Eosinophilic Esophagitis: Symptoms, Types, Causes and Treatment

Eosinophilic esophagitis (EoE) is a chronic, immune-driven disorder characterized by inflammation of the esophagus due to an accumulation of eosinophils—a type of white blood cell involved in allergic responses. Once considered rare, EoE is now recognized as a major cause of swallowing difficulties and food impactions in both children and adults. This article offers an in-depth exploration of EoE, focusing on its symptoms, types, underlying causes, and available treatment strategies, drawing on the latest research and clinical sources.

Symptoms of Eosinophilic Esophagitis

EoE presents with a diverse range of symptoms that can often be mistaken for other gastrointestinal conditions, particularly gastroesophageal reflux disease (GERD). Recognizing the pattern and context of these symptoms is crucial for early diagnosis and effective management.

Symptom	Age Group	Typical Features	Sources
Dysphagia	Adults, Adolescents	Difficulty swallowing, food sticking	2 3 5 6 8
Food Impaction	Adults, Older Children	Sudden blockage of food in esophagus	1 5 8 9
Heartburn	All	Burning chest discomfort	2 5 16
Chest/Epigastric Pain	All	Discomfort not relieved by antacids	2 3 8
Vomiting/Nausea	Children	Often with feeding issues	3 4 8
Feeding Difficulties	Infants/Children	Poor feeding, failure to thrive	3 4 8
Allergic Disorders	All	Asthma, eczema, rhinitis, food allergy	2 4 6

Table 1: Key Symptoms

Overview of Symptom Patterns

EoE can mimic or overlap with more common conditions like GERD, which often leads to delays in diagnosis. The hallmark symptom in adults and older children is dysphagia—difficulty swallowing, especially with solid foods. Food impaction, where food becomes stuck in the esophagus, can lead to emergency visits and is a red flag for EoE. In younger children and infants, symptoms are more likely to include vomiting, feeding difficulties, and poor weight gain, often with a background of other allergic conditions such as asthma or eczema 2 3 4 5 6 8.

Age-Dependent Manifestations

• Adults and Adolescents:
  • Predominantly present with dysphagia, food impaction, or chest pain 2 5 6.
  • Heartburn is present but less likely to respond to conventional antacid therapy 2 5 16.
  • Up to half have concurrent allergic diseases 2 4 6.
• Children:
  • More likely to show vomiting, nausea, feeding refusal, or failure to thrive 3 4 8.
  • Symptoms may be subtle or non-specific, making diagnosis challenging 3 8.

Endoscopic and Histological Clues

Endoscopy may reveal features such as linear furrows, concentric rings, white exudates, and strictures, though in up to a quarter of patients, the esophagus may appear normal 2 6 9 16. Biopsy is essential for diagnosis, confirming increased eosinophils (>15 per high-power field) 1 5 7 9.

Types of Eosinophilic Esophagitis

EoE is a heterogeneous disease, and its manifestations can change over time. Understanding the different types or phenotypes helps tailor treatment and predict disease progression.

Type	Age/Onset	Clinical Features	Sources
Inflammatory	Children, Early	Edema, exudate, pain, vomiting	8 13 16
Fibrostenotic	Adolescents, Adults	Rings, strictures, dysphagia	6 9 10 13
Atopic-Associated	All	Overlap with allergies/asthma	2 4 11
PPI-responsive	All	Symptoms/biopsy improve with PPIs	5 14 16

Table 2: EoE Types/Phenotypes

The Spectrum of EoE Phenotypes

EoE can present as a primarily inflammatory disease, especially in younger patients, or progress to a fibrostenotic (scarring) form in older individuals 8 10 13 16.

Inflammatory vs. Fibrostenotic

• Inflammatory EoE:
  • Typically seen in children or at early disease stages 8 13.
  • Characterized by active inflammation—edema, white exudates, and mucosal furrows seen on endoscopy 13 16.
• Fibrostenotic EoE:
  • More common in adolescents and adults or in long-standing cases 6 9 10.
  • Features include esophageal rings (trachealization), strictures, and narrowing, leading to severe dysphagia 6 9 10 13.
  • The risk of stricture increases with the duration of untreated disease 10.
• Atopic-Associated EoE:
  • Many patients have coexisting allergic conditions, which can influence symptom severity and response to therapy 2 4 11.

PPI-Responsive EoE

A subset of patients shows marked improvement in symptoms and esophageal eosinophilia with proton pump inhibitor (PPI) therapy. This group, previously thought to be a separate entity (PPI-responsive esophageal eosinophilia), is now considered part of the EoE spectrum, provided other causes are excluded 5 14 16.

Causes of Eosinophilic Esophagitis

EoE is a multi-factorial disease, with genetic predisposition, environmental exposures, and immune responses all playing important roles. Dissecting these causes helps in understanding risk and prevention strategies.

