Eosinophilic Esophagitis

Eosinophilic Esophagitis: Excerpt from The 5-Minute Pediatric Consult

James P. Franciosi, MD, MSChris A. Liacouras, MD

Eosinophilic Esophagitis - BASICS

Eosinophilic Esophagitis - description

• Eosinophilic esophagitis (EE) is a clinical, pathologic diagnosis characterized by a localized eosinophilic inflammation of the esophagus.
  • Symptoms can be similar to gastroesophageal reflux.
  • Esophageal endoscopic biopsies are required to establish the diagnosis.
  • On endoscopic mucosal biopsies, EE is defined as the presence of at least 15 eosinophils per high-powered field isolated to the esophagus that does not respond to acid blockade medication.

Eosinophilic Esophagitis - pathophysiology

The pathophysiology of EE is unknown; however, it has been linked to an allergic response to food antigens that does not follow a typical IgE mediated pattern. As of 2003, the incidence and prevalence of EE in children 0–19 years of age is thought to be respectively 1 and 4.3 per 10,000 children.

Eosinophilic Esophagitis - DIAGNOSIS

Eosinophilic Esophagitis - signs & symptoms

• Vomiting
• Regurgitation
• Dysphagia
• Nausea
• Epigastric pain
• Heartburn
• Chest pain
• Esophageal food impaction
• Irritability/Feeding difficulties
• Nighttime cough
• Other complications that can occur with EE include failure to thrive, malnutrition, feeding intolerance, esophageal strictures, hiatal hernia, small caliber esophagus and esophageal perforation. Esophageal fungal or viral superinfection may also occur. Any patient who is being considered for surgical correction of gastroesophageal reflux (fundoplication) should first be evaluated endoscopically for EE to prevent unnecessary surgery.

Eosinophilic Esophagitis - history

• EE in the infant and younger child:
  • Pay attention to infants with chronic gastroesophageal reflux
  • Pay attention to irritability, weight, vomiting, feeding difficulties
  • Personal or family history of atopic disease (asthma, allergic rhinitis, eczema)
  • Typically occurs in toddlers and older children, and is considered distinct from infant milk protein allergy
  • Should exclude other causes of vomiting, and failure to thrive
  • Special questions:
    • Personal or family history of atopic disease?
    • Family history of EE?
    • No relief/response to acid blockade medication?
• EE in the older child and adolescent:
  • Pay attention to heartburn, nausea, vomiting, epigastric pain
  • EE should be considered in any child presenting with dysphagia or food impaction
  • Diet history is important with notation of particular foods avoided or difficulty with eating
  • Should investigate for personal and family history of atopic disease, food allergy and esophageal food impaction
  • Special questions
    • Personal or family history of atopic disease?
    • Family history of EE?
    • No relief/response to acid blockade medication?
    • Are they a slow eater or do they avoid specific foods?
    • Any sensation of food sticking or difficulty swallowing?
    • Personal or family history of esophageal food impaction?

Eosinophilic Esophagitis - physical exam

• Typically normal
• Growth failure (rare, occurs if appetite decreased significantly)
• Allergic shiners, reactive airway disease
• Eczema

Eosinophilic Esophagitis - tests

• Upper endoscopy (Esophagastroduodenoscopy, EGD):
  • The gold standard for diagnosis of eosinophilic esophagitis. Typically performed after a trial of acid blockade medication for 6–8 weeks. Endoscopic mucosal biopsies should be obtained from the proximal and distal esophagus (2 from each location), the stomach and duodenum. A pathologist who has experience with EE should examine the mucosal biopsies for the presence of 15 or more eosinophils per high-powered field isolated to the esophagus.
• Upper GI series radiography:
  • Typically performed before endoscopy to exclude other etiologies of vomiting. May demonstrate esophageal stricture, corrugated esophagus, foreshortened esophagus, hiatal hernia, a small caliber esophagus or esophageal perforation. However, none of these findings are specific to EE.
• pH Probe:
  • Should be normal in patients with EE. Considered a gold standard for evaluation of acid reflux disease, but of limited role in the evaluation of EE
• Blood tests:
  • Peripheral eosinophilia is present in <50% of patients. Currently, there are no widely available serum markers for EE.
• Food allergy testing:
  • RAST (radio-allergo-sorbent test): Serum testing for food specific IgE antibodies. Low sensitivity, limited role.
  • Skin testing: Percutaneous prick puncture testing for food specific IgE mediated reactions
  • Patch testing (atopy patch testing): Allergen in prolonged contact with the skin in patches with the same concentration of food that is ingested. Testing for food specific non-IgE mediated reactions.
  • Food allergy testing is typically only performed after endoscopic confirmation of EE. Causative food antigens can be identified in 70% of patients through skin prick and patch testing. Although EE is considered a mixed IgE and non-IgE mediated disease, most reactions occur through non-IgE mediated pathways.

