Eosinophilic esophagitis (EoE) has been defined as a ‘chronic, immune/antigen-mediated, esophageal disease, characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation'. A peak value of ≥15 eosinophils/high power field has been defined as histologic diagnostic cutoff. Other conditions associated with esophageal eosinophilia, such as gastro-esophageal reflux disease, PPI-responsive esophageal eosinophilia or Crohn's disease, need to be ruled out before EoE can be diagnosed. Males are affected more frequently than females and most of the patients have concomitant allergies. Currently, the EoE prevalence is about 1 of 2,000 inhabitants in Westernized countries. The first EoE patients were described only 2 decades ago. Despite this short period, considerable progress has been made regarding the understanding of the pathophysiology, natural history, assessment of disease activity and with respect to evaluating different therapeutic options. Untreated EoE can lead to esophageal remodeling with reduced compliance and stricture formation, which represents the main risk factor for food bolus impactions. The therapeutic options can be summarized with the 3 D's, which stand for drugs, diets and dilation. Of note, as of yet, there is no EoE-specific drug that has been approved by regulatory authorities. This is, among other reasons, related to the lack of validated outcome measurement instruments until recently. Swallowed topical steroids such as budesonide or fluticasone represent the standard of care for treating symptomatic pediatric and adult EoE patients with inflammatory activity. Several trials have already evaluated different biologic therapies, such as anti-interleukin-5 or anti-IgE. Further studies are on the way. As a non-pharmacologic alternative, different dietary regimens exist. Dilation can offer long-lasting symptomatic response in case of stricturing EoE but does not have any impact on the underlying inflammation. This review highlights the latest insights regarding pathophysiology and its impact regarding current and future therapeutic strategies.

Attwood SE, Smyrk TC, Demeester TR, Jones JB: Esophageal eosinophilia with dysphagia. A distinct clinicopathologic syndrome. Dig Dis Sci 1993;38:109-116.
Straumann A, Spichtin HP, Bernoulli R, Loosli J, Vögtlin J: [Idiopathic eosinophilic esophagitis: a frequently overlooked disease with typical clinical aspects and discrete endoscopic findings]. Schweiz Med Wochenschr 1994;124:1419-1429.
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;133:1342-1363.
Liacouras CA, Furuta GT, Hirano I, et al: Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol 2011;128:3-20.e6; quiz 21-22.
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;125:1660-1669.
Hruz P, Straumann A, Bussmann C, et al: Escalating incidence of eosinophilic esophagitis: a 20-year prospective, population-based study in Olten County, Switzerland. J Allergy Clin Immunol 2011;128:1349-1350.
Arias Á, Lucendo AJ: Prevalence of eosinophilic oesophagitis in adult patients in a central region of Spain. Eur J Gastroenterol Hepatol 2013;25:208-212.
Van Rhijn BD, Verheij J, Smout AJ, Bredenoord AJ: Rapidly increasing incidence of eosinophilic esophagitis in a large cohort. Neurogastroenterol Motil 2013;25:47-52.e5.
DeBrosse CW, Collins MH, Buckmeier Butz BK, et al: Identification, epidemiology, and chronicity of pediatric esophageal eosinophilia, 1982-1999. J Allergy Clin Immunol 2010;126:112-119.
Prasad GA, Alexander JA, Schleck CD, et al: Epidemiology of eosinophilic esophagitis over three decades in Olmsted County, Minnesota. Clin Gastroenterol Hepatol 2009;7:1055-1061.
Straumann A, Simon HU: Eosinophilic esophagitis: escalating epidemiology? J Allergy Clin Immunol 2005;115:418-419.
Giriens B, Yan P, Safroneeva E, et al: Escalating incidence of eosinophilic esophagitis in Canton of Vaud, Switzerland, 1993-2013: a population-based study. Allergy 2015;70:1633-1639.
Dellon ES, Jensen ET, Martin CF, Shaheen NJ, Kappelman MD: Prevalence of eosinophilic esophagitis in the United States. Clin Gastroenterol Hepatol 2014;12:589-596.e1.
Spergel JM, Brown-Whitehorn T, Beausoleil JL, et al: Predictive values for skin prick test and atopy patch test for eosinophilic esophagitis. J Allergy Clin Immunol 2007;119:509-511.
Kagalwalla AF, Sentongo TA, Ritz S, et al: Effect of six-food elimination diet on clinical and histologic outcomes in eosinophilic esophagitis. Clin Gastroenterol Hepatol 2006;4:1097-1102.
Gonsalves N, Yang GY, Doerfler B, et al: Elimination diet effectively treats eosinophilic esophagitis in adults; food reintroduction identifies causative factors. Gastroenterology 2012;142:1451-1459.e1; quiz e14-e15.
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;131:797-804.
Rothenberg ME: Eosinophilic gastrointestinal disorders (EGID). J Allergy Clin Immunol 2004;113:11-28.
Sherrill JD, Kc K, Wu D, et al: Desmoglein-1 regulates esophageal epithelial barrier function and immune responses in eosinophilic esophagitis. Mucosal Immunol 2014;7:718-729.
Abdulnour-Nakhoul SM, Al-Tawil Y, Gyftopoulos AA, et al: Alterations in junctional proteins, inflammatory mediators and extracellular matrix molecules in eosinophilic esophagitis. Clin Immunol 2013;148:265-278.
Hogan SP, Mishra A, Brandt EB, et al: A critical role for eotaxin in experimental oral antigen-induced eosinophilic gastrointestinal allergy. Proc Natl Acad Sci U S A 2000;97:6681-6686.
