Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis
Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
European Respiratory Society
2019-10-01
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| Series: | European Respiratory Review |
| Online Access: | http://err.ersjournals.com/content/28/153/190022.full |
| _version_ | 1818299985447354368 |
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| author | Bridget F. Collins Ganesh Raghu |
| author_facet | Bridget F. Collins Ganesh Raghu |
| author_sort | Bridget F. Collins |
| collection | DOAJ |
| description | Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifesting other “non-IPF progressive fibrotic interstitial lung diseases” (non-IPF-PF) may be similar to those in patients with IPF. Thus, it is biologically plausible that pharmacological agents with antifibrotic properties may be efficacious in non-IPF-PF. Indeed, studies are underway or planned to assess the safety and efficacy of nintedanib or pirfenidone among patients with several non-IPF fibrotic lung diseases. In this review, we briefly summarise the use of pirfenidone and nintedanib in IPF as well as the rationale and potential for use of these medications in non-IPF-PF that are being investigated in ongoing and upcoming clinical trials. |
| first_indexed | 2024-12-13T04:59:55Z |
| format | Article |
| id | doaj.art-15ec4620d4e747428e0a500ce996f0ec |
| institution | Directory Open Access Journal |
| issn | 0905-9180 1600-0617 |
| language | English |
| last_indexed | 2024-12-13T04:59:55Z |
| publishDate | 2019-10-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | European Respiratory Review |
| spelling | doaj.art-15ec4620d4e747428e0a500ce996f0ec2022-12-21T23:58:48ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172019-10-012815310.1183/16000617.0022-20190022-2019Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosisBridget F. Collins0Ganesh Raghu1 Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA, USA Center for Interstitial Lung Diseases, University of Washington Medical Center, Seattle, WA, USA Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifesting other “non-IPF progressive fibrotic interstitial lung diseases” (non-IPF-PF) may be similar to those in patients with IPF. Thus, it is biologically plausible that pharmacological agents with antifibrotic properties may be efficacious in non-IPF-PF. Indeed, studies are underway or planned to assess the safety and efficacy of nintedanib or pirfenidone among patients with several non-IPF fibrotic lung diseases. In this review, we briefly summarise the use of pirfenidone and nintedanib in IPF as well as the rationale and potential for use of these medications in non-IPF-PF that are being investigated in ongoing and upcoming clinical trials.http://err.ersjournals.com/content/28/153/190022.full |
| spellingShingle | Bridget F. Collins Ganesh Raghu Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis European Respiratory Review |
| title | Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis |
| title_full | Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis |
| title_fullStr | Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis |
| title_full_unstemmed | Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis |
| title_short | Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis |
| title_sort | antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis |
| url | http://err.ersjournals.com/content/28/153/190022.full |
| work_keys_str_mv | AT bridgetfcollins antifibrotictherapyforfibroticlungdiseasebeyondidiopathicpulmonaryfibrosis AT ganeshraghu antifibrotictherapyforfibroticlungdiseasebeyondidiopathicpulmonaryfibrosis |