2f:I[703058,["/_next/static/immutable/chunks/3bhq_5eh1d760.js","/_next/static/immutable/chunks/1855-ogyi8eh4.js","/_next/static/immutable/chunks/2pfsjpn6k6zlg.js","/_next/static/immutable/chunks/26c7v3_3uwnwu.js","/_next/static/immutable/chunks/25a3ozcrvi1ff.js","/_next/static/immutable/chunks/2r4wx50nvjh_4.js","/_next/static/immutable/chunks/23fsw06hsi5b1.js","/_next/static/immutable/chunks/06_yt270h41rg.js","/_next/static/immutable/chunks/3ezsiz-7shkna.js","/_next/static/immutable/chunks/20x2y96ldu5wx.js","/_next/static/immutable/chunks/2lxntcm3pd1_h.js","/_next/static/immutable/chunks/00dgco8irq3jw.js","/_next/static/immutable/chunks/00nwe-kq0rboc.js","/_next/static/immutable/chunks/2051ffx3xzio9.js"],"PaperListPageClient"] 30:T535,Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease marked by aberrant fibroblast activation, resulting in excessive proliferation, survival, and accumulation of extracellular matrix (ECM). A critical barrier to developing effective therapies for IPF is the limited understanding of druggable molecular regulators that control fibroblast activation. In this study, we identify the proto-oncogene MYCN as a key driver upregulated in dysregulated fibroblasts from IPF lungs. Mechanistically, we show that TGFα induces MYCN expression via the profibrotic transcription factor Wilms tumor 1 (WT1). Notably, the knockdown of MYCN significantly attenuated fibroblast proliferation, survival, and ECM production. We further show that MYCN positively regulates the mitotic kinase, Polo-like kinase (PLK1), and that pharmacological inhibition of PLK1 using volasertib reduced expression of MYCN, WT1, and PLK1 and mitigated fibroblast activation. In vivo, volasertib treatment attenuated fibroblast activation and collagen deposition during TGFα-induced pulmonary fibrosis. Together, these findings identify a pathogenic role for the WT1-MYCN-PLK1 axis in fibroblast activation and provide proof-of-concept evidence supporting PLK1 inhibition with volasertib as a potential therapeutic strategy for IPF.31:T546,Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease marked by aberrant fibroblast activation, resulting in excessive proliferation, survival, and accumulation of extracellular matrix (ECM). A critical barrier to developing effective therapies for IPF is the limited understanding of druggable molecular regulators that control fibroblast activation. In this study, we identify the proto-oncogene MYCN as a key driver upregulated in dysregulated fibroblasts from IPF lungs. Mechanistically, we show that TGFα induces MYCN expression via the profibrotic transcription factor Wilms tumor 1 (WT1). Notably, the knockdown of MYCN significantly attenuated fibroblast proliferation, survival, and ECM production. We further show that MYCN positively regulates the mitotic kinase, Polo-like kinase (PLK1), and that pharmacological inhibition of PLK1 using volasertib reduced expression of MYCN, WT1, and PLK1 and mitigated fibroblast activation. In vivo, volasertib treatment attenuated fibroblast activation and collagen deposition during TGFα-induced pulmonary fibrosis. Together, these findings identify a pathogenic role for the WT1-MYCN-PLK1 axis in fibroblast activation and provide proof-of-concept evidence supporting PLK1 inhibition with volasertib as a potential therapeutic strategy for IPF.32:Tf17, Background The association between immune-cell-specific transcriptomic profiles and mortality in IPF is unknown. Methods We profiled peripheral blood mononuclear cells (PBMC) by single-cell RNA sequencing (scRNA-seq) and investigated which immune-cell-specific transcriptomic profile predicted IPF outcomes consistently. Prognostic accuracy was investigated in PBMC, Bronchoalveolar Lavage (BAL) and lung tissue. Findings were validated by flow cytometry, analysis of independent scRNA-seq datasets and cellular deconvolution. We investigated the function of this transcriptomic profile and its cellular source in lung tissue (overall sample size:1054, IPF:555, other:499). Connectivity map and LASSO regression were used to identify drug candidates and a subset of genes with prognostic potential, respectively. Results A 230-gene-up-score (Pittsburgh-PBMC) from CD14 + CD163 − HLA-DR low monocytes predicted mortality in Chicago PBMC cohort (HR: 6.58, 95%CI:2.15–20.13, p=0.001), in BAL pooled analysis (HR: 2.20, 95%CI: 1.44–3.37, p=0.0003) and negatively correlated with Forced Vital Capacity (FVC) in lung tissues (ρ=−0.2, p=0.02). CD14 + CD163 − HLA-DR low monocytes were higher in progressive versus stable IPF (12.59%, 95%CI:9.66–16.23, versus 7.61%, 95%CI:6.68–10.21, p=0.014). High risk IPF patients had decreased expression of T-cell co-stimulatory genes (Pittsburgh and Chicago p<0.01). CD14 + HLA-DR low monocytes had higher expression of profibrotic, proangiogenic and chemotactic factors compared to CD14 + HLA-DR hi monocytes (p<0.05). The 230-gene-up-score correlated with the SPP1 + fibrosis-associated macrophages-gene-score in lung tissues (ρ=0.19, p<2.2e − 16). Connectivity map identified drug categories to reverse the 230-gene-signature. A subset of six genes retained predictive performance (pooled PBMC cohorts -HR: 4.79, 95%CI:2.58–8.92, p<0.0001). Conclusions The transcriptome of CD14 + CD163 − HLA-DR low monocytes is associated with increased mortality in patients with IPF. Its reversal should be investigated as a precision-based therapy in IPF. 25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"Idiopathic Pulmonary Fibrosis: condition Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on Idiopathic Pulmonary Fibrosis (condition). Clinician-written summaries and actionable practice insights. Updated daily.