The Steakhouse Syndrome
Mazer R. Ally, M.D.
Case
•  24yo male presents to the ER with a meat
impaction
•  3hrs ago he was eating a NY strip steak at
Morton’s Steakhouse
•  Midway into the meal he felt a piece of meat
get stuck. He immediately drinks some water
but that doesn’t help.
•  He points to the sternal notch where he feels
this sensation of impaction
•  His airway is intact, but noticing that he
cannot tolerate secretions
Case (cont)
•  The patient has no significant medical
history, no reflux symptoms and is not
on any medications.
•  He reports occasional episodes where
food gets “hung up”, but usually can
wash it down with water.
•  The patient is taken to the ER
–  Glucagon is given but ineffective
•  GI is called for help…
Endoscopy
•  Is this Eosinophilic Esopahgitis (EoE)?
Outline
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Definition
Epidemiology
Pathogenesis
Clinical Presentation
Endoscopic findings
Diagnosis
Management
What is Eosinophilic Esophagitis?
•  Chronic, immune/antigen mediated
esophageal disease
•  Clinically associated with symptoms
related to esophageal dysfunction
AND
•  Histologically associated with
eosinophil-predominant inflammation.
•  Seen in both children and adults
J Allergy Clin Immunol 2011
Publications
Eosinophilic Esophagitis
PubMed Citations 1978-2009
Epidemiology:
Incidence of EE at University of
Pennsylvania (1994-2003)
Liacouras CA et al. Clinical Gastro Hep 2005
Epidemiology:
The Prevalence of EE
Who gets EoE?
Young
Caucasian
Males
Moawad et al. Gastroenterology 2009; 136 (Suppl 1):S1857
Clinical Presentation (N=127)
Clinical Presentation
Endoscopic Findings:
1. Concentric rings
Trachealization
Concentric rings:
not always EE
Endoscopic Findings:
2. Longitudinal furrows
Endoscopic Findings:
3. White lesions
Endoscopic Findings:
4. Friability/Crepe-like
Endoscopic Findings:
5. Narrow lumen esophagus
EoE does not always have
endoscopic features
•  10% of patients demonstrate a normal
appearing esophagus
Pathogenesis
Moawad et al. Dig Dis Sci 2009
Food Allergens Associated with
EE in Children
•  Skin prick test performed on 42 subjects with EE plus 4 controls
•  Positive reactions for 62% of the EE group and 25% of the control group
Number of patients testing positive
15
14
12
11
10
10
7
5
4
3
2
2
2
2
2
2
Almond
Beef
Chicken
Corn
Potato
Rye
0
Milk
Peanut
Egg
Soybean
Wheat
Codf ish
Pea
Baxi S, et al. Gastrointest Endosc. 2006
Richter J. Eosinophilic Esophagitis. Gastroenterology Current Hot Topics 2008
Seasonal Variation
Peds EE
Adult EE
Wang et al. J Clin Gastroenterol 2007
Almansa et al. Am J Gastroenterol 2009
Aeroallergens and EE
EoE(%Pt)
Trees *
Grass**
Weed**
15
40
33%
12
30
20
27%
24%
16%
9
6
10
3
0
0
Jan-­‐Mar
Apr-­‐Jun
Jul-­‐Sep
Oct-­‐Dec
Moawad et al. Gastroenterology 2009; 136 (Suppl 1):S1857
Mean Pollen Count (Grains per m3)
Mean Pollen Count (Grains per m3/10)
*
**
*
History of Atopic Diseases
Walter Reed Population
Atopic dermatitis
6%
Asthma
12%
Food allergies
13%
33%
Seasonal allergies
0
10
20
% EE Patients (n=127)
30
40
Does Acid Exposure Play a Role?
Eosinophilic Esophagitis
Oct 2 1997
Erosive Esophagitis
March 4 2009
EoE and GERD
have a complex association
•  GERD causes esophageal injury with
eosinophilic infiltration
•  GERD and EE coexist but are unrelated
•  EE causes GERD
•  GERD causes EE
Spechler et al. Am J Gastroenterol 2007
Diagnosis
•  Made by multiple biopsies from the
proximal and distal esophagus
•  Histologic findings:
–  Increase number of eosinophils (>15 eos)
–  Microabscesses
–  Spongiosis
–  Basal zone hyperplasia
–  Degranulation
–  Subepitheilial fibrssis is characteristic
Odze RD. Am J Gastroenterol. 2009
Nutrients 2013, 5
Number of biopsies to establish
diagnosis
Gonsalves N et al. Gastrointest Endoscop 2006
Management
•  Pharmacologic therapy
–  Proton pump inhibitors
–  Corticosteroids (topical and systemic)
–  Leukotriene receptor antagonists
•  Dietary
–  Elemental
–  Six-food elimination (Eggs, Wheat, Peanuts, Seafood,
Soy, Milk)
–  Elimination based on testing
•  Mechanical
–  Dilation
PPI therapy
•  A trial of PPI therapy is recommended
for patients with EoE even if the
diagnosis is clear cut
•  PPI responsive esophageal eosinohilia
–  Have typical EoE symptoms and histology
–  Do not have GERD by endoscopy or pH
monitoring
–  Exhibit a clinical and histological response
to PPI’s
PPI-REE
•  Pathogenesis
–  Patients have GERD causing esophageal
eosinophilia (Non-erosive reflux disease)
–  Patients have EoE that responds to an
anti-inflammatory effect of PPIs
PPI Response in Children with
EoE (N=43)
Dranove JE et al. J Pediatr. 2009
Approaches to Diet therapy
•  Elemental diet
–  Amino acid based formulas
–  Gold standard
•  Empiric Elimination Diet (Six Food
Elimation Diet)
–  Avoidance of milk, soy, eggs, wheat, nuts,
seafood
•  Direct Elimination Diet
–  Skin prick testing
–  75% success in children
Elemental Diet
•  Amino-acid based formula
•  Effective in children but costly
•  >95% success in children, not studied
in adults
Introduction to Elemental Diet
Kelly KJ et al. Gastroenterology 1995
6 food elimination diet
50 patients
Dysphagia Symptom Score
Effect of a SFED for weeks on
Effect of Six-Food Elimination Diet (SFED) for 6
Dysphagia
Symptom
Score
Weeks on Dysphagia Symptom Score in 50 Adults
16
14
12
12
10
8
6
4
3.5*
2
0
Pre-SFED
Post-SFED
Gonsalves N. Gastroenterology 2012;142:1451.
