Cystic fibrosis liver disease progression in the era of elexacaftor-tezacaftor-ivacaftor.

ABSTRACT:

Background & aims

The effect of elexacaftor-tezacaftor-ivacaftor (ETI) on cystic fibrosis liver disease (CFLD) outcomes remains unknown. Thus, we investigated the association between the ETI rollout and trends in CFLD progression in a nationwide cohort.

Methods

Using the French National Hospital Discharge Database (2014-2023), we measured the incidence of CFLD progression (decompensated cirrhosis, gastroesophageal variceal bleeding, primary liver cancer, or liver transplantation) before and after ETI became available (December 2019) in patients with cystic fibrosis (pwCF) aged ≥12 years. Death without CFLD progression and lung transplantation were treated as competing events. Incidence rates were compared across ETI calendar eras in both the full cohort and a 1:1 propensity

SUBMITTER: Mouliade C 

PROVIDER: S-EPMC12506492 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature