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:T6e6,In fibrotic lung disease, radiological features of usual interstitial pneumonia (UIP) are associated with adverse outcomes. The aim of this study was to evaluate the likelihood of definite UIP on HRCT as a determinant of outcome in patients with pulmonary fibrosis. All available HRCT from the Australian IPF Registry (AIPFR) were retrospectively reviewed by two radiologists. The radiologists were asked to assign a likelihood (%) within each of the four ATS/ERS/JRS/ALAT 2018 UIP categories, summating to 100%, enabling them to not be constrained by a single UIP category (e.g., 5% definite UIP, 95% alternative to UIP). Likelihood of UIP was defined as the percentage likelihood score for the definite UIP category. Semi-quantitative features were also estimated. Cox proportional hazards regression was performed to evaluate the prognostic utility of the likelihood scores. HRCT was available for analysis in 515 patients. Median follow-up of the cohort was 4.1 years (IQR 2.0-6.2 years). Median total ILD extent was 30% (IQR20-45%), with traction bronchiectasis and honeycombing present in 96.3% and 45.4% of cases respectively, and all three variables were associated with worse transplant-free survival. A pattern of definite or probable UIP was favoured in 26.4% and 32.4% of cases respectively. Likelihood of definite UIP was independently associated with both reduced transplant-free survival (HR1.12, 95% CI 1.04-1.20; p = 0.002) and 12-month progression (HR1.19, 95% CI 1.04-1.35; p = 0.009) and was superior to the ATS/ERS/JRS/ALAT 2018 guideline-based categories for these associations. We demonstrate that estimation of the likelihood of definite UIP is strongly associated with transplant-free survival and 12-month disease progression.31:Taa4,ABSTRACT Background and Objectives In fibrotic lung disease, radiological features of usual interstitial pneumonia (UIP) are associated with adverse outcomes. The aim of this study was to evaluate the likelihood of definite UIP on HRCT as a determinant of outcome in patients with pulmonary fibrosis . Methods All available HRCT from the Australian IPF Registry (AIPFR) were retrospectively reviewed by two radiologists. The radiologists were asked to assign a likelihood (%) within each of the four ATS/ERS/JRS/ALAT 2018 UIP categories, summating to 100%, enabling them to not be constrained by a single UIP category (e.g., 5% definite UIP, 95% alternative to UIP). Likelihood of UIP was defined as the percentage likelihood score for the definite UIP category. Semi‐quantitative features were also estimated. Cox proportional hazards regression was performed to evaluate the prognostic utility of the likelihood scores. Results HRCT was available for analysis in 515 patients. Median follow‐up of the cohort was 4.1 years (IQR 2.0–6.2 years). Median total ILD extent was 30% (IQR20‐45%), with traction bronchiectasis and honeycombing present in 96.3% and 45.4% of cases respectively, and all three variables were associated with worse transplant‐free survival. A pattern of definite or probable UIP was favoured in 26.4% and 32.4% of cases respectively. Likelihood of definite UIP was independently associated with both reduced transplant‐free survival (HR1.12, 95% CI 1.04–1.20; p = 0.002) and 12‐month progression (HR1.19, 95% CI 1.04–1.35; p = 0.009) and was superior to the ATS/ERS/JRS/ALAT 2018 guideline‐based categories for these associations. Conclusions We demonstrate that estimation of the likelihood of definite UIP is strongly associated with transplant‐free survival and 12‐month disease progression. 32:T6e9,Pulmonary fibrosis (PF) is a severe extra-articular manifestation of rheumatoid arthritis (RA). The study aimed to externally validate a genetic risk score (GRS) and a combined risk score for predicting the risk of RA-associated PF in an independent cohort of early-RA patients. This study utilized an inception cohort of 1118 patients diagnosed with RA from northern Sweden between 1996 and 2016. Clinical data were systematically collected, and genotyping was performed for 12 single-nucleotide polymorphisms (SNPs) associated with idiopathic pulmonary fibrosis. Statistical analyses, including logistic regression and area under the curve (AUC) assessments, were conducted to evaluate the performance of the GRS and in combination with clinical data as combined risk score in predicting RA-PF development. Of the 1115 patients with complete data, 60 (5.6%) were diagnosed with PF. PF was significantly associated with age, rheumatoid factor positivity, disease activity, and MUC5B (rs35705950) and FAM13A(rs2609255) SNPs. The GRS demonstrated a significant association with RA-PF (odds ratio 2.6, (95%CI 1.6, 4.5), while the combined risk score exhibited superior performance (AUC 0.75, p<0.001) compared to the GRS alone (AUC 0.62). The combined risk score outperformed the GRS in discriminating RA-PF, indicating its potential utility in clinical practice. This study provides external validation of the VARA-ILD-GRS and VARA-ILD combined risk score in an RA cohort, demonstrating their generalizability and effectiveness in identifying high-risk individuals for RA-ILD. The findings support the integration of genetic and clinical data in risk stratification models, which