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

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

Chief Complaint his is a 37 y.o. WM who presents wit ysphagia of solid foods for three year

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 Normal Exam Barium swallow: narrowing of distal esophagus. Differential diagnosis: 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

Schatzki Ring

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

Ringed Esophagus

Esophageal nodules

Whitish exudates

Esophageal Stricture with Food Impaction

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

Eosinophilic Esophagitis Referred for allergy evaluation

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

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

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

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

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

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

Blood results Food Egg Peanut Fish (cod, tuna,salm on) Hazelnut kU/L 4.88 2.56 0.35 Food Malt Wheat kU/L 3.32 12.3 3.32 Almond 3.37 Pork 4.57 Total IgE 627(0114) Serum 871 (15Eos 500) WBC 6.7K,13%

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

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

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

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

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

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

Treatment Food avoidance Effective in children Elemental formula reduced eosinophils and symptoms1 Food avoidance based on all positive skin test and food patch test:2 1 8/10 resolution, other 2 improved 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 Kelly, Gastroenterology, 1995, 1503-1512 2 Spergel, JACI 2002,

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

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

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