\",\"url\":\"https://www.reachrx.ai/hub/tag/condition/Idiopathic%20Pulmonary%20Fibrosis\",\"breadcrumb\":{\"@type\":\"BreadcrumbList\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https://www.reachrx.ai\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Research Hub\",\"item\":\"https://www.reachrx.ai/hub\"},{\"@type\":\"ListItem\",\"position\":3,\"name\":\"Conditions\",\"item\":\"https://www.reachrx.ai/hub/tag/condition\"},{\"@type\":\"ListItem\",\"position\":4,\"name\":\"Idiopathic Pulmonary Fibrosis\",\"item\":\"https://www.reachrx.ai/hub/tag/condition/Idiopathic%20Pulmonary%20Fibrosis\"}]}}"}}]],["$","$L2f",null,{"title":"Idiopathic Pulmonary Fibrosis: Research & Clinical Takeaways","description":"Discover peer-reviewed research on Idiopathic Pulmonary Fibrosis with concise clinician summaries and next-step guidance.","papers":[{"doi":"10.1002/resp.70309","pmid":"42706010","title":"Contemporary Concise Review 2025: Interstitial Lung Disease","journal_title":"Respirology","journal_issn":"1323-7799","published_at":"2026-09-07T00:00:00.000Z","url":"https://doi.org/10.1002/resp.70309","source_score":6,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-09-13T06:21:49.904Z","updated_at":"2026-09-13T06:21:49.904Z","tags":[{"paper_doi":"10.1002/resp.70309","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"condition","value":"Interstitial Lung Abnormalities","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"condition","value":"Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"modality","value":"Antifibrotic Therapy","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"warning","value":"No specific drugs, brand names, or trial-level data are named in the abstract; tags reflect only what is directly stated.","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"warning","value":"Publication date (2026-09-07) is in the future relative to the review year cited (2025); this appears to be a 2025 literature review published in 2026, which is consistent with today's date.","value_numeric":null},{"paper_doi":"10.1002/resp.70309","tag_type":"warning","value":"This is a narrative review without systematic search criteria or meta-analytic methods; level of evidence is low (expert opinion/review).","value_numeric":null}],"summaries":{"what_studied":"A contemporary concise review (2025) summarizing recent advances in interstitial lung disease (ILD), covering updated nomenclature, early detection, interstitial lung abnormalities (ILAs), multi-omic biomarkers, quantitative imaging, and novel antifibrotic/targeted therapies.","key_result":"Key themes include: updated ILD classification aligning terminology with morphology and pathobiology; growing focus on early ILD detection and risk stratification in ILAs; emerging multi-omic biomarkers and quantitative imaging for prognostic stratification; and expanding antifibrotic/targeted therapies beyond IPF, with unmet needs in patient selection and treatment response.","limitations":"As a narrative review, it does not provide primary data, pooled effect sizes, or systematic search methodology, limiting its evidentiary weight. Specific patient populations, sample sizes, and outcome data are not reported.","practice_takeaway":"Multidisciplinary discussion remains the foundation of ILD care; clinicians should be aware of updated ILD nomenclature and the expanding therapeutic landscape beyond IPF, including novel antifibrotics and targeted agents. Early identification of high-risk ILA patients and integration of molecular/imaging biomarkers may help guide individualized management."}},{"doi":"10.1002/resp.70305","pmid":"42706029","title":"Contemporary Fibrotic Interstitial Lung Disease Outcomes: A Multicentre National Cohort Study","journal_title":"Respirology","journal_issn":"1323-7799","published_at":"2026-09-07T00:00:00.000Z","url":"https://doi.org/10.1002/resp.70305","source_score":6,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-09-11T06:20:00.870Z","updated_at":"2026-09-11T06:20:00.870Z","tags":[{"paper_doi":"10.1002/resp.70305","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"condition","value":"Connective Tissue Disease-Associated Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"condition","value":"Fibrotic Hypersensitivity Pneumonitis","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"condition","value":"Unclassifiable Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"sample_size","value":"n=4236","value_numeric":4236},{"paper_doi":"10.1002/resp.70305","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"study_design","value":"Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"warning","value":"fHP (fibrotic hypersensitivity pneumonitis) subgroup represents only 9% of the cohort; subgroup-specific estimates may have wider uncertainty.","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"warning","value":"No drug or therapeutic intervention was evaluated; modality, brand, and generic tags were omitted as not applicable.","value_numeric":null},{"paper_doi":"10.1002/resp.70305","tag_type":"warning","value":"The study population is Canadian and registry-based, which may limit generalizability to other healthcare systems or ILD referral patterns.","value_numeric":null}],"summaries":{"what_studied":"Transplant-free survival and prognostic factors were assessed in 4,236 adults with fibrotic ILD (IPF, CTD-ILD, fHP, uILD) prospectively enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE-PF), with outcomes tracked at 1, 3, and 5 years from enrollment. Performance of the ILD-GAP index was also evaluated.","key_result":"Overall transplant-free survival was 91% at 1 year, 72% at 3 years, and 57% at 5 years. IPF had the worst outcomes (87%, 61%, 39%), while CTD-ILD had the best (95%, 81%, 70%; adjusted HR vs. IPF: 0.64 [95% CI 0.55–0.76]). The ILD-GAP index showed moderate discrimination (Harrell's C = 0.75 at 1 year, 0.73 at 3 years).","limitations":"Registry-based observational design limits causal inference. Enrollment was used as time zero, which may introduce selection bias toward patients already engaged in specialist care. The ILD-GAP index showed only moderate discrimination, suggesting prognostic gaps remain.","practice_takeaway":"CTD-ILD carries meaningfully better long-term prognosis than IPF — roughly twice the 5-year transplant-free survival (70% vs. 39%) — reinforcing the importance of accurate ILD subtyping. The ILD-GAP index performs modestly in contemporary cohorts and should not be used alone for major clinical decisions."