Concept:
Eosinophilic Esophagitis (EoE) is a chronic, immune-mediated, antigen-driven esophageal disorder characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation.
The diagnostic threshold is defined as $\ge 15\text{ eosinophils/high-power field (HPF)}$ on mucosal biopsy in the absence of other systemic causes of eosinophilia.
Explanation:
• Common endoscopic features in pediatric EoE include linear vertical furrows, circular mucosal rings ("trachealization" of the esophagus), white plaques/exudates (eosinophil microabscesses), and strictures.
• First-line medical therapy traditionally begins with high-dose Proton Pump Inhibitor (PPI) therapy ($1\text{--}2\text{ mg/kg/day}$) for an 8- to 12-week trial.
• In patients who remain symptomatic and fail to achieve histological remission ($>15\text{ eos/HPF}$) after an adequate 8-week course of PPI therapy (PPI-refractory EoE), the next guideline-recommended step (NASPGHAN/ESPGHAN/AGA) is:
1. Topical (Swallowed) Corticosteroids: Viscous oral budesonide slurry or swallowed fluticasone propionate from a metered-dose inhaler (MDI without spacer) swallowed onto the esophageal mucosa.
2. Alternatively, dietary elimination therapies (e.g., 6-food elimination diet).
• Systemic corticosteroids are reserved only for acute severe dysphagia causing significant dehydration/weight loss or critical narrowing.
• Esophageal dilation is indicated only for persistent, high-grade fibrostenotic strictures causing obstruction that fail medical anti-inflammatory therapy.
Final Answer:
The appropriate next step in PPI-unresponsive eosinophilic esophagitis is the initiation of topical (swallowed) corticosteroids such as fluticasone or budesonide.