Straumann A, Bauer M, Fischer B, et al: Idiopathic eosinophilic esophagitis is associated with a T(H)2-type allergic inflammatory response. J Allergy Clin Immunol 2001;108:954-961.
Clayton F, Fang JC, Gleich GJ, et al: Eosinophilic esophagitis in adults is associated with IgG4 and not mediated by IgE. Gastroenterology 2014;147:602-609.
Straumann A, Aceves SS, Blanchard C, et al: Pediatric and adult eosinophilic esophagitis: similarities and differences. Allergy 2012;67:477-490.
Assa'ad AH, Putnam PE, Collins MH, et al: Pediatric patients with eosinophilic esophagitis: an 8-year follow-up. J Allergy Clin Immunol 2007;119:731-738.
Helou EF, Simonson J, Arora AS: 3-yr-follow-up of topical corticosteroid treatment for eosinophilic esophagitis in adults. Am J Gastroenterol 2008;103:2194-2199.
Spergel JM, Brown-Whitehorn TF, Beausoleil JL, et al: 14 years of eosinophilic esophagitis: clinical features and prognosis. J Pediatr Gastroenterol Nutr 2009;48:30-36.
Kagalwalla AF, Akhtar N, Woodruff SA, et al: Eosinophilic esophagitis: epithelial mesenchymal transition contributes to esophageal remodeling and reverses with treatment. J Allergy Clin Immunol 2012;129:1387-1396.e7.
Schoepfer AM, Safroneeva E, Bussmann C, et al: Delay in diagnosis of eosinophilic esophagitis increases risk for stricture formation in a time-dependent manner. Gastroenterology 2013;145:1230-1236.e1-e2.
Lipka S, Kumar A, Richter JE: Impact of diagnostic delay and other risk factors on eosinophilic esophagitis phenotype and esophageal diameter. J Clin Gastroenterol 2016;50:134-140.
Dellon ES, Kim HP, Sperry SL, Rybnicek DA, Woosley JT, Shaheen NJ: A phenotypic analysis shows that eosinophilic esophagitis is a progressive fibrostenotic disease. Gastrointest Endosc 2014;79:577-585.e4.
Van Rhijn BD, Oors JM, Smout AJ, Bredenoord AJ: Prevalence of esophageal motility abnormalities increases with longer disease duration in adult patients with eosinophilic esophagitis. Neurogastroenterol Motil 2014;26:1349-1355.
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;108:679-692; quiz 693.
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;146:1639-1648.
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;131:797-804.
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;63:3-12.
Spechler SJ, Genta RM, Souza RF: Thoughts on the complex relationship between gastroesophageal reflux disease and eosinophilic esophagitis. Am J Gastroenterol 2007;102:1301-1306.
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;62:824-832.
Krarup AL, Villadsen GE, Mejlgaard E, et al: Acid hypersensitivity in patients with eosinophilic oesophagitis. Scand J Gastroenterol 2010;45:273-281.
Moawad FJ, Veerappan GR, Dias JA, et al: Randomized controlled trial comparing aerosolized swallowed fluticasone to esomeprazole for esophageal eosinophilia. Am J Gastroenterol 2013;108:366-372.
Straumann A, Conus S, Degen L, et al: Budesonide is effective in adolescent and adult patients with active eosinophilic esophagitis. Gastroenterology 2010;139:1526-1537, 1537.e1.
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;10:742-749.e1.
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;139:418-429.
Arora AS, Perrault J, Smyrk TC: Topical corticosteroid treatment of dysphagia due to eosinophilic esophagitis in adults. Mayo Clin Proc 2003;78:830-835.
Hirano I: Therapeutic end points in eosinophilic esophagitis: is elimination of esophageal eosinophils enough? Clin Gastroenterol Hepatol 2012;10:750-752.
Schoepfer A, Safroneeva E, Straumann A: How to measure disease activity in eosinophilic esophagitis. Dis Esophagus 2015, Epub ahead of print.
Schroeder S, Fleischer DM, Masterson JC, et al: Successful treatment of eosinophilic esophagitis with ciclesonide. J Allergy Clin Immunol 2012;129:1419-1421.
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;6:165-173.
Attwood SE, Lewis CJ, Bronder CS, et al: Eosinophilic oesophagitis: a novel treatment using Montelukast. Gut 2003;52:181-185.
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;56:3551-3558.
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;19:865-869.
Straumann A, Bussmann C, Conus S, et al: Anti-TNF-alpha (infliximab) therapy for severe adult eosinophilic esophagitis. J Allergy Clin Immunol 2008;122:425-427.
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;59:21-30.
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;141:1593-1604.
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;129:456-463, 463.e1-e3.
Rocha R, Vitor AB, Trindade E, et al: Omalizumab in the treatment of eosinophilic esophagitis and food allergy. Eur J Pediatr 2011;170:1471-1474.
Straumann A, Hoesli S, Bussmann CH, et al: Anti-eosinophil activity and clinical efficacy of the CRTH2 antagonist OC000459 in eosinophilic esophagitis. Allergy 2013;68:375-385.
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;105:1062-1070.
Moawad FJ, Cheatham JG, DeZee KJ: Meta-analysis: the safety and efficacy of dilation in eosinophilic oesophagitis. Aliment Pharmacol Ther 2013;38:713-720.
Lipka S, Keshishian J, Boyce HW, Estores D, Richter JE: The natural history of steroid-naïve eosinophilic esophagitis in adults treated with endoscopic dilation and proton pump inhibitor therapy over a mean duration of nearly 14 years. Gastrointest Endosc 2014;80:592-598.
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