Eosinophilic Esophagitis and the Eosinophilic Gastrointestinal Diseases: Approach to Diagnosis and Management

Abstract

The eosinophilic gastrointestinal diseases (EGIDs) represent disorders of the gastrointestinal (GI) tract that result from the local infiltration and aberrant activity of eosinophils and other immune cells. Eosinophilic esophagitis (EoE) is the most well-characterized EGID and is defined by the presence of intraepithelial eosinophils in the esophagus (≥15 eosinophils per high-powered field) and clinical symptoms associated with esophageal dysfunction. The other EGIDs are rare and lack strong data regarding pathogenesis and management. The incidence and prevalence of EoE are increasing, and EoE is now a major cause of upper GI morbidity. Management is multidisciplinary, with collaboration between gastroenterologists, allergists, pathologists, and dieticians, and is aimed at amelioration of symptoms and prevention of long-term complications such as esophageal stricture. Treatment options for EoE include proton pump inhibitors, swallowed topical corticosteroids, and elimination diets. Esophageal dilation is used when esophageal strictures or fibrostenotic changes are present. Additional therapies targeting eosinophils and other mediators of Th2 inflammation are under development and are promising. Treatment options for other EGIDs typically involve corticosteroids or dietary elimination.

Keywords: Eosinophilic esophagitis; Eosinophilic gastrointestinal disease.

Figure 1.

A range of endsocopic findings in EoE with application of the EREFS classification, which measures the 5 main endoscopic features of EoE: Edema, Rings, Exudates, Furrows, and Strictures. Edema is graded as absent (0) or present (1); Rings are graded as absent (0), mild (1), moderate (2), or severe (3); Exudates are graded as absent (0), mild (1), or severe (2); Furrows are graded as absent (0), mild (1), or severe (2); Stricture are graded as absent (0) or present (1), and if present the inner diameter can also be reported. (A) A patient with suspected EoE, but with a normal endoscopy. (B) A patient with edema, exudates, and furrows. (C) A patient with edema, rings, exudates, furrows, and a stricture. (D) A patient with edema, with edema, rings, exudates, furrows, and a stricture. (E) A patient with edema and furrows. (F) A patient with edema, rings, exudates, and furrows.

Figure 2.

Dietary reintroduction of food allergen groups for patients with EoE. Modified with permission from Gastrointest Endosc Clin N Am. 2008;18(1):179–94; xi (148) and Gastroenterology. 2014;147(6):1238–54 (96).

Figure 3.

Algorithm for diagnosis and management of EoE. “Response” refers to <15 eos/hpf with resolution of symptoms. “No Response” refers to >15 eos/hpf and persistence of symptoms. For patients with a histologic response but with ongoing symptoms, assessment should be made for a persistent esopahgeal stricture or alternative cause of symptoms (dysmotility, viserceral hypersensitivity, etc). Patients with no histologic reponse but with resolution of symptoms may have undergone prior esophageal dilation and should be assessed for dietary avoidance or modification behaviors.