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Biomedicines
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New Advances in Pulmonary Fibrosis
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New Advances in Pulmonary Fibrosis

A special issue of Biomedicines (ISSN 2227-9059). This special issue belongs to the section "Molecular and Translational Medicine".

Deadline for manuscript submissions: 31 August 2026 | Viewed by 8060

Editor

Dr. Mari Ozaki

E-Mail Website
Guest Editor
Department of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland
Interests: pulmonary fibrosis; familial pulmonary fibrosis; induced pluripotent stem cell-derived organoid models; telomere biology; sex hormones; alveolar epithelial type 2 cells

Special Issue Information

Dear Colleagues,

We are delighted to invite you to submit an article to this Special Issue on “New Advances in Pulmonary Fibrosis”.

Pulmonary fibrosis represents a group of debilitating interstitial lung diseases marked by progressive scarring of the lung parenchyma, with rising global incidence and limited treatment options. Despite the availability of FDA-approved antifibrotic drugs, pirfenidone and nintedanib, morbidity and mortality remain high. As a result, there is an urgent need for improved diagnostic tools and novel treatments with better efficacy and safety profiles.

Recent advances in diagnostics include transbronchial cryobiopsy and genomic classifier testing, which offer less invasive approaches with favourable safety and accuracy. Artificial intelligence-assisted imaging is also emerging as a promising tool for early detection, though challenges in standardization persist. On the therapeutic front, new agents targeting alternative pathways are in development. Notably, nerandomilast, a selective PDE4B inhibitor, has shown encouraging results in early trials, both as monotherapy and in combination with current antifibrotics.

This Special Issue aims to bring together cutting-edge research and expert reviews, highlighting the latest progress in pulmonary fibrosis research, from pathobiology, evolving diagnostic methods to innovative therapeutic strategies and personalized medicine approaches to provide a comprehensive overview of the evolving landscape of pulmonary fibrosis and its implications for both clinical practice and translational research.

Dr. Mari Ozaki
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-anonymized peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Biomedicines is an international peer-reviewed open access monthly 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 2600 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
  • interstitial lung disease
  • antifibrotic therapy
  • precision medicine
  • imaging diagnostics
  • novel therapeutics
  • pathobiology
  • artificial intelligence

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Further information on MDPI's Special Issue policies can be found here.

Published Papers (3 papers)

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Research

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16 pages, 761 KB  
Article
Lung Transplantation in Idiopathic Pulmonary Fibrosis Patients in the European MultiPartner IPF Registry: Challenges for Health Equity
by Nóra M. Tóth, Mordechai R Kramer, Martina Šterclová, Veronika Müller, Katarzyna B. Lewandowska, Nesrin Mogulkoc, Marta Hájková, Michael Studnicka, Jasna Tekavec-Trkanjec, Sanja Dimic-Janjic, Anton Penev, Zoran Arsovski, Jakub Gregor, Petra Ovesná and Martina Koziar Vašáková
Biomedicines 2025, 13(11), 2684; https://doi.org/10.3390/biomedicines13112684 - 31 Oct 2025
Viewed by 2245
Abstract
Background: Despite advancements in pharmacological therapy, lung transplantation (LuTX) remains the only life-prolonging treatment in end-stage idiopathic pulmonary fibrosis (IPF). However, real-world referral patterns in Central and Eastern European (CEE) countries remain poorly characterized. We aimed to comprehensively review factors influencing referral and [...] Read more.
Background: Despite advancements in pharmacological therapy, lung transplantation (LuTX) remains the only life-prolonging treatment in end-stage idiopathic pulmonary fibrosis (IPF). However, real-world referral patterns in Central and Eastern European (CEE) countries remain poorly characterized. We aimed to comprehensively review factors influencing referral and identify systemic barriers to LuTX access. Methods: Baseline characteristics of IPF patients potentially eligible for LuTX, enrolled in the European MultiPartner IPF Registry between 2012 and 2022 (n = 1256), were retrospectively analyzed. LuTX (n = 94) and potentially eligible but not transplanted (n = 1162) subgroups were compared. National experts also completed a questionnaire assessing transplant referral and listing practices across different healthcare systems. Results: Only 7.5% of potentially eligible subjects were transplanted, revealing substantial geographic disparities, with Israel having the highest rates (43.1%), followed by Austria (9.5%), Hungary (7.8%), and the Czech Republic (4.6%). LuTX patients were younger (60.2 ± 7.4 vs. 62.6 ± 6.2 years, p < 0.001), had worse lung function (FVC 60 ± 15 vs. 74 ± 21% predicted; p < 0.001, TLCO 41 ± 15 vs. 49 ± 19% predicted; p < 0.001), and were more likely to receive antifibrotic and oxygen therapies. The most frequent reasons for exclusion from referral/listing were age > 70 years and concomitant heart/renal failure. Conclusions: This first comprehensive CEE analysis demonstrates low IPF transplant rates with high inter-country variability. Patients presenting early with functionally advanced disease are more likely transplanted, while advanced age remains the primary exclusion factor, highlighting critical access gaps potentially contributing to regional outcome differences. Full article
(This article belongs to the Special Issue New Advances in Pulmonary Fibrosis)
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Review

