Eosinophilic Esophagitis Seema S. Aceves,
MD, PhD
KEYWORDS Eosinophilic esophagitis Pathogenesis Clinical features Treatment options Fibrosis Remodeling KEY POINTS Eosinophilic esophagitis (EoE) is a clinicopathologic disease of increasing prevalence in adults and children. EoE is a chronic disease and as such its prevalence will continue to rise. Antigen triggers drive eosinophilic and T helper cell type 2 inflammation, resulting in subepithelial fibrosis; this esophageal remodeling is the likely underlying pathogenesis for complications. Management strategies include dietary antigen elimination and topical corticosteroids. Long-term therapy and repeated endoscopy are needed in most subjects so consideration must be given to maintenance regimens and side effects.
INTRODUCTION
Eosinophilic esophagitis (EoE) is a chronic, antigen-mediated immune disease leading to esophageal stricture and dysmotility.1,2 EoE is a clinicopathologic diagnosis that requires endoscopy with biopsy that demonstrates more than 15 eosinophils per high power field.1 Other causes of esophageal eosinophilia including acid-induced eosinophilia, drug reaction, hypereosinophilic syndrome, infection, and celiac disease, and must be ruled out before making a diagnosis of primary EoE.1,3–7 Complications of EoE include stricture formation and adult studies clearly demonstrate that the absence of inflammatory control leads to progressive fibrostenosis with reduced esophageal compliance, increased esophageal rigidity, and risk of food impactions.8–11 Symptoms in adults, adolescents, and children with longstanding or difficult-to-control EoE include dysphagia; younger children have vomiting, poor appetite, poor weight gain, and abdominal pain.1,12 The mechanisms of fibrostenosis lie in esophageal remodeling with epithelial–mesenchymal transformation,13 increased collagen and fibronectin deposition,14,15 subepithelial fibrosis, inflammation and angiogenesis,14,16 and altered smooth muscle function.15,17
Funding Sources: NIH/NIAID AI 092135, DOD FA100044. Department of Pediatrics and Medicine, Division of Allergy and Immunology, Center for Infection, Inflammation, and Immunology, 9500 Gilman Drive, MC-0760, La Jolla, CA 92093, USA E-mail address: [email protected] Immunol Allergy Clin N Am 35 (2015) 145–159 http://dx.doi.org/10.1016/j.iac.2014.09.007 immunology.theclinics.com 0889-8561/15/$ – see front matter Ó 2015 Elsevier Inc. All rights reserved.
146
Aceves
This review describes the clinical features, treatment options, epidemiology, and pathogenesis of EoE in children and adults. CLINICAL FEATURES
The EoE population is clinically allergic in multiple end organs.1 Up to 70% of subjects have asthma, 4% to 75% have allergic rhinitis, up to 5% to 43% have reported food allergies with anaphylaxis, and more than 80% have sensitization to foods using serum, skin prick, or atopy patch tests to foods.1 This high predisposition to other allergic diatheses must be considered during disease management. Symptoms of EoE shift with age—the youngest children have subjective symptoms of vomiting, poor appetite, and poor growth; older children complain of abdominal pain; and adolescents and adults complain mainly of dysphagia.1,18 Dysphagia is the single most common reason for endoscopy in EoE adults and EoE accounts for 46% to 63% of subjects who are evaluated for food impactions.19 Although EoE can be diagnosed at any age, it is most common in a younger population (
MD, PhD
KEYWORDS Eosinophilic esophagitis Pathogenesis Clinical features Treatment options Fibrosis Remodeling KEY POINTS Eosinophilic esophagitis (EoE) is a clinicopathologic disease of increasing prevalence in adults and children. EoE is a chronic disease and as such its prevalence will continue to rise. Antigen triggers drive eosinophilic and T helper cell type 2 inflammation, resulting in subepithelial fibrosis; this esophageal remodeling is the likely underlying pathogenesis for complications. Management strategies include dietary antigen elimination and topical corticosteroids. Long-term therapy and repeated endoscopy are needed in most subjects so consideration must be given to maintenance regimens and side effects.
INTRODUCTION
Eosinophilic esophagitis (EoE) is a chronic, antigen-mediated immune disease leading to esophageal stricture and dysmotility.1,2 EoE is a clinicopathologic diagnosis that requires endoscopy with biopsy that demonstrates more than 15 eosinophils per high power field.1 Other causes of esophageal eosinophilia including acid-induced eosinophilia, drug reaction, hypereosinophilic syndrome, infection, and celiac disease, and must be ruled out before making a diagnosis of primary EoE.1,3–7 Complications of EoE include stricture formation and adult studies clearly demonstrate that the absence of inflammatory control leads to progressive fibrostenosis with reduced esophageal compliance, increased esophageal rigidity, and risk of food impactions.8–11 Symptoms in adults, adolescents, and children with longstanding or difficult-to-control EoE include dysphagia; younger children have vomiting, poor appetite, poor weight gain, and abdominal pain.1,12 The mechanisms of fibrostenosis lie in esophageal remodeling with epithelial–mesenchymal transformation,13 increased collagen and fibronectin deposition,14,15 subepithelial fibrosis, inflammation and angiogenesis,14,16 and altered smooth muscle function.15,17
Funding Sources: NIH/NIAID AI 092135, DOD FA100044. Department of Pediatrics and Medicine, Division of Allergy and Immunology, Center for Infection, Inflammation, and Immunology, 9500 Gilman Drive, MC-0760, La Jolla, CA 92093, USA E-mail address: [email protected] Immunol Allergy Clin N Am 35 (2015) 145–159 http://dx.doi.org/10.1016/j.iac.2014.09.007 immunology.theclinics.com 0889-8561/15/$ – see front matter Ó 2015 Elsevier Inc. All rights reserved.
146
Aceves
This review describes the clinical features, treatment options, epidemiology, and pathogenesis of EoE in children and adults. CLINICAL FEATURES
The EoE population is clinically allergic in multiple end organs.1 Up to 70% of subjects have asthma, 4% to 75% have allergic rhinitis, up to 5% to 43% have reported food allergies with anaphylaxis, and more than 80% have sensitization to foods using serum, skin prick, or atopy patch tests to foods.1 This high predisposition to other allergic diatheses must be considered during disease management. Symptoms of EoE shift with age—the youngest children have subjective symptoms of vomiting, poor appetite, and poor growth; older children complain of abdominal pain; and adolescents and adults complain mainly of dysphagia.1,18 Dysphagia is the single most common reason for endoscopy in EoE adults and EoE accounts for 46% to 63% of subjects who are evaluated for food impactions.19 Although EoE can be diagnosed at any age, it is most common in a younger population (
