Lung transplantation for idiopathic pulmonary fibrosis enriches for individuals with telomere-mediated disease.

ABSTRACT:

Background

Idiopathic pulmonary fibrosis (IPF) is the most common indication for lung transplantation in North America and variants in telomere-maintenance genes are the most common identifiable cause of IPF. We reasoned that younger IPF patients are more likely to undergo lung transplantation and we hypothesized that lung transplant recipients would be enriched for individuals with telomere-mediated disease due to the earlier onset and more severe disease in these patients.

Methods

Individuals with IPF who underwent lung transplantation or were evaluated in an interstitial lung disease specialty clinic who did not undergo lung transplantation were examined. Genetic evaluation was completed via whole genome sequencing (WGS) of 426 individuals and targeted sequencing for 5 in

SUBMITTER: Alder JK 

PROVIDER: S-EPMC9038609 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature