Idiopathic chronic pancreatitis.

Idiopathic pancreatitis is diagnosed in up to 25% of patients with chronic pancreatitis by exclusion of other potential causes including rare ones. It has been shown that idiopathic pancreatitis comprises two clinically distinct entities characterised as early-onset and late-onset disease and that the natural courses of both forms differ from that of alcoholic chronic pancreatitis. Due to considerable progress in our understanding of hereditary and autoimmune mechanisms for development of chronic pancreatitis, a specific aetiology of chronic pancreatitis can be determined in an increasing proportion of cases. Nevertheless, the aetiopathogenesis of idiopathic chronic pancreatitis frequently remains obscure. This review focuses on the pathogenetic relevance of various endogenous and exogenous (co-)factors for the manifestation and the natural course of the disease. Moreover, it presents a multifactorial model for understanding the development of idiopathic chronic pancreatitis.