Allergy Grand Rounds Michael Goldman M D Johns

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Allergy Grand Rounds Michael Goldman, M. D. Johns Hopkins Asthma & Allergy Center April

Allergy Grand Rounds Michael Goldman, M. D. Johns Hopkins Asthma & Allergy Center April 2, 2004

Chief Complaint This is a 37 y. o. WM who presents with dysphagia of

Chief Complaint This is a 37 y. o. WM who presents with dysphagia of solid foods for three years

HPI Complains of difficulty swallowing solid foods. Steak, chicken, pork, and bread get stuck

HPI Complains of difficulty swallowing solid foods. Steak, chicken, pork, and bread get stuck in the base of throat emesis, with relief of obstruction No pain on swallowing, no heartburn, no wt. loss No difficulty swallowing liquids Only slight improvement with Nexium. Stopped due to nausea. Referred to GI

Work up n n n Normal Exam Barium swallow: narrowing of distal esophagus. Differential

Work up n n n Normal Exam Barium swallow: narrowing of distal esophagus. Differential diagnosis: n n Peptic stricture (from reflux esophagitis), but no history of reflux Motility disorder (eg achalasia, esophageal spasm) but no mention of abnormal motility or dilated esophagus on barium swallow Hypertensive lower esophageal sphincter Schatzki ring (Lower esophageal mucosal ring)

Achalasia

Achalasia

Schatzki Ring

Schatzki Ring

Work up n Endoscopy: “Ringed” esophagus n Several “polypoid/nodular” areas, biopsied n Narrowing of

Work up n Endoscopy: “Ringed” esophagus n Several “polypoid/nodular” areas, biopsied n Narrowing of the distal esophagus n n Not consistent with n Stricture dilated Schatzki ring

Ringed Esophagus

Ringed Esophagus

Esophageal nodules

Esophageal nodules

Whitish exudates

Whitish exudates

Esophageal Stricture with Food Impaction

Esophageal Stricture with Food Impaction

Biopsy Results n Esophagus: Moderate chronic inflammation n Focally parakeratotic (excessive keratin) papillary tissue

Biopsy Results n Esophagus: Moderate chronic inflammation n Focally parakeratotic (excessive keratin) papillary tissue with increased areas of eosinophils in subepithelium n Read as compatible with squamous papilloma with eosinophilia described in reflux esophagitis. n No dysplasia n

Eosinophilic Esophagitis Referred for allergy evaluation

Eosinophilic Esophagitis Referred for allergy evaluation

Allergy Consultation Dysphagia of solids persists, but no further vomiting episodes since esophageal dilitation.

Allergy Consultation Dysphagia of solids persists, but no further vomiting episodes since esophageal dilitation. n No history of food allergy, but on careful questioning reports slight itchy throat to peanuts, eggs, possibly nuts. n Beer causes facial flushing and mild throat constriction. No reactions to other forms of alcohol. n

Medical/Social History Allergic rhinitis as a child, treated with immunotherapy. Mild symptoms presently except

Medical/Social History Allergic rhinitis as a child, treated with immunotherapy. Mild symptoms presently except around pets. n Mild asthma around pets and with exercise in cold air. Uses albuterol prn. No steroids or ER visits since childhood. n Otherwise healthy: no cardiac disease, rashes, arthritis, fevers, chills, diarrhea, travel n No pets, non smoker, no ETOH abuse. n

Physical Exam n Normal No edema of nasal mucosa n Clear lungs n Cardiac

Physical Exam n Normal No edema of nasal mucosa n Clear lungs n Cardiac without murmurs n Normal abdominal exam n Diagnostic testing performed n What would you test for? n

Skin Testing-scratch Food Wheal/flare Egg 10/45 Malt 5/12 Peanut 7/20 Wheat 3/5 Fish Mix

Skin Testing-scratch Food Wheal/flare Egg 10/45 Malt 5/12 Peanut 7/20 Wheat 3/5 Fish Mix 6/30 Beef 0 Hazelnut 10/30 Chicken 0 Almond 4/8 Milk 0 Other nuts 0 Soy 0 Pork 4/12 Shellfish mix 0

Now What? More skin testing? n Confirm with RAST? n Food patch testing? n

Now What? More skin testing? n Confirm with RAST? n Food patch testing? n Other blood tests? n Food avoidance? n Epipen? n Medicines? n

My recommendations n Food avoidance for 1 month: all positive skin tests except wheat

My recommendations n Food avoidance for 1 month: all positive skin tests except wheat (borderline) n Egg, peanut, beer (malt), hazelnut, almond, pork, lamb. Confirm positive tests with cap-Rast. n Check CBC, eosinophil count, total Ig. E n No meds prescribed n

