.
Ciclesonide — Ciclesonide, a topical glucocorticoid with less systemic absorption than fluticasone, has been evaluated in small case series [59-61]. In one report, four children who had either failed therapy with fluticasone or dietary restriction, or whose parents were concerned about steroid exposure, were treated with swallowed topical ciclesonide (80 or 160 mcg, two sprays twice daily) for two months . Symptoms resolved in all four patients and there was a significant decrease in eosinophil counts in both proximal and distal esophageal biopsy specimens at two months (proximal: 71±25.5 versus 1.8±2 eosinophils/hpf before and after treatment; distal: 76.3±33 versus 0.75±1.5 eosinophils/hpf before and after treatment). Further studies are needed before ciclesonide can be recommended for patients with eosinophilic esophagitis.
Maintenance therapy — Maintenance therapy with topical steroids and/or dietary restriction should be considered for all patients, but particularly in those with severe dysphagia or food impaction, high-grade esophageal stricture, and rapid symptomatic/histologic relapse following initial therapy . The lack of symptoms does not reliably predict the absence of biologic disease activity [62-64].
Optimal approaches to maintenance therapy have not been well established. Thus, the approach should consider the clinical setting, patient preferences, and available resources. Long-term dietary restriction is effective in maintaining remission in patients in whom dietary triggers have been identified. In patients unwilling to maintain a dietary approach and those in whom a trigger cannot be identified, topical glucocorticoids can be used at the lowest dose that allows patients to remain asymptomatic. In adults, a guideline issued by the ACG suggests a maintenance dose of fluticasone (880 mcg daily in divided doses) or oral viscous budesonide (1 mg daily). (See 'Dietary therapy' above and 'Fluticasone propionate' above.)
One of the only controlled trials of maintenance therapy included 28 adults with eosinophilic esophagitis in clinical remission who were randomly assigned to twice daily budesonide (0.25 mg) or placebo for a total of 50 weeks . The eosinophil load increased over the course of treatment in both groups, but the increase was less in those treated with budesonide (from 0.4 to 32 eosinophils/hpf) than in those treated with placebo (from 0.7 to 65 eosinophils/hpf).
Over the course of the study, patients treated with budesonide did not report a statistically significant increase in symptoms, whereas those who received placebo did. At the end of the study, patients who received budesonide were more likely to be in clinical remission (64 versus 36 percent). Patients treated with budesonide also showed evidence of esophageal remodelling. At baseline, patients with eosinophilic esophagitis had esophageal walls that were twice as thick as those seen in healthy controls (mean thickness 4.2 versus 2.2 mm). After treatment, patients in the budesonide group showed decreases in the thickness of all wall layers, though only the decrease in the thickness of the mucosa was statistically significant. No adverse events were noted.
Topical versus systemic glucocorticoids — Systemic glucocorticoids have a limited role in eosinophilic esophagitis, except possibly in patients with severe disease in whom other approaches are not feasible . Oral prednisone may be slightly more effective than topical fluticasone for the treatment of eosinophilic esophagitis, but the degree of benefit probably does not justify routine use of prednisone considering the greater likelihood of side effects . Furthermore, because of the high relapse rate, chronic or repeated therapy may be needed, which may also support the preferential use of swallowed fluticasone. If systemic steroids are used, the typical dose is 1 to 2 mg/kg/day in divided doses (maximum 60 mg per day).
A randomized trial compared topical with systemic glucocorticoids . The trial included 80 children with eosinophilic esophagitis who were randomly assigned to oral prednisone or swallowed fluticasone. Almost all of the patients, regardless of treatment, were symptom free by four weeks. Histologic improvement was seen to a greater degree in the prednisone group. Relapse was observed in 45 percent of patients in both groups within 24 weeks of stopping therapy. Glucocorticoid side effects occurred in 40 percent of patients in the prednisone arm, whereas candidal esophagitis was seen in 15 percent in the fluticasone arm.
ESOPHAGEAL DILATION — Dilation of esophageal strictures is effective for relieving dysphagia, but has no effect on underlying inflammation [66,67]. It is often reserved for patients who have failed more conservative therapy, but may be required as initial therapy in patients with high-grade strictures [8,68]. We generally reserve dilation for patients with strictures or rings who have not responded to medical therapy.
