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A TFGED diet achieves EoE remission in 43% of children and adults. A step-up approach results in early identification of a majority of responders to an empiric diet with few food triggers, avoiding unnecessary dietary restrictions, saving endoscopies, and shortening the diagnostic process.

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Objectives: Epidemiologic studies on eosinophilic esophagitis (EoE) are scarce and patient responders to proton pump inhibitor (PPI) therapy have usually been excluded. We aimed to evaluate population-based incidence rates, prevalence and trends in adult EoE over the past decade, including responders to PPI therapy. Methods: We conducted an analysis of a prospectively established case registry in the health area of Cáceres, located in midwestern Spain. From the first EoE case diagnosed in 2007, endoscopy and pathology reports up to December 2016 were manually reviewed. A baseline diagnosis of EoE was confirmed upon symptoms of esophageal dysfunction (dysphagia/food impaction) and esophageal eosinophilia 15 eos/HPF. All patients were re-evaluated on PPI therapy during follow-up. Results: A total of 137 patients were diagnosed with EoE during the study period, of whom 63 (46%) achieved clinicohistologic remission on PPI therapy. The prevalence of autoimmune disorders was low. Mean incidence rate was 8.09 new cases/100,000 inhabitants/year, increasing to 9.95 during the last lustrum and peaking in 2016 with 13.7. This trend coincided with late declining of esophageal biopsies rate. Overall prevalence in 2016 was 81.73 patients/100,000 inhabitants, with the highest prevalence in males between age 35 and 44 years (273 cases/100,000). No seasonal variation was observed in the diagnosis of EoE (53% during pollen season vs. 47%, p ¼ 0.4). Conclusions: In midwestern Spain, incidence (13.7 cases/100,000 inhabitants/year) and prevalence (81.73 patients/100,000 inhabitants) in 2016 have grown remarkably in just one decade, coming closer to those figures recently reported for Crohn's disease in Spain.

Eosinophilic esophagitis (EoE) is a chronic inflammatory esophageal disease triggered predominantly, but not excusively, by food antigens. Elimination diet thus remains the only therapy targeting the cause of the disease. Importantly, EoE is a unique form of non-IgE mediated food allergy, largely dependant upon delayed, cell-mediated hypersensitivity. Areas covered: A comprehensive review of literature to summarize and update the most relevant advances on dietary therapy for pediatric and adult EoE patients is conducted. Expert commentary: None of the currently available food allergy tests adequately predict food triggers for EoE, especially in adults. Elemental diet (exclusive feeding with aminoacid-based formulas) and empiric six-food elimination diet, withdrawing cow´s milk, wheat, egg, soy, nuts and fish/seafood for 6 weeks, have consistently shown the best cure rates. However, their high level of restriction and need for multiple endoscopies (top-down approach) have been a deterrent for patients and physicians. Less restrictive empiric schemes, like a four-food (animal milk, gluten-containing cereals, egg, legumes) or a two-food (animal milk and gluten-containing cereals) elimination diet have lately shown encouraging results. Therefore, a novel step-up strategy (2-4-6) may enhance patient uptake and promptly identify most responders to empiric diets with few food triggers, besides saving unnecessary dietary restrictions and endoscopic procedures.

Proton pump inhibitor-responsive esophageal eosinophilia is an inappropriate disease descriptor, arbitrarily based on a response to a single drug, and should be abandoned. Patients who have esophageal eosinophilia and esophageal symptoms that resolve with PPI therapy have phenotypic, molecular, mechanistic, and therapeutic features indistinguishable from similar patients who do not respond to PPIs. These patients with PPI responsiveness should be considered within the spectrum of EoE.

Eosinophilic esophagitis (EoE) is a chronic esophageal disease characterized by a Th2 inflammatory response triggered by food/environmental allergens. Solid data confirm that up to half of patients with suspected EoE achieve complete remission on proton pump inhibitors (PPI) therapy. This disease phenotype is currently labelled as PPI-responsive esophageal eosinophilia (PPI-REE). Albeit initially believed to represent gastro-esophageal reflux disease (GERD), evolving evidence has underscored that PPI-REE and EoE show a significant overlap regarding clinic, endoscopic, histologic, Th2 immune-mediated inflammation and gene expression features. Moreover, PPI therapy can effectively reverse Th2 inflammation and the EoE transcriptome expression in PPI-REE patients. Therefore, EoE and PPI-REE likely represent a common allergic disorder, where PPI therapy should be considered a short- and long-term therapeutic asset, along with diet and topical steroids.

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