Hereditary pancreatitis: a model for inflammatory diseases of the pancreas

Acute and chronic pancreatitis remain among the most recalcitrant of all diseases to investigation and intervention. In the majority of patients, excessive alcohol consumption is associated with development of the disease. Therefore, several theories have been proposed seeking to explain the relationship between alcohol and the development of acute and chronic pancreatitis. However, recent investigations in hereditary pancreatitis provided important insights into chronic pancreatitis pathogenesis and offer an important model for understanding pancreatic inflammation. This article highlights several advances gained from investigating hereditary pancreatitis kindreds, and reviews the TIGAR-O risk/aetiology classification system. Finally, the major independent theories on development of chronic pancreatitis are reviewed with respect to the SAPE hypothesis of chronic pancreatitis pathogenesis.