Eosinophilic Esophagitis - differencial diagnosis

• Gastroesophageal reflux
• Inflammatory bowel disease
• Eosinophilic gastroenteritis
• Celiac disease
• Food allergy
• Connective tissue disease
• Drug allergy
• Churg-Strauss syndrome
• Hypereosinophilic syndrome
• Autoimmune enteropathy
• Candida esophagitis
• Viral esophagitis (Herpes or CMV)

Eosinophilic Esophagitis - TREATMENT

Eosinophilic Esophagitis - general measures

Several modes of dietary and medication therapy are available depending on disease presentation and therapy.

Eosinophilic Esophagitis - diet

• Elimination diet has been shown to be effective for treatment of eosinophilic esophagitis. Whenever possible, specific allergen testing (skin and patch testing) should be performed to identify and remove specific causative food antigens.
  • Food allergen testing may be falsely negative, and empiric removal of highly antigenic foods has also had some success.
  • The 6 foods that are considered the most antigenic for EE include milk, soy, nuts, egg, wheat, and fish/shellfish.
• Some patients require an elemental diet.
  • Patients are allowed to drink only water and an amino acid based formula until the esophageal eosinophilia resolves; subsequently, food is gradually reintroduced.
  • This method frequently requires nasogastric tube feeding as these formulas are often unpalatable. Success with the elemental diet occurs in >95% of patients.

Eosinophilic Esophagitis - special therapy

• Endoscopic therapy: Esophageal dilatation is a useful therapy for EE patients with fixed esophageal strictures.
  • Care must be taken when performing dilatation as significant esophageal mucosal lacerations, tearing, and perforation have been reported.
  • Whenever possible, diagnostic endoscopy followed by a trial of dietary or steroid therapy is recommended prior to esophageal stricture dilation.
  • Patients presenting with esophageal food impaction should have the food bolus removed endoscopically, and esophageal biopsies should be obtained to investigate for EE.

Eosinophilic Esophagitis - medication

• Fluticasone propionate: A topical ingested steroid that is an alternative to dietary therapy. Aerosolized fluticasone spray is swallowed instead of being inhaled. At high doses, this therapy achieves histologic remission in >50% of patients. However, with discontinuation of the medication, almost all patients relapse. Reported side effects include esophageal candidiasis, epistaxis and dry mouth. Long-term effects on growth, bone health and esophageal fibrosis are currently not known. Recommended doses are 110–220 mcg (swallowed) b.i.d. for children <6 years of age, and 220–440 mcg b.i.d. for children >6 years of age.
• Corticosteroids: An effective treatment for EE, but symptoms and histologic eosinophilia return when these medications are discontinued. Given the extensive side effect profile, long-term corticosteroids are not considered an ideal treatment option for EE. Short courses of oral steroids may have a role in treating emergent EE patients who present with significant esophageal strictures, severe weight loss or the inability to eat most foods/liquids. The recommended dose of prednisone is 1–2 mg/kg/d (maximum 60 mg/d).
• Budesonide (Entocort): A steroid formulation with less systemic toxicity. There have been only case reports of its usage in EE.
• Leukotriene receptor antagonists (Montelukast): A therapy limited to adult EE studies, shown to improve clinical symptoms but not tissue histology.
• Oral cromolyn sodium: Not shown to be effective
• Monoclonal antibodies (anti-IL-5): Promising therapy with ongoing clinical trials

