Changes in liver biochemistry and tacrolimus levels following the introduction of elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis and liver transplant

• Published in: Journal of cystic fibrosis Vol. 23; no. 1; pp. 99 - 102
• Main Authors: Tachtatzis, P, Spoletini, G, Clifton, I, Etherington, C, Peckham, D
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.01.2024
• Subjects: Aminophenols; Benzodioxoles; Cystic fibrosis; Cystic Fibrosis - diagnosis; Cystic Fibrosis - drug therapy; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Elexacaftor; Female; Humans; Indoles; Ivacaftor; Liver; Liver biopsy; Liver function test; Liver transplant; Liver Transplantation - adverse effects; Male; Mutation; Pyrazoles; Pyridines; Pyrrolidines; Quinolones; Tacrolimus - adverse effects; Tezacaftor; Liver biopsy; Liver function test; Ivacaftor; Liver transplant; Cystic fibrosis; Elexacaftor; Tezacaftor
• ISSN: 1569-1993; 1873-5010
• DOI: 10.1016/j.jcf.2023.04.023

•CFTR modulators can cause abnormal LFTs in CF post liver transplant patients.•Abnormal LFTs in this setting need to be assessed further.•Liver biopsy may be considered to exclude other liver pathology.•Normal histology provides re-assurance to continue with CFTR modulator therapy. Elevated liver function tests (LFTs) are reported in individuals with cystic fibrosis (CF) starting elexacaftor/tezacaftor/ivacaftor (ETI). We report our experience with ETI in CF liver transplant patients. All CF liver transplant patients under the care of the Leeds CF team were commenced on ETI. Liver biopsies were performed when ALT >3 times upper limit of normal with or without bilirubin elevation. Treatment was guided by transplant hepatology and CF teams. Clinical data including lung function, LFTs and tacrolimus levels were collected. Four patients (3 male, 1 female) on tacrolimus were commenced on ETI. Median time post liver transplantation was 6.5 years. Three patients underwent liver biopsy. One biopsy was abnormal with immune-mediated liver injury, which responded to increased immunosuppression. Management of tacrolimus levels proved straightforward. ETI therapy in CF post liver transplant recipients was encouraging. Normal liver biopsy provides re-assurance to continue treatment despite elevated LFTs.