CF Center, Department of Pediatrics, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.
Department Radiology, Hadassah Medical Center and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel.
J Cyst Fibros. 2023 Jul;22(4):777-779. doi: 10.1016/j.jcf.2023.02.013. Epub 2023 Mar 11.
Cystic fibrosis transmembrane conductance regulator modulator therapy is associated with substantial clinical benefit and improved quality of life in patients with cystic fibrosis (CF). While their effect on lung function has been clearly reported, we are still in the process of unraveling the full impact they have on the pancreas. We present two cases of pancreatic-insufficient CF patients who presented with acute pancreatitis shortly after commencing elexacaftor/tezacaftor/ivacaftor modulator therapy. Both patients were treated with ivacaftor for 5 years prior to elexacaftor/tezacaftor/ivacaftor initiation, but had no previous episodes of acute pancreatitis. We suggest that highly effective modulator combination therapy may restore additional pancreatic acinar activity, resulting in the development of acute pancreatitis in the interim until ductal flow is improved. This report adds to the growing evidence for possible restoration of pancreatic function in patients receiving modulator therapy, and highlights that treatment with elexacaftor/tezacaftor/ivacaftor may be associated with acute pancreatitis until ductal flow is restored, even in pancreatic-insufficient CF patients.
囊性纤维化跨膜电导调节因子调节剂治疗可显著改善囊性纤维化（CF）患者的临床获益和生活质量。虽然它们对肺功能的影响已被明确报道，但我们仍在深入研究它们对胰腺的全面影响。我们报告了两例胰腺功能不全的 CF 患者，他们在开始接受依伐卡托/泰他卡托/艾氟卡托调节剂治疗后不久就出现了急性胰腺炎。这两名患者在开始接受依伐卡托/泰他卡托/艾氟卡托治疗之前已经接受了 5 年的依伐卡托治疗，但没有发生过急性胰腺炎。我们推测，高效的调节剂联合治疗可能会恢复额外的胰腺腺泡活性，从而导致在导管血流改善之前出现急性胰腺炎。该报告进一步证实了接受调节剂治疗的患者胰腺功能可能得到恢复，并且强调即使在胰腺功能不全的 CF 患者中，艾氟卡托/泰他卡托/依伐卡托治疗也可能与急性胰腺炎相关，直至导管血流恢复。
