Long-term disease progression in pediatric acute recurrent and chronic pancreatitis: A report from INSPPIRE

Background Acute recurrent pancreatitis (ARP) in childhood can rapidly progress to chronic pancreatitis (CP). Prospectively-collected data from the IN ternational S tudy group of P ediatric P ancreatitis: I n search for a cu RE (INSPPIRE) provides novel insight into disease progression. Methods INSPPIRE subjects were categorized as persistent ARP (pARP = remained ARP through last follow-up), incident CP (iCP = ARP at enrollment, developed CP), or prevalent CP (pCP = CP at enrollment). Time-to-sequelae and risk factors were analyzed. Results Of 626 total children, 384 (61%) were ARP at baseline; of these, 81 (21.1%) were iCP at follow-up. iCP were more likely to have PRSS1 mutations, obstructive risk factors, and more acute pancreatitis episodes (AP) vs. pARP, but didn't differ in age at first AP. Exocrine pancreatic insufficiency (EPI) developed in 24% during follow-up, 10% of pARP, 32% of iCP. In all CP, 50% had EPI by 17.7yrs, median 10yrs after first AP. Diabetes mellitus (DM) developed in 8% during follow-up, 6% of pARP, 5% of iCP. In all CP, median event-free survival from birth and after first AP was not reached. Conclusions Prospective follow-up of children with ARP revealed nearly 1 in 5 progressed to CP, with a subset developing irreversible sequelae, and highlighted risk factors associated with progression including genetics, obstructive disease, and episode frequency.