Eosinophilic Esophagitis - bibliography

1. Aceves SS, Dohil R, Newbury RO, et al. Topical viscous budesonide suspension for treatment of eosinophilic esophagitis. J Allergy Clin Immunol. 2005;116(3):705–6.
2. Arora AS, Perrault J, Smyrk TC. Topical corticosteroid treatment of dysphagia due to eosinophilic esophagitis in adults. Mayo Clin Proc. 2003;78(7):830–5.
3. Attwood SE, Lewis CJ, Bronder CS, et al. Eosinophilic oesophagitis: a novel treatment using Montelukast. Gut. 2003;52(2):181–5.
4. Kagalwalla AF, Sentongo TA, Ritz S, et al. Effect of six-food elimination diet on clinical and histologic outcomes in eosinophilic esophagitis. Clin Gastroenterol Hepatol. 2006;4(9):1097–102.
5. Kelly KJ, Lazenby AJ, Rowe PC, et al. Eosinophilic esophagitis attributed to gastroesophageal reflux: improvement with an amino acid-based formula. Gastroenterology. 1995;109(5):1503–12.
6. Konikoff MR, Noel RJ, Blanchard C, et al. A randomized, double-blind, placebo-controlled trial of fluticasone propionate for pediatric eosinophilic esophagitis. Gastroenterology. 2006;131(5):1381–91.
7. Liacouras CA. Eosinophilic esophagitis: treatment in 2005. Curr Opin Gastroenterol. 2006;22(2):147–52.
8. Liacouras CA, Spergel JM, Ruchelli E, et al. Eosinophilic esophagitis: a 10-year experience in 381 children. Clin Gastroenterol Hepatol. 2005;3(12):1198–206.
9. Liacouras CA, Wenner WJ, Brown K, et al. Primary eosinophilic esophagitis in children: successful treatment with oral corticosteroids. J Pediatr Gastroenterol Nutr. 1998;26(4):380–5.
10. Markowitz JE, Spergel JM, Ruchelli E, et al. Elemental diet is an effective treatment for eosinophilic esophagitis in children and adolescents. Am J Gastroenterol. 2003;98(4):777–82.
11. Noel RJ, Putnam PE, Collins MH, et al. Clinical and immunopathologic effects of swallowed fluticasone for eosinophilic esophagitis. Clin Gastroenterol Hepatol. 2004;2(7):568–75.
12. Spergel JM, Andrews T, Brown-Whitehorn TF, et al. Treatment of eosinophilic esophagitis with specific food elimination diet directed by a combination of skin prick and patch tests. Ann Allergy Asthma Immunol. 2005;95(4):336–43.
13. Stein ML, Collins MH, Villanueva JM, et al. Anti-IL-5 (inepolizumab) therapy for eosinophilic esophagitis. J Allergy Clin Immunol. 2006;118(6):1312–9.
14. Straumann A, Spichtin HP, Grize L, et al. Natural history of primary eosinophilic esophagitis: a follow-up of 30 adult patients for up to 11.5 years. Gastroenterology. 2003;125(6):1660–9.
15. Teitelbaum JE, Fox VL, Twarog FJ, et al. Eosinophilic esophagitis in children: immunopathological analysis and response to fluticasone propionate. Gastroenterology. 2002;122(5):1216–25.

Eosinophilic Esophagitis - CODES

Eosinophilic Esophagitis - icd9

530.1 Esophagitis

Eosinophilic Esophagitis - FAQ

• Q: Is EE considered a life-long disease or will it resolve over time?
• A: The long-term outcome for children with EE is unknown. Currently, the natural history of the disease suggests that EE is a chronic disease that can be controlled with diet or medical therapy.
• Q: Is there any harm in not treating asymptomatic patients with ongoing histologic EE?
• A: There is much debate about the progression of untreated EE. The concerns are that, if left untreated, ongoing esophageal eosinophilia may lead to dysphagia, strictures and esophageal fibrosis.
• Q: Is EE a genetic or environmental disease?
• A: EE is more common in white males, >50% of patients have atopic disease (such as asthma, eczema or allergic rhinitis), and 30–50% of patients with EE have a family history of EE or atopic disease.
• Q: Is the incidence of EE increasing?
• A: There has been a significant rise in the number of pediatric and adult EE diagnoses over the last 5 years. Although the exact explanation is unknown, the most likely reasons include both an improved recognition of the disease among gastroenterologists, allergists and pathologists as well as a rising incidence of new cases (similar to the increase seen in other allergic disorders).

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

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More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

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