Progressive Fibrosing Interstitial Lung Diseases: A Current Perspective

Abstract

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.

High-Resolution Computed Tomography and Lung Ultrasound in Patients with Systemic Sclerosis: Which One to Choose?

“…Goh et al and Warrick et al proposed various semiquantitative scoring systems to visually quantify the extent of ILD. These can provide prognostic information, e.g., limited or extensive disease and follow-up data in SSc patients with ILD [ 55 , 56 , 57 ]. The semiquantitative scoring systems can visually quantify the extent of ILD, with a categorical cut-off of 20% to distinguish limited and extensive parenchymal involvement with prognostic implications.…”

Molecular Mechanisms and Cellular Contribution from Lung Fibrosis to Lung Cancer Development

“…The recent PROFILE (Prospective Study of Fibrosis in Lung Endpoints) study that analyzed the molecular profile of more than 100 serum proteins showed a significantly higher level of MMP1, MMP7, and SP-D in IPF patients compared with healthy people. Furthermore, it showed that oncostatin M and cytokeratin 19 fragments (CYFRA-21-1) were markers for IPF patients [54,[93][94][95][96][97][98]. These biomarkers may be an important aspect to investigate in the future even for lung cancer.…”

Nintedanib in chronic fibrosing interstitial lung diseases. A case series

“…First-line treatment of fibrosis in PPF was traditionally with corticosteroids (CS) [ 2 ], although antifibrotic drugs have been used in recent years [ 6 ]. Nintedanib is the only recommended antifibrotic therapy for PPF-ILDs in the 2022 ATS/ERS/JRS/ALAT guidelines [ 6 ].…”

“…First-line treatment of fibrosis in PPF was traditionally with corticosteroids (CS) [ 2 ], although antifibrotic drugs have been used in recent years [ 6 ]. Nintedanib is the only recommended antifibrotic therapy for PPF-ILDs in the 2022 ATS/ERS/JRS/ALAT guidelines [ 6 ]. Another antifibrotic drug, pirfenidone, has been studied in PPFs [ 7 ], although evidence on its efficacy is not as consistent as for nintedanib, and more research is needed [ 6 ].…”