Blood results Food k. U/L Egg 4. 88 Malt 3. 32 Peanut 2. 56

Blood results Food k. U/L Egg 4. 88 Malt 3. 32 Peanut 2. 56 Wheat 12. 3 Fish (cod, <0. 35 tuna, salmon ) Hazelnut 3. 32 Total Ig. E 627(0 -114) Almond 3. 37 Serum Eos 871 (15 -500) Pork 4. 57 WBC 6. 7 K, 13%eo s

Follow Up Improved but not resolved n Still with some dysphagia but no choking

Follow Up Improved but not resolved n Still with some dysphagia but no choking or vomiting (since dilitation) n Avoiding egg, pork, lamb, fish, malt, peanuts, and nuts n Skin tests to individual fish all + except tuna n Skin tests to inhalants +cat, dog, DM, trees, grass. n No seasonal worsening of dysphagia n

Now What? Recommended wheat avoidance for 2 weeks, symptoms partially improved n Pt not

Now What? Recommended wheat avoidance for 2 weeks, symptoms partially improved n Pt not interested in neocate trial n To start Flovent 220 2 p bid, without spacer, swallowed. n GI follow up in 2 months. n

Adult Eosinophilic Esophagitis Typically seen in young adults (mean age 34) n Dysphagia with

Adult Eosinophilic Esophagitis Typically seen in young adults (mean age 34) n Dysphagia with bolus impaction is most common symptom n Esophageal strictures common (unlike kids) n Esophageal biopsy necessary for diagnosis n Exclude secondary causes: HES, parasitic disease, connective tissue dz (scleroderma), drug reaction n

Adult Eosinophilic Esophagitis Mean duration to diagnosis 4 years n Male : Female =

Adult Eosinophilic Esophagitis Mean duration to diagnosis 4 years n Male : Female = 3 : 1 n 50 – 75% atopic n Food sensitization common, but not well studied n

Pediatric Eosinophilic Esophagitis n Reflux symptoms most common presentation Vomiting n Regurgitation n Abdominal

Pediatric Eosinophilic Esophagitis n Reflux symptoms most common presentation Vomiting n Regurgitation n Abdominal pain n Dysphagia n Food refusal/poor wt. gain n Unresponsive to PPIs for GERD n Strictures less common n

Pediatric Eosinophilic Esophagitis Endoscopy and biopsy needed for diagnosis n Food sensitization very common

Pediatric Eosinophilic Esophagitis Endoscopy and biopsy needed for diagnosis n Food sensitization very common n 60 -75% skin test positive (egg, milk most common) n 80% in one study positive patch test to foods (wheat most common)1 n 1 Spergel, JACI 2002, 109: 363 -368

Treatment n Food avoidance n Effective in children n Elemental formula reduced eosinophils and

Treatment n Food avoidance n Effective in children n Elemental formula reduced eosinophils and symptoms 1 n 8/10 resolution, other 2 improved n Food avoidance based on all positive skin test and food patch test: 2 n n n resolved symptoms in 18/24 kids w/ EE and improved symptoms other 6. Milk, egg most common, but many others implicated. Average of 3. 6+/-2. 1 foods Mean esophageal eosinophils decreased 55. 8/hpf 8. 4/hpf No studies in adults 1 Kelly, Gastroenterology, 1995, 1503 -1512 2 Spergel, JACI 2002, 109: 363 -

Treatment n Oral corticosteroids n n Effective but side effects Topical Steroids (swallowed “inhaled”

Treatment n Oral corticosteroids n n Effective but side effects Topical Steroids (swallowed “inhaled” steroids) Swallow FP 220 2 pbid n Improvement in both adults and children n Esophageal candidiasis (2/13 kids)1 n 1 Teitlebaum, Gastroenterology 2002; 122: 1216

Treatment n Esophageal dilitation n n Relieves obstruction, dysphagia often persists PPIs for EE

Treatment n Esophageal dilitation n n Relieves obstruction, dysphagia often persists PPIs for EE generally ineffective

EE vs. GERD Characteristic Atopy Food sensitization Histology Peripheral eosinophilia Esophageal p. H PPI

EE vs. GERD Characteristic Atopy Food sensitization Histology Peripheral eosinophilia Esophageal p. H PPI Steroids Food allergen elimination EE High >24 eos/hpf ~50% Normal Usually not helpful Helpful Sometimes helpful GERD Nml 0 -7 eos/hpf rare Abnormal Helpful Not helpful