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Table of Contents

What is an Eosinophil?
What is EE?
What are the Symptoms of EE?
How is EE Diagnosed?
Why is it so Difficult to Obtain a Diagnosis?
Treatment, Dietary, Medications
      Restricted diets
      Elemental diets
      Feeding tubes 
Resources for Healthcare Professionals and Consumers
Recipes
Frequently Asked Questions Answered by Doctors on our Medical Board

What is an Eosinophil?
Eosinophils, a type of white blood cell, are an important part of the immune system, helping us fight off certain types of infections, such as parasites. Many different problems can cause high numbers of eosinophils in the blood including allergies (food and environ-
mental), certain infections (caused by parasites), eosinophil associated gastrointestinal disorders, leukemia, and other problems. When eosinophils occur in higher than normal numbers in the body, without a known cause, an eosinophilic disorder may be present.

Eosinophilic disorders are further defined by the area affected. For instance, eosinophilic esophagitis means abnormal numbers of eosinophils in the esophagus.
What is EE?
Eosinophilic esophagitis is characterized by the infiltration of a large number of eosinophils, a type of white blood cell, in the esophagus (the tube connecting the mouth to the stomach). Eosinophils are an important part of the immune system, helping us fight off certain types of infections, such as parasites. A variety of stimuli may trigger this abnormal production and accumulation of eosinophils, including certain foods. Eosinophilic esophagitis means eosinophils infiltrating the esophagus, –itis means inflammation.
Eosinophil, Courtesy of Dr. Margaret Collins
People with EE commonly have other allergic diseases such as asthma or eczema. EE affects people of all ages, gender and ethnic backgrounds. In certain families, there may be an inherited (genetic) tendency. EE is thought to be the most common type of eosinophil-associated gastrointestinal disorder.
Learn more: Eosinophilic Esophagitis.pdf
Eosinophils are not normally present in the esophagus, although they may be found in small numbers in other areas of the gastrointestinal tract. Diseases other than EE can cause eosinophils in the esophagus including gastroesophageal reflux diseases (GERD), food allergy, and inflammatory bowel disease.

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What are the Symptoms of EE?
Symptoms vary from one individual to the next and may differ depending on age. Vomiting may occur more commonly in young children and difficulty swallowing in older individuals.

Common symptoms include:
• Reflux that does not respond to usual therapy
  (which includes proton pump inhibitors, a med-
  icine which stops acid production in the stomach)
• Dysphagia (difficulty swallowing)
• Food impactions (food gets stuck in the throat)
• Nausea and vomiting
• Failure to thrive (poor growth or weight loss)
• Abdominal or chest pain
• Poor appetite
• Malnutrition
• Difficulty sleeping
Upper Gastrointestinal Tract

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How is EE Diagnosed?
In individuals with symptoms consistent with EE, an upper endoscopy with biopsies is needed for the diagnosis. The endoscopy is often performed after treatment with reflux medications have failed to relieve the symptoms. Medications for reflux include proton pump inhibitors or histamine-2 receptor blockers.

During an upper endoscopy, the gastroenterologist looks at the esophagus, stomach and duodenum (first part of the small bowel) through an endoscope (small tube inserted through the mouth) and takes multiple small tissue samples (biopsies) which the pathologist reviews under the microscope. Even if the esophagus appears normal, the biopsies may show EE. A high number of eosinophils (counted per high power field) suggest the diagnosis of EE. GERD also causes eosinophils in the esophagus, but typically far fewer. The pathologist will also look for tissue injury, swelling and thickening of the esophageal layers. With EE, the eosinophils are limited to the esophagus and not found in other areas. Once the diagnosis of EE is confirmed, food allergy testing is typically recommended to guide treatment. Skin prick testing to different foods is the most common type of allergy testing.

The examples below are of endoscopic findings that may be seen in eosinophilic esophagitis*.
Furrows
Rings
White plaques
Images courtesy of Dr. Chris Liacouras, Children’s Hospital of Philadelphia.

For more detailed information on colonoscopy and upper endoscopy visit the American Gastroenterological Association.

Allergy Testing (skin prick, patch testing and RAST)
Once the diagnosis of EE is confirmed, allergy testing is typically requested. In many situations, avoiding ‘allergens’ that trigger the eosinophils will be effective treatment. The reactions to foods are not always ‘immediate hypersensitivity’ (IgE-mediated). This means that a food can be consumed with no obvious reaction to it, but over a period of days to weeks the eosinophils triggered by the food will cause inflammation and injury to the esophagus. For this reason, food logs (keeping track of foods and symptoms) may not identify the offending food. The skin testing will include skin prick testing and may also include patch testing (to look for delayed reactions).

Skin prick testing is for IgE- mediated reactions (‘immediate hypersensitivity’). Skin prick testing involves ‘scratching’ small amounts of pure food or environmental allergens into the skin. A ‘wheal’ (bump) greater than the negative control indicates a positive test. Both a positive control (one that should cause a wheal) and negative control (should not cause a wheal) are used.
Skin patch testing can be used when testing for delayed food reactions. Skin patch testing is most commonly used to test for dermatologic (skin) reactions. When used for food reactions, small amounts of a pure food are placed in tiny cups, which are then taped to the back. The foods will be chosen based on the patient’s diet, previous reactions, and prior skin prick test results. The patches are removed after 48 hours and read at 72 hours.

Example of Patch Testing
RAST (Radioallergosorbent test) is not as helpful for identifying foods that cause EE. Instead, RAST may be used to confirm an immediate reaction to a food (for instance, hives following a peanut butter sandwich). RAST testing identifies IgE antibodies for a specific food.
Skin Patch Testing



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Why is it so Difficult to Obtain a Diagnosis?
EE is a relatively uncommon disorder that doctors may not encounter often. The diagnosis of EE is often delayed, sometimes for years, because of lack of awareness of these disorders.

Although doctors may have minor disagreements concerning specific criteria, the diagnosis can be confirmed with biopsies in the majority of cases. In rare situations, it may be difficult to distinguish eosinophilic esophagitis from gastroesophageal reflux disease (GERD). Working closely with your health care team is the best way to ensure a proper and timely diagnosis.

View Images of Upper and Lower Endoscopy
View and upper endoscopy and balloon dilatation of an esophageal stricture
Learn more: EGID Diagnosis
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Treatment
• Dietary
• Medications
Most children and adults with EE respond favorably to dietary treatments. The dietary restrictions are guided by food allergy testing and ‘fine-tuned’ with food trials once the symptoms have resolved.

Single ingredient cooking for food trial.pdf

Elimination diets, in which all "positive" foods on allergy testing are removed from the diet, are one type of dietary treatment. An elimination diet may be the only treatment needed for some individuals with eosinophilic esophagitis.
Learn more: Restricted Diets.pdf

Elemental diets, in which all sources of protein are removed from the diet, are another dietary therapy. The elemental diet includes only an amino acid formula (building blocks of protein), no whole or partial proteins. Simple sugars, salt and oils are permitted on an elemental diet. Learn more: Elemental Diets

Children and adults who rely in part, or completely, on an elemental amino acid based formula may have a difficult time drinking enough of the formula. To maintain proper nutrition, some may require tube feedings directly into the stomach (enteral feeds).
View an Illustration of an NG Tube Learn more: Living with Feeding Tubes.pdf

Food trials involve adding back one ingredient at a time to determine specific foods causing a reaction. Food trials begin after symptoms resolve and eosinophils have cleared.

Medications for EE most commonly include steroids to control inflammation and suppress the eosinophils. Steroids are used if dietary measures do not resolve the symptoms. Steroids can be taken orally or topically (swallowed from an asthma inhaler).

For more detailed information on the treatment of EE and related disorders
Learn more: Treatment of EGID

Patients with EE may require additional endoscopies and biopsies to assess how the esophagus is responding to specific treatment. The initial diagnosis of EE can be overwhelming and often affects the entire family. A positive attitude and a focus on non-food activities go a long way in learning to live with EE. With proper treatment, individuals with EE can lead a normal life.

