EOSINOPHILIC ESOPHAGITISAND OTHER EOSINOPHILIC DISORDERS OF GI TRACT

Saransh jain

Preceptor Dr Anoop saraya

Outline

Introduction Eosinophilic esophagitis

Pathophysiology Clinical features Endoscopic findings Histopathology Differential diagnosis Management

Other EGID Eosinophilic gastroenteritis FPIEC Eosinophilic proctitis

Classification of EGID

Definition

Chronic immune/ antigen-mediated clinicopathological condition diagnosed based on Symptoms of esophageal dysfunction HPE at least 15 eosinophill / HPF Exclusion of other competing causes of

eosinophilia including PPI-REE

Eosinophilic esophagitisEpidemiology

Prevalence 0.5 to 1 case / 1000 persons (general population)1

12-22% (in person symptomatic with dysphagia)2

46-63% (food impaction)3

Incidence 10/10000 person/year Prevalence increasing No reported cohorts in India and Africa All ages but peak children and adults < 40 yrs 3-4 X male > female White > non white

Dellon ESet al. Prevalence of eosinophilic esophagitis in the United States. CGH2013Mackenzie SH et al. Clinical trial: eosinophilic esophagitis in patients presenting with dysphagia: a prospective analysis. Desai TK et al. Association of eosinophilic inflammation with esophageal food impaction in adults. Gastrointest Endosc 2005;

Pathogenesis

Clinical presentation

Children Nonspecific

Vomiting Regurgitation Abdominal pain Decreased appetite Fever / weight loss

Atopy 50-75% allergic rhinitis 30-50 % asthma

Clinical presentation

Adults Solid food dysphagia 60 – 100%

Difficulty swallowing Dietary modification Lubricating foods

Heartburn 30 – 60% Non cardiac chest pain 8- 44 %

Atopy (allergic rhinitis , asthma) Abdominal pain , diarrhea, weight loss

Dellon ES, Gibbs WB, Fritchie KJ, Rubinas TC, Wilson LA, Woosley JT, Shaheen NJ. Clinical, endoscopic, and histologic findings distinguish eosinophilic esophagitis from gastroesophageal reflux disease. Clin Gastroenterol Hepatol 2009;7:1305-1313.

Mechanism of differential presentation

Endoscopic findings

Concentric ringsTransient - felinization

Longitudinal furrows

White exudate Decreased vascularity

Crepe paper mucosa

Endoscopic finding : salient features Finding may occur in isolation or

together Children normal / edema / plaque Adult rings / strictures However 7-10 % cases normal

esophagus on luminal imaging Therefore the need for obtaining tissue

diagnosis EoE endoscopic reference score (EREFS)

Exudates ,rings , edema ,furrows, strictures Hirano I, Moy N, Heckman MG, Thomas CS, Gonsalves N, Achem SR. Endoscopicassessment of the oesophageal features of eosinophilic oesophagitis: validation of a novelclassification and grading system. Gut 2013;62:489-95.

Histopathology

Distal esophagus Proximal esophagus

At least 15 eosinophils/hpf must be present to consider a diagnosis of EoE (peak count)

Other findings Eosinophilic degranulation Eosinophil micro-abscesses Basal layer hyperplasia Elongation of the rete pegs Dilated intracellular spaces or spongiosis Lamina propria fibrosis

None of these findings are pathognomonic for EoE, and histopathologic findings alone cannot diagnose EoE

Changes are patchy

Histopathological criteria

Greater than or equal to 15 intraepithelial eosinophils per HPF in at least one esophageal site

Additional sections should be obtained from nondiagnostic but highly suggestive biopsies, and fewer eosinophils than the recommended threshold value may not eliminate the diagnosis in patients who otherwise would qualify for the diagnosis

Altered eosinophil character manifest as surface layering and abscesses.

Epithelial changes such as basal layer hyperplasia, dilated intercellular spaces.

Thickened lamina propria fibers.