}},{"doi":"10.1038/s41590-026-02616-1","pmid":"42629416","title":"Collaborative functionality between three types of inflammation in idiopathic pulmonary fibrosis","journal_title":"Nature Immunology","journal_issn":"1529-2908","published_at":"2026-08-21T00:00:00.000Z","url":"https://doi.org/10.1038/s41590-026-02616-1","source_score":15,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-29T06:19:14.047Z","updated_at":"2026-08-29T06:19:14.047Z","tags":[{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"category","value":"Immunology","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"modality","value":"Immunomodulatory Therapy","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"warning","value":"No patient population size is applicable — this is a review article with no primary data.","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"warning","value":"The paper's DOI contains a future-looking identifier (2026); confirmed as published August 21, 2026 — consistent with today's date.","value_numeric":null},{"paper_doi":"10.1038/s41590-026-02616-1","tag_type":"warning","value":"The 'three types of inflammation' framework is central to the review but is not explicitly named in the abstract; tag classifications reflect inferred standard immunological types (Type 1/2/3).","value_numeric":null}],"summaries":{"what_studied":"This review examines how three distinct types of inflammation (and their key cellular and soluble mediators) collaborate to drive progression of idiopathic pulmonary fibrosis (IPF), focusing on the multicellular fibrotic niche rather than isolated cytokines or immune cells.","key_result":"The authors argue that IPF progression is regulated by spatially and kinetically overlapping inflammatory types acting in concert — including epithelial dysfunction, peripheral immune cell infiltration, fibroblast/immune cell differentiation, and extracellular matrix remodeling — rather than a single dominant inflammatory program.","limitations":"As a narrative review, no original data are presented and conclusions reflect the authors' synthesis of existing literature, which may introduce selection bias. The mechanistic overlap between inflammatory types in IPF is largely inferred from experimental models and may not fully translate to human disease.","practice_takeaway":"Current IPF therapies only slow decline; this review highlights that targeting multiple overlapping inflammatory pathways simultaneously — rather than single cytokines — may be needed to achieve meaningful disease modification. Clinicians should watch for emerging combination or multi-target therapeutic strategies."}},{"doi":"10.1016/j.arbres.2026.07.002","pmid":"42595561","title":"Combined Assessment of Frailty and Respiratory Function in Idiopathic Pulmonary Fibrosis: A Prospective Cohort Study","journal_title":"Archivos de Bronconeumología","journal_issn":"0300-2896","published_at":"2026-08-13T00:00:00.000Z","url":"https://doi.org/10.1016/j.arbres.2026.07.002","source_score":2,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-20T06:47:54.864Z","updated_at":"2026-08-20T06:47:54.864Z","tags":[{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"condition","value":"Frailty","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"modality","value":"Geriatric Assessment","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"sample_size","value":"n=140","value_numeric":140},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"study_design","value":"Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"warning","value":"The composite outcome (mortality or acute exacerbation) combines events of differing clinical severity; separate outcome analyses are not reported in the abstract.","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"warning","value":"The 'impact_flag' of practice_changing is applied cautiously — findings are promising but stem from a single center with a modest sample size; external validation is needed before widespread adoption.","value_numeric":null},{"paper_doi":"10.1016/j.arbres.2026.07.002","tag_type":"warning","value":"Three frailty tools were compared for concordance, but full concordance statistics (e.g., kappa coefficients) are not reported in the abstract, limiting interpretation of agreement between instruments.","value_numeric":null}],"summaries":{"what_studied":"This prospective single-center cohort study evaluated the prevalence and concordance of frailty tools (Fried Frailty Phenotype, CGA-derived Frailty Index, and Clinical Frailty Scale) in 140 IPF patients aged ≥65, and assessed whether combining frailty measures with the GAP index improved prediction of all-cause mortality or acute exacerbation over a median follow-up of 465 days.","key_result":"Frailty prevalence ranged from 16.4% (CGA-FI) to 22.3% (FFP); 25% of patients hit the composite outcome. CFS ≥5 (HR 3.94; 95% CI 1.81–8.58) and GAP stage II–III (HR 3.11; 95% CI 1.39–7.00) were independently predictive. The combined GAP-CFS model achieved a bootstrap Harrell C-index of 0.72 (95% CI 0.62–0.81).","limitations":"Single-center design limits generalizability. Relatively small sample (n=140) with only 35 events may reduce statistical power. Median follow-up of ~15 months may miss longer-term outcomes.","practice_takeaway":"Add the Clinical Frailty Scale (CFS) to routine GAP staging in older IPF patients — a CFS ≥5 nearly quadruples the risk of death or acute exacerbation. Use this combined GAP-CFS assessment to guide personalized, multidimensional care planning."}},{"doi":"10.1016/j.rmed.2026.109086","pmid":"42567369","title":"Comprehensive assessment of complete blood count as a prognostic biomarker in interstitial lung diseases","journal_title":"Respiratory Medicine","journal_issn":"0954-6111","published_at":"2026-08-07T00:00:00.000Z","url":"https://doi.org/10.1016/j.rmed.2026.109086","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-13T13:22:55.959Z","updated_at":"2026-08-13T13:22:55.959Z","tags":[{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"condition","value":"Connective Tissue Disease-Associated Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"condition","value":"Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"modality","value":"Laboratory Biomarker","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"sample_size","value":"n=1065","value_numeric":1065},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"study_design","value":"Retrospective Cohort Study (Derivation-Validation)","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"warning","value":"Study design label 'Retrospective Cohort Study (Derivation-Validation)' is used; the authors also describe it as 'registry-based,' which may imply some methodological nuances not fully captured by a single design label.","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"warning","value":"Subgroup sample sizes for specific ILD categories (e.g., CTD-ILD) are not reported in the abstract, so the robustness of subgroup findings is unclear.","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"warning","value":"The study is registry-based at tertiary referral centers, which may limit generalizability to community practice settings.","value_numeric":null},{"paper_doi":"10.1016/j.rmed.2026.109086","tag_type":"warning","value":"Total sample size of n=1065 is derived by combining the derivation (n=369) and validation (n=696) cohorts, as no single combined N is stated in the abstract.","value_numeric":null}],"summaries":{"what_studied":"This retrospective derivation-validation registry study assessed the prognostic value of complete blood count (CBC) parameters for a composite outcome of lung transplantation or death in consecutive ILD patients at tertiary referral centers. It included a derivation cohort (n=369, median follow-up 20 months) and an independent validation cohort (n=696, median follow-up 60 months).","key_result":"Neutrophil count >5.6 K/μL was confirmed as an independent predictor of worse outcomes across most ILD subtypes in both cohorts (except CTD-ILD). Platelet count <149 K/μL also showed prognostic significance, though it was not an independent predictor in multivariate analysis.","limitations":"- Retrospective registry design limits causal inference and may introduce selection bias toward tertiary-center patients.\n- CBC cutoffs were data-derived (Youden's index), which may not generalize to other populations.\n- CTD-ILD was an exception to the neutrophil finding, but subgroup sizes were not reported, limiting interpretation.","practice_takeaway":"At ILD diagnosis, check a CBC: a neutrophil count above 5.6 K/μL independently signals higher risk of death or lung transplantation across most ILD types. Consider closer monitoring or earlier referral for advanced therapies in patients who cross this threshold — but note this threshold does not apply to CTD-ILD."}},{"doi":"10.1183/13993003.00338-2026","pmid":"42532543","title":"Effect of nerandomilast on survival in patients with pulmonary fibrosis","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2026-07-30T00:00:00.000Z","url":"https://doi.org/10.1183/13993003.00338-2026","source_score":11,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-04T18:18:08.130Z","updated_at":"2026-08-04T18:18:08.130Z","tags":[{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"condition","value":"Progressive Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"generic","value":"nerandomilast","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"modality","value":"PDE4B Inhibitor","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"study_design","value":"Pooled Analysis of Randomized Controlled Trials","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"warning","value":"Sample size is not explicitly reported in the abstract; the sample_size tag has been omitted.","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"warning","value":"The modality tag 'PDE4B Inhibitor' is inferred from nerandomilast's known pharmacology, not stated explicitly in this abstract.","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"warning","value":"The primary (key secondary) endpoint was not met in either individual trial; the survival benefit is based on a pooled post-hoc analysis of a secondary endpoint, which warrants caution in interpretation.","value_numeric":null},{"paper_doi":"10.1183/13993003.00338-2026","tag_type":"warning","value":"The study was published July 30, 2026 — very recent; regulatory status of nerandomilast is not addressed in the abstract.","value_numeric":null}],"summaries":{"what_studied":"Pooled survival analysis from two placebo-controlled trials (FIBRONEER-IPF and FIBRONEER-ILD) evaluating nerandomilast (9 mg or 18 mg twice daily) versus placebo on time to death in patients with IPF and PPF over a mean follow-up of 16.7 months.","key_result":"Nerandomilast 18 mg bid reduced the risk of death by 43% vs. placebo (HR 0.57, 95% CI 0.41–0.78; p<0.001); 9 mg bid reduced it by 33% (HR 0.67, 95% CI 0.49–0.90; p=0.009). Sensitivity, tipping point, and exposure-response analyses all supported these findings.","limitations":"The key secondary endpoint was not met in either individual trial; this analysis is a pooled, post-hoc investigation of a secondary endpoint, which limits causal inference. Missing data assumptions were explored but not eliminated. Lung transplant-related deaths were excluded from the primary analysis, which could affect generalizability.","practice_takeaway":"Nerandomilast 18 mg bid showed a robust 43% reduction in mortality risk across IPF and PPF patients in pooled analysis — clinicians managing these patients may consider this emerging evidence, though regulatory approval and primary endpoint data should be reviewed before adopting it into practice."}},{"doi":"10.1093/ajrccm/aamag396","pmid":"42518202","title":"ADAMTS14 is a Novel Modulator of Fibroblast Mechanoactivation in Pulmonary Fibrosis","journal_title":"American Journal of Respiratory and Critical Care Medicine","journal_issn":"1073-449X","published_at":"2026-07-28T00:00:00.000Z","url":"https://doi.org/10.1093/ajrccm/aamag396","source_score":8,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-01T13:21:56.438Z","updated_at":"2026-08-01T13:21:56.438Z","tags":[{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"generic","value":"ADAMTS14 siRNA","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"modality","value":"Gene Silencing (siRNA)","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"study_design","value":"Translational/Mechanistic Study","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"warning","value":"No specific sample size (N) is reported in the abstract or results for patient cohorts or in vitro experiments; sample_size tag was omitted.","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"warning","value":"Study design is primarily in vitro and transcriptomic — no dedicated animal model for ADAMTS14 was described, so level of evidence is set at 5 (preclinical/mechanistic).","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag396","tag_type":"warning","value":"The paper was published 2026-07-28 and is very recent; clinical translation remains highly speculative at this stage.","value_numeric":null}],"summaries":{"what_studied":"This study investigated the role of ADAMTS14 in YAP-mediated fibroblast mechanoactivation in IPF, using siRNA screens in primary human lung fibroblasts (HLFs), transcriptomic analysis of IPF patient cohorts, proteomics, and mechanobiology assays to map ADAMTS14's substrate interactions and downstream signaling.","key_result":"An unbiased siRNA screen identified ADAMTS14 as a key regulator of YAP-driven fibroblast activation; knockdown in IPF patient-derived HLFs reduced pro-fibrotic gene expression, blunted TGFβ response, and disrupted FAK-AKT signaling via an ADAMTS14–collagen V–focal adhesion axis.","limitations":"Findings are primarily based on in vitro siRNA knockdown and transcriptomic patient data without a dedicated in vivo animal model for ADAMTS14 loss-of-function; causal directionality in human disease remains unproven. No therapeutic agent targeting ADAMTS14 was tested.","practice_takeaway":"ADAMTS14 is not yet a clinical target, but this work identifies it as a potential node linking ECM remodeling to fibroblast mechanoactivation in IPF — worth monitoring as a future therapeutic or biomarker candidate."