Learn more about school issues:
Guide to Celebrating Without Food.pdf
IEP/504 Plans.pdf
Post-Secondary School.pdf
Student Health Forms.pdf Health Information Sheet, Patient.pdf
Guide for Students with Chronic Illness.pdf
No-food Birthday cakes.pdf

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Resources for Healthcare Professionals and Consumers
Reading List for Healthcare Professionals.pdf
Link to Clinical Trials page.
Link to Healthcare Professionals page.

References
(Updated 5-30-05, Author: Wendy Book, email the author)
1. Liacouras CA, Ruchelli E. Eosinophilic esophagitis. Curr Opin Pediatr. 2004 Oct;16(5):560-6.
2. Noel RJ, Putnam PE, Rothenberg ME. Eosinophilic esophagitis. N Engl J Med. 2004 Aug 26;351(9):940-1.
3. Dahms BB. Reflux esophagitis: sequelae and differential diagnosis in infants and children including eosinophilic esophagitis. Pediatr Dev Pathol. 2004 Jan-Feb;7(1):5-16. Review
4. Arora AS, Yamazaki K. Eosinophilic esophagitis: asthma of the esophagus? Clin Gastroenterol Hepatol. 2004 Jul;2(7):523-30. Review.
5. Ahmad M, Soetikno R, Ahmed A. The differential diagnosis of eosinophilic esophagitis. J Allergy Clin Immunol 2004;30(3):242-244
6. Fox V, Nurko S, Furuta G. Eosinophilic Esophagitis: It’s not just kid’s stuff Gastrointestinal Endoscopy 260-270, Vol 56, No 2, 2002
7. Furuta GT, Nurko S, Bousvaros A, et al. The spectrum of pediatric gastroesophageal reflux. JAMA 2000;284(24)
8. Liacouras CA. Eosinophilic esophagitis in children and adults. J Pediatr Gastroenterol Nutr. 2003 Nov-Dec;37 Suppl 1:S23-8
9. Liacouras C, Markowitz JE. Eosinophilic Esophagitis: A Subset of Eosinophilic Gastroenteritis. Current Gastroenterology Reports, 1999 1:253-258
10. Markowitz JE and Liacouras CA. Eosinophilic Esophagitis. Gastroenterology Clinics of NA. Sept 2003
11. Orenstein S, Shalaby T, DiLorenzo C, Putnam PE. The Spectrum of Pediatric Eosinophilic Esophagitis Beyond Infancy: A Clinical Series of 30 Children. American Journal of Gastroenterology 2000; 95:1422-1430
12. Sampson HA. Food Allergy. J Allergy Clin Immunol. 2003;111:S540-547
13. Straumann A, Spichtin HP, Grize L, Bucher KA, Beglinger C, Simon HU. Natural history of primary eosinophilic esophagitis: a follow-up of 30 adult patients for up to 11.5 years. Gastroenterology. 2003 Dec;125(6):1660-9
14. Walsh SV, Antonioli DA, Goldmann H, Fox VL, Bousvaros A, Leichtner AM, and Furuta GT. Allergic Esophagitis in Children. A Clinicopathological Entity. American Journal of Surgical Pathology1999, 23(4):390-396

