Idiopathic pulmonary fibrosis

被引：2

作者：

Hennion, Nathan ^{[1
]}

Desseyn, Jean-Luc ^{[1
]}

Gottrand, Frederic ^{[1
]}

Wemeau-Stervinou, Lidwine ^{[2
]}

Gouyer, Valerie ^{[1
]}

机构：

[1] Univ Lille, INSERM, CHU Lille, U1286 Infinite, F-59000 Lille, France

[2] CHU Lille, Ctr reference constitutif Ies Malad pulmonaires r, Serv pneumol & immuno allergol, F-59000 Lille, France

来源：

M S-MEDECINE SCIENCES | 2022年 / 38卷 / 6-7期

关键词：

INTERSTITIAL LUNG-DISEASE; DOUBLE-BLIND; PIRFENIDONE;

D O I：

10.1051/medsci/2022084

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

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.

引用

页码：579 / 584

页数：6

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