Therapeutic hopes for CFTR-related recurrent pancreatitis: a case-series.

The presence of CFTR variants increases the risk of pancreatitis in pancreatic sufficient people with cystic fibrosis (pwCF) or with CFTR-related disorder (pwCFTR-RD). Modulators have been showed to decrease pancreatitis episodes in pwCF presenting recurrent pancreatitis. Whether CFTR modulators are efficient to prevent recurrent episodes of pancreatitis in pwCFTR-RD is not known. We collected clinical, biological, imaging and CFTR functional data in 4 patients with recurrent pancreatitis and CFTR-RD, treated with CFTR modulators and assessed the efficacy of the treatment. With a follow-up of minimum 9 months, we found that ETI and ivacaftor reduced or even resolved the number of acute pancreatitis episodes.