Clinical Features
15. Sant’Anna AM, Rolland S, Fournet JC, Yazbeck S, Drouin E. Eosinophilic Esophagitis in Children: Symptoms, Histology and pH Probe Results. J Pediatr Gastroenterol Nutr. 2004 Oct;39(4):373-377
16. Lim JR, Gupta SK, Croffie JM, Pfefferkorn MD, Molleston JP, Corkins MR, Davis MM, Faught PP, Steiner SJ, Fitzgerald JF.. White specks in the esophageal mucosa: An endoscopic manifestation of non-reflux eosinophilic esophagitis in children. Gastrointest Endosc. 2004 Jun;59(7):835-8.
17. Straumann A, Spichtin HP, Bucher KA, Heer P, Simon HU. Eosinophilic esophagitis: red on microscopy, white on endoscopy. Digestion. 2004;70(2):109-16. Epub 2004 Sep 20.
18. Steiner SJ, Gupta SK, Croffie JM, Fitzgerald JF. Correlation between number of eosinophils and reflux index on same day esophageal biopsy and 24 hour esophageal pH monitoring. Am J Gastroenterol. 2004 May;99(5):801-5.
19. Attwood SEA, Smyrk TC, Demeester TR, Jones JB. Esophageal Eosinophilia with Dysphagia. A Distinct Clinicopathologic Syndrome. Digestive Disease Science, 1993, 38: 109-116
20. Brown LF, Goldman H, Antonioli DA. Intraepithelial Eosinophils in Endoscopic Biopsies of Adults with Reflux Esophagitis. American Journal of Pathology, 1984 8:899-905
21. Cheung KM, Oliver MR, Cameron DJ, Catto-Smith AG, Chow CW. Esophageal eosinophilia in children with dysphagia. J Pediatr Gastroenterol Nutr. 2003 Oct;37(4):498-503
22. Croese J, Fairley SK, Masson JW, Chong AK, Whitaker DA, Kanowski PA, Walker NI. Clinical and endoscopic features of eosinophilic esophagitis in adults. Gastrointestinal Endosc. 2003 Oct;58(4):516-22
23. Cury EK, Schraibman V, Faintuch S. Eosinophilic infiltration of the esophagus: gastroesophageal reflux versus eosinophilic esophagitis in children--discussion on daily practice. J Pediatr Surg. 2004 Feb;39(2):e4-7
24. Desai T, Goldstein N, Stecevic V, Badizadegan K, Furuta GT. Esophageal Eosinophilia is Common among Adults with Esophageal Food Impaction. Gastroenterology, 2002; 122:M1723
25. Khan S, Orenstein SR, DiLorenzo C, Kocoshis SA, Putnam PE, Sigurdsson L, Shalaby TM. Eosinophilic esophagitis: strictures, impactions, dysphagia. Dig Dis Sci. 2003 Jan;48(1):22-9
26. Potter JW, Saeian K, Staff D, Massey BT, Komorowski RA, Shaker R, Hogan WJ. Eosinophilic esophagitis in adults: An emerging problem with unique esophageal features. Gastrointestinal Endosc. 2004 Mar;59(3):355-61
27. Rothenberg ME, Mishra A, Collins MH, Putnam PE. Pathogenesis and Clinical Features of Eosinophilic Gastroenteritis. Journal of Allergy and Clinical Immunology, 2001, 108:891-894
28. Straumann A, Rossi L, Simon HU, Heer P, Spichtin HP, Beglinger C.. Fragility of the esophageal mucosa: a pathognomonic endoscopic sign of primary eosinophilic esophagitis? Gastrointestinal Endosc 2003 Mar;57(3:407-12
29. Vasilopoulos S, Murphy P, Auerbach A, Massey BT, Shaker R, Stewart E. et al. The Small-Caliber Esophagus: An Unappreciated Cause of Dysphagia for Solids in Patients with Eosinophilic Esophagitis. Gastrointestinal Endoscopy, 2002, 55:99-106

Etiology
30. Braun-Fahrlander C, Rieldler J, Herz U, et al. Environmental exposure to endotoxin and its relation to asthma in school –age children. N Engl J Med 2002;347(12):869-876
31. Cury EK, Schraibman V, Faintuch S. Eosinophilic infiltration of the esophagus: gastroesophageal reflux versus eosinophilic esophagitis in children--discussion on daily practice. J Pediatr Surg. 2004 Feb;39(2):e4-7.
32. Fogg, Rachelli, and Spergel. Pollen and Eosinophilic Esophagitis. Jounal of Allergy and Clinical Immunology. 2003 Oct;112(4):796-7.
33. Fox V, Nurko S, Teitelbaum JE, Badizadegan K, Furuta G. High resolution EUS in Children with Eosinophilic “Allergic” Esophagitis Gastrointestinal Endoscopy, 30-36, Vol. 57, No. 1 2003
34. Jean-Francois B. Mechanisms of disease - The effect of infections on susceptibility to autoimmune and allergic diseases N Engl J Med 2002;347(12):911-918
35. Latcham F, Merino F, Lang A, et al. A consistence pattern of immunodeficiency and subtle enteropathy in children with multiple food allergy. Journal of Pediatrics 2003;143(1)
36. Spergel JM, Beausoleil JL, Mascarenhas M, et al. The use of Skin Prick Tests and Patch Tests to identify causative foods in Eosinophilic Esophagitis. Journal of Clinical Immunology 2002, 109:363-368
37. Straumann A, Bauer M, Fischer B, Blaser K, Simon HU. Idiopathic Eosinophilic Esophagitis is Associated with a Th2-type Allergic Inflammatory Response. Journal of Allergy and Clinical Immunology 2001: 108:954-961
38. Straumann A, Simon H.-U. The physiological and pathophysiological roles of eosinophils in the gastrointestinal tract. Allergy 2004;59:15-25
39. Weiss ST. Eat Dirt- The hygiene hypothesis and allergic diseases. N Engl J Med 2002;347(12)

