Journal Article:

nejmra1502863

Summary:

Daniel DeMarco                  Journal Article Assignment                         Rotation #5: Peds

 

Eosinophilic Esophagitis

Glenn T. Furuta, MD and David A Katzka MD

New England Journal of Medicine

 

What is it?:

• “Chronic, immune-mediated or antigen-mediated esophageal disease characterized by symptoms related to esophageal dysfunction and eosinophil-predominant inflammation”
• Predominant antigens = food-based antigens

 

Epidemiology?:

• Western World prevalence estimate = 0.4% of all children and adults
• Increasing prevalence in Asia
• Male predominance: 3:1; predominantly white men with onset from school age to midlife
• Factors associated with increased risk of eosinophilic esophagitis (EOE): birth by C-section, prematurity, antibiotic exposure during infancy, food allergy, lack of breast-feeding, living in areas of lower population density, personal or family history of atopic disorders: asthma, eczema, rhinitis, anaphylactic food allergy
• Genetic Component = family history of EOE, three genes altered in EOE based on genome studies
• Co-existing conditions = connective-tissue disease, celiac disease, Crohn’s disease

 

Symptoms?:

• In Children?: feeding problems, failure to thrive, vomiting, nausea, heartburn, abdominal pain
• In Adolescents and Adults?: dysphagia, food impaction
• In All?: chronic reflux symptoms

 

DDx?:

• GERD
• Parasitic infection
• Allergic vasculitis
• Esophageal leomyomatosis
• Crohn’s disease

 

Pathogenesis?:

• Combination of:
  • Impaired Barrier Function: dilated interepithelial spaces, altered epithelial barrier function, down-regulation of proteins with barrier function which alter the epithelial permeability = increased antigen presentation = increased recruitment of eosinophils
  • Enhanced TH2 Activity and Allergic Susceptibility
  • IL-5 and IL-13: treatment with anti-IL5 and IL-13 antibodies decreases esophageal eosinophils in children and adults
  • Food-specific IgG4

 

Procedures/Imaging?:

• Upper Endoscopy + Biopsy
• Intraluminal Balloon Device to measure wall compliance
• Contrast Esophagography

 

Endoscopic Findings?:

• “White Specks” = eosinophilic exudates
• Mucosal edema
• Linear furrows
• Esophageal rings
• Strictures
• “Crepe-Paper Esophagus” = linear tears 2’ to trauma
• “Tug Sign” = firm feeling felt by endoscopist
• Development of validated endoscopic scoring system = EREFS
  • Edema
  • Rings
  • Exudates
  • Furrows
  • Strictures

 

Barium Esophagography?:

• Strictures/narrowing may not be detected on endoscopy = often there is evidence of this on barium esophagography

 

Findings on Histology?:

• Hallmark = Esophageal mucosa with > or = to 15 eosinophils per high-power field
  • Sensitivity = 100%
  • Specificity = 96%
• Other:
  • Aggregates of eosinophils = microabscess
  • Eosinophil layers along luminal surface
  • Dilated intercellular spaces
  • Rete-peg elongation
  • Basal-cell hyperplasia
  • Increased number of other inflammatory cells = lymphocytes, mast cells, basophils

 

Complications:

• Esophageal stricture
• Food impaction
• Perforation
• Malnutrition
• EOE IS NOT PRE-MALIGNANT

 

Treatment:

• Goals = alleviate symptoms, control inflammation, restore function
• Modalities = diet, drugs, dilation
• Team = gastroenterologist, allergist, nutritionist
• Diet
  • Amino-acid based formula (elemental diet)
  • Allergen-targeted diets
  • Six-food elimination: wheat, milk, soy, nuts, eggs, seafood
  • Challenges: inc. cost of food, poor adherence, nutritional deficiencies
• Drugs
  • Proton Pump Inhibitors = decrease cytokine secretion from esophageal epithelium possibly conferring anti-inflammatory effect
    • “Proton-pump inhibitor-responsive EOE”
  • Topical Glucocorticoids = decrease inflammatory cells, decrease fibrosis, reversal of IL-13 and EOE transcriptome
    • Fluticasone orally (MDI)
    • Budesonide orally (Viscous Liquid)
  • S/E: local candida infection, adrenal axis suppression, bone demineralization, diminished growth
• Dilation
  • Alleviation of esophageal narrowing
  • No effect on inflammatory process