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Pulmonary Fibrosis: Molecular Mechanisms and Therapeutic Strategies

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: 28 March 2026 | Viewed by 374

Special Issue Editor

Special Issue Information

Dear Colleagues,

Pulmonary fibrosis is a complex and multipathways process which encompasses a broad spectrum of chronic lung diseases including interstitial lung diseases (ILDs). In recent years, the landscape of these diseases has been evolving significantly. The ongoing advancements of precision medicine, along with the increasing application of artificial intelligence, machine learning and genetic profiling as well as the growing use of omics science has deeply reshaped our approach to ILDs. Simultaneously, a deeper understanding of the molecular mechanisms and signalling underlying ILD pathogenesis is emerging, are paving the way for new therapeutic target. All in all, these developments are opening promising advances for earlier diagnosis, personalized treatment strategies, and improved clinical outcomes.

This Special Issue is supervised by Prof. Dr. Barbara Ruaro and assisted by Dr. Giorgio Monteleone (Catholic University of Sacred Heart). It aims at pointing out the pathogenetic mechanisms and signal pathways involved in pulmonary fibrosis and fibrotic interstitial lung diseases, as well as the application of cutting-edge tools and technologies to advance our understanding of their pathobiology.  

We warmly invite to submit original research articles both in vitro and in vivo, systematic or narrative review that explore these critical aspects.

Prof. Dr. Barbara Ruaro
Guest Editor

Dr. Giorgio Monteleone
Guest Editor Assistant

Manuscript Submission Information

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Keywords

  • machine learning
  • pulmonary fibrosis
  • interstitial lung diseases
  • molecular mechanisms
  • precision medicine

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Published Papers (1 paper)

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Research

26 pages, 5909 KB  
Article
Vitamin D3-Deficient Diet Promotes Pulmonary Fibrosis Development in Murine Model of Hypersensitivity Pneumonitis
by Marta Kinga Lemieszek, Michał Chojnacki, Iwona Paśnik, Wiktoria Gawryś, Alicja Wilczyńska, Ilona Leśniowska, Jakub Anisiewicz and Michał Kiełbus
Int. J. Mol. Sci. 2025, 26(24), 11770; https://doi.org/10.3390/ijms262411770 - 5 Dec 2025
Viewed by 196
Abstract
Although vitamin D3 (VD3) deficiency has been recognized as a harmful agent in several respiratory diseases, the present study is the first one to investigate its influence on the development of hypersensitivity pneumonitis (HP). This research was conducted in a murine model of [...] Read more.
Although vitamin D3 (VD3) deficiency has been recognized as a harmful agent in several respiratory diseases, the present study is the first one to investigate its influence on the development of hypersensitivity pneumonitis (HP). This research was conducted in a murine model of HP, wherein pulmonary fibrosis was induced by antigen of Pantoea agglomerans. VD3 deficiency was provoked by diet with 10-times less cholecalciferol than feed given to VD3-sufficient mice. Before and after 14 and 28 days of nebulization, lung function was evaluated. Moreover, at indicated time points, lungs were collected and subjected to histological assessment, flow cytometry, gene expression assays, and ELISA. The performed research showed a higher sensitivity of VD3-deficient mice to fibrosis response to P. agglomerans antigen, which was strongly associated with enhanced epithelial-to-mesenchymal transition, the signs of which were over-expression of EMT-transcription factors (Snail2, Zeb1, Zeb2) and mesenchymal cell markers (Cdh2/N-cadherin, Acta2/SMA, Fn1/Fibronectin, Vim/Vimentin). Indicated negative changes in VD3-deficient mice with developed HP were supported by deepening calcitriol deficiency and worsening respiratory functions, including the frequency of breathing, minute volume, total cycle times, expiratory and inspiratory time. Moreover, typical for VD3-deficient mice with HP, there was also an increased influx of immune cells into the lungs (especially neutrophils, macrophages, dendritic cells and lymphocytes Tc), a disturbed cytokine profile with over-production of growth factors favoring fibrosis (FGF2 and TGFβ), and lowered synthesis of several cytokines (IL1β, IL6, IL12, IL4 IL10, IL13). The present study reveals that VD3 deficiency promotes the development of pulmonary fibrosis in the murine model of HP. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Molecular Mechanisms and Therapeutic Strategies)
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