When Food Keeps Getting Stuck: Recognizing and Understanding
Eosinophilic Esophagitis in Children
Jenifer R. Lightdale, MD, MPH, FASGEDivision Chief, Pediatric GastroenterologyUMass Memorial Children’s Medical Center
DisclosuresA. I have the following financial relationships with the manufacturers
of commercial products and/or providers of commercial services:– Mead Johnson ‐ Honorarium– Perrigo ‐ Paid Consultant– Medtronic ‐ Paid Consultant– Norgine ‐ Paid Consultant
B. I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation
Eosinophilic Esophagitis• “Chronic, immune/antigen‐mediated esophageal disease characterized– Clinically by symptoms related to esophageal dysfunction AND
– Histologically by eosinophil‐predominant inflammation”• >1 biopsy showing 15+ eos/high power field
• Exclusion of other causes• PPI‐REE
Dellon, AJG, 2013
Learning Objectives• Recognize clinical symptoms in children associated with EoE
• Understand the diagnostic approach to children with EoE
• Discuss guidelines for appropriately treating EoE, as a chronic condition in children
Natural History of EoE• Not completely understood• Chronic inflammation leads to
– Esophageal wall remodeling– Fibrostenosis– Stricture formation
• EoE accounts for 80% of food impactions in adults
Liacouras, J Allergy Clin Immun, 2011
Typical Patient with EoE• Male (3:1)• Thin body habitus• Atopic• Asthma• History of food allergy• Family history of allergic and/or atopic disorders
• Peripheral eosinophilia on CBC
Typical Patient with EoE• Complain of persistent reflux symptoms, • Vomiting, dysphagia, food impaction
– “Slow eater”
– “Last at the table”
– “Chews carefully, cuts food into small pieces…”
– Gagging, food refusal, feels “food going down”
EoE Overlap with GERD Symptoms/Heartburn
Studies of EoE % of EoE study sample who c/o GERD (n)
Alexander JA et al, 2012 29% of 21
Gonsalves N et al, 2012 94% of 50
Spergel J et al, 2012 39% of 169
Iwanczak B et al, 2011 54% of 74
Assa’ d et al, 2011 20% of 149
• Esophageal rings• Linear Furrows• Edema• White plaques/exudates
• Can occur in isolation or combination
Endoscopic Features of EoE
Endoscopic Features of EoE
Histological Features of EoE• Prominent eosinophilic infiltrate (H&E staining)
• Eosinophilic degranulation• Eosinophilic microabscesses• Basal layer hyperplasia• Dilated intracellular spaces
• May be a transmural process
Histological Features of EoE
2013 Diagnostic Criteria for EoE from the American College of Gastroenterology (ACG)
1. Clinical symptoms of esophageal dysfunction2. Pathological findings isolated to the
esophagus– >1 biopsy of an eosinophil predominant
inflammation (15+ eosinophils/hpf)
3. Exclusion of other causes (i.e. PPI‐REE)– Lack of response to high dose PPI
PPI‐Responsive Esophageal Eosinophilia• Condition in which esophageal eosinophilia is highly responsive to treatment with PPI
• PPI‐REE currently considered ‘distinct” from EoE• Mechanism remains unclear
– Gastroesophageal reflux responsive to acid suppression?
– Evidence of anti‐inflammatory effect of PPI?– Combination of GERD and EoE?
2013 American College of Gastroenterology Guidelines
• “All patients with suspected EoE should receive a two‐month course of PPI, followed by endoscopy with biopsies [as a repeat procedure if necessary] to exclude PPI‐REE…”
Dellon, AJG, 2013
Initial Steps in Evaluation• Refer to GI for endoscopy• Start PPI• Consider UGI imaging
Small Caliber Esophagus
Ringed Esophagus
Options for Clinical Management• Dietary exclusions
– Elemental diet– 6 food elimination
• Pharmacologic– PPI– Steroids (Topical, Systemic)
• Endoscopic dilation
Six food elimination diet (SFED)• Milk, soy, egg, wheat, beef, fish• 6 weeks • Clinicopathological remission with SFED• Eosinophilia returns when diet liberalized
Gonsalves et al, Gastroenterology 2012
Fast Forward to 2016• EoE is a chronic immune‐mediated inflammatory condition with no current curative therapy– At risk for fibrostenosis and stricture development
• Current palliative approaches– Elimination diet– PPI– Topical steroids
• Treatments used alone or in combination– To minimize disease risks, while preserving quality of life
Immune Cells in EoE• Eosinophils• Th2 cells• Mast Cells• Basophils
Potential Therapeutic Targets• Immune therapy directed at IL‐13 and eotaxin• Prostaglandin D2 inhibitor – CRTH2• Other FDA approved Phase I trials ongoing
Current Pharmacologic Therapy• PPI may be effective adjunct• Topical glucocorticoids
– Fluticasone (220mcg inhaler)– Budesonide (1‐2mg daily)
• Lead to decrease in eosinophil counts• Recurrence of symptoms when discontinued• Associated with candidal esophagitis
• Systemic steroids effective, but NOT for maintenance
Budesonide• “Oral viscous” budesonide (2mg slurry)
– Randomized placebo controlled study– OVB=15, placebo‐9– Significant reduction in symptoms and eosinophilia
Dohil 2010
Treatment End Points• Ideal = Complete resolution of symptoms, inflammation and remodeling
• Reality = EoE is currently a chronic disease with no curative treatment
• High likelihood of symptom recurrence after discontinuing treatment
Treatment End Points• Reasonable = A balance
– Use treatment options to minimize symptoms and prevent disease complications
– Preserve quality of life– Limit adverse effects of treatment
Changes You May Want to Make in Your Practice
• Recognize the presenting symptoms of EoE – Chronic inflammatory disease– Immune mediated, food allergen induced– Highly associated with atopy– Relatively common worldwide
• Understand there is no current curative therapy
Take Home Points• Diagnosis of EoE is clinicopathologic• Consider other “ee’s”
• Particularly PPI‐REE• Tailor therapies to minimize disease‐associated inflammation and fibrostenosis
• Treatment should minimize disease risks, while preserving quality of life
Thank you!