Antifibrotic therapy in familial idiopathic pulmonary fibrosis: a comparative cohort study.

ABSTRACT: Before the introduction of antifibrotic therapy, patients with familial pulmonary fibrosis (FPF) were reported to have a higher mortality risk than those with sporadic disease. Genetic predisposition plays an important role in the development of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF). The 2023 European Respiratory Society consensus statement defined FPF and recommended antifibrotic drugs for treatment; however, their efficacy in FPF remains unclear. We conducted a retrospective multicohort study of 280 patients with IPF receiving antifibrotic therapy (exploratory cohort, n = 141; validation cohort, n = 139). FPF was defined as fibrotic ILD in at least two first- or second-degree relatives. We examined tolerability, causes of drug discontinuation, inc

SUBMITTER: Morikawa K 

PROVIDER: S-EPMC12706045 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature