CFTR modulators increase risk of acute pancreatitis in pancreatic insufficient patients with cystic fibrosis.

Patients with pancreatic insufficient cystic fibrosis rarely develop acute pancreatitis due to insufficient acinar reserve. We describe a series of five patients under the age of 18 (range 8-16 years) with pancreatic insufficient cystic fibrosis who developed a phenotype in keeping with acute pancreatitis following initiation of CFTR modulator therapy. This occurred at a median of 30 months following CFTR modulator initiation. 3/5 of these patients also developed pancreatic sufficiency or at least an intermediary pancreas status, indicated by fecal elastases above 100 μg/g. This series highlights a mostly unrecognized potential side effect of this therapy as well as the potential of CFTR modulator therapies to improve exocrine pancreatic function, even in adolescent patients.

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Déclaration de conflit d'intérêts

Declaration of Competing Interest T.G. has provided consultation to Vertex Pharmaceutical Inc. No other conflicts to declare. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.