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Management Guidelines of Eosinophilic Esophagitis in Childhood

Papadopoulou, A.*; Koletzko, S.; Heuschkel, R.; Dias, J.A.§; Allen, K.J.||; Murch, S.H.; Chong, S.#; Gottrand, F.**; Husby, S.††; Lionetti, P.‡‡; Mearin, M.L.§§; Ruemmele, F.M.||||; Schäppi, M.G.¶¶; Staiano, A.##; Wilschanski, M.***; Vandenplas, Y.†††for the ESPGHAN Eosinophilic Esophagitis Working Group and the Gastroenterology Committee

Journal of Pediatric Gastroenterology and Nutrition: January 2014 - Volume 58 - Issue 1 - p 107–118
doi: 10.1097/MPG.0b013e3182a80be1
Medical Position Paper

Objectives: Eosinophilic esophagitis (EoE) represents a chronic, immune/antigen-mediated esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation. With few exceptions, 15 eosinophils per high-power field (peak value) in ≥1 biopsy specimens are considered a minimum threshold for a diagnosis of EoE. The disease is restricted to the esophagus, and other causes of esophageal eosinophilia should be excluded, specifically proton pump inhibitor–responsive esophageal eosinophilia. This position paper aims at providing practical guidelines for the management of children and adolescents with EoE.

Methods: Relevant literature from searches of PubMed, CINAHL, and recent guidelines was reviewed. In the absence of an evidence base, recommendations reflect the expert opinion of the authors. Final consensus was obtained during 3 face-to-face meetings of the Gastroenterology Committee and 1 teleconference.

Results: The cornerstone of treatment is an elimination diet (targeted or empiric elimination diet, amino acid–based formula) and/or swallowed, topical corticosteroids. Systemic corticosteroids are reserved for severe symptoms requiring rapid relief or where other treatments have failed. Esophageal dilatation is an option in children with EoE who have esophageal stenosis unresponsive to drug therapy. Maintenance treatment may be required in case of frequent relapse, although an optimal regimen still needs to be determined.

Conclusions: EoE is a chronic, relapsing inflammatory disease with largely unquantified long-term consequences. Investigations and treatment are tailored to the individual and must not create more morbidity for the patient and family than the disease itself. Better maintenance treatment as well as biomarkers for assessing treatment response and predicting long-term complications is urgently needed.

*Division of Gastroenterology & Nutrition, First Department of Pediatrics, University of Athens, Children's Hospital Agia Sophia, Athens, Greece

Dr. von Haunersches Kinderspital, Ludwig-Maximilians-University, Munich, Germany

Department of Pediatric Gastroenterology, Addenbrookes Hospital, Cambridge, UK

§Department of Pediatrics, Hospital S. João, Porto, Portugal

||Department of Allergy and Immunology, Department of Gastroenterology, University of Melbourne Department of Paediatrics, Murdoch Children's Research Institute, Royal Children's Hospital, Parkville, Victoria, Australia

Division of Metabolic and Vascular Health, Warwick Medical School, University of Warwick, Coventry, UK

#Queen Mary's Hospital for Children, Epsom & St Helier University Hospitals NHS Trust, Carshalton, Surrey, UK

**Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Jeanne de Flandre University Hospital, University of Lille, Lille, France

††Hans Christian Andersen Children's Hospital, OUH, Odense, Denmark

‡‡Pediatric Gastroenterology & Nutrition Unit, Department of Sciences for Woman and Child Health, University of Florence, Meyer Children's Hospital, Florence, Italy

§§Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands

||||Université Paris Descartes, Sorbonne Cité, Paris, and APHP, Hôpital Necker Enfants Malades, Pediatric Gastroenterology, Paris, France

¶¶Pediatric Center, Clinique des Grangettes, Geneva and Centre Médical Universitaire, Geneva, Switzerland

##Department of Pediatrics, University of Naples “Federico II,” Naples, Italy

***Pediatric Gastroenterology Unit, Division of Pediatrics, Hadassah University Hospital, Jerusalem, Israel

†††Vrije Universiteit Brussel, Brussels, Belgium.

Address correspondence and reprint requests to Alexandra Papadopoulou, Division of Gastroenterology and Nutrition, First Department of Pediatrics, University of Athens, Children's Hospital Agia Sophia, Thivon & Papadiamantopoulou, 11527 Athens, Greece (e-mail:

Accepted 14 July, 2013

A.P. has received speaker's honoraria from Danone and Ferring and a research grant from Biogaia. S.K. is a consultant and speaker for Abbott, Danone (Nutricia), Merck-Sharpe-Dohme, and Nestlé Nutrition, and has received research grants from Mead Johnson and Nestlé Nutrition. R.H. is a lecturer for Danone, Mead Johnson, and Merck-Sharpe-Dohme, and has received unrestricted support for educational events from Nestle, Biogaia, and Merck-Sharpe-Dohme. J.A.D. is a lecturer for Danone, Mead Johnson, and United Pharmaceuticals (Novolac). K.J.A. has received speaker's honoraria from Nutricia, Abbott, and Pfizer. S.H.M. has received compensation for lectures and is a member of advisory panels for Danone, Nutricia, and Mead Johnson. F.G. is a consultant for Nutricia Clinical Nutrition and has received research grants from Danone and Nestlé. S.H. has received speaker's honorarium from Thermo-Fisher. P.L. serves on the advisory board of Abvie and is a lecturer for Danone, Nutricia, and Nestlé Nutrition. F.M.R. is a consultant, advisory board member, or speaker for Merck-Sharpe-Dohme, Janssen, Nestlé, Danone, and Biocodex. A.S. is on the advisory board of Movetis, is a consultant for D.M.G. Italy, and serves on the speakers’ bureaus of Valeas and Mead Johnson. Y.V. lectures for Abbott, Biocodex, Danone (Nutricia), Mead Johnson, Nestlé Nutrition, and United Pharmaceuticals (Novalac). The other authors report no conflicts of interest.

© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,