Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention
Author:
D’Agnano Vito1, Mariniello Domenica Francesca1, Ruotolo Michela1, Quarcio Gianluca1, Moriello Alessandro1, Conte Stefano1, Sorrentino Antonio1, Sanduzzi Zamparelli Stefano2, Bianco Andrea1ORCID, Perrotta Fabio1ORCID
Affiliation:
1. Department of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, Italy 2. Division of Pneumology, A. Cardarelli Hospital, 80131 Naples, Italy
Abstract
Interstitial lung diseases comprise a heterogenous range of diffuse lung disorders, potentially resulting in pulmonary fibrosis. While idiopathic pulmonary fibrosis has been recognized as the paradigm of a progressive fibrosing interstitial lung disease, other conditions with a progressive fibrosing phenotype characterized by a significant deterioration of the lung function may lead to a burden of significant symptoms, a reduced quality of life, and increased mortality, despite treatment. There is now evidence indicating that some common underlying biological mechanisms can be shared among different chronic fibrosing disorders; therefore, different biomarkers for disease-activity monitoring and prognostic assessment are under evaluation. Thus, understanding the common pathways that induce the progression of pulmonary fibrosis, comprehending the diversity of these diseases, and identifying new molecular markers and potential therapeutic targets remain highly crucial assignments. The purpose of this review is to examine the main pathological mechanisms regulating the progression of fibrosis in interstitial lung diseases and to provide an overview of potential biomarker and therapeutic options for patients with progressive pulmonary fibrosis.
Funder
University of Campania L. Vanvitelli “Progetti di ricerca fondamentale ed applicata dedicato ai giovani Ricercatori D.R. n. 509”
Subject
Paleontology,Space and Planetary Science,General Biochemistry, Genetics and Molecular Biology,Ecology, Evolution, Behavior and Systematics
Reference165 articles.
1. An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias;Travis;Am. J. Respir. Crit. Care Med.,2013 2. [Epidemiology of interstitial lung diseases];Valeyre;Presse Med.,2010 3. Piciucchi, S., Tomassetti, S., Ravaglia, C., Gurioli, C., Gurioli, C., Dubini, A., Carloni, A., Chilosi, M., Colby, T.V., and Poletti, V. (2016). From “Traction Bronchiectasis” to Honeycombing in Idiopathic Pulmonary Fibrosis: A Spectrum of Bronchiolar Remodeling Also in Radiology?. BMC Pulm. Med., 16. 4. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline;Raghu;Am. J. Respir. Crit. Care Med.,2022 5. Rapidly Non-Ipf Progressive Fibrosing Interstitial Lung Disease: A Phenotype with an Ipf-like Behavior;Khine;Sarcoidosis Vasc. Diffus. Lung Dis. Off. J. WASOG,2020
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