Biliary disease and cholecystectomy after initiation of elexacaftor/ivacaftor/tezacaftor in adults with cystic fibrosis |
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| Authors: | Julie Safirstein Jonathan J. Grant Emily Clausen Deepika Savant Rebecca Dezube Gina Hong |
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| Affiliation: | 1. Department of Pharmacy, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA;2. Johns Hopkins Homecare Group, 1800 Orleans Street, Baltimore, MD 21287, USA;3. Department of Medicine, University of Pennsylvania, Perelman School of Medicine, 3400 Spruce Street, Philadelphia, PA 19104, USA;4. Department of Pathology, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA 19104, USA;5. Division of Pulmonary and Critical Care Medicine, The Johns Hopkins University School of Medicine, 1800 Orleans Street, Baltimore, MD 21287, USA |
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| Abstract: | Individuals with cystic fibrosis (CF) have an increased risk for gallbladder abnormalities and biliary tract disease, but the reported incidence of these manifestations of CF varies widely in the literature. With the approval of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA), increasing numbers of CF patients have been initiated on highly effective cystic fibrosis transmembrane regulator (CFTR) modulator therapy. While elevations in hepatic panel are known potential side effects of CFTR modulators, there have been no published cases of biliary disease or acute cholecystitis attributed to these medications. In this case series, we describe seven patients at two adult CF centers with biliary colic shortly after initiation with ELX/TEZ/IVA, six of whom required cholecystectomy. |
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