Idiopathic Pulmonary Fibrosis
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  • ||||||||||  bleomycin / Generic mfg.
    Journal:  Dysregulated alveolar type 2 epithelial cell proteostasis promotes fibrogenic macrophage migration inhibitory factor-CD74 signaling. (Pubmed Central) -  Dec 3, 2025   
    Modeling UPS disruption with an AEC2-specific cullin 3 (Cul3) deletion produced spontaneous fibrosis in a physiological aging mouse model and exacerbated fibrosis in the bleomycin-induced lung injury model...In participants with IPF, BALF MIF greater than 4000 picograms per milliliter was associated with increased mortality compared with participants with IPF with lower MIF. Together, these findings define a UPS-sensitive epithelial-macrophage signaling connection and identify MIF-CD74 cross-talk as a potential therapeutic target in fibrotic lung disease.
  • ||||||||||  Biomarker, Journal:  Idiopathic Pulmonary Fibrosis Identification and Prediction of Disease Severity via Combination of Different Biomarker Types. (Pubmed Central) -  Dec 3, 2025   
    A Random Forest was trained with forward feature selection and tested on validation subsets. The results reveal novel combinations of biomarkers at different timescales reflecting pulmonary inflammation, oxidative stress, and metabolic dysregulation, and altogether capable of accurately distinguishing between idiopathic pulmonary function (IPF) vs. connective tissue disease ILD (CTD-ILD) and predicting disease severity as seen with pulmonary function testing (PFT).Clinical Relevance- This proof-of-concept study shows that fibrotic ILD type and disease severity can be predicted via novel combinations of biomarkers spanning a broad range of timescales, offering the possibility to gain insight into the physiological processes underlying the disease.
  • ||||||||||  Journal:  Antagonistic Regulation of Pro-Inflammatory AnnexinA2 and Anti-Inflammatory AnnexinA1 in the Pathogenesis of Idiopathic Pulmonary Fibrosis. (Pubmed Central) -  Dec 3, 2025   
    The results reveal novel combinations of biomarkers at different timescales reflecting pulmonary inflammation, oxidative stress, and metabolic dysregulation, and altogether capable of accurately distinguishing between idiopathic pulmonary function (IPF) vs. connective tissue disease ILD (CTD-ILD) and predicting disease severity as seen with pulmonary function testing (PFT).Clinical Relevance- This proof-of-concept study shows that fibrotic ILD type and disease severity can be predicted via novel combinations of biomarkers spanning a broad range of timescales, offering the possibility to gain insight into the physiological processes underlying the disease. No abstract available
  • ||||||||||  Journal:  VWF Deficiency Inhibits EndoMT to Attenuate Pulmonary Fibrosis. (Pubmed Central) -  Dec 1, 2025   
    Our findings provided evidence for the pivotal role of ECs in IPF and revealed VWF might be a driving factor of EndoMT, suggesting that VWF can develop as a potential therapeutic target against IPF. This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/).
  • ||||||||||  Journal:  Addressing advance care planning for idiopathic pulmonary fibrosis: a call to action. (Pubmed Central) -  Dec 1, 2025   
    While IPF research has resulted in huge progress in the understanding of disease pathobiology and the expansion of treatment options, perhaps the most patient-centered portion of care remains under-studied. We must prioritize research to better understand an interdisciplinary system of iterative ACP that gives IPF patients a clear voice until the last moment of life.
  • ||||||||||  NXP900 / Nuvectis Pharma
    Characterisation of Src-family kinase inhibitors in lung fibroblasts shows distinct signalling complexes required for matrix synthesis and differentiation (Abbey/4th) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_471;    
    Cell-free kinome scan assays and cell-based phosphoproteomics revealed that more selective SFK inhibition does not affect TGF-?1-mediated mTOR signalling required for ECM synthesis, but does modulate cell signalling complexes associated with cytoskeletal organisation, focal adhesion, and cell-cell/substrate junctions. These results suggest specific roles for the SFKs in myofibroblast differentiation with implications for therapeutically targeting functional heterogeneity in pathological cell sub-populations in IPF.
  • ||||||||||  Effect of dietary nitrate supplementation on exercise performance in hypoxic IPF: the EDEN-Ox 3 study (Moore/4th) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_244;    
    Download figure Open in new tab Download powerpoint Abstract P30 Figure 1 Conclusion These findings suggest benefit from acute dietary nitrate supplementation with NR-BRJ in IPF patients with hypoxia on exercise and vascular function, consistent with previous findings in COPD and pulmonary hypertension. Longer term studies are needed to see if this translates into sustained clinical benefit.
  • ||||||||||  CT-P27 / Celltrion
    Can antifibrotic side-effect decision aids improve patient tolerance and continuation of antifibrotic medication? (Moore/4th) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_241;    
    There was no increase in patient contacts outside the established antifibrotic pathway, with a higher proportion of patients not utilising the contact line within the first 3 months (p=0.004). View this table: View inline View popup Download powerpoint Abstract P27 Table 1 Discussion The Decision Aids have had a positive impact on patient tolerance and treatment continuation during the initial 3-month period without a significant increase in patient contacts to the specialist nurse contact line.