Formula, Corticosteroids and other treatment
40. Markowitz JE, Spergel JM, Ruchelli E, Liacouras CA. Elemental Diet is an Effective Treatment for Eosinophilic Esophagitis in Children and Adolescents. American Journal of Gastroenterology, 2003 Vol 98 No. 4, 777-782
41. Kelly K, Lazenby AJ, Rowe PC, Yardley JH, Perman JA, Sampson HA. Eosinophilic Esophagitis Attributed to Gastroesophageal Reflux: Improvement with an Amino Acid-Based Formula. Gastroenterology, 1995, 1503-1512
42. Gawrieh S, Shaker R. Treatment options for eosinophilic esophagitis: montelukast. Curr Gastroenterol Rep. 2004 Jun;6(3):189
43. Attwood SE, Lewis CJ, Bronder CS, Morris CD, Armstrong GR, Whittam J. Eosinophilic oesophagitis: a novel treatment using Montelukast. Gut. 2003 Feb;52(2):181-5.
44. Elkon KB, Sher R, Seftel HC. Immunological studies of eosinophilic gastro-enteritis and treatment with disodium cromoglycate and beclomethasone dipropionate. S Afr Med J. 1977 Nov 12;52(21):838-41.
45. Friesen CA, Kearns GL, Andre L, et al. Clinical efficacy and pharmacokinetics of montelukast in Dyspeptic children with duodenal eosinophilia. Journal of Ped Gastr and Nutr. 2004;38:343-351
46. Heine RG. Pathophysiology, diagnosis and treatment of food protein-induced gastrointestinal diseases. Curr Opin Allergy Clin Immunol. 2004 Jun;4(3):221-9.
47. Khan S, Henderson WA. Treatment of Eosinophilic Esophagitis in Children. Curr Treat Options Gastroenterol. 2002 Oct;5(5):367-376
48. Khan S, Orenstein SR. Eosinophilic gastroenteritis: epidemiology, diagnosis and management. Paediatr Drugs. 2002;4(9:563-70. Review.
49. Kukuruzovic R, Elliott E, O’Loughlin E, Markowitz J. Non-surgical interventions for eosinophilic oesophagitis. Cochrane Database Sys Rev 2004;3:CD004065
50. Liacouras CA, Wenner WJ, Brown K, Ruchelli E. Primary Eosinophilic Esophagitis in Children: Successful Treatment with Oral Corticosteroids. Journal of Pediatric Gastroenterology and Nutriion. 1998, 26:380-385
51. Ruchelli E, Wenner W, Voytek T, Brown K, Liacouras C. Severity of Esophageal Eosinophilia Predicts Response to Conventional Gastroesophageal Reflux Therapy.Pediatric and Developmental Pathology, 1999. 2, 15-18.

