What is the management and treatment for food protein enterocolitis syndrome in adults?

Management and Treatment of Food Protein-Induced Enterocolitis Syndrome (FPIES) in Adults

The primary management of FPIES in adults involves strict dietary elimination of identified trigger foods, appropriate emergency treatment of acute reactions, and regular monitoring for resolution under medical supervision. 1, 2

Diagnosis in Adults

Adult FPIES presents differently from pediatric cases:

• Common symptoms in adults:

  • Abdominal pain (96.3%) 3
  • Diarrhea (72-92.9%) 4, 3
  • Abdominal cramps (71.4%) 4
  • Vomiting (59-60.7%) 4, 3

• Most common triggers in adults:

  • Shellfish (45.2%) 4
  • Fish (45.2%) 4
  • Seafood (59.8%) 3
  • Egg (14%) 3
  • Milk (10.3%) 3

• Diagnostic criteria: Based on clinical history of characteristic symptoms occurring 1-4 hours after food ingestion, with improvement after withdrawal of the suspected trigger food 1

Management Protocol

1. Dietary Management

• Complete elimination of identified trigger foods is the cornerstone of FPIES management 1, 2
• Nutritional consultation is strongly recommended for all patients to ensure adequate nutrition despite dietary restrictions 1, 2
• Be aware that 43.9% of adult patients react to more than one food group 3

2. Acute Reaction Management

For mild episodes (1-2 episodes of emesis, no lethargy):

• Attempt oral rehydration with clear fluids
• Consider ondansetron 0.15 mg/kg IM (max 16 mg) if ≥6 months old
• Monitor for 4-6 hours from onset 1, 2

For moderate episodes (>3 episodes of emesis with mild lethargy):

• Administer ondansetron 0.15 mg/kg IM (max 16 mg)
• Establish IV access for normal saline bolus (20 mL/kg)
• Transfer to ED/ICU if persistent hypotension, shock, lethargy, or respiratory distress 1, 2

For severe episodes (>3 episodes of emesis with severe lethargy, hypotonia, ashen/cyanotic appearance):

• Establish IV access with rapid normal saline bolus (20 mL/kg), repeat as needed
• Administer IV ondansetron 0.15 mg/kg (max 16 mg)
• Consider IV methylprednisolone (1 mg/kg; max 60-80 mg)
• Monitor and correct acid-base and electrolyte abnormalities
• Correct methemoglobinemia if present 1, 2

3. Monitoring and Follow-up

• Regular follow-up to assess for resolution
• Oral food challenges (OFCs) to confirm diagnosis if history is unclear and to evaluate for tolerance development 1
• Location for OFCs:
  • Hospital setting with IV access for patients with history of severe FPIES
  • Physician's office for those without history of severe reactions 1, 2

Prognosis in Adults

• Adult FPIES generally has a lower resolution rate than pediatric cases
• After a median follow-up of 3.5 years, only 16.8% of adults achieve tolerance 3
• Resolution correlates inversely with:
  • Duration of the disease
  • Seafood as a trigger 3
• Some patients may outgrow adult-onset FPIES (40% in one study) after a mean of 17.8 months (range 6-36 months) 4

Special Considerations

• Comorbidities: Adults with FPIES have a higher prevalence of gastrointestinal pathologies including irritable bowel syndrome, eosinophilic esophagitis, inflammatory bowel disease, and celiac disease 3
• Gender disparity: FPIES in adults shows a strong female predominance (83.3-93.5%) 4, 3
• Ondansetron use: Use with caution in patients with heart disease due to potential QT interval prolongation 1, 2

Emerging Concepts

• The pathophysiology remains poorly understood, but involves activation of the innate immune compartment 5
• No strategies currently exist to accelerate tolerance development 6, 5
• Prevention, laboratory diagnostic testing, and strategies to accelerate tolerance development are urgent unmet needs 6

Adult FPIES requires a high index of suspicion for diagnosis, as patients typically experience multiple reactions (mean 6.3) before diagnosis 4.