  • ||||||||||  nintedanib / Generic mfg., pirfenidone / Generic mfg.
    Impact of tiered service delivery model on antifibrotic adherence and mortality among patients with interstitial lung disease (Moore/4th) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_239;    
    View this table: View inline View popup Download powerpoint Abstract P27 Table 1 Discussion The Decision Aids have had a positive impact on patient tolerance and treatment continuation during the initial 3-month period without a significant increase in patient contacts to the specialist nurse contact line. While there was no difference in discontinuation rate between tiers, pirfenidone had a significantly higher discontinuation risk compared to nintedanib (HR 1.60, 95% CI 1.05
  • ||||||||||  Targeted clinical management of probands and at-risk relatives in familial pulmonary fibrosis: a regional model with national relevance (Abbey/4th) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_189;    
    View this table: View inline View popup Abstract S156 Table 1 Demographic, clinical, functional, and genetic characteristics of patients with ILD and suspected genetic predisposition evaluated in our Regional Familial Pulmonary Fibrosis Service Conclusion Over the initial 6-month service evaluation, we identified a high prevalence of first-degree relatives potentially at risk of developing ILD through our dedicated FPF MDT. Further regional and national referrals will likely emphasize this unmet clinical need and support development of a formal NHS pathway/screening programme for first-degree relatives at risk of developing ILD.
  • ||||||||||  treprostinil / Generic mfg.
    Defining the antifibrotic mechanisms of treprostinil in pulmonary fibrosis (Albert/2nd) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_113;    
    Antifibrotic effects of Treprostinil were detected in IPF derived PCLS. Further studies are ongoing to establish the underlying mechanism as there were additive effects of Treprostinil with established antifibrotic agents which may have important clinical implications.
  • ||||||||||  deoxyglucose / Generic mfg.
    The impact of a hypoxic environment on alveolar macrophage immunometabolism: implications for lung fibrosis (Albert/2nd) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_112;    
    AMs were treated with oligomycin, an inhibitor of ATP synthase and the electron transport chain, or 2-deoxyglucose, a non-metabolisable glucose analogue, and changes in metabolic activity were measured...Decreased efferocytosis in hypoxia may be attributed to dysregulated efferocytosis receptor expression or decreased metabolic activity. Further research is needed to dissect the mechanism behind this dysfunction and to model how it could influence the pathogenesis of IPF.
  • ||||||||||  Exploring the effect of PKN2 knockdown on idiopathic pulmonary fibrosis (Albert/2nd) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_111;    
    Knockdown of PKN2 enhances pro-fibrotic processes, including wound healing and disrupts the expression of fibrosis associated genes, proteins and pathways. Collectively suggesting that PKN2 plays a role in protecting against fibrosis within the lungs.
  • ||||||||||  Quantitative CT is associated with progression free survival in idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis (Moore/4th) -  Nov 28, 2025 - Abstract #BTSWM2025BTS_WM_23;    
    View this table: View inline View popup Abstract S13 Table 1 Patients included in Cox proportional hazards model of progression free survival in idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis Conclusions WRVS correlates with PFTs and is negatively associated with lymphocytosis on BAL, highlighting the potential for WRVS to assess non-fibrotic burden in fHP patients. Baseline WRVS is associated with progression-free survival in both IPF and fHP and may aid prognostication in these patient groups.
  • ||||||||||  prednisone / Generic mfg., pirfenidone / Generic mfg.
    P2 data, Journal:  Pirfenidone for grade 2 and grade 3 radiation-induced lung injury: a multicentre, open-label, randomised, phase 2 trial. (Pubmed Central) -  Nov 27, 2025   
    P2
    Pirfenidone in combination with glucocorticoids provides a potential therapeutic strategy for grade 2 or grade 3 radiation-induced lung injury, addressing the unmet clinical need for effective antifibrotic therapy in patients receiving thoracic radiotherapy. Further investigation is needed to validate these findings in patients with worse radiation-induced lung injury than was studied here.
  • ||||||||||  Biomarker, Review, Journal:  Predicting and Treating Pulmonary Fibrosis with Proteomic Biomarker Investigations. (Pubmed Central) -  Nov 27, 2025   
    To date, specific molecular pathways have been implicated in the onset and progression of idiopathic pulmonary fibrosis, including abnormal wounding, fibroblast proliferation, inflammation, deposition of the extracellular matrix, oxidative stress, endoplasmic reticulum stress, and the coagulation system. This review highlights the role of proteomics in identifying key biomarkers for IPF, focusing on their clinical relevance, including diagnosis, prognosis, disease progression, and the identification of new therapeutic options, in light of the most recent technological advancements in mass spectrometry.