Topical Steroid Therapy and EE
52. Noel RJ, Putnam PE, Collins MH, Assa’ad AH, Guajardo JR, Jameson SC, Rothenberg ME. Clinical and immunopathologic effects of swallowed fluticasone for eosinophilic esophagitis. Clin Gastroenterol Hepatol. 2004 Jul;2(7):568-75.
53. Arora AS, Perrault J, Smyrk TC. Topical corticosteroid treatment of dysphagia due to eosinophilic esophagitis. Mayo Clinic Proc.2003 Jul;78(7);830-5
54. Teitelbaum JE, Fox VL, Twarog FJ, Nurko S, Antonioli D, Gleich G, Badizadegan K, and Furuta GT. Eosinophilic Esophagitis in Children: Immunopathological Analysis and Response to Fluticasone Propionate. Gastroenterology 2002. 122:1216-1225
55. Cave A, Arlett P, Lee E. Inhaled and nasal corticosteroids: factors affecting the risks of systemic adverse effects. Pharmacol Ther. 1999;83(3):153-79
56. Drake AJ, Howells RJ, Shield JPH, et al. Symptomatic adrenal insufficiency presenting with hypoglycemia in children with asthma receiving high dose inhaled fluticasone proprionate. British Medical Journal 2002;324(7345):1081-1082
57. Eid N, Morton R, Olds B, et al. Decreased morning serum cortisol levels in children with asthma treated with inhaled fluticasone proprionate. Pediatrics 2002;110(5):1030-1
58. England RW, Nugent JS, Grathwohl KW, et al. High-dose inhaled fluticasone and delayed hypersensitivity skin testing. Chest 2003;123(4):1014-1017
59. Falcoz C, Oliver R, McDowall JE, et al. Bioavailability of orally administerd micronised fluticasone proprionate. Clin Pharmacokinet 2002;39 Suppl 1:9-15
60. Faubion, Jr. WA, Perrault J, Burgart LJ, Zein NN, Clawson M, Freese DK. Treatment of Eosinophilic Esophagitis with Inhaled Corticosteroids. Journal of Pediatric Gastroenterology and Nutriion 27:90-93, July 1998
61. Harrison TW. Systemic availability of inhaled budesonide and fluticasone proprionate: healthy versus asthmatic lungs. Bio Drugs 2001;15(6):405-11
62. Howland WC 3rd. Fluticasone proprionate: topical or systemic effects? Clin Exp 1996;26 Suppl 3:18-22
63. Kemp JP, Osur S, Shrewsbury SB, et al. Potential effects of fluticasone proprionate on bone mineral density in patients with asthma: A 2-year randomized, double –blind, placebo-controlled trial. Mayo Clinic Proc. 2004;79:458-466
64. Krishnaswami S, Hochhaus G, Derendorf H. An interactive algorithm for the cumulative cortisol suppression during inhaled corticosteroid therapy. AAPS PharmSci. 2002;2(3):E22
65. Mohammed A. Adrenal insufficiency after treatment with fluticasone: Dose-response curve should have been highlighted. Briish Medical Journal 2002;325(7368):837
66. Nguyen KL, Lauver D, Kim I, et al. The effect of a steroid “burst” and long-term, inhaled fluticasone proprionate on adrenal reserve. Ann Allergy Asthma Immunol. 2003;9(1):38-43
67. Noel RJ, Putnam PE, Collins MH, et al. A subset of children with refractory allergic eosinophilic esophagitis are resistant to swallowed fluticasone proprionate. Pediatric Gastroenterol Nutr 2002;35(3): 447-456
68. Patel L, Clayton P. Adrenal insufficiency after treatment with fluticasone: Lowest possible dose of Glucocorticoids should be given. British Medical Journal 2002;325(7368):837-838
69. Thorsson L, Dahlstrom K, Edsbacker S, et al. Pharmacokinetics and systemic effects of inhaled fluticasone proprionate in healthy subjects. Br. Journ Clin Pharmacol 1997;43(2):155-61

Food Allergies
70. Sampson HA. Food Allergies. J Allergy Clin Immunol 2004;113:805-819.
71. Heine RG. Pathophysiology, diagnosis and treatment of food protein-induced gastrointestinal diseases. Curr Opin Allergy Clin Immunol. 2004 Jun;4(3):221-9.
72. Munoz-Furlong A. Daily Coping Strategies for Patients and their Families. Pediatrics 2002;111:165

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