Hereditary pancreatitis is a rare subtype of chronic pancreatitis that has an autosomal dominant inheritance. As a congenital condition, presentation with acute pancreatitis attacks typically occurs in childhood, and features of chronic pancreatitis with parenchymal and ductal calcifications and atrophy are commonly seen when patients become adolescents.
Imaging plays a role in excluding underlying abnormalities. The diagnosis is clinically considered when dealing with idiopathic chronic pancreatitis in young patients, especially when a family history is present.
The condition has been reported with a 50-70-fold increased risk of pancreatic cancer 1.
- 1. Shanbhogue AK, Fasih N, Surabhi VR, Doherty GP, Shanbhogue DK, Sethi SK. A clinical and radiologic review of uncommon types and causes of pancreatitis. Radiographics : a review publication of the Radiological Society of North America, Inc. 29 (4): 1003-26. doi:10.1148/rg.294085748 - Pubmed
- 2. Rebours V, Lévy P, Ruszniewski P. An overview of hereditary pancreatitis. Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver. 44 (1): 8-15. doi:10.1016/j.dld.2011.08.003 - Pubmed