2012-03-26 16:00:15 UTC

Diagnosis and Management of EoE in Children and Adults

March 26, 2012

NAME

Khoa Tran, MD

Pediatric Gastroenterologist, Massachusetts General Hospital for Children, Boston

NAME

Braden Kuo, MD

Assistant Physician, GI Unit, Massachusetts General Hospital, Boston

 It is only recently that eosinophilic esophagitis (EoE) has emerged as an independent clinical-pathologic diagnosis. Recent animal and human studies have shown that EoE represents an antigen-driven immunologic process with multiple pathogenic pathways. The key characteristics of EoE are clinical symptoms related to esophageal dysfunction (dysphagia, chest pain, heartburn, impaction) and histological findings of eosinophilic inflammation.

Diagnosis

Endoscopy with esophageal biopsies is the most reliable method to diagnose EoE. The classic endoscopic features are fixed or transient esophageal rings, vertical furrowing and presence of whitish exudates. Other associated findings include diffuse esophageal narrowing, or narrow caliber esophagus, and mucosal fragility. However, as these findings are present in other esophageal conditions, none are pathognomic for EoE. Current guidelines use an eosinophil count of greater than 15 per high-power field as a cutoff for diagnosis of EoE.1 Importantly, PPI therapy is used to differentiate PPI-responsive esophageal eosinophilia from EoE. If the upper intestinal symptoms and eosinophilia improve on antacids, the diagnosis is likely GERD and not EoE.

Until recently, many gastroenterologists who treat adults did not routinely perform esophageal biopsies. There was a time when we did not obtain esophageal biopsies in a symptomatic patient if the esophagus appeared normal endoscopically. Now if a patient presents with upper intestinal symptoms, we will obtain at least two biopsies of the distal and proximal esophagus. In a study of 222 patients with dysphagia who underwent endoscopy, including esophageal biopsy, 21 patients had endoscopic features suggestive of EoE, yet only eight (38 percent) had histological evidence of EoE. Of 102 patients with a visually normal endoscopy, 10 (9.8 percent) had histologic evidence of EoE.2 Both findings underscore the importance of biopsies in diagnosing EoE. As more adult gastroenterologists biopsy normal-appearing tissue, a commonplace practice in pediatrics, the incidence of EoE will likely increase.

Ultimately, EoE is a clinic-pathologic diagnosis. Esophageal eosinophilia in an asymptomatic patient is not EoE. Likewise, the presence of symptoms without identifiable esophageal eosinophils should prompt other considerations such as esophageal dysmotility or visceral hypersensitivity.

Management

Acid suppression with high-dose PPI continues to be an option in the management of EoE, as some patients with secondary GERD can receive symptomatic relief with antacids. We usually treat as recommended with PPIs at a dose of 20 to 40 mg once to twice daily for eight to 12 weeks.1 Topical corticosteroids continue to be a mainstay of effective therapy in treating EoE. Budesonide (swallowed as a suspension) and fluticasone (puffed and swallowed through a metered dose inhaler) are the two most commonly used corticosteroids. Both can induce clinical and histological improvements of EoE, yet the disease has a high recurrence rate when discontinuing steroid therapy. Often, patients will need to be on maintenance steroid therapy for months to years. As a result, they are at risk for steroid-related complications such as local fungal infection, decreased bone density or growth retardation in the pediatric population. The challenge is finding the equipoise between symptomatic relief and risks of long-term treatment.

In pediatric patients, a key treatment modality for EoE is dietary therapy. Approaches include the use of an amino acid-based formula, directed dietary restriction based on allergy testing or empiric elimination of the most common food antigens (cow’s milk, soy, wheat, egg, peanut/tree nut and seafood). Dietary therapy can often lead to a full resolution of the clinical and histological signs of EoE,3 with the elemental formula diet being the most effective.

In contrast, dietary therapy is not widely adopted in adults. Practitioners who treat adults have less experience with dietary restriction as it relates to allergens even though these issues are commonly managed by pediatricians and allergists. A recent study has shown that in adults with EoE, an allergy evaluation yielded identification of one or more allergens in 81 percent of referred patients, and one or more skin tests to foods were positive in 50 percent.4 A preliminary report from 2008 showed that in 23 adults placed on the six-food elimination diet, 94 percent had improved symptoms and 78 percent had histological improvement. In six patients who underwent food reintroduction, a specific food antigen was identified in triggering the esophageal eosinophilia.5 These preliminary observations highlight the potential of dietary therapy as an effective treatment strategy for adult EoE. Though further research needs to be undertaken, adults with EoE who are motivated and compliant should be a candidate for dietary therapy.1 Ironically, maybe it is time for the adult gastroenterologists to “grow up” and consider some of the practices of our pediatric colleagues regarding this treatment option.

References

1. Liacouras CA, Furuta GT, Hirano I, et al. Eosinophilic esophagitis: updated consensus recommendations for children and adults. J Allergy Clin Immunol. 2011 Jul;128(1):3-20.

2. Prasad GA, Talley NJ, Romero Y, Arora AS, Kryzer LA, Smyrk TC, et al. Prevalence and predictive factors of eosinophilic esophagitis in patients presenting with dysphagia: a prospective study. Am J Gastroenterol 2007;102:2627-32.

3. Spergel JM, Brown-Whitehorn TF, Beausoleil JL, Franciosi J, Shuker M, Verma R, et al. 14 years of eosinophilic esophagitis: clinical features and prognosis. J Pediatr Gastroenterol Nutr 2009;48:30-6.

4. Penfield JD, Lang DM, Goldblum JR, et al. The role of allergy evaluation in adults with eosinophilic esophagitis. J Clin Gastroenterol 2010. 44(1), 22–27 (2010).

5. Gonsalves N, Yang GY, Doerfler B, et al. A prospective clinical trial of six food elimination diet and reintroduction of causative agents in adults with eosinophilic esophagitis. Gastroenterology. 2008;134 (Suppl):A104.

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