Hereditary chronic pancreatitis

What is Hereditary chronic pancreatitis?

A rare gastroenterologic disease characterized by recurrent acute pancreatitis and/or chronic pancreatitis in at least 2 first-degree relatives, or 3 or more second-degree relatives in 2 or more generations, for which no predisposing factors are identified. This rare inherited form of pancreatitis leads to irreversible damage to both exocrine and endocrine components of the pancreas.

Inheritance type

Autosomal dominant


  • Europe: 1-9 in 1,000 000

Age of Onset

  • Adolescent
  • Childhood
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "". Data version 1.3.16 / 4.1.7.