Eosinophilic Esophagitis Management

Introduction

Eosinophilic Esophagitis (EoE) is a chronic inflammatory disease characterized by the presence of eosinophils in the esophagus, leading to symptoms such as dysphagia, abdominal pain, and diarrhea, as stated by the American Gastroenterological Association (AGA) 1, 2, 3

Histological assessment for EoE includes peak eosinophil count per 0.3 mm², basal cell hyperplasia, edema, eosinophil microabscesses, eosinophil layering, eosinophil degranulation, and subepithelial sclerosis, as recommended by the American Gastroenterological Association (AGA) 1
Initial workup for eosinophilia should include a comprehensive history, physical examination, CBC with differential, comprehensive metabolic panel with uric acid, lactate dehydrogenase, liver function tests, serum tryptase levels, and vitamin B12 levels, as recommended by the National Comprehensive Cancer Network 4

The following tests are recommended for comprehensive evaluation:

Test	Description
Complete blood count with differential	To confirm eosinophilia
Peripheral blood smear	To evaluate blood cell morphology
Comprehensive metabolic panel with liver function tests	To assess organ function
Urinalysis with protein-to-creatinine ratio	To evaluate kidney function
C-reactive protein	To assess inflammation
Serum tryptase	To evaluate mast cell activation
Vitamin B12 levels	To assess nutritional status

Concentrated stool microscopy (multiple samples, at least 3 specimens on different days) is recommended for accurate diagnosis, with strongyloides serology having a high yield across all regions and stool PCR for parasites having a higher sensitivity than microscopy 6

Proton pump inhibitors (PPIs) should be administered twice daily for 8-12 weeks before assessing histological response, and can be used as maintenance therapy in patients who achieve histological response, as recommended by the American Gastroenterological Association 1
Topical corticosteroids are supported by high-quality evidence for inducing histological and clinical remission, and maintenance treatment is recommended to prevent clinical and histological relapse, according to the American Gastroenterological Association 1
Dietary therapy (elimination diets or elemental formula) is effective in 92-98% of children, according to the American Gastroenterological Association (AGA) 3
First-line therapy options for EoE include proton pump inhibitors, topical corticosteroids (strong recommendation, moderate quality evidence), and dietary therapy (elimination diets or elemental formula) 1

Endoscopic dilatation is effective and safe for improving symptoms in patients with fibrostenotic disease, and clinical outcomes are better when dilatation is combined with effective anti-inflammatory therapy, as recommended by the American Gastroenterological Association 1

For refractory disease, consider biologic therapy with dupilumab, which shows promise based on current evidence, and endoscopic dilation if fibrostenotic disease is present 1
Dupilumab, an anti-IL-4 receptor-α, has shown significant reduction in dysphagia symptoms and esophageal eosinophilia in phase 2 trials, with a mean reduction of 3.0 vs 1.3 in placebo (p=0.0304) and reduced peak mean eosinophil count by 86.8 eosinophils/hpf compared to placebo (p<0.001) 1
Cendakimab (RPC4046) has shown significant reduction in mean eosinophil count and dysphagia scores in phase 2 trials, and is effective even in steroid-refractory cases (n=47) 1
Mepolizumab and Reslizumab, anti-IL-5 antibodies, have shown reduction in tissue eosinophilia but limited clinical improvement in early studies 2
Infliximab, a TNF-α blocker, has shown little improvement in symptom scores, endoscopic assessment, or eosinophil count in severe corticosteroid-dependent EoE 1
Vedolizumab has shown response in case reports of eosinophilic gastroenteritis and concomitant EoE/Crohn's disease, but has no high-quality data supporting its use in EoE 1
Omalizumab has shown no change in esophageal eosinophilia or symptom reduction in one RCT, and the AGA/JTF suggests against its use for EoE (conditional recommendation, very low-quality evidence) 2

Patients with refractory disease or significant concomitant atopic disease should be jointly managed by a gastroenterologist and specialist allergist, according to the American Gastroenterological Association 1
Regular assessment of both symptoms and eosinophil counts is recommended every 1-3 months 1
Histological remission is defined as complete remission (peak eosinophil count <5 eosinophils per 0.3 mm²) or partial remission (peak eosinophil count between 5-15 eosinophils per 0.3 mm²) 1
Regular monitoring of eosinophil counts and assessment for resolution of symptoms are crucial in managing eosinophilia, with a frequency of monitoring every 1-3 months, as recommended by the National Kidney Foundation 6
Vigilance for end-organ damage, particularly cardiac complications, is essential in patients with persistent eosinophilia, and consideration of bone marrow examination to rule out hematologic malignancy may be necessary, with a frequency of monitoring every 3-6 months, as recommended by the American College of Cardiology 6

Underestimating the frequency of strictures and narrow lumen esophagus in EoE, as warned by the American Gastroenterological Association 1
Failing to combine therapeutic dilatation with effective anti-inflammatory therapy, as cautioned by the American Gastroenterological Association 1
Not recognizing that EoE is the most common cause of spontaneous perforation of the esophagus, as highlighted by the American Gastroenterological Association 1
Discontinuing topical steroid treatment prematurely, which can lead to high rates of clinical and histological relapse, as advised by the American Gastroenterological Association 1

Candida infection may occur in a small proportion of patients treated with topical corticosteroids, and should be managed with topical antifungals while continuing steroids 1
Systemic side effects of topical steroids have not been documented during long-term treatment of EoE patients, but continued monitoring of bone mineral density and adrenal suppression is recommended in children and adolescents 1
The psychological impact of dietary therapy should be discussed with patients, and alternative treatments such as diet or topical steroids should be considered if PPI therapy causes unwanted side effects 1
Long-term systemic corticosteroid use can lead to significant side effects, including growth abnormalities, bone abnormalities, mood disturbances, and adrenal suppression, as reported by the American Gastroenterological Association 3