| Below is a summary version of the 2011 consensus recommendations for eosinophilic esophagitis (EoE) diagnosis and treatment.[1] Since 2007, the number of EoE publications doubled, providing new disease insight. A panel of 33 physicians with expertise in pediatric and adult allergy/immunology, gastroenterology and pathology conducted a systematic review of the EoE literature (since September 2006) using electronic databases. Based on the literature review and expertise of the panel, a summary of the recommendations is provided here. |
| Conceptual definition - To refine perceptions and hypotheses for future EoE studies, the following conceptual definition was developed: "Eosinophilic esophagitis represents a chronic, immune/antigen mediated, esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation." |
| Diagnostic guidelines - Taking into account increasing clinical experiences and research, the following guidelines were proposed: "Eosinophilic esophagitis is a clinico-pathological disease. Clinically, EoE is characterized by symptoms related to esophageal dysfunction. Pathologically, one or more biopsies must show eosinophil predominant inflammation.* With few exceptions, 15 eosinophils/hpf (peak value) is considered a minimum threshold for a diagnosis of EoE. The disease is isolated to the esophagus and other causes of esophageal eosinophilia should be excluded, specifically proton pump inhibitor (PPI)-responsive esophageal eosinophilia.¶ (Table A). The disease should remit with treatments of dietary exclusion and/or topical corticosteroids. EoE should be diagnosed by clinicians taking into consideration all clinical and pathologic information; neither of these parameters should be interpreted in isolation. |
| Summary statements |
| History and physical - History should focus on difficulties with eating and swallowing (Table B) and a thorough physical examination should focus on growth and nutrition parameters and to assess potential other causes of esophagitis (Table A). |
| Endoscopy - Endoscopy with esophageal biopsy is considered the only reliable EoE diagnostic. Two to four biopsies each from the proximal and distal esophagus should be obtained. Gastric and duodenal biopsies should be examined to exclude other potential causes of eosinophil associated gastrointestinal disease (Table A). Endoscopic features can suggest but cannot diagnose EoE. |
| Radiography - An upper gastrointestinal series is useful to characterize anatomic abnormalities that may escape endoscopic detection such as proximal strictures and long segment narrowing. |
| Histopathology - See Diagnostic guidelines and Table C. |
| Allergic evaluation - An evaluation by an allergist or immunologist is recommended to document aeroallergen sensitization and seasonal variability as it may pertain to EoE and to control concurrent atopic diseases. Serum IgE and/or skin prick testing for immediate type hypersensitivity reactions to foods are warranted to help identify food allergic disease in patients with EoE. Medically supervised food reintroduction may be necessary for patients with previous allergic reactions to a food or IgE-mediated sensitivity documented by IgE testing. Skin prick tests, serum IgE tests, and food patch tests may be used to help identify foods that are associated with EoE, but are not sufficient to make the diagnosis of food allergy driven EoE. Foods that trigger EoE can only be identified by documenting disease remission and recrudescence after specific food elimination and addition. |
| Genetics - EoE runs in families and although specific genes that pre-dispose to EoE susceptibility have been identified (thymic stromal lymphopoietin [TSLP], eotaxin-3), they are not yet ready for usage in clinical settings. |
| Treatments - (Table D). |
| Dietary therapy - Amino acid based formulas and dietary elimination are effective therapies for children with EoE and their use in adults requires further study. Patient's lifestyle, adherence to therapy and family resources should be considered when instituting these treatments. Consultation with a registered dietitian is strongly encouraged. EoE foods triggers may need to be restricted indefinitely. |
| Steroids - Topical corticosteroids are effective therapy for EoE in children and adults. Systemic corticosteroids may be used for emergent situations (severe dysphagia, hospitalization, weight loss) but caution is warranted for chronic management of EoE. |
| Other treatments - Cromolyn sodium, leukotriene receptor antagonists, and immunosuppressives (azathioprine or 6-mercaptopurine) are not recommended treatments for EoE. Biologic agents require further clinical studies and are currently not recommended for routine use. |
| Dilation - Esophageal dilation can provide relief of dysphagia in selected EoE patients. If high-grade esophageal stenosis is not present, a trial of medical or dietary therapy prior to esophageal dilation is reasonable. |
| Table A.[1] Conditions associated with esophageal eosinophilia |
| Gastroesophageal reflux disease (GERD) |
| Eosinophilic esophagitis (EoE) |
| Eosinophilic gastrointestinal diseases (EGIDs) |
| Celiac disease |
| Crohn's disease |
| Infection |
| Hypereosinophilic syndrome (HES) |
| Achalasia |
| Drug hypersensitivity |
| Vasculitis |
| Pemphigoid vegetans |
| Connective tissue disease |
| Graft versus host disease |
| Table B.[1] Symptoms related to EoE |
| Dysphagia and feeding dysfunction |
| Coping mechanisms - Avoiding highly textured foods such as meats and bulky foods such as bagels, cutting food in small pieces, lubricating foods before eating with liquids or butter, extensive chewing of foods, washing food down with liquids, prolongation of mealtimes |
| Food impaction |
| Coping mechanisms - Drinking liquid to wash food down, raising hands above head, jumping up and down, waiting for food to dissolve or to pass |
| Chest pain |
| Coping mechanisms - Avoiding foods or liquids that exacerbate pain such as highly textured or bulky foods, alcohol or acidic drinks |
| GERD like symptoms recalcitrant to medical and surgical GERD management |
| Abdominal pain |
| Vomiting |
| Anorexia and early satiety |
| Table C.[1] Histological characteristics of EoE |
| Mucosal eosinophilia |
| Eosinophil microabscesses |
| Superficial layering of eosinophils |
| Extracellular eosinophil granules |
| Surface epithelial desquamation |
| Basal zone hyperplasia |
| Rete peg elongation |
| Dilated intercellular spaces |
| Subepithelial fibrosis/sclerosis/lamina propria fibrosis |
| Table D.[1] Recommended initial treatments for EoE |
| Topical swallowed corticosteroids (initial doses)[2-4] |
| Fluticasone (spray metered dose inhaler directly in mouth then swallow) |
| Adults: 440 to 880 mcg twice daily |
| Children: 88 to 440 mcg twice to four times daily (maximum 1760 mcg per day) |
| Budesonide (as a compounded viscous suspensionΔ) |
| Children (<10 years): 1 mg daily |
| Older children and adults: 2 mg daily |
| Following administration, patients should not rinse the mouth or eat or drink for 30 minutes |
| Systemic corticosteroids (severe disease) |
| Prednisone: 1 to 2 mg/kg per day by mouth in one or two divided doses (maximum 60 mg per day), taper after week 4[4] |
| Education, advocacy, and/or research support resources: |
| American Academy of Allergy, Asthma, and Immunology: www.aaaai.org |
| American Partnership for Eosinophilic Disorders: www.apfed.org |
| Campaign Urging Research for Eosinophilic Disorders: www.curedfoundation.org |
| Children's Digestive Health and Nutrition Foundation: https://naspghan.org/naspghan-foundation/ |
| Food Allergy & Anaphylaxis Network: www.foodallergy.org |
| North American Society of Pediatric Gastroenterology and Nutrition: www.naspghan.org |
| Registry for Eosinophilic Gastrointestinal Disorders: https://www.rarediseasesnetwork.org/cms/cegir |
| TIGERS is grateful to the American Partnership for Eosinophilic Disorders for providing financial support of this summary document. APFED (www.apfed.org) is a 501(c)3 non-profit dedicated to education, advocacy, support and advancing research to improve the lives of those with eosinophil associated diseases. Content summarized by TIGERS. |
