Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis

Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis

Abstract Background Pleuroparenchymal fibroelastosis (PPFE) has a variable disease course with dismal prognosis in the majority of patients with no validated drug therapy. This study is to evaluate the effect of nintedanib in patients with idiopathic and secondary PPFE. Patients admitted to a tertia...

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Bibliographic Details
Main Authors: Mouhamad Nasser, Salim Si-Mohamed, Ségolène Turquier, Julie Traclet, Kaïs Ahmad, François Philit, Philippe Bonniaud, Lara Chalabreysse, Françoise Thivolet-Béjui, Vincent Cottin
Format: Article
Language:English
Published: BMC 2021-10-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Pulmonary fibrosis
Pleuroparenchymal fibroelastosis
Pulmonary function tests
CT volumetry
Nintedanib
Progression
Online Access:https://doi.org/10.1186/s13023-021-02043-5

Internet

https://doi.org/10.1186/s13023-021-02043-5

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