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Cells
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Advances in Pulmonary Fibrosis
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Advances in Pulmonary Fibrosis

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: 10 June 2026 | Viewed by 4158

Special Issue Editor

Dr. Sergio Piñeiro Hermida

E-Mail Website
Guest Editor
Ramón y Cajal Research Fellow, Head of the Telomeres and Tumor Microenvironment Unit, Fundación Rioja Salud, Piqueras 98, 26006, Logroño, Spain
Interests: lung

Special Issue Information

Dear Colleagues, 

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease characterized by a progressive decline in exercise capacity, difficulty breathing, recurrent infections, and a severe impairment in lung function, which makes the patients dependent on long-term oxygen treatment. Due to the lack of effective treatments, the median survival time of patients from diagnosis is 2–4 years. Currently, lung transplantation is the only therapeutic option and is carried out in fewer than 5% of IPF patients with a very severe stage of disease. Thus, there is an urgent need to identify novel mechanisms implicated in the onset and development of this disease, as well as potential therapies. 

Potential topics for inclusion in this Special Issue include the following:

  • Novel biomarkers;
  • Experimental therapeutics;
  • Animal models (cellular and molecular pathophysiology and experimental therapies);
  • The identification of novel mechanisms in IPF.

Dr. Sergio Piñeiro Hermida
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pulmonary fibrosis
  • animal models
  • biomarkers
  • experimental therapeutics

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Published Papers (3 papers)

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Research

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15 pages, 5064 KB  
Article
Mitochondria-Dependent Metabolic Reprogramming Enhances Myofibroblast Differentiation and Aggravates Bleomycin-Induced Pulmonary Fibrosis
by Kai Yazaki, Yosuke Matsuno, Yuki Yabuuchi, Sosuke Matsumura, Kenya Kuramoto, Kazufumi Yoshida, Masashi Matsuyama, Takumi Kiwamoto, Yuko Morishima, Yukio Ishii, Kaori Ishikawa, Kazuto Nakada and Nobuyuki Hizawa
Cells 2026, 15(7), 582; https://doi.org/10.3390/cells15070582 - 25 Mar 2026
Viewed by 883
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease characterized by irreversible fibrosis. Aberrant cell differentiation plays a crucial role in the development of IPF. Although recent studies have suggested that mitochondrial dysfunction may play a role in IPF, its direct impact [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease characterized by irreversible fibrosis. Aberrant cell differentiation plays a crucial role in the development of IPF. Although recent studies have suggested that mitochondrial dysfunction may play a role in IPF, its direct impact on fibrosis remains unclear. This study aimed to clarify the role of mitochondria in lung cell differentiation and pulmonary fibrosis development by employing mito-mice ND6M, in which the activity of respiratory chain complex I is decreased due to a mitochondrial DNA mutation (G13997A). Pulmonary fibrosis was induced by administering bleomycin (BLM) to both wild-type and mito-mice ND6M. Bone marrow-derived macrophages and primary lung fibroblasts, generated from both types of mice, were analyzed to evaluate M1/M2 polarization and myofibroblast differentiation, respectively. Compared to wild-type mice, mito-mice ND6M exhibited more severe fibrosis and lower survival rates following BLM inoculation. Lactate production in the lungs after BLM administration was significantly higher in mito-mice ND6M than in wild-type mice. TGF-β1-treated fibroblasts from mito-mice ND6M exhibited increased α-smooth muscle actin expression. While type I collagen expression was not different between these mice, TGF-β1-induced expression of phosphoserine phosphatase and serine hydroxymethyltransferase2, two of the enzymes involved in the serine–glycine pathway, was significantly higher in mito-mice ND6M than in wild-type mice. On the other hand, mitochondrial dysfunction had a small effect on pulmonary inflammation and on M1/M2 macrophage polarization. In conclusion, mitochondrial dysfunction promotes TGF-β1-induced myofibroblast differentiation and BLM-induced pulmonary fibrosis. Mitochondria-dependent metabolic reprogramming may therefore represent a promising therapeutic target in IPF. Full article
(This article belongs to the Special Issue Advances in Pulmonary Fibrosis)
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18 pages, 3381 KB  
Article
EPDR1 Links Fibroblast Dysfunction to Disease Severity in Idiopathic Pulmonary Fibrosis
by Jong-Uk Lee, Seung-Lee Park, Min Kyung Kim, Eunjeong Seo, Hun-Gyu Hwang, Jung Hyun Kim, Hun Soo Chang and Choon-Sik Park
Cells 2025, 14(19), 1515; https://doi.org/10.3390/cells14191515 - 28 Sep 2025
Cited by 1 | Viewed by 1842
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by aberrant fibroblast activation, lysosomal dysfunction, and cellular senescence. Transcriptomic analyses have identified ependymin-related 1 (EPDR1) as a fibroblast-enriched gene in IPF, but its biological function remains unclear. EPDR1 expression was assessed in [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterized by aberrant fibroblast activation, lysosomal dysfunction, and cellular senescence. Transcriptomic analyses have identified ependymin-related 1 (EPDR1) as a fibroblast-enriched gene in IPF, but its biological function remains unclear. EPDR1 expression was assessed in lung fibroblasts, lung tissues, bronchoalveolar lavage fluid (BALF), and serum from IPF patients and controls using qPCR, Western blotting, ELISA, and immunohistochemistry. Lysosomal function, autophagic flux, and senescence markers were analyzed in primary fibroblasts following siRNA-mediated EPDR1 knockdown. EPDR1 was significantly upregulated in IPF-derived fibroblasts and localized to fibrotic regions enriched with α-SMA+, COL1A1+, and FN1+ myofibroblasts of IPF-derived lung tissues. EPDR1 levels were markedly elevated in the BALF and serum of IPF patients and correlated with increased mortality. IPF fibroblasts exhibited reduced lysosomal acidification and impaired autophagic flux, indicated by p62 and LC3B accumulation. EPDR1 knockdown restored lysosomal function; enhanced autophagic degradation; and reduced senescence markers, including p21, p16, and SA-β-gal activity. EPDR1 drives lysosomal dysfunction and fibroblast senescence in IPF. Its elevated expression in lung tissue and biological fluids, together with its association with prognosis, highlights EPDR1 as a potential biomarker and therapeutic target in IPF. Full article
(This article belongs to the Special Issue Advances in Pulmonary Fibrosis)
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Review

