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Review
. 2022 Jun-Jul;38(6-7):579-584.
doi: 10.1051/medsci/2022084. Epub 2022 Jun 29.

[Idiopathic pulmonary fibrosis]

[Article in French]
Nathan Hennion  1 , Jean-Luc Desseyn  1 , Frédéric Gottrand  1 , Lidwine Wémeau-Stervinou  2 , Valérie Gouyer  1
Affiliations
Free article
Review

[Idiopathic pulmonary fibrosis]

[Article in French]
Nathan Hennion et al. Med Sci (Paris). 2022 Jun-Jul.
Free article

Abstract
in English, French

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal lung disease of unknown origin. It is characterized by aberrant scarring of the alveolar epithelium leading to an accumulation of extracellular matrix (ECM). Fibroblastic foci, consisting of fibroblasts and myofibroblasts, are responsible for the excessive production of ECM. The two therapeutic molecules available on the market to date only allow to slow down the evolution of the disease. In this review, we present the mechanisms involved in the progression of the disease, its treatments and the study models.

Title: La fibrose pulmonaire idiopathique.

Abstract: La fibrose pulmonaire idiopathique (FPI) est une maladie pulmonaire chronique, évolutive et mortelle dont l’origine est inconnue. Elle se caractérise par une cicatrisation aberrante de l’épithélium alvéolaire aboutissant à une accumulation de matrice extracellulaire (MEC). Les foyers fibroblastiques, constitués de fibroblastes et de myofibroblastes, sont responsables de la production excessive de MEC. Les deux seules molécules thérapeutiques disponibles sur le marché permettent seulement de ralentir l’évolution de la maladie. Dans cette revue, nous présentons les mécanismes impliqués dans la progression de la maladie, ses traitements et les modèles d’étude.

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