Elemental vs. Six Food
Elimination Diet
Six foods
60 EoE patients
Wheat
Eggs
Milk
Soy
Peanuts
35 SFED*
25 elemental diet
6 weeks
Seafood
26/35 with < 10 eos/hpf
74%
22/25 < 10 eos/hpf
88%
Kagalwalla et al. Clin Gastroenterol Hepatol 2006
Pre and Post Eosinophil Counts
SFED
Kagalwalla et al. Clin Gastroenterol Hepatol 2006
Elemental Diet
What can
we eat?
Salad without
croutons
Water
Elimination Diet Based on Skin
Prick Testing (N=75)
Liacouras CA et al. Clinical Gastro Hep 2005
Topical Steroids- Fluticasone
•  Fluticasone 440 mcg twice daily
•  Treatment given for 6-8 weeks
•  Ensure delivery to esophagus by
removing spacer
•  Rinse mouth with water and avoid food
for 2 hours
•  Side effects include candidiasis
•  Effective in inducing remission
Esophageal Candidiasis
Topical Steroids
Konikoff et al. Gastroenterology 2006
Oral Viscous Budesonide
Eos 0-7
Eos 8-23
Eos ≥ 24
Children < 10 – 1mg/day
Children > 10 – 2mg/day
Aceves S et al. Am J Gastroenterol. 2007
Systemic Steroids
•  21 children found to have EoE
•  Treated with 1.5mg/kg oral
methylprednisolone for 4 weeks
•  20/21 had clinical improvement within
7 days and histology within 4 weeks
Liacouras et al. J Pediatr Gastroenterol Nutr 1998
Targeting Degranulation
Leukotriene Receptor Antagonists
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•
•
•
•
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12 patients with EoE (mean age 40)
Mean eosinophil count 56/HPF
Treated with Singulair for 14 months
7/8 with improvement in symptoms
No change in eosinophilia
6/8 with recurrence of symptoms once
treatment discontinued
Attwood et al. Gut 2003
Dilation in EoE
•  Generally used when medical therapy
“fails”
•  When initially reported was felt that
dilation led to increased complications
–  Reports of spontaneous esophageal
perforations
–  Perforations during endoscopy
–  Tears with dilation
Dilation: Initial Description
•  10/11 patients successfully treated
•  Symptoms recurred in 8 months on
average
•  Perforation has been reported, but
mucosal wall disruption more common
Straumann et al. Gastroenterology 2003
Dilation in EoE
•  No single dilator type has proved
superior to another in safety or efficacy
•  Esophageal strictures are dilated
progressively rather than abruptly
–  Rule of 3’s
Improvement in Symptoms
Following Dilation
Moawad FJ et al. Am J Gastroenterol. 2010 (submitted)
CRE vs Bougie
•  Controlled radial expansion
–  Dilate dominant or focal stricture
–  Limit esophageal wall disruption
–  Allows direct visualization
•  Bougie
–  Dilate entire esophagus
–  No visualization (Maloney)
Dilation
Complications with Dilation
(N=36)
Cohen et al. Clin Gastro Hep 2007
Recommendations for Dilation
•  Perform carefully and gradually
•  Begin small (8-10 mm bougie)
•  2 consecutive dilators in 1mm increments
•  3 consecutive dilators if no resistance
encountered
Back to that case…
•  Patient had
biopsies takenmax was 63 EOS/
HPF
•  Started on a PPI
•  Plan for repeat
EGD in 8 weeks
Key Points
•  EoE is a common disease in adults and
children
•  EoE is a clinicopathologic disease
•  The prevalence appears to be increasing
•  Food impaction and dysphagia are the most
common presenting symptoms in adults
•  EoE should be considered in all patients with
dysphagia
•  Treatments are effective but relapse is
common
Questions