Cystic fibrosis patients on cystic fibrosis transmembrane conductance regulator modulators have a reduced incidence of cirrhosis.
Cirrhosis
Cystic fibrosis
Cystic fibrosis related liver disease
Market scan
Transmembrane
Ursodiol
Journal
World journal of hepatology
ISSN: 1948-5182
Titre abrégé: World J Hepatol
Pays: United States
ID NLM: 101532469
Informations de publication
Date de publication:
27 Feb 2022
27 Feb 2022
Historique:
received:
21
10
2021
revised:
15
12
2021
accepted:
16
02
2022
entrez:
23
3
2022
pubmed:
24
3
2022
medline:
24
3
2022
Statut:
ppublish
Résumé
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators significantly improve pulmonary function in patients with cystic fibrosis (CF) but the effect on hepatobiliary outcomes remains unknown. We hypothesized that CF patients on CFTR modulators would have a decreased incidence of cirrhosis compared to patients not on CFTR modulators or on ursodiol. To investigate the effect of CFTR modulators on the development of cirrhosis in patients with CF. A retrospective analysis was performed using Truven MarketScan from January 2012 through December 2017 including all patients with a diagnosis of CF. Patients were excluded if they underwent a liver transplantation or if they had other etiologies of liver disease including viral hepatitis or alcohol use. Subjects were grouped by use of CFTR modulators, ursodiol, dual therapy, or no therapy. The primary outcome was development of cirrhosis. Kaplan-Meier curves estimated the incidence of cirrhosis and log-rank tests compared incidence curves between treatment groups. A total of 7201 patients were included, of which 955 (12.6%) used a CFTR modulator, 529 (7.0%) used ursodiol, 105 (1.4%) used combination therapy, and 5612 (74.3%) used neither therapy. The incidence of cirrhosis was 0.1% at 1 year and 0.7% at 4 years in untreated patients, 5.9% and 10.1% in the Ursodiol group, and 1.0% and 1.0% in patients who received both therapies. No patient treated with CFTR modulators alone developed cirrhosis. Patients on CFTR modulators alone had lower cirrhosis incidence than untreated patients ( CFTR modulators are associated with a reduction in the incidence of cirrhosis compared to other therapies in patients with CF.
Sections du résumé
BACKGROUND
BACKGROUND
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators significantly improve pulmonary function in patients with cystic fibrosis (CF) but the effect on hepatobiliary outcomes remains unknown. We hypothesized that CF patients on CFTR modulators would have a decreased incidence of cirrhosis compared to patients not on CFTR modulators or on ursodiol.
AIM
OBJECTIVE
To investigate the effect of CFTR modulators on the development of cirrhosis in patients with CF.
METHODS
METHODS
A retrospective analysis was performed using Truven MarketScan from January 2012 through December 2017 including all patients with a diagnosis of CF. Patients were excluded if they underwent a liver transplantation or if they had other etiologies of liver disease including viral hepatitis or alcohol use. Subjects were grouped by use of CFTR modulators, ursodiol, dual therapy, or no therapy. The primary outcome was development of cirrhosis. Kaplan-Meier curves estimated the incidence of cirrhosis and log-rank tests compared incidence curves between treatment groups.
RESULTS
RESULTS
A total of 7201 patients were included, of which 955 (12.6%) used a CFTR modulator, 529 (7.0%) used ursodiol, 105 (1.4%) used combination therapy, and 5612 (74.3%) used neither therapy. The incidence of cirrhosis was 0.1% at 1 year and 0.7% at 4 years in untreated patients, 5.9% and 10.1% in the Ursodiol group, and 1.0% and 1.0% in patients who received both therapies. No patient treated with CFTR modulators alone developed cirrhosis. Patients on CFTR modulators alone had lower cirrhosis incidence than untreated patients (
CONCLUSION
CONCLUSIONS
CFTR modulators are associated with a reduction in the incidence of cirrhosis compared to other therapies in patients with CF.
Identifiants
pubmed: 35317183
doi: 10.4254/wjh.v14.i2.411
pmc: PMC8891668
doi:
Types de publication
Journal Article
Langues
eng
Pagination
411-419Informations de copyright
©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
Déclaration de conflit d'intérêts
Conflict-of-interest statement: None of the authors have relevant financial relationships to disclose.
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