Management Guidelines of Eosinophilic Esophagitis in Childhood
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MEDICAL POSITION PAPER Management Guidelines of Eosinophilic Esophagitis in Childhood ÃA. Papadopoulou, yS. Koletzko, zR. Heuschkel, §J.A. Dias, jjK.J. Allen, ôS.H. Murch, #S. Chong, ÃÃF. Gottrand, yyS. Husby, zzP. Lionetti, §§M.L. Mearin, jjjjF.M. Ruemmele, ôôM.G. Scha¨ppi, ##A. Staiano, ÃÃÃM. Wilschanski, and yyyY. Vandenplas, for the ESPGHAN Eosinophilic Esophagitis Working Group and the Gastroenterology Committee ABSTRACT 07/08/2020 on BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3SN2oLfdKwVfOd8N1BUgoFLqFCJm0ieBLGeEi8TPX5tc= by https://journals.lww.com/jpgn from Downloaded Downloaded esophageal stenosis unresponsive to drug therapy. Maintenance treatment Objectives: Eosinophilic esophagitis (EoE) represents a chronic, immune/ may be required in case of frequent relapse, although an optimal regimen from antigen-mediated esophageal disease characterized clinically by symptoms still needs to be determined. https://journals.lww.com/jpgn related to esophageal dysfunction and histologically by eosinophil-predo- Conclusions: EoE is a chronic, relapsing inflammatory disease with largely minant inflammation. With few exceptions, 15 eosinophils per high-power unquantified long-term consequences. Investigations and treatment are field (peak value) in 1 biopsy specimens are considered a minimum tailored to the individual and must not create more morbidity for the threshold for a diagnosis of EoE. The disease is restricted to the esophagus, patient and family than the disease itself. Better maintenance treatment and other causes of esophageal eosinophilia should be excluded, specifically as well as biomarkers for assessing treatment response and predicting long- proton pump inhibitor–responsive esophageal eosinophilia. This position by term complications is urgently needed. BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3SN2oLfdKwVfOd8N1BUgoFLqFCJm0ieBLGeEi8TPX5tc= paper aims at providing practical guidelines for the management of children and adolescents with EoE. Key Words: amino acid–based formula, empiric elimination diet, Methods: Relevant literature from searches of PubMed, CINAHL, and eosinophilic esophagitis, local steroids, systemic steroids, targeted recent guidelines was reviewed. In the absence of an evidence base, elimination diet recommendations reflect the expert opinion of the authors. Final consensus was obtained during 3 face-to-face meetings of the (JPGN 2014;58: 107–118) 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, osinophilic esophagitis (EoE) is a chronic immune/antigen- topical corticosteroids. Systemic corticosteroids are reserved for severe E mediated esophageal inflammatory disease associated with symptoms requiring rapid relief or where other treatments have failed. esophageal dysfunction resulting from severe eosinophil-predomi- Esophageal dilatation is an option in children with EoE who have nant inflammation (1,2). In 2007, a multidisciplinary group of Accepted July 14, 2013. From the ÃDivision of Gastroenterology & Nutrition, First Department of Pediatrics, University of Athens, Children’s Hospital Agia Sophia, Athens, Greece, the yDr. von Haunersches Kinderspital, Ludwig-Maximilians-University, Munich, Germany, the zDepartment of Pediatric Gastroenterology, Adden- brookes Hospital, Cambridge, UK, the §Department of Pediatrics, Hospital S. Joa˜o, Porto, Portugal, the jjDepartment 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, the ôDivision of Metabolic and Vascular Health, Warwick Medical School, University of Warwick, Coventry, UK, the #Queen Mary’s Hospital for Children, Epsom & St Helier University Hospitals NHS Trust, Carshalton, Surrey, UK, the ÃÃDepartment of Pediatric Gastroenterology, Hepatology, and Nutrition, Jeanne de Flandre University Hospital, University of Lille, Lille, France, the yyHans Christian Andersen Children’s Hospital, OUH, Odense, Denmark, the zzPediatric Gastroenterology & Nutrition Unit, Department of Sciences for Woman and Child Health, University of Florence, Meyer Children’s Hospital, Florence, Italy, the §§Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands, the jjjjUniversite´ Paris Descartes, Sorbonne Cite´, Paris, and APHP, Hoˆpital Necker Enfants Malades, Pediatric Gastroenterology, Paris, France, the ôôPediatric Center, Clinique des Grangettes, Geneva and Centre Me´dical Universitaire, Geneva, Switzerland, the ##Department of Pediatrics, on 07/08/2020 University of Naples ‘‘Federico II,’’ Naples, Italy, the ÃÃÃPediatric Gastroenterology Unit, Division of Pediatrics, Hadassah University Hospital, Jerusalem, Israel, and the yyyVrije 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: [email protected]). 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 Nestle´ Nutrition, and has received research grants from Mead Johnson and Nestle´ 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 Nestle´. 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 Nestle´ Nutrition. F.M.R. is a consultant, advisory board member, or speaker for Merck-Sharpe-Dohme, Janssen, Nestle´, 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, Nestle´ Nutrition, and United Pharmaceuticals (Novalac). The other authors report no conflicts of interest. Copyright # 2013 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition DOI: 10.1097/MPG.0b013e3182a80be1 JPGN Volume 58, Number 1, January 2014 107 Copyright 2013 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited. ESPGHAN EoE Working Group/GI Committee JPGN Volume 58, Number 1, January 2014 experts published the first consensus recommendations for the failure to thrive. During childhood, vomiting and/or abdominal or diagnosis and treatment of EoE (1), which were recently updated retrosternal pain are reported, whereas during adolescence, gastro- (2). The updated definition of the disease includes the histological esophageal reflux disease (GERD) symptoms, dysphagia, and food presence of 15 eosinophils per high power field (eos/hpf) in at impaction are the most frequent symptoms (9,11). least 1 endoscopic esophageal mucosal biopsy (peak value) taken at Peripheral eosinophilia (>700 cells/mm3) has been reported upper gastrointestinal endoscopy; and/or the presence of other in children with EoE (12). Furthermore, specific immunoglobulin E microscopic features of eosinophilic inflammation such as eosino- (IgE) antibodies to foods (13) may be found in children with EoE philic microabscesses, superficial layering, or extracellular eosino- identifying sensitization to foods which may (or may not) be the phil granules (2). These publications provide extensive information causative foods of the disease. Typical endoscopic findings include on pathogenesis, epidemiology, clinical presentation, diagnosis, esophageal rings, a thickened, sometimes pale mucosa with linear and management of EoE in both adults and children; however, a furrows and white exudates and less often, narrowing of the caliber practical algorithm on the optimal treatment of children with EoE, of the esophagus. A normal esophagus at endoscopy does not to guide clinical practice, is lacking. This position paper of the exclude the diagnosis of EoE. Mucosal breaks (erosions or ulcera- Eosinophilic Esophagitis Working Group (see Appendix) and the tion) are not findings of EoE and are indicative for GERD, Crohn Gastroenterology Committee of ESPGHAN aims at providing disease, or other diagnoses. According to Shah et al, at least 3 practical guidelines for the management of children and adolescents esophageal biopsy specimens taken from different parts of the with EoE, based on available evidence where possible. If sufficient esophagus are necessary to achieve a diagnosis of EoE in 97% evidence is lacking, our recommendations are based on expert of patients (14). According to Gonsalves et al (15), 1 biopsy opinion and personal practice. specimen has a sensitivity of only 55%, whereas 5 biopsies increase this to 100%. To maximize diagnostic sensitivity,