Dilation should be performed carefully since it has been associated with deep mucosal tears and esophageal perforation [69-72]. It has been recommended that the progression of dilation per session be limited to 3 mm or less . Because of this, multiple dilations are often required to attain a goal esophageal diameter of 15 to 18 mm [66,73,74]. Patients should be forewarned of the risks of esophageal dilation including chest pain, which occurs in 75 percent of patients, and bleeding .
Whether patients with eosinophilic esophagitis are at higher risk of perforation due to esophageal dilation than other patients undergoing dilation is not clear. Older studies suggested that patients with eosinophilic esophagitis are at increased risk for perforation, with perforation rates of 5 to 7 percent . More recent studies suggest that the rate of perforation is considerably lower [66,70,76,77]. A 2013 meta-analysis that included 860 patients, of whom 525 underwent at least one dilation, and a total of 992 dilations found that there were only three perforations (2 percent, 95% CI 0-0.9) and one hemorrhage (0 percent, 95% CI 0-0.8) . Clinical improvement with dilation occurred in 75 percent of patients (95% CI 58-93).
Tearing and perforation can occur without perceived resistance when passing a dilator or the endoscope (picture 1) . As a result, it may be reasonable to gently inspect the esophagus after passing each dilator. Rigid endoscopy has been associated with a high rate of perforation and should be avoided . The use of glucocorticoids before dilation has not been extensively studied ; it may offer a small benefit prior to dilation by diminishing inflammation. (See "Management of benign esophageal strictures", section on 'Complex strictures'.)
EXPERIMENTAL AND INEFFECTIVE TREATMENTS — Other treatments for eosinophilic esophagitis have been examined.
Prostaglandin D2 receptor antagonist — Chemoattractant receptor-homologous molecule on Th2 cells (CRTH2) is a receptor expressed by Th2 cells, eosinophils, and other inflammatory cells that mediates chemotaxis of these cells in response to prostaglandin D2. OC000459 is a selective, orally bioavailable, CRTH2 antagonist. In a randomized controlled trial, 26 adults with steroid-dependent or steroid-refractory eosinophilic esophagitis were randomly assigned to treatment with OC000459 (100 mg twice daily) or placebo . At eight weeks, treatment with OC000459, but not placebo, was associated with a modest but significant reduction in esophageal eosinophil counts and an improvement in symptoms as compared with baseline.
Antihistamines and cromolyn — Little benefit has been seen in patients treated with medications aimed at controlling allergies, including antihistamines and cromolyn sodium (a mast cell stabilizer) [81,82].
Montelukast — Initial experience suggested that montelukast (a leukotriene inhibitor that has been used in eosinophilic gastroenteritis) may be helpful for symptom reduction in patients with eosinophilic esophagitis but subsequent experience has been mixed for either induction or maintenance of remission [83-87]. Thus, its role, if any, remains unclear. The following are illustrative of the range of observations.
●One series examined eight adults who were treated with montelukast . The dose used was much higher than the standard 10 mg dose used for the treatment of asthma in adults. While patients started at a dose of 10 mg per day, the patient increased the dose up to a suprapharmacologic dose totaling 100 mg daily based upon symptom relief. Once symptom relief had been achieved, the dose was reduced to maintenance levels where symptom control was maintained (20 to 40 mg daily). Six of eight patients had complete symptom relief. However, there was no effect on esophageal eosinophilia. Several side effects were observed, including nausea and myalgias. The safety of the high doses used in this study is unclear.
●In a retrospective series of eight pediatric patients who were maintained on montelukast (4 to 10 mg daily), three patients had a clinical response that was attributed to montelukast (one complete response and two partial responses) . Four patients responded clinically but had also been started on other therapies in addition to the montelukast. The study was not able to confirm if montelukast had an effect on esophageal histology. No side effects were reported related to the montelukast.
●In a small randomized trial, 41 patients with eosinophilic esophagitis were assigned to maintenance treatment with montelukast (20 mg daily) or placebo for 26 weeks following steroid-induced symptomatic remission . There were no significant differences in the proportion of patients that remained in remission between the two groups (40 versus 24 percent). Endoscopic and histological outcomes were not reported.
Mepolizumab — Mepolizumab is a humanized monoclonal antibody against interleukin (IL)-5, which has a central role in eosinophil recruitment. (See "Eosinophilic esophagitis (EoE): Genetics and immunopathogenesis", section on 'Role of the immune system and environmental factors'.)
Studies of mepolizumab have had variable results:
●A case series found clinical benefits in four patients with eosinophilic esophagitis .