Diagnostic criteria

Diagnosis based on Symptoms of esophageal dysfunction HPE at least 15 eosionophil / HPF

Peak count Size of HPF described 2-4 biopsy separate location

(distal and mid /proximal) Exclusion of other competing causes of

eosinophilia including PPI-REE

Differential diagnosis

GERD PPI-REE Secondary eosinophilia

HES CTD Eosinophillic gastroentritis Crohn’s disease

EoE Vs. PPI-REE

Patients suspected of having EoE Symptoms Endoscopic findings ≥ 15 eosinophils/hpf in esophageal

biopsies Undergo clinical and histologic

resolution following PPI therapy (2 weeks)

EoE Vs. GERD

Symptoms overlap esp. heartburn GERD much more prevalent HPE in GERD can have eosinophil infiltration(< 15

cells /HPF)

Presence of GERD does not preclude a diagnosis of EoE

Determine contribution of of reflux towards patients symptoms

GERD Eosinophilic esophagitis

Impaired esophageal clearance of

physiologic reflux

Reflux leads to a leaky epithelial barrier

Management

Pharmcological therapy Steroids

Non pharmacological therapy Diet elimination therapy Endoscopic dilatation

Maintenance therapy Need Options

NONE FDA approved

Steroids

Topical corticosteroids Fluticasone MDI swallow no spacer

Dose 88-1760 µg/day Budesonide OVB (slurry mixed with sucralose)

Dose 2 mg/day 3-5 gm of sucralose per 2 ml of aqueous solution

Proper technique important MDI end expiration during breath hold After meals Do not eat /drink anything for 60 min after

swallowing

Side effects of local steroids No reports of adrenal axis suppression

after 8-12 weeks course No long term follow-up study Avoid grape fruit (- CYP3A ) Oral candidiasis uncommon Esophagial candidiasis 15-20%

Diet elimination therapy

Principle -Removal of allergens Amino acid based Directed elimination diets based on

allergy test results Non directed elimination diets

Elemental diet

Remission 85-95% Absence of complication Issues

Palatablity Need for enteral feeding tubes Patient complaince Cost

Baseline Introduce

diet 4-6 week

Reintroduce group

A

2-3 mon Endoscopy

Reintroduce group B and so

on

Elimination diet

Directed based on Skin prick test not effective Atopy patch test effective Remission 35-56%

Empiric elimination diet 6 food elimination diet Cow milk protein , soy , egg , peanut and

fish Remission 66-78% Allows variety of foods

Endoscopic dilatation

Treats symptoms not inflammation Done via balloons or wire guided bougie Goal- mucosal tear (NOT a complication) Symptoms improve 50% symptom free

after 1 session for 1 year S/E 75% chest pain (expect it ) Perforation

Previous series as high as 8 % Recent data 0.3%1

Egan JV, Baron TH, Adler DG, Davila R, Faigel DO, Gan SL, Hirota WK, Leighton JA,RD. Esophageal dilation. Gastrointest Endosc 2006;63:755-60.

Eosinophilic gastroenteritis

Rare prevalence 22-28/100000 persons 20- 60 years M=F rare children Atopy 40-50% Symptoms protean vary by site and depth of

involvement small intestine > stomach Mucosal abdominal pain vomiting diarrhea Muscular GOO intestinal obstruction (but

stricture RARE) Serosal 10% ascites protracted course

Management : mainly case series Systemic corticosteroids 0.5 to 1 mg/ kg

induction Budesonide 9 mg/day solubilized OD HS Dietary therapy elemental and 6 food

elimination diet anti IgE , monteleukast , cromolyn Na

not effective

Food protein induced enterocolitis Presents in Infancy, 1st few weeks of life Trigger cow milk protein based formula Profuse bloody mucoid diarhoea wieght

loss Rx elimination of causal milk and soy

proteins 90% can tolerate milk by 3 years of age

Eosinophilic proctitis

Children younger than 2 years Bloody stool, lack any systematic

symptoms Endoscopy focal rectal erythma ,

erosions HPE eosinophil in mucosa minimum 6

eosinophils /HPF and /or eosinophils invading crypts or muscularis mucosae

Rx elimination diet

Summary and take home points

• Over the past ten years, EoE has become a major cause of GI symptoms,including dysphagia and food impaction in adolescents and adults, and feeding intolerance, failure-to-thrive, regurgitation, heartburn, and vomiting in children.

• EoE is a clinicopathologic condition, so the entire clinical and histologic picture must to be considered in order to make a diagnosis; no single feature is diagnostic on its own.

• EoE is now diagnosed based on consensus guidelines requiring symptoms of esophageal dysfunction, at least 15 eosinophils per high-power microscopy field on esophageal biopsy, and eosinophilia limited to the esophagus with other causes of esophageal eosinophilia (including proton pump inhibitor responsive esophageal eosinophilia) excluded.

• Effective first line treatment strategies include topical steroids, such as swallowed fluticasone or budesonide, or dietary therapy with either an elemental formula, a six-food elimination diet, or a targeted elimination diet.

THANK YOU

Maintenance therapy

Chronic disease Long standing disease higher risk of

stictures Elemental diet and Local steroids

prevent fibrosis Options diet / local steroids Unanswered questions

Duration Side effects