期刊
BIOMEDICINES
卷 9, 期 9, 页码 -出版社
MDPI
DOI: 10.3390/biomedicines9091237
关键词
antifibrotic therapy; fibrosis; idiopathic pulmonary fibrosis; interstitial lung disease; nintedanib; pirfenidone; progressive fibrosing interstitial lung disease
资金
- Boehringer Ingelheim
Interstitial lung diseases (ILDs) encompass a diverse group of rare and chronic respiratory disorders, with idiopathic pulmonary fibrosis (IPF) being the most studied member. Recent development of antifibrotic drugs has shown benefits in ILDs characterized by progressive fibrosis, leading to significant changes in management.
Interstitial lung diseases (ILDs) are a large and diverse group of rare and chronic respiratory disorders, with idiopathic pulmonary fibrosis (IPF) being the most common and best-studied member. Increasing interest in fibrosis as a therapeutic target and the appreciation that fibrotic mechanisms may be a treatable target of IPF prompted the development and subsequent approval of the antifibrotics, pirfenidone and nintedanib. The management of ILDs has changed considerably following an understanding that IPF and some ILDs share similar disease behavior of progressive fibrosis, termed progressive fibrosing phenotype. Indeed, antifibrotic treatment has shown to be beneficial in ILDs characterized by the progressive fibrosing phenotype. This narrative review summarizes current knowledge in the field of progressive fibrosing ILDs. Here, we discuss the clinical characteristics and pathogenesis of lung fibrosis and highlight relevant literature concerning the mechanisms underlying progressive fibrosing ILDs. We also summarize current diagnostic approaches and the available treatments of progressive fibrosing ILDs and address the optimization of treating progressive fibrosing ILDs with antifibrotics in clinical practice.
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