●A placebo-controlled trial in 11 adults found a reduction in esophageal eosinophilia in patients treated with mepolizumab compared with those who received placebo . Clinically, there was a small improvement in dysphagia in the mepolizumab arm, but it did not differ significantly from improvement also seen the placebo arm.
●A third report included 57 children with eosinophilic esophagitis who were randomly assigned to three infusions of mepolizumab at different doses (0.55, 2.5, or 10 mg/kg) . There was a significant decrease in the peak and mean eosinophil counts and improvement in endoscopic findings in all three groups. However, symptoms did not improve, possibly because most patients did not have severe symptoms at baseline. Whether the observed histologic end endoscopic benefits could be attributed to mepolizumab is uncertain since the study did not include a placebo arm and because dietary interventions were permitted.
Reslizumab — Reslizumab is an IL-5 neutralizing antibody that is undergoing clinical trials in eosinophilic esophagitis. In a controlled trial involving 226 children and adolescents with eosinophilic esophagitis, there was a significant reduction in peak eosinophil counts compared with placebo (59, 67, and 64 percent for the three doses tested versus 24 percent for placebo) . However, all treatment groups had significant improvement in symptoms as assessed by a physician global assessment score and the differences were not significantly different than placebo. Thus, its role remains uncertain.
Anti-IgE monoclonal antibody — In a report of two patients with multiple food allergies and eosinophilic esophagitis, treatment with the anti-IgE monoclonal antibody omalizumab was associated with an improvement in allergic symptoms but not in endoscopic or histologic features of eosinophilic esophagitis .
PROGNOSIS — The long-term prognosis of eosinophilic esophagitis is unclear. Untreated, patients may remain symptomatic or have episodic symptoms. As noted above, symptoms frequently recur in patients treated with a short course of topical glucocorticoids .
Whether the disease persists into adulthood in affected children has not been extensively studied, although the available data suggest that it does. In a report of 620 children evaluated over a 14-year period, eosinophilic esophagitis persisted, with only 10 percent developing tolerance to their food allergies . No children progressed to other gastrointestinal diseases.
In adults, anecdotal observations suggest that the disease may progress to a fibrostenotic stage in which the predominant symptom is intermittent dysphagia . Patients with extreme narrow caliber esophagus, a subtype of eosinophilic esophagitis in which the esophagus cannot be traversed with an adult endoscope, often require multiple dilations, are more refractory to treatment with steroids, and have lower response rates to treatment . However, the proportion of patients with progressive disease is unknown. The largest study on the natural history in adults focused on 30 adults who were followed for an average of seven years . Patients underwent a follow-up examination consisting of a structured interview, laboratory testing, and an upper endoscopy with biopsies. The majority of patients had persistent dysphagia. Attacks of dysphagia were more common in patients who had peripheral eosinophilia. Eosinophilic infiltration persisted in all symptomatic patients, but the degree of tissue eosinophilia appeared to decrease. The inflammatory process remained confined to the esophagus without gastric or duodenal involvement. No cases of dysplasia or esophageal malignancy were observed.
PATIENT ADVOCACY — The American Partnership for Eosinophilic Disorders is an advocacy group for patients with eosinophilic gastrointestinal diseases.
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
●Basics topic (see "Patient education: Eosinophilic esophagitis (The Basics)")
SUMMARY AND RECOMMENDATIONS — The management of eosinophilic esophagitis includes pharmacologic, endoscopic, and dietary interventions. Our recommendations below are consistent with consensus guidelines (table 1 and table 2).
●We refer both adults and children to an allergist with expertise in the evaluation of food allergies. We suggest avoidance of known allergens (both food and environmental) for patients in whom specific allergies can be identified after discussion of the pros and cons of the dietary approach as described above (Grade 2C). (See 'Dietary therapy' above.)
●Dietary approaches have been best studied in children, in whom they are used routinely as an option for primary therapy. Emerging data in adults suggest a six-food elimination diet can improve symptoms and esophageal eosinophilia and help identify causative foods. As a result, some authorities have begun recommending this approach as a primary option for their adult patients. If a dietary approach is taken, it is important to coordinate it with a nutritionist since elimination and elemental diets can result in important restrictions of calories and nutrients. (See 'Dietary therapy' above and "Dietary management of eosinophilic esophagitis".)