}},{"doi":"10.1002/resp.70285","pmid":"42417564","title":"Clinical Outcomes and Acute Exacerbation Incidence in Combined Pulmonary Fibrosis and Emphysema","journal_title":"Respirology","journal_issn":"1323-7799","published_at":"2026-07-08T00:00:00.000Z","url":"https://doi.org/10.1002/resp.70285","source_score":3,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-17T13:24:06.191Z","updated_at":"2026-07-17T13:24:06.191Z","tags":[{"paper_doi":"10.1002/resp.70285","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"condition","value":"Chronic Obstructive Pulmonary Disease","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"condition","value":"Combined Pulmonary Fibrosis and Emphysema","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"condition","value":"Idiopathic Interstitial Pneumonia","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"sample_size","value":"n=1016","value_numeric":1016},{"paper_doi":"10.1002/resp.70285","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"study_design","value":"Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"warning","value":"Antifibrotic therapy use (e.g., nintedanib, pirfenidone) is not reported, which may be a material confounder for survival in the IPF/CPFE subgroup.","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"warning","value":"The cohort is exclusively Japanese, which may limit applicability to non-Asian populations with different CPFE prevalence or smoking patterns.","value_numeric":null},{"paper_doi":"10.1002/resp.70285","tag_type":"warning","value":"Visual CT assessment for emphysema extent may introduce inter-reader variability; quantitative CT thresholds were not used.","value_numeric":null}],"summaries":{"what_studied":"This multicentre prospective cohort study enrolled 1,016 patients with idiopathic interstitial pneumonias (IIPs) or COPD across 29 Japanese hospitals (2013–2016), followed for 5 years to evaluate survival, acute exacerbation incidence, and disease progression in CPFE patients defined by ≥5% emphysema on visual CT assessment.","key_result":"Among 528 IIP patients, 92 (17.4%) had CPFE. CPFE was independently associated with increased mortality (HR 1.85, 95% CI 1.27–2.69; p<0.001), with 5-year survival of 51.4% vs. 63.1% in non-CPFE IIP patients and 84.0% in COPD patients. CPFE patients also had significantly higher acute exacerbation rates; outcomes were worst in the CPFE/IPF subgroup.","limitations":"- Single-country cohort (29 Japanese hospitals) limits generalizability to other ethnicities and healthcare settings.\n- Emphysema extent determined by visual CT assessment, which carries inter-reader variability.\n- No antifibrotic therapy data reported, which could confound survival outcomes in the IPF subgroup.","practice_takeaway":"Screen all IIP patients for CPFE using visual CT assessment with a ≥5% emphysema threshold, as CPFE—especially combined with IPF—carries markedly worse survival and higher acute exacerbation risk. These patients warrant closer surveillance and early consideration of tailored management strategies."}},{"doi":"10.1183/13993003.01557-2025","pmid":"42276742","title":"Progressive pulmonary fibrosis: a state-of-the-art review","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2026-06-11T00:00:00.000Z","url":"https://doi.org/10.1183/13993003.01557-2025","source_score":1,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-23T13:25:18.949Z","updated_at":"2026-06-23T13:25:18.949Z","tags":[{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"brand","value":"Ofev","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"condition","value":"Fibrosing Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"condition","value":"Progressive Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"generic","value":"nerandomilast","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"generic","value":"nintedanib","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"generic","value":"pirfenidone","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"modality","value":"Antifibrotic Agent","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"modality","value":"Phosphodiesterase 4B Inhibitor","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"modality","value":"Tyrosine Kinase Inhibitor","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"warning","value":"Nerandomilast is described as reducing disease progression in a 2025 trial but is not yet guideline-endorsed; regulatory approval status is not specified in the abstract.","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"warning","value":"Sample sizes of the individual trials referenced are not provided in the abstract.","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"warning","value":"Sex of the studied populations is not reported in the abstract, so a sex tag was omitted.","value_numeric":null},{"paper_doi":"10.1183/13993003.01557-2025","tag_type":"warning","value":"This is a narrative review; conclusions about treatment efficacy are drawn from summarized trial data rather than a pooled meta-analysis — individual trial effect sizes should be checked in primary sources.","value_numeric":null}],"summaries":{"what_studied":"This state-of-the-art review evaluates the diagnosis, disease course, and emerging treatment landscape of progressive pulmonary fibrosis (PPF) — a progressive fibrosing phenotype seen in non-IPF interstitial lung diseases — covering key trials through 2025.","key_result":"Nintedanib (a tyrosine kinase inhibitor) halved FVC decline over one year in a 2019 phase III trial and holds a conditional guideline recommendation for PPF (2022). In 2025, the PDE4B inhibitor nerandomilast further reduced disease progression in PPF. Smaller trials also suggest a benefit with pirfenidone.","limitations":"This is a narrative review, not a systematic review or meta-analysis, limiting strength of evidence synthesis. Many supporting trials are described as 'smaller,' and most treatments carry only conditional recommendations. The PPF concept itself is still being validated.","practice_takeaway":"Identify PPF early — worsening symptoms, declining FVC, and radiologic progression despite conventional therapy — and initiate antifibrotic therapy (nintedanib is guideline-supported; nerandomilast is emerging) promptly. Refer eligible patients for lung transplant evaluation as part of holistic care."