Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis.
Fiche publication
Date publication
octobre 2021
Journal
Orphanet journal of rare diseases
Auteurs
Membres identifiés du Cancéropôle Est :
Pr BONNIAUD Philippe
Tous les auteurs :
Nasser M, Si-Mohamed S, Turquier S, Traclet J, Ahmad K, Philit F, Bonniaud P, Chalabreysse L, Thivolet-Béjui F, Cottin V
Lien Pubmed
Résumé
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 tertiary care center (2010-2019) were included into this retrospective analysis if they had a multidisciplinary diagnosis of PPFE, had been followed-up for 3 months or more, and had lung function tests and chest CTs available for review. Changes in pulmonary function tests were assessed using non-parametric tests and linear mixed effect model. Lung volumes were measured with lobar segmentation using chest CT.
Mots clés
CT volumetry, Nintedanib, Pleuroparenchymal fibroelastosis, Prognosis, Progression, Pulmonary fibrosis, Pulmonary function tests
Référence
Orphanet J Rare Dis. 2021 Oct 9;16(1):419