Fluticasone is administered using a metered dose inhaler without a spacer. The medication is sprayed into the patient’s mouth and then swallowed. Patients should not use a spacer or inhale while the medication is being delivered, and they should not eat or drink for 30 minutes following administration. The dose used varies with the age of the patient.
We suggest initial treatment for six to eight weeks (Grade 2C). Optimal strategies after initial treatment are unsettled. Thus, the decision to continue treatment should be individualized. Because symptoms and esophageal eosinophilia almost universally return when treatment is discontinued, we suggest ongoing treatment, whether medical or nutritional (Grade 2C). Some authorities treat on an as-needed basis. Such an approach is particularly useful in patients who have identifiable, seasonal triggers. Others use the lowest dose possible of topical glucocorticoids that allow patients to remain asymptomatic. Our approach is to continue nutritional therapy, using topical glucocorticoids as needed in doses and intervals that keep patients asymptomatic.
Treatment is generally well tolerated. However, worsening of dysphagia during treatment should alert to the possibility of candidal esophagitis.
●In patients who do not respond to topical fluticasone, we suggest either an elimination diet (if not already tried) or a trial of topical budesonide (Grade 2C). (See 'Budesonide' above and 'Dietary therapy' above and "Dietary management of eosinophilic esophagitis", section on 'Elimination diets'.)
●Adult patients with esophageal rings or strictures may require dilation. Dilation is associated with mucosal tears and esophageal perforation and thus should be performed extremely cautiously. We suggest that, if possible, dilation be avoided until patients have been given a course of fluticasone, which may relieve dysphagia and thus avoid the need for dilation (Grade 2C). Dilation has generally not been needed in children in most reports. (See 'Esophageal dilation' above.)
●While a two-month course of proton pump inhibitor therapy followed by endoscopy and biopsy (or with a normal pH study) are recommended to establish the diagnosis of eosinophilic esophagitis, the role of acid suppression in the treatment of established eosinophilic esophagitis is unclear. Acid suppression is reasonable in patients in whom reflux is suspected to be contributing to symptoms, and there are occasional patients who have a good clinical response to proton pump inhibitors. Thus, we suggest a trial of a proton pump inhibitor except in patients who have already tried one and did not respond (Grade 2C). (See 'Acid suppression' above and "Clinical manifestations and diagnosis of eosinophilic esophagitis", section on 'Diagnosis'.)
●Whether patients should undergo surveillance endoscopy is unclear. Although a malignant potential has not been reported, follow-up has generally been short. We generally repeat upper endoscopy for patients in whom symptoms have changed or who require esophageal dilation.
- MacCarty RL, Talley NJ. Barium studies in diffuse eosinophilic gastroenteritis. Gastrointest Radiol 1990; 15:183.
- Dobbins JW, Sheahan DG, Behar J. Eosinophilic gastroenteritis with esophageal involvement. Gastroenterology 1977; 72:1312.
- Furuta GT, Katzka DA. Eosinophilic Esophagitis. N Engl J Med 2015; 373:1640.
- Kukuruzovic RH, Elliott EE, O'Loughlin EV, Markowitz JE. Non-surgical interventions for eosinophilic oesophagitis. Cochrane Database Syst Rev 2004; :CD004065.
- Rothenberg ME. Biology and treatment of eosinophilic esophagitis. Gastroenterology 2009; 137:1238.
- Dellon ES. Diagnosis and management of eosinophilic esophagitis. Clin Gastroenterol Hepatol 2012; 10:1066.
- Liacouras CA, Furuta GT, Hirano I, et al. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol 2011; 128:3.
- Dellon ES, Gonsalves N, Hirano I, et al. ACG clinical guideline: Evidenced based approach to the diagnosis and management of esophageal eosinophilia and eosinophilic esophagitis (EoE). Am J Gastroenterol 2013; 108:679.
- Papadopoulou A, Koletzko S, Heuschkel R, et al. Management guidelines of eosinophilic esophagitis in childhood. J Pediatr Gastroenterol Nutr 2014; 58:107.
- Straumann A, Spichtin HP, Grize L, et al. Natural history of primary eosinophilic esophagitis: a follow-up of 30 adult patients for up to 11.5 years. Gastroenterology 2003; 125:1660.
- Lieberman JA, Morotti RA, Konstantinou GN, et al. Dietary therapy can reverse esophageal subepithelial fibrosis in patients with eosinophilic esophagitis: a historical cohort. Allergy 2012; 67:1299.
