Eosinophilic oesophagitis

A chronic, immune/antigen-mediated, oesophageal disease characterised clinically by symptoms related to oesophageal dysfunction and histologically by eosinophil-mediated inflammation

• 'EoE' in USA

Epidemiology

• Detected in most countries
• Maybe more common in urban areas
• Seasonal exacerbations in keeping with atopy
• Probable increasing incidence over time

Risk factors

• Limited data
• Not particularly increased with smoking or NSAIDs or H. pylori
• Strongly associated with atopic disorders - food allergies, environmental allergies, asthma, atopic dermatitis

Pathophysiology

• Subtypes:
  • 1: mild, with normal-appearing oesophagus, and mild histological changes
  • 2: inflammatory subtype with highest expression of cytokines
  • 3: fibrostenotic subtype with a narrow-calibre oesophagus, and highest degree of endoscopic and histological severity

Presentation

• Adults and teenagers frequently present with dysphagia to solids (most commonly), food impaction, central chest pain, GORD, upper abdominal pain
• Children present with feeding difficulties, GORD and abdominal pain

Diagnosis

• Eosinophils present on biopsy (essential)
  • Total of at least 6 biopsies: 2-4 biopsies each from the distal oesophagus and another 2-4 from the mid or proximal oesophagus, along with gastric and duodenal biopsies looking for other diseases
  • The vast majority of patients have at least 15 eosinophils per hpf in at least one biopsy specimen
  • Also look for eosinophil microabscesses, superficial layering of eosinophils, sheets of eosinophils, extracellular eosinophil granules, subepithelial and lamina propria fibrosis and inflammation, basal cell hyperplasia, papillary lengthening, and increased numbers of mast cells/B cells/IgE-bearing cells
• Accompanied by characteristic symptoms (essential)
• Endoscopic appearance (not essential)
  • Feline oesophagus (stacked circular rings) (44%)
  • Strictures, especially proximal (21%)
  • Attenuation of the subepithelial vascular pattern (41%)
  • Linear furrows (48%)
  • Whitish specks representing eosinophil micro-abscesses (27%)
  • Small calibre oesophagus (9%)
• Other causes of oesophageal eosinophilia have been excluded (essential)
  • GORD
    • Favour EoE if proximal oesophageal involvement, subepithelial lamina propria fibrosis, eosinophilic abscesses, more severe basal cell hyperplasia, activated mast cells
    • If histological findings resolve with PPI alone, probably more GORD rather than EoE
    • EoE shouldn't be responsive to PPI
  • Recurrent vomiting from other causes
  • Parasitic and fungal infections
  • Allergic vasculitis
  • Drug injury
  • Connective tissue diseases
  • Bullous pemphigoid
  • Pemphigoid vegetans
  • Graft-versus-host disease
  • Achalasia
  • Drug hypersensitivity
  • Coeliac
  • Vasculitis
  • Carcinoma

Management

Dietary

• • Identify allergens and avoid
  • Generally refer to allergist

Pharmacological

• • Start PPI on diagnosis for 8/52 - 40mg daily
  • If symptoms fail to improve after 4/52, increase to 40mg BD

Endoscopic

• • Repeat endoscopy after 8/52 of PPI
    • If there is good response (71%), continue PPI at lowest effective dose
    • If no response, pursue dietary modification or topical glucocorticoid (oral budesonide 2mg BD is best-studied). Those with strictures on index endoscopy are less likely to respond. Take the drink slowly over 5 minutes, and don't consume anything for 30 minutes afterwards.

Complications

• Strictures
  • Dilation is effective, but has no impact on underlying inflammation
  • Try to reserve it for strictures that don't respond to medical therapy.
  • Perform carefully - can cause deep mucosal tears and perforation. Limit progression per session to 3mm or less. Multiple dilations are often required to attain a diameter of 15-18mm.
  • Unclear whether patients with EoE are at higher risk of perforation - older studies suggested the rate is 5-7%, but the rates are 0.03-0.7% in modern series

Natural history

• Chronic disease with recurrence likely on stopping treatment
• Does not appear to significantly shorten lifespan
• Long-term prognosis of dysphagia is unknown