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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 id...

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Main Authors: Keisuke Morikawa, Yuzo Suzuki, Shinpei Kato, Masato Kono, Dai Hashimoto, Koshi Yokomura, Yusuke Inoue, Hideki Yasui, Hironao Hozumi, Masato Karayama, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Naoki Inui, Takafumi Suda
Format: Artigo
Language:Inglês
Published: Nature Portfolio 2025-12-01
Series:Scientific Reports
Subjects:
Familial pulmonary fibrosis
Idiopathic pulmonary fibrosis
Antifibrotic therapy
Mortality
Acute exacerbation
Tolerability
Online Access:https://doi.org/10.1038/s41598-025-27730-2
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