- Spergel JM, Brown-Whitehorn TF, Beausoleil JL, et al. 14 years of eosinophilic esophagitis: clinical features and prognosis. J Pediatr Gastroenterol Nutr 2009; 48:30.
- Spergel JM. Eosinophilic esophagitis in adults and children: evidence for a food allergy component in many patients. Curr Opin Allergy Clin Immunol 2007; 7:274.
- Gonsalves N, Yang GY, Doerfler B, et al. Elimination diet effectively treats eosinophilic esophagitis in adults; food reintroduction identifies causative factors. Gastroenterology 2012; 142:1451.
- Lucendo AJ, Arias Á, González-Cervera J, et al. Empiric 6-food elimination diet induced and maintained prolonged remission in patients with adult eosinophilic esophagitis: a prospective study on the food cause of the disease. J Allergy Clin Immunol 2013; 131:797.
- Markowitz JE, Spergel JM, Ruchelli E, Liacouras CA. Elemental diet is an effective treatment for eosinophilic esophagitis in children and adolescents. Am J Gastroenterol 2003; 98:777.
- Peterson KA, Byrne KR, Vinson LA, et al. Elemental diet induces histologic response in adult eosinophilic esophagitis. Am J Gastroenterol 2013; 108:759.
- Wolf WA, Jerath MR, Sperry SL, et al. Dietary elimination therapy is an effective option for adults with eosinophilic esophagitis. Clin Gastroenterol Hepatol 2014; 12:1272.
- Arias A, González-Cervera J, Tenias JM, Lucendo AJ. Efficacy of dietary interventions for inducing histologic remission in patients with eosinophilic esophagitis: a systematic review and meta-analysis. Gastroenterology 2014; 146:1639.
- Straumann A, Bussmann C, Zuber M, et al. Eosinophilic esophagitis: analysis of food impaction and perforation in 251 adolescent and adult patients. Clin Gastroenterol Hepatol 2008; 6:598.
- Klinnert MD. Psychological impact of eosinophilic esophagitis on children and families. Immunol Allergy Clin North Am 2009; 29:99.
- Santangelo CM, McCloud E. Nutritional management of children who have food allergies and eosinophilic esophagitis. Immunol Allergy Clin North Am 2009; 29:77.
- Remedios M, Campbell C, Jones DM, Kerlin P. Eosinophilic esophagitis in adults: clinical, endoscopic, histologic findings, and response to treatment with fluticasone propionate. Gastrointest Endosc 2006; 63:3.
- Spechler SJ, Genta RM, Souza RF. Thoughts on the complex relationship between gastroesophageal reflux disease and eosinophilic esophagitis. Am J Gastroenterol 2007; 102:1301.
- Cheng E, Zhang X, Huo X, et al. Omeprazole blocks eotaxin-3 expression by oesophageal squamous cells from patients with eosinophilic oesophagitis and GORD. Gut 2013; 62:824.
- Vazquez-Elizondo G, Ngamruengphong S, Khrisna M, et al. The outcome of patients with oesophageal eosinophilic infiltration after an eight-week trial of a proton pump inhibitor. Aliment Pharmacol Ther 2013; 38:1312.
- Orenstein SR, Shalaby TM, Di Lorenzo C, et al. The spectrum of pediatric eosinophilic esophagitis beyond infancy: a clinical series of 30 children. Am J Gastroenterol 2000; 95:1422.
- Krarup AL, Villadsen GE, Mejlgaard E, et al. Acid hypersensitivity in patients with eosinophilic oesophagitis. Scand J Gastroenterol 2010; 45:273.
- Peterson KA, Thomas KL, Hilden K, et al. Comparison of esomeprazole to aerosolized, swallowed fluticasone for eosinophilic esophagitis. Dig Dis Sci 2010; 55:1313.
- Moawad FJ, Veerappan GR, Dias JA, et al. Randomized controlled trial comparing aerosolized swallowed fluticasone to esomeprazole for esophageal eosinophilia. Am J Gastroenterol 2013; 108:366.
- Molina-Infante J, Katzka DA, Gisbert JP. Review article: proton pump inhibitor therapy for suspected eosinophilic oesophagitis. Aliment Pharmacol Ther 2013; 37:1157.
- Molina-Infante J, Rodriguez-Sanchez J, Martinek J, et al. Long-Term Loss of Response in Proton Pump Inhibitor-Responsive Esophageal Eosinophilia Is Uncommon and Influenced by CYP2C19 Genotype and Rhinoconjunctivitis. Am J Gastroenterol 2015; 110:1567.
- Konikoff MR, Noel RJ, Blanchard C, et al. A randomized, double-blind, placebo-controlled trial of fluticasone propionate for pediatric eosinophilic esophagitis. Gastroenterology 2006; 131:1381.
- Alexander JA, Jung KW, Arora AS, et al. Swallowed fluticasone improves histologic but not symptomatic response of adults with eosinophilic esophagitis. Clin Gastroenterol Hepatol 2012; 10:742.
- Aceves SS, Furuta GT, Spechler SJ. Integrated approach to treatment of children and adults with eosinophilic esophagitis. Gastrointest Endosc Clin N Am 2008; 18:195.
- Noel RJ, Putnam PE, Collins MH, et al. Clinical and immunopathologic effects of swallowed fluticasone for eosinophilic esophagitis. Clin Gastroenterol Hepatol 2004; 2:568.
- Faubion WA Jr, Perrault J, Burgart LJ, et al. Treatment of eosinophilic esophagitis with inhaled corticosteroids. J Pediatr Gastroenterol Nutr 1998; 27:90.
- Langdon DE. Fluticasone in eosinophilic corrugated ringed esophagus. Am J Gastroenterol 2001; 96:926.
- Perrault J, Smyrk T, Burgart L, Arora A. The varied presentations of eosinophilic esophagitis in adults: GERD it is not! (abstract). Gastroenterology 2001; 120:A250.
- Teitelbaum JE, Fox VL, Twarog FJ, et al. Eosinophilic esophagitis in children: immunopathological analysis and response to fluticasone propionate. Gastroenterology 2002; 122:1216.
- Helou EF, Simonson J, Arora AS. 3-yr-follow-up of topical corticosteroid treatment for eosinophilic esophagitis in adults. Am J Gastroenterol 2008; 103:2194.
- Dohil R, Newbury R, Fox L, et al. Oral viscous budesonide is effective in children with eosinophilic esophagitis in a randomized, placebo-controlled trial. Gastroenterology 2010; 139:418.
- Hirano I. Therapeutic end points in eosinophilic esophagitis: is elimination of esophageal eosinophils enough? Clin Gastroenterol Hepatol 2012; 10:750.
- Wolf WA, Cotton CC, Green DJ, et al. Predictors of response to steroid therapy for eosinophilic esophagitis and treatment of steroid-refractory patients. Clin Gastroenterol Hepatol 2015; 13:452.
- Arora AS, Perrault J, Smyrk TC. Topical corticosteroid treatment of dysphagia due to eosinophilic esophagitis in adults. Mayo Clin Proc 2003; 78:830.
- Schaefer ET, Fitzgerald JF, Molleston JP, et al. Comparison of oral prednisone and topical fluticasone in the treatment of eosinophilic esophagitis: a randomized trial in children. Clin Gastroenterol Hepatol 2008; 6:165.
- Lindberg GM, Van Eldik R, Saboorian MH. A case of herpes esophagitis after fluticasone propionate for eosinophilic esophagitis. Nat Clin Pract Gastroenterol Hepatol 2008; 5:527.
- Cumming RG, Mitchell P, Leeder SR. Use of inhaled corticosteroids and the risk of cataracts. N Engl J Med 1997; 337:8.
- Wagener JS, Wojtczak HA. Inhaled steroids in children: risks versus rewards. J Pediatr 1998; 132:381.
- Golekoh MC, Hornung LN, Mukkada VA, et al. Adrenal Insufficiency after Chronic Swallowed Glucocorticoid Therapy for Eosinophilic Esophagitis. J Pediatr 2016; 170:240.
- Aceves SS, Dohil R, Newbury RO, Bastian JF. Topical viscous budesonide suspension for treatment of eosinophilic esophagitis. J Allergy Clin Immunol 2005; 116:705.
- Aceves SS, Bastian JF, Newbury RO, Dohil R. Oral viscous budesonide: a potential new therapy for eosinophilic esophagitis in children. Am J Gastroenterol 2007; 102:2271.
- Karnam U, Hirano I. Effectiveness of oral budesonide suspension in adult patients with eosinophilic esophagitis (abstract). Gastroenterology 2008; 134 (Suppl):A289.
- Netzer P, Gschossmann JM, Straumann A, et al. Corticosteroid-dependent eosinophilic oesophagitis: azathioprine and 6-mercaptopurine can induce and maintain long-term remission. Eur J Gastroenterol Hepatol 2007; 19:865.
- Straumann A, Degen L, Felder S. Budesonide as induction treatment for active eosinophilic esophagitis in adolescents and adults: a randomized, double-blind, placebo-controlled study (Bee-I trial) (abstract). Gastroenterology 2008; 134 (Suppl):A104.
- Straumann A, Conus S, Degen L, et al. Long-term budesonide maintenance treatment is partially effective for patients with eosinophilic esophagitis. Clin Gastroenterol Hepatol 2011; 9:400.
- Straumann A, Conus S, Degen L, et al. Budesonide is effective in adolescent and adult patients with active eosinophilic esophagitis. Gastroenterology 2010; 139:1526.
- Dellon ES, Sheikh A, Speck O, et al. Viscous topical is more effective than nebulized steroid therapy for patients with eosinophilic esophagitis. Gastroenterology 2012; 143:321.
- Stoeck M, Riedel R, Hochhaus G, et al. In vitro and in vivo anti-inflammatory activity of the new glucocorticoid ciclesonide. J Pharmacol Exp Ther 2004; 309:249.
- Schroeder S, Fleischer DM, Masterson JC, et al. Successful treatment of eosinophilic esophagitis with ciclesonide. J Allergy Clin Immunol 2012; 129:1419.
- Lee JJ, Fried AJ, Hait E, et al. Topical inhaled ciclesonide for treatment of eosinophilic esophagitis. J Allergy Clin Immunol 2012; 130:1011; author reply 1011.
- Safroneeva E, Straumann A, Coslovsky M, et al. Symptoms Have Modest Accuracy in Detecting Endoscopic and Histologic Remission in Adults With Eosinophilic Esophagitis. Gastroenterology 2016; 150:581.
- Schoepfer AM, Straumann A, Panczak R, et al. Development and validation of a symptom-based activity index for adults with eosinophilic esophagitis. Gastroenterology 2014; 147:1255.
- Richter JE. New questionnaire for eosinophilic esophagitis: will it measure what we want? Gastroenterology 2014; 147:1212.
- Liacouras CA, Spergel JM, Ruchelli E, et al. Eosinophilic esophagitis: a 10-year experience in 381 children. Clin Gastroenterol Hepatol 2005; 3:1198.
- Schoepfer AM, Gonsalves N, Bussmann C, et al. Esophageal dilation in eosinophilic esophagitis: effectiveness, safety, and impact on the underlying inflammation. Am J Gastroenterol 2010; 105:1062.
- Robles-Medranda C, Villard F, le Gall C, et al. Severe dysphagia in children with eosinophilic esophagitis and esophageal stricture: an indication for balloon dilation? J Pediatr Gastroenterol Nutr 2010; 50:516.
- Furuta GT, Liacouras CA, Collins MH, et al. Eosinophilic esophagitis in children and adults: a systematic review and consensus recommendations for diagnosis and treatment. Gastroenterology 2007; 133:1342.
- Kaplan M, Mutlu EA, Jakate S, et al. Endoscopy in eosinophilic esophagitis: "feline" esophagus and perforation risk. Clin Gastroenterol Hepatol 2003; 1:433.
- Jung KW, Gundersen N, Kopacova J, et al. Occurrence of and risk factors for complications after endoscopic dilation in eosinophilic esophagitis. Gastrointest Endosc 2011; 73:15.
- Cohen MS, Kaufman AB, Palazzo JP, et al. An audit of endoscopic complications in adult eosinophilic esophagitis. Clin Gastroenterol Hepatol 2007; 5:1149.
- Eisenbach C, Merle U, Schirmacher P, et al. Perforation of the esophagus after dilation treatment for dysphagia in a patient with eosinophilic esophagitis. Endoscopy 2006; 38 Suppl 2:E43.
- Bohm M, Richter JE, Kelsen S, Thomas R. Esophageal dilation: simple and effective treatment for adults with eosinophilic esophagitis and esophageal rings and narrowing. Dis Esophagus 2010; 23:377.
- Richter JE. Esophageal dilation in eosinophilic esophagitis. Best Pract Res Clin Gastroenterol 2015; 29:815.
- Hirano I. Dilation in eosinophilic esophagitis: to do or not to do? Gastrointest Endosc 2010; 71:713.