Cause	Mechanism/Trigger	Notes	Sources
Food Allergens	Immune reaction to food proteins	Most common trigger	4 5 11 12 16
Genetic Factors	Susceptibility loci (TSLP, CAPN14)	Family history increases risk	5 11 12
Environmental	Antibiotic use, hygiene hypothesis, aeroallergens	Early life exposures matter	5 10 11 12
Atopy	Allergic diseases (asthma, eczema)	High prevalence among EoE patients	2 4 11
Barrier Dysfunction	Esophageal epithelial defects	Facilitates allergen entry	11 12

Table 3: Major Causes and Risk Factors

Food and Environmental Allergens: The Main Culprits

• Food Antigens:

  • The esophagus responds abnormally to specific foods—dairy, wheat, eggs, soy, nuts, and seafood are common triggers 4 5 16.
  • Removal of triggering foods often leads to disease remission 4 5 16.

• Aeroallergens:

  • Environmental allergens (pollens, dust mites) can also contribute, explaining seasonal variation in some cases 4 11.

Genetic and Environmental Influences

• Genetic Susceptibility:

  • Specific gene variants (e.g., TSLP, CAPN14) are linked to increased risk 5 11 12.
  • Family history is common, suggesting heritable risk 5 11.

• Environmental Factors:

  • Early antibiotic exposure, mode of birth, and microbial environment may influence risk via immune priming 5 10 11 12.
  • The "hygiene hypothesis" suggests that less exposure to microbes early in life may predispose to allergic diseases like EoE 10 12.

Atopy and Barrier Dysfunction

• Atopic Disorders:
  • Up to half of EoE patients have other allergic diseases—this interplay may amplify esophageal inflammation 2 4 11.
• Epithelial Barrier Dysfunction:
  • Abnormal esophageal lining allows allergens to penetrate, triggering an immune response 11 12.
  • Inflammatory cytokines like IL-5 and IL-13 play a key role in recruiting eosinophils and driving chronic inflammation 11 12.

Treatment of Eosinophilic Esophagitis

Treatment aims to control symptoms, heal esophageal inflammation, and prevent long-term complications like strictures. Management is typically tailored to disease severity, phenotype, and patient preferences.

Treatment	Approach/Mechanism	Indication/Effectiveness	Sources
Proton Pump Inhibitors (PPIs)	Acid suppression, anti-inflammatory	First-line, 40-70% respond	1 5 14 16
Topical Steroids	Swallowed corticosteroids (fluticasone, budesonide)	Most effective for remission	1 5 14 16
Dietary Therapy	Elimination of food allergens	Empirical or allergy-guided	4 5 14 16
Esophageal Dilation	Mechanical widening of strictures	For fibrostenotic disease or strictures	1 5 9 13 16
Maintenance Therapy	Long-term use of above strategies	Prevents relapse, especially in chronic cases	8 9 15 16

Table 4: Main Treatment Strategies

Three Pillars: Drugs, Diet, and Dilation

EoE management is often summarized as the "three D's": Drugs, Diet, and Dilation 16.

Drug Therapy

• Proton Pump Inhibitors (PPIs):
  • First-line therapy due to ease of use and safety profile 5 14 16.
  • About half of patients achieve remission with PPIs 5 14.
• Topical Corticosteroids:
  • Swallowed steroids (fluticasone, budesonide) are the most effective agents for inducing and maintaining remission, with 65–80% response rates 5 14 16.
  • Minimal systemic absorption, so side effect risk is low 5 14 16.
• Other Agents:
  • Systemic steroids and immunosuppressants are reserved for severe/refractory cases 16.

Dietary Therapy

• Elemental Diets:
  • Use of amino acid-based formulas is highly effective but rarely practical for long-term use 5 16.
• Empirical Food Elimination:
  • Stepwise removal of common allergens (e.g., six-food elimination diet) can induce remission in over half of patients 4 5 14 16.
• Allergy Testing-Guided Elimination:
  • Identifies specific triggers, though effectiveness varies 4 16.

Endoscopic Dilation

• For Fibrostenotic EoE:
  • Indicated when strictures cause significant dysphagia or food impactions 1 5 9 16.
  • Does not address underlying inflammation, so should be combined with medical therapy 9 16.

Maintenance and Monitoring

• Long-Term Therapy:

  • High relapse rates require ongoing therapy—either pharmacologic or dietary 8 9 15 16.

• Assessment of Response:

  • Combination of symptom improvement, endoscopic findings, and histology is used to gauge treatment success 15.

• Personalized Approach:

  • Phenotype (inflammatory vs. fibrostenotic), patient preference, and presence of allergies guide therapy selection 14 16.

Conclusion

Eosinophilic esophagitis is a complex and increasingly recognized disorder with important implications for gastrointestinal health. Understanding its symptoms, types, causes, and treatment options enables earlier diagnosis and more effective management.

Summary of Key Points:

• Symptoms vary by age, with dysphagia and food impaction in adults, and feeding issues and vomiting in children 2 3 4 5 6 8.
• Types include inflammatory and fibrostenotic forms; atopic associations and PPI-responsive variants are also recognized 6 8 9 10 11 13 14 16.
• Causes involve a mix of food/environmental allergens, genetic susceptibility, and epithelial barrier dysfunction 4 5 10 11 12 16.
• Treatment revolves around PPIs, topical steroids, dietary elimination, and dilation for strictures, with maintenance therapy needed to prevent relapse 1 4 5 9 13 14 15 16.

Early recognition and tailored therapy can dramatically improve quality of life for those living with EoE, and ongoing research continues to refine our understanding of this fascinating disease.