}},{"doi":"10.1093/ajrccm/aamag249","pmid":"42155010","title":"Gut microbiota associate with disease severity and survival in idiopathic pulmonary fibrosis","journal_title":"American Journal of Respiratory and Critical Care Medicine","journal_issn":"1073-449X","published_at":"2026-05-19T00:00:00.000Z","url":"https://doi.org/10.1093/ajrccm/aamag249","source_score":3,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-12T13:24:32.159Z","updated_at":"2026-06-12T13:24:32.159Z","tags":[{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"brand","value":"Bactrim","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"generic","value":"pirfenidone","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"generic","value":"trimethoprim-sulfamethoxazole","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"modality","value":"Antibiotic","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"modality","value":"Antifibrotic","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"sample_size","value":"n=411","value_numeric":411},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"study_design","value":"Prospective Cohort Study (Secondary Analysis of RCT)","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"warning","value":"Co-trimoxazole is listed under the brand name 'Bactrim' for recognition; the generic used in the paper is 'co-trimoxazole' (trimethoprim-sulfamethoxazole).","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"warning","value":"Study design tag notes: this is a secondary/post-hoc analysis of an RCT cohort, not a primary RCT — level of evidence reflects the observational/exploratory nature of this analysis.","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag249","tag_type":"warning","value":"This is a post-hoc exploratory analysis of the CleanUP-IPF trial; findings are hypothesis-generating and should not be used to guide clinical decisions without further prospective validation.","value_numeric":null}],"summaries":{"what_studied":"This study examined associations between gut microbiota composition (via 16S rRNA and shotgun metagenomic sequencing of fecal swabs) and disease severity, lung transplant-free survival, and treatment heterogeneity in 411 IPF patients enrolled in the CleanUP-IPF trial.","key_result":"Higher abundance of *Lachnospiraceae* unclassified genus was linked to improved transplant-free survival in patients not on anti-microbials (HR 0.34, 95% CI 0.14–0.87, P=.02), but to worse survival in those receiving co-trimoxazole (HR 6.09, 95% CI 1.36–27.27, P=.02). Gut microbial diversity and composition also correlated significantly with impaired gas exchange (ppDLCO).","limitations":"- Exploratory post-hoc analysis limits causal inference and increases risk of false-positive findings.\n- Fecal swabs (vs. fresh stool) may yield lower-quality microbiome data.\n- Confounders such as PPI use, sex, and age influenced microbiota composition and may not be fully adjusted.","practice_takeaway":"Gut microbiota — particularly *Lachnospiraceae* abundance — may help predict survival and modulate response to co-trimoxazole or pirfenidone in IPF patients. These findings are hypothesis-generating; routine microbiome testing in IPF is not yet supported by this evidence alone."}},{"doi":"10.1056/nejmoa2501488","pmid":"42149993","title":"Phase 3 Trials of Inhaled Treprostinil for Idiopathic Pulmonary Fibrosis","journal_title":"New England Journal of Medicine","journal_issn":"0028-4793","published_at":"2026-05-18T00:00:00.000Z","url":"https://doi.org/10.1056/nejmoa2501488","source_score":128,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-15T13:26:15.310Z","updated_at":"2026-07-15T13:26:15.310Z","tags":[{"paper_doi":"10.1056/nejmoa2501488","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"brand","value":"Tyvaso","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"category","value":"Medicine, General & Internal","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"generic","value":"treprostinil","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"level_of_evidence","value":"1","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"modality","value":"Prostacyclin Analogue","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"sample_size","value":"n=598","value_numeric":598},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"study_design","value":"Randomized Controlled Trial","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"warning","value":"The brand name 'Tyvaso' is associated with treprostinil inhalation solution; confirm whether TETON-1 used the same inhaled formulation (Tyvaso DPI or liquid nebulizer) as labeled commercially.","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"warning","value":"The high dropout rate (~40% in the treatment arm) and the cough side effect asymmetry raise a risk of functional unblinding, which is not explicitly addressed in the abstract.","value_numeric":null},{"paper_doi":"10.1056/nejmoa2501488","tag_type":"warning","value":"The trial was funded by United Therapeutics (the manufacturer of treprostinil), which is a potential source of industry bias.","value_numeric":null}],"summaries":{"what_studied":"Phase 3 double-blind RCT (TETON-1) comparing inhaled treprostinil (12 breaths four times daily) vs. placebo in 598 patients with IPF over 52 weeks; 77.6% were on background antifibrotic therapy. Results are also reported combined with the previously published TETON-2 trial.","key_result":"Treprostinil significantly reduced FVC decline vs. placebo (median change: -43.3 ml vs. -196.2 ml; difference +130.1 ml; 95% CI 82.2–178.1; P<0.001). Clinical worsening (death, respiratory hospitalization, or ≥10% relative FVC decline) occurred in 31.8% vs. 44.5% (HR 0.67; 95% CI 0.52–0.88; P=0.003). No significant difference was seen in time to acute IPF exacerbation.","limitations":"- High discontinuation rate (40.5% treprostinil vs. 32.8% placebo), with adverse events as the primary driver, may bias efficacy estimates.\n- Cough occurred in 54.8% of the treprostinil group vs. 33.1% placebo, raising concerns about unblinding.\n- No significant benefit on acute IPF exacerbation, limiting the scope of secondary endpoint conclusions.","practice_takeaway":"Inhaled treprostinil added to background antifibrotic therapy meaningfully slows lung function decline and reduces clinical worsening events in IPF over 1 year. Clinicians should counsel patients about a high rate of cough (~55%) and a 1-in-5 chance of stopping the drug due to side effects."