11/11/2011
Management of Exocrine Insufficiency David C Whitcomb MD PhD Giant Eagle g Foundation Professor of Cancer Genetics Professor of Medicine, ...
Management of Exocrine Insufficiency David C Whitcomb MD PhD Giant Eagle g Foundation Professor of Cancer Genetics Professor of Medicine, Cell Biology & Physiology, and Human Genetics Chief, Division of Gastroenterology, Hepatology and Nutrition University of Pittsburgh and UPMC.
Outline: EPI in pancreatic disease Syndromes with EPI Need for PERT Goals for PERT Recommendations
1
11/11/2011
Clinical Course of CP Where do you start treatment? How much is enough? Should the dose be changed?
Pancreatic Function
Time (years)
Clinical Course of CP
Patient B Pancreatic Function Patient C
Patient A
Time (years)
2
11/11/2011
PEI – major question • • • • •
Which disorders commonly have PEI? What are the signs that PEI is present? Is PEI a sign of an irreversible state? What are the goals of treatment? How should the patient’s condition be monitored?
Syndromes with Pancreatic Insufficiency: AIP • Autoimmune pancreatitis Type 1: lymphoplasmacytic-sclerosis l h l i l i x Elevated IgG4 x IgG4-associated Systemic Disease (ISD)
Type 2: granulocyte epithelial lesion (GEL) x IgG4 may be normal x Associated with IBD (especially UC)
May present with vague abdominal discomfort and symptoms of malabsorption Pancreatic exocrine insufficiency may be reversed with steroid treatment Zhang et al Pancreas 2011;40: 1172-1179
4
11/11/2011
Syndromes with Pancreatic Insufficiency: IBD • Inflammatory Bowel Disease Pancreatic diseases is common in both Crohn’s disease and UC Acute pancreatitis is common during treatment with immunomodulators (e.g. azothioprine) p y, p pancreatic p pathology gy is p present in 4 40% At autopsy, of people with Crohn’s disease Pancreatic exocrine insufficiency may be present in 10-15% of patients with IBD, especially in those with diarrhea.
Syndromes with Pancreatic Insufficiency: Celiac Disease • Celiac Disease Complex pathophysiology of pancreatic insufficiency x Mucosal injury and decreased CCK release x Poor nutritional status – especially protein malnutrition x Coexisting chronic pancreatitis
Diarrhea from exocrine pancreatic insufficiency improves with pancreatic enzyme replacement therapy. Pancreatic Insufficiency is reversible!