- Jacobs JW Jr, Spechler SJ. A systematic review of the risk of perforation during esophageal dilation for patients with eosinophilic esophagitis. Dig Dis Sci 2010; 55:1512.
- Dellon ES, Gibbs WB, Rubinas TC, et al. Esophageal dilation in eosinophilic esophagitis: safety and predictors of clinical response and complications. Gastrointest Endosc 2010; 71:706.
- Moawad FJ, Cheatham JG, DeZee KJ. Meta-analysis: the safety and efficacy of dilation in eosinophilic oesophagitis. Aliment Pharmacol Ther 2013; 38:713.
- Schoepfer AM, Gschossmann J, Scheurer U, et al. Esophageal strictures in adult eosinophilic esophagitis: dilation is an effective and safe alternative after failure of topical corticosteroids. Endoscopy 2008; 40:161.
- Straumann A, Hoesli S, Bussmann Ch, et al. Anti-eosinophil activity and clinical efficacy of the CRTH2 antagonist OC000459 in eosinophilic esophagitis. Allergy 2013; 68:375.
- Losurdo J, Bruninga K, Dobozi B. Idiopathic eosinophilic esophagitis: A new cause of "feline" esophagus (abstract). Gastroenterology 1999; 116:A239.
- Walsh SV, Antonioli DA, Goldman H, et al. Allergic esophagitis in children: a clinicopathological entity. Am J Surg Pathol 1999; 23:390.
- Attwood SE, Lewis CJ, Bronder CS, et al. Eosinophilic oesophagitis: a novel treatment using Montelukast. Gut 2003; 52:181.
- Stumphy J, Al-Zubeidi D, Guerin L, et al. Observations on use of montelukast in pediatric eosinophilic esophagitis: insights for the future. Dis Esophagus 2011; 24:229.
- Vanderhoof JA, Young RJ, Hanner TL, Kettlehut B. Montelukast: use in pediatric patients with eosinophilic gastrointestinal disease. J Pediatr Gastroenterol Nutr 2003; 36:293.
- Daikh BE, Ryan CK, Schwartz RH. Montelukast reduces peripheral blood eosinophilia but not tissue eosinophilia or symptoms in a patient with eosinophilic gastroenteritis and esophageal stricture. Ann Allergy Asthma Immunol 2003; 90:23.
- Lucendo AJ, De Rezende LC, Jiménez-Contreras S, et al. Montelukast was inefficient in maintaining steroid-induced remission in adult eosinophilic esophagitis. Dig Dis Sci 2011; 56:3551.
- Alexander JA, Ravi K, Enders FT, et al. Montelukast Does not Maintain Symptom Reductions After Topical Steroid Therapy for Eosinophilic Esophagitis. Clin Gastroenterol Hepatol 2016.
- Stein ML, Collins MH, Villanueva JM, et al. Anti-IL-5 (mepolizumab) therapy for eosinophilic esophagitis. J Allergy Clin Immunol 2006; 118:1312.
- Straumann A, Conus S, Grzonka P, et al. Anti-interleukin-5 antibody treatment (mepolizumab) in active eosinophilic oesophagitis: a randomised, placebo-controlled, double-blind trial. Gut 2010; 59:21.
- Assa'ad AH, Gupta SK, Collins MH, et al. An antibody against IL-5 reduces numbers of esophageal intraepithelial eosinophils in children with eosinophilic esophagitis. Gastroenterology 2011; 141:1593.
- Spergel JM, Rothenberg ME, Collins MH, et al. Reslizumab in children and adolescents with eosinophilic esophagitis: results of a double-blind, randomized, placebo-controlled trial. J Allergy Clin Immunol 2012; 129:456.
- Rocha R, Vitor AB, Trindade E, et al. Omalizumab in the treatment of eosinophilic esophagitis and food allergy. Eur J Pediatr 2011; 170:1471.
- Straumann A, Bussmann C, Conus S, et al. Anti-TNF-alpha (infliximab) therapy for severe adult eosinophilic esophagitis. J Allergy Clin Immunol 2008; 122:425.
- Attwood SE, Smyrk TC, Demeester TR, Jones JB. Esophageal eosinophilia with dysphagia. A distinct clinicopathologic syndrome. Dig Dis Sci 1993; 38:109.
- Eluri S, Runge TM, Cotton CC, et al. The extremely narrow-caliber esophagus is a treatment-resistant subphenotype of eosinophilic esophagitis. Gastrointest Endosc 2016; 83:1142.