}},{"doi":"10.1183/13993003.00349-2026","pmid":"42097674","title":"Reading the monocyte code in idiopathic pulmonary fibrosis","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2026-05-08T00:00:00.000Z","url":"https://doi.org/10.1183/13993003.00349-2026","source_score":3,"is_retracted":false,"is_open_access":false,"raw_metadata":{"ooir":{"day":"2026-05-08","doi":"10.1183/13993003.00349-2026","days":"7","issn":"0903-1936","rank":"9","field":"Clinical Medicine","score":"3","category":"Respiratory System","categories":["Respiratory System"],"_categories":["Respiratory System"]},"pubmed":{"doi":"10.1183/13993003.00349-2026","title":"Reading the monocyte code in idiopathic pulmonary fibrosis.","journal":"The European respiratory journal","methods":"","results":"","abstract":"","keywords":[],"pubmed_id":"42097674","conclusions":"","publication_date":"2026-05-08T00:00:00"},"crossref":{"DOI":"10.1183/13993003.00349-2026","URL":"https://doi.org/10.1183/13993003.00349-2026","ISSN":["0903-1936","1399-3003"],"link":[{"URL":"https://syndication.highwire.org/content/doi/10.1183/13993003.00349-2026","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":"2600349","type":"journal-article","issue":"5","score":1,"title":["Reading the monocyte code in idiopathic pulmonary fibrosis"],"author":[{"given":"Anna V.","family":"Curioni","sequence":"first","affiliation":[]},{"given":"Bruno","family":"Crestani","sequence":"additional","affiliation":[]},{"given":"Doumet Georges","family":"Helou","sequence":"additional","affiliation":[]}],"issued":{"date-parts":[[2026,5]]},"member":"81","prefix":"10.1183","source":"Crossref","volume":"67","created":{"date-time":"2026-05-07T10:10:19Z","timestamp":1778148619000,"date-parts":[[2026,5,7]]},"indexed":{"date-time":"2026-05-07T11:09:58Z","timestamp":1778152198822,"date-parts":[[2026,5,7]]},"license":[{"URL":"https://publications.ersnet.org/user-licence","start":{"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000,"date-parts":[[2026,5,1]]},"delay-in-days":0,"content-version":"vor"}],"subject":[],"abstract":null,"accepted":{"date-parts":[[2026,3,2]]},"language":"en","relation":{},"resource":{"primary":{"URL":"https://publications.ersnet.org/lookup/doi/10.1183/13993003.00349-2026"}},"subtitle":[],"deposited":{"date-time":"2026-05-07T10:10:25Z","timestamp":1778148625000,"date-parts":[[2026,5,7]]},"issn-type":[{"type":"print","value":"0903-1936"},{"type":"electronic","value":"1399-3003"}],"published":{"date-parts":[[2026,5]]},"publisher":"European Respiratory Society (ERS)","reference":[{"DOI":"10.1038/s41577-026-01268-4","key":"2026050703100988000_67.5.2600349.1","year":null,"issue":null,"author":null,"volume":null,"first-page":null,"unstructured":"Bell PT , Belz GT . 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Fibrosis","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"generic","value":"volasertib","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"modality","value":"PLK1 Inhibitor","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"study_design","value":"Preclinical Study","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"warning","value":"Abstract does not report sample size, species, or effect sizes.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"warning","value":"In vivo results are from a TGFα-induced pulmonary fibrosis model, not patients.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00204.2025","tag_type":"warning","value":"Preclinical design; not classifiable under standard clinical study types.","value_numeric":null}],"summaries":{"what_studied":"A preclinical study mapped the WT1–MYCN–PLK1 pathway driving fibroblast activation in idiopathic pulmonary fibrosis and tested PLK1 inhibition with volasertib. Experiments included in vitro fibroblast assays and an in vivo TGFα‑induced pulmonary fibrosis model, assessing proliferation, survival, ECM production, and collagen deposition.","key_result":"Volasertib reduced expression of MYCN, WT1, and PLK1, mitigated fibroblast activation in vitro, and attenuated fibroblast activation and collagen deposition in a TGFα‑induced pulmonary fibrosis model in vivo. MYCN knockdown also decreased fibroblast proliferation, survival, and ECM production.","limitations":"Findings are limited to in vitro systems and an in vivo TGFα‑induced fibrosis model rather than human clinical studies. The abstract reports no sample sizes, species, effect sizes, or duration of treatment.","practice_takeaway":"PLK1 inhibition with volasertib shows antifibrotic activity in preclinical IPF models, highlighting the WT1–MYCN–PLK1 axis as a druggable target. This does not change clinical care; clinical trials are needed to assess safety and efficacy in patients."}},{"doi":"10.1183/13993003.00804-2025","pmid":"41360505","title":"The transcriptome of CD14\n ⁺\n CD163\n ^-\n HLA-DR\n ^low\n monocytes predicts mortality in Idiopathic Pulmonary Fibrosis","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2025-12-09T00:00:00.000Z","url":"https://doi.org/10.1183/13993003.00804-2025","source_score":18,"is_retracted":false,"is_open_access":false,"raw_metadata":{"ooir":{"day":"2025-12-11","doi":"10.1183/13993003.00804-2025","days":"30","issn":"0903-1936","rank":"6","field":"Clinical Medicine","score":"18","category":"Respiratory System","categories":["Respiratory System"],"_categories":["Respiratory System"]},"pubmed":{"doi":"10.1183/13993003.00804-2025","title":"The transcriptome of CD14","journal":"The European respiratory journal","methods":"","results":"A 230-gene-up-score (Pittsburgh-PBMC) from CD14","abstract":"The association between immune-cell-specific transcriptomic profiles and mortality in IPF is unknown.\nWe profiled peripheral blood mononuclear cells (PBMC) by single-cell RNA sequencing (scRNA-seq) and investigated which immune-cell-specific transcriptomic profile predicted IPF outcomes consistently. Prognostic accuracy was investigated in PBMC, Bronchoalveolar Lavage (BAL) and lung tissue. Findings were validated by flow cytometry, analysis of independent scRNA-seq datasets and cellular deconvolution. We investigated the function of this transcriptomic profile and its cellular source in lung tissue (overall sample size:1054, IPF:555, other:499). Connectivity map and LASSO regression were used to identify drug candidates and a subset of genes with prognostic potential, respectively.