Publication
Title
Progression in the management of non-idiopathic pulmonary fibrosis interstitial lung diseases, where are we now and where we would like to be
Author
Abstract
A significant proportion of patients with interstitial lung disease (ILD) may develop a progressive fibrosing phenotype characterized by worsening of symptoms and pulmonary function, progressive fibrosis on chest computed tomography and increased mortality. The clinical course in these patients mimics the relentless progressiveness of idiopathic pulmonary fibrosis (IPF). Common pathophysiological mechanisms such as a shared genetic susceptibility and a common downstream pathway—self-sustaining fibroproliferation—support the concept of a progressive fibrosing phenotype, which is applicable to a broad range of non-IPF ILDs. While antifibrotic drugs became the standard of care in IPF, immunosuppressive agents are still the mainstay of treatment in non-IPF fibrosing ILD (F-ILD). However, recently, randomized placebo-controlled trials have demonstrated the efficacy and safety of antifibrotic treatment in systemic sclerosis-associated F-ILD and a broad range of F-ILDs with a progressive phenotype. This review summarizes the current pharmacological management and highlights the unmet needs in patients with non-IPF ILD.
Language
English
Source (journal)
Journal of Clinical Medicine
Publication
2021
ISSN
2077-0383
DOI
10.3390/JCM10061330
Volume/pages
10 :6 (2021) , p. 1-16
Article Reference
1330
ISI
000652002700001
Pubmed ID
33807034
Full text (Publisher's DOI)
Full text (open access)
UAntwerpen
Research group
Publication type
Subject
External links
Web of Science
Record
Identifier
Creation 25.01.2024
Last edited 26.01.2024
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