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22 pages, 1034 KB  
Review
A Scoping Review of Emerging Treatments in the Pipeline for Idiopathic Pulmonary Fibrosis: Future Perspectives
by Maria Eugenia Novara, Martina Chirulli, Patrizio Vitulo, Anna Carollo and Alessio Provenzani
Biomedicines 2026, 14(6), 1293; https://doi.org/10.3390/biomedicines14061293 - 5 Jun 2026
Viewed by 577
Abstract
Background: Idiopathic pulmonary fibrosis (IPF) is an incurable disease with limited therapeutic options and a poor prognosis. Current standard therapies are characterized by drugs or surgical strategies with limited effects, as they are either not curative or their use is restricted to [...] Read more.
Background: Idiopathic pulmonary fibrosis (IPF) is an incurable disease with limited therapeutic options and a poor prognosis. Current standard therapies are characterized by drugs or surgical strategies with limited effects, as they are either not curative or their use is restricted to a specific subset of the population. The aim of this scoping review is to evaluate the drugs currently under investigation in Phase II and Phase III trials and provide an overview of the mechanisms of new therapeutic strategies for IPF. Methods: The search strategy was conducted in accordance with PRISMA guidelines and included studies conducted on adults with IPF retrieved from the registered ClinicalTrials.gov database up to 31 December 2025. Results: Nineteen studies were included. The clinical trials investigate key signaling pathways and molecular targets, including MAPK, RhoA/ROCK, PDE4B/cAMP, Wnt/β-catenin, Hedgehog/SMO, IL-11/STAT3, and LPA/autotaxin, as well as extracellular receptors and mediators such as CSF1R, TBXA2R, and WISP1. Conclusions: Ongoing clinical research in IPF reflects a broad diversification of molecular targets; however, translational success remains limited. Current evidence suggests that biological complexity, pathway redundancy, and systemic constraints significantly restrict the clinical impact of single-target strategies. Future progress will likely depend on improved patient stratification, combination approaches, and biomarker-guided trial design rather than isolated pathway modulation. Full article
(This article belongs to the Special Issue New Advances in Pulmonary Fibrosis)
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29 pages, 1280 KB  
Review
Idiopathic Pulmonary Fibrosis: A Comprehensive Review of Risk Factors, Genetics, Diagnosis, and Therapeutic Approaches
by Lamiyae Senhaji, Nadia Senhaji, Meriame Abbassi, Mariem Karhate, Mounia Serraj, Mohammed El Biaze, Mohamed Chakib Benjelloun, Karim Ouldim, Laila Bouguenouch and Bouchra Amara
Biomedicines 2026, 14(1), 90; https://doi.org/10.3390/biomedicines14010090 - 1 Jan 2026
Cited by 4 | Viewed by 4642
Abstract
Idiopathic Pulmonary Fibrosis (IPF) is a severe, chronic, progressive lung disease classified within interstitial lung disorders. It predominantly affects individuals aged 50 to 70 years, with a prognosis of 3–5 years post-diagnosis. The pathophysiology of IPF is complex, involving an interplay of genetic [...] Read more.
Idiopathic Pulmonary Fibrosis (IPF) is a severe, chronic, progressive lung disease classified within interstitial lung disorders. It predominantly affects individuals aged 50 to 70 years, with a prognosis of 3–5 years post-diagnosis. The pathophysiology of IPF is complex, involving an interplay of genetic predisposition, environmental exposures, and age-related factors. A significant genetic component is evident, with key contributions from rare variants in telomere maintenance genes (e.g., TERT and TERC) and surfactant protein genes (e.g., SFTPA and SFTPC), as well as a strong association with a common promoter variant in the MUC5B gene. The diagnosis is established through high-resolution computed tomography (HRCT) and, when necessary, histopathological analysis. The search for reliable biomarkers is a key area of research, with molecules such as KL-6, SP-A, SP-D, and MMP-7 showing potential for aiding in diagnosis, prognosis, and monitoring disease activity. While antifibrotic therapies (Pirfenidone and Nintedanib) have revolutionized management by slowing the decline in lung function, the therapeutic landscape continues to evolve. Ongoing research efforts are focused on integrating clinical, radiological, genetic, and biomarker data to facilitate early diagnosis and develop personalized treatment strategies to improve patient outcomes. Full article
(This article belongs to the Special Issue New Advances in Pulmonary Fibrosis)
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