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23 pages, 681 KB  
Review
Alveolar Lipid–Macrophage Networks at the Intersection of Pulmonary Fibrosis
by Simon H. Apte, Viviana P. Lutzky, Penny L. Groves and Daniel C. Chambers
Cells 2026, 15(8), 668; https://doi.org/10.3390/cells15080668 - 9 Apr 2026
Viewed by 851
Abstract
Idiopathic pulmonary fibrosis (IPF) is characterised by progressive parenchymal remodelling, driven by epithelial dysfunction, fibroblast activation, and altered immune regulation within the distal lung. Alveolar macrophages (AMs) reside in a surfactant-rich environment and are specialised for continuous lipid handling, yet the significance of [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is characterised by progressive parenchymal remodelling, driven by epithelial dysfunction, fibroblast activation, and altered immune regulation within the distal lung. Alveolar macrophages (AMs) reside in a surfactant-rich environment and are specialised for continuous lipid handling, yet the significance of this metabolic role for macrophage heterogeneity and fibrotic progression has remained incompletely integrated across studies. In this review, we synthesise evidence from human lung tissue, experimental models, lipidomic analyses, and clinical investigations to place macrophage populations described in IPF—including FABP4-high homeostatic cells and SPP1-associated disease-enriched states—within a unified lipid-metabolic context. We show that macrophage heterogeneity in IPF can be understood as a variation within a core lipid-handling programme rather than the emergence of distinct macrophage lineages. Profibrotic macrophage states are characterised by altered lipid processing and signalling, including dysregulated sterol handling, lysophospholipid pathways, and eicosanoid balance, which impair surfactant turnover and contribute to fibroblast activation. Importantly, experimental and clinical data indicate that macrophage lipid-metabolic programmes remain modifiable, although definitive disease-modifying efficacy in IPF has yet to be established. Framing macrophage states within a lipid-metabolic framework provides a coherent basis for interpreting heterogeneous datasets and supports the rationale for therapeutic strategies aimed at stabilising or restoring macrophage lipid handling in fibrotic lung disease. Full article
(This article belongs to the Special Issue Advances in Pulmonary Fibrosis)
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