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Progressive Fibrosing Interstitial Lung Diseases: A Current Perspective
| Content Provider | MDPI |
|---|---|
| Author | Albera, Carlo Verri, Giulia Sciarrone, Federico Sitia, Elena Mangiapia, Mauro Solidoro, Paolo |
| Copyright Year | 2021 |
| Description | 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. |
| Starting Page | 1237 |
| e-ISSN | 22279059 |
| DOI | 10.3390/biomedicines9091237 |
| Journal | Biomedicines |
| Issue Number | 9 |
| Volume Number | 9 |
| Language | English |
| Publisher | MDPI |
| Publisher Date | 2021-09-16 |
| Access Restriction | Open |
| Subject Keyword | Biomedicines Respiratory System Antifibrotic Therapy Fibrosis Idiopathic Pulmonary Fibrosis Interstitial Lung Disease Nintedanib Pirfenidone Progressive Fibrosing Interstitial Lung Disease |
| Content Type | Text |
| Resource Type | Article |
