Role of hereditary pancreatitis and CFTR gene mutations in the aetiology of acute relapsing pancreatitis of unknown origin. How are they important?

From the clinical standpoint, the causes of acute relapsing pancreatitis (ARP) are either immediately identifiable by their history and a few standard investigations, less obvious and requiring more detailed investigations or obscure and even conjectural. When no immediate cause is found, the recurrent episode is classified as "idiopathic ARP", accounting for 10 to 30% of all recurrences [1]. Over the last few years this so-called idiopathic pancreatitis has been decreasing as a result of increased recognition of causes such as biliary sludge or microlithiasis, sphincter of Oddi dysfunction, autoimmune diseases, and genetic disorders. This contribution will attempt to determine the role of the two most important hereditary pancreatic diseases, namely, hereditary pancreatitis (HP) and cystic fibrosis (CF), in the etiology of ARP of unknown origin.