\nA 230-gene-up-score (Pittsburgh-PBMC) from CD14\nThe transcriptome of CD14","keywords":[],"pubmed_id":"41360505","conclusions":"","publication_date":"2025-12-09T00:00:00"},"crossref":{"DOI":"10.1183/13993003.00804-2025","URL":"https://doi.org/10.1183/13993003.00804-2025","ISSN":["0903-1936","1399-3003"],"link":[{"URL":"https://syndication.highwire.org/content/doi/10.1183/13993003.00804-2025","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":"2500804","type":"journal-article","issue":null,"score":1,"title":["The transcriptome of CD14\n ⁺\n CD163\n ^-\n HLA-DR\n ^low\n monocytes predicts mortality in Idiopathic Pulmonary Fibrosis"],"author":[{"given":"Theodoros","family":"Karampitsakos","sequence":"first","affiliation":[]},{"given":"Minxue","family":"Jia","sequence":"additional","affiliation":[]},{"given":"Bochra","family":"Tourki","sequence":"additional","affiliation":[]},{"given":"Zainab","family":"Fatima","sequence":"additional","affiliation":[]},{"given":"Bogdan","family":"Visinescu","sequence":"additional","affiliation":[]},{"given":"Carole Y.","family":"Perrot","sequence":"additional","affiliation":[]},{"given":"Tamer","family":"Fadli","sequence":"additional","affiliation":[]},{"given":"Amy","family":"Zhao","sequence":"additional","affiliation":[]},{"given":"Avraham","family":"Unterman","sequence":"additional","affiliation":[]},{"given":"Marc A.","family":"Schneider","sequence":"additional","affiliation":[]},{"given":"Debabrata","family":"Bandyopadhyay","sequence":"additional","affiliation":[]},{"given":"Brenda M.","family":"Juan-Guardela","sequence":"additional","affiliation":[]},{"given":"Ioannis","family":"Vamvakaris","sequence":"additional","affiliation":[]},{"given":"Antje","family":"Prasse","sequence":"additional","affiliation":[]},{"given":"Imre","family":"Noth","sequence":"additional","affiliation":[]},{"given":"Stephen","family":"Liggett","sequence":"additional","affiliation":[]},{"given":"Michael","family":"Kreuter","sequence":"additional","affiliation":[]},{"given":"Wonder","family":"Drake","sequence":"additional","affiliation":[]},{"given":"Argyris","family":"Tzouvelekis","sequence":"additional","affiliation":[]},{"given":"Joe G. N.","family":"Garcia","sequence":"additional","affiliation":[]},{"given":"Erica","family":"Herzog","sequence":"additional","affiliation":[]},{"given":"Naftali","family":"Kaminski","sequence":"additional","affiliation":[]},{"given":"Panayiotis V.","family":"Benos","sequence":"additional","affiliation":[]},{"given":"Jose D.","family":"Herazo-Maya","sequence":"additional","affiliation":[]}],"funder":[{"name":"USF Foundation","award":["Ubben Family Fund"],"award-info":[{"award-number":["Ubben Family Fund"]}]},{"id":[{"id":"10.13039/100000002","id-type":"DOI","asserted-by":"publisher"}],"DOI":"10.13039/100000002","name":"National Institutes of Health","award":["R21HL161723, R01HL127349, R01HL141852, U01HL145567"],"award-info":[{"award-number":["R21HL161723, R01HL127349, R01HL141852, U01HL145567"]}],"doi-asserted-by":"publisher"}],"issued":{"date-parts":[[2025,12,5]]},"member":"81","prefix":"10.1183","source":"Crossref","volume":null,"created":{"date-time":"2025-12-08T05:24:52Z","timestamp":1765171492000,"date-parts":[[2025,12,8]]},"indexed":{"date-time":"2025-12-11T18:38:56Z","timestamp":1765478336002,"date-parts":[[2025,12,11]]},"license":[{"URL":"http://creativecommons.org/licenses/by-nc/4.0/","start":{"date-time":"2025-12-05T00:00:00Z","timestamp":1764892800000,"date-parts":[[2025,12,5]]},"delay-in-days":0,"content-version":"am"}],"subject":[],"abstract":"$32","accepted":{"date-parts":[[2025,11,7]]},"language":"en","relation":{},"resource":{"primary":{"URL":"https://publications.ersnet.org/lookup/doi/10.1183/13993003.00804-2025"}},"subtitle":[],"deposited":{"date-time":"2025-12-11T18:35:24Z","timestamp":1765478124000,"date-parts":[[2025,12,11]]},"issn-type":[{"type":"print","value":"0903-1936"},{"type":"electronic","value":"1399-3003"}],"published":{"date-parts":[[2025,12,5]]},"publisher":"European Respiratory Society (ERS)","reference":null,"short-title":[],"update-policy":"https://doi.org/10.1183/ers-crossmark-policy","alternative-id":["10.1183/13993003.00804-2025"],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"original-title":[],"container-title":["European Respiratory Journal"],"reference-count":0,"published-online":{"date-time":null,"timestamp":null,"date-parts":[[2025,12,5]]},"references-count":0,"short-container-title":["Eur Respir J"],"is-referenced-by-count":0},"unpaywall":{"doi":"10.1183/13993003.00804-2025","is_oa":false,"title":"The transcriptome of CD14\n ⁺\n CD163\n ^-\n HLA-DR\n ^low\n monocytes predicts mortality in Idiopathic Pulmonary Fibrosis","updated":"2025-12-13T00:32:06+00:00","published_date":"2025-12-05","best_oa_location":null}},"created_at":"2026-01-11T11:19:33.869Z","updated_at":"2026-01-11T11:19:33.869Z","tags":[{"paper_doi":"10.1183/13993003.00804-2025","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/13993003.00804-2025","tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1183/13993003.00804-2025","tag_type":"sample_size","value":"n=1054","value_numeric":1054},{"paper_doi":"10.1183/13993003.00804-2025","tag_type":"study_design","value":"Cohort Study","value_numeric":null},{"paper_doi":"10.1183/13993003.00804-2025","tag_type":"warning","value":"Abstract/results text appears truncated; no effect sizes, hazard ratios, or accuracy metrics are provided.","value_numeric":null},{"paper_doi":"10.1183/13993003.00804-2025","tag_type":"warning","value":"Study design classification is inferred from limited abstract details and may not reflect the authors’ terminology.","value_numeric":null}],"summaries":{"what_studied":"PBMC were profiled by scRNA-seq to identify immune-cell–specific transcriptomic profiles that consistently predict outcomes in Idiopathic Pulmonary Fibrosis; prognostic accuracy was examined in PBMC, BAL, and lung tissue and validated by flow cytometry, independent scRNA-seq datasets, and cellular deconvolution. Overall sample size was 1054 (IPF 555; other 499).","key_result":"A 230-gene up-score (Pittsburgh-PBMC) derived from CD14+CD163- HLA-DRlow monocytes predicted mortality in IPF."}}],"initialOffset":16,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"condition","value":"Idiopathic Pulmonary Fibrosis"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Conditions","href":"/hub/tag/condition"},{"name":"Idiopathic Pulmonary Fibrosis"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"condition","hub_tag_value":"Idiopathic Pulmonary Fibrosis"}}}]]

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