How is exocrine pancreatic insufficiency (EPI) best managed?

Management of Exocrine Pancreatic Insufficiency (EPI)

Pancreatic enzyme replacement therapy (PERT) is the cornerstone of EPI management, with an initial dose of 40,000 USP units of lipase per main meal and half that amount for snacks, taken during meals to ensure optimal efficacy. 1

Diagnosis of EPI

Before initiating treatment, proper diagnosis is essential:

High-Risk Populations

• High-risk conditions: Chronic pancreatitis, relapsing acute pancreatitis, pancreatic ductal adenocarcinoma, cystic fibrosis, and previous pancreatic surgery 1
• Moderate-risk conditions: Duodenal diseases (celiac, Crohn's), previous intestinal surgery, longstanding diabetes mellitus, and hyper-secretory states 1

Clinical Features

• Steatorrhea with or without diarrhea
• Weight loss despite adequate caloric intake
• Bloating and excessive flatulence
• Fat-soluble vitamin deficiencies (A, D, E, K)
• Protein-calorie malnutrition 1, 2

Diagnostic Testing

• Fecal elastase test: Primary diagnostic tool
  • <100 μg/g: Good evidence of EPI
  • 100-200 μg/g: Indeterminate for EPI

  • 200 μg/g: Normal pancreatic function 1, 2

• Important: Must be performed on semi-solid or solid stool specimen 1
• Note: Can be performed while on PERT 1

Treatment Algorithm

1. Pancreatic Enzyme Replacement Therapy (PERT)

• Initial dosing: 40,000 USP units of lipase per main meal, 20,000 USP units for snacks 1, 2
• Administration: Take during meals, not before or after 1, 2
• Formulation: pH-sensitive, enteric-coated microspheres (all derived from porcine sources) 1, 2
• Dosage adjustment: Based on meal size and fat content 1
• Maximum dose: Can increase up to 2-3 times initial dose before pursuing adjunctive therapies 3

2. Adjunctive Treatments

• Acid suppression: Add proton pump inhibitors or H2-blockers when response to PERT is suboptimal 2
• Fat-soluble vitamin supplementation: Routinely monitor and supplement vitamins A, D, E, and K 1, 2
• Dietary modifications:
  • Low-moderate fat diet (avoid very-low-fat diets)
  • Frequent smaller meals
  • Protein intake of 1.0-1.5 g/kg body weight 1, 2

Monitoring Treatment Response

Measures of Success

• Reduction in steatorrhea and GI symptoms
• Weight gain and improved nutritional status
• Improvement in fat-soluble vitamin levels 1, 2

Follow-up Schedule

• Baseline measurements: BMI, quality-of-life measure, fat-soluble vitamin levels
• Bone health: Baseline DEXA scan, repeated every 1-2 years 1
• Regular monitoring: Every 6 months for adults 2

Common Pitfalls and How to Avoid Them

Inadequate Dosing

Many patients with EPI are underdosed 3. Ensure adequate initial dosing and be prepared to increase as needed.

Improper Timing

PERT must be taken during meals, not before or after, to ensure enzymes mix properly with food 2.

Diagnostic Errors

Relying solely on a therapeutic trial of pancreatic enzymes is unreliable for EPI diagnosis 1. Always confirm with fecal elastase testing.

Imaging Limitations

Cross-sectional imaging (CT, MRI, endoscopic ultrasound) cannot identify EPI directly but helps diagnose underlying pancreatic disease 1.

Vitamin Deficiencies

Neglecting vitamin supplementation can lead to serious nutritional deficiencies. Regular monitoring is essential 1, 2.

Special Considerations

Enteral Nutrition

For patients requiring enteral nutrition, special consideration is needed as standard PERT products are not indicated for mixing with enteral formulas. Consider specialized products like RELiZORB (immobilized lipase) designed for enteral nutrition 4.

Repeat Testing

Patients with very low initial fecal elastase (<15 μg/g) are unlikely to be reclassified on repeat testing. Those with abnormal pancreatic imaging are more likely to respond to PERT 5.