Step-Down Dose Reduction Dupilumab Maintenance is an Effective Long-Term Treatment Strategy in Eosinophilic Esophagitis.

ACG 2025 (GRADE) is still the governing guideline. The ASGE 2026 consensus Part 2 (modified Delphi, 28 statements at ≥80% agreement; not GRADE) adds endoscopic and pediatric detail without contradicting it. DIAGNOSIS: symptoms of esophageal dysfunction plus ≥15 eos/hpf after excluding other causes of esophageal eosinophilia. A PPI trial is not a diagnostic criterion (PPI-REE abandoned). Do the initial diagnostic EGD off PPI, topical steroid and dietary therapy (ACG key concept; ASGE consensus, which gives no washout duration). Initial assessment is a standard EGD, not a less-invasive modality, so that full biopsies and dilation are possible (ASGE). Take ≥6 esophageal biopsies from ≥2 levels in anyone with symptoms or endoscopic findings consistent with EoE, targeting visible lesions. In CHILDREN with suspected EoE, also biopsy the stomach and duodenum (ASGE). Use EREFS at every endoscopy and report a quantified peak eosinophil count (ACG strong/low; ASGE). TREATMENT (ACG): swallowed topical steroids STRONG/moderate; high-dose PPI CONDITIONAL/low; empiric elimination diet CONDITIONAL/low. Choose a single initial therapy by shared decision-making. Fluticasone or budesonide are both reasonable. Adult doses: PPI double the reflux dose (e.g., omeprazole 20 mg BID or 40 mg daily); budesonide 2-4 mg/day (BOS 2 mg BID); fluticasone 1,760 mcg/day divided. Pediatric doses: PPI 2 mg/kg/day; budesonide 1-2 mg/day; fluticasone 110-880 mcg/day. Allergy testing is not used to direct diets. A 1FED/2FED step-up diet with a dietitian is acceptable, with endoscopic and biopsy confirmation after each reintroduced food. Dupilumab is CONDITIONAL for PPI-nonresponsive patients aged ≥12 (moderate) and for ages 1-11 at ≥15 kg (low). Weight-band dosing: 15-<30 kg 200 mg q2w; 30-<40 kg 300 mg q2w; ≥40 kg 300 mg weekly. Recommendations AGAINST: omalizumab, cromolyn, montelukast. No recommendation for cendakimab, benralizumab, lirentelimab, mepolizumab or reslizumab. DILATION: an adjunct to anti-inflammatory therapy for strictures causing dysphagia (ACG conditional/low). Start low and go slow; target ≥16 mm in adults and adolescents over ≥1 session. In children, ASGE supports dilation of symptomatic strictures or a narrow-caliber esophagus regardless of disease activity, using the same start-low/go-slow approach. MAINTENANCE: continue effective therapy (ACG STRONG despite low evidence) at the lowest effective dose. MONITORING: judge response on symptoms, endoscopy and histology together, never symptoms alone. Assess response endoscopically at 8-12 weeks after starting a PPI or topical steroid. For diet, ACG says 8-12 weeks and ASGE says 6-8 weeks. For dupilumab or biologics, assess at 12-24 weeks (ASGE: up to 24 weeks). Allow appropriate washout or timing after any treatment change before the follow-up EGD (ASGE). Take monitoring biopsies from the most severe-appearing lesions (rings, furrows, exudates) (ASGE). Monitoring biopsies may be limited to the esophagus when prior gastric and duodenal biopsies were normal and nothing new suggests other pathology (ASGE). The histologic response threshold of <15 eos/hpf is a 'may consider' target, read alongside endoscopic and symptom findings. Shorten follow-up after complications. Care gaps of ≥2 years are associated with fibrostenosis. PEDIATRIC (ACG): esophagram for children with dysphagia to assess fibrostenotic disease; feeding therapy and dietitian for feeding dysfunction; growth, development and nutrition are treatment goals.