could significantly improve screening strategies for RA patients at risk of developing PF.33:Tbeb, Objectives Pulmonary fibrosis (PF) is a severe extra‐articular manifestation of rheumatoid arthritis (RA). The study aimed to externally validate a genetic risk score (GRS) and a combined risk score for predicting the risk of RA‐associated PF in an independent cohort of early‐RA patients. Methods This study utilized an inception cohort of 1118 patients diagnosed with RA from northern Sweden between 1996 and 2016. Clinical data were systematically collected, and genotyping was performed for 12 single‐nucleotide polymorphisms (SNPs) associated with idiopathic pulmonary fibrosis. Statistical analyses, including logistic regression and area under the curve (AUC) assessments, were conducted to evaluate the performance of the GRS and in combination with clinical data as combined risk score in predicting RA‐PF development. Results Of the 1115 patients with complete data, 60 (5.6%) were diagnosed with PF. PF was significantly associated with age, rheumatoid factor positivity, disease activity, and MUC5B (rs35705950) and FAM13A (rs2609255) SNPs. The GRS demonstrated a significant association with RA‐PF (odds ratio 2.6, (95%CI 1.6, 4.5), while the combined risk score exhibited superior performance (AUC 0.75, p<0.001) compared to the GRS alone (AUC 0.62). The combined risk score outperformed the GRS in discriminating RA‐PF, indicating its potential utility in clinical practice. Conclusion This study provides external validation of the VARA‐ILD‐GRS and VARA‐ILD combined risk score in an RA cohort, demonstrating their generalizability and effectiveness in identifying high‐risk individuals for RA‐ILD. The findings support the integration of genetic and clinical data in risk stratification models, which could significantly improve screening strategies for RA patients at risk of developing PF.

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25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"Pulmonary Fibrosis: condition Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on Pulmonary Fibrosis (condition). Clinician-written summaries and actionable practice insights. Updated daily.\",\"url\":\"https://www.reachrx.ai/hub/tag/condition/Pulmonary%20Fibrosis\",\"breadcrumb\":{\"@type\":\"BreadcrumbList\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https://www.reachrx.ai\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Research Hub\",\"item\":\"https://www.reachrx.ai/hub\"},{\"@type\":\"ListItem\",\"position\":3,\"name\":\"Conditions\",\"item\":\"https://www.reachrx.ai/hub/tag/condition\"},{\"@type\":\"ListItem\",\"position\":4,\"name\":\"Pulmonary Fibrosis\",\"item\":\"https://www.reachrx.ai/hub/tag/condition/Pulmonary%20Fibrosis\"}]}}"}}]],["$","$L2f",null,{"title":"Pulmonary Fibrosis: Research & Clinical Takeaways","description":"Discover peer-reviewed research on Pulmonary Fibrosis with concise clinician summaries and next-step guidance.","papers":[{"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":"10.1136/rmdopen-2026-006943","pmid":"42508943","title":"Characteristics and outcomes of anti-Ku antibody–associated interstitial lung disease: a retrospective study of 60 patients","journal_title":"RMD Open","journal_issn":"2056-5933","published_at":"2026-07-27T00:00:00.000Z","url":"https://doi.org/10.1136/rmdopen-2026-006943","source_score":7,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-04T18:18:05.680Z","updated_at":"2026-08-04T18:18:05.680Z","tags":[{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"condition","value":"Idiopathic Inflammatory Myopathy","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"condition","value":"Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"condition","value":"Sjögren's Disease","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"condition","value":"Systemic Lupus Erythematosus","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"condition","value":"Systemic Sclerosis","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"azathioprine","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"cyclophosphamide","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"methotrexate","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"mycophenolate mofetil","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"nintedanib","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"pirfenidone","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"generic","value":"rituximab","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"modality","value":"Antifibrotic Agent","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"modality","value":"Immunosuppressive Therapy","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"sample_size","value":"n=154","value_numeric":154},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"study_design","value":"Retrospective Cohort Study","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"warning","value":"The ILD progression rate of 75% likely overestimates what would be found under strict 12-month ATS/ERS/JRS/ALAT criteria (only 6 patients met that stricter definition); direct comparison with other ILD cohorts using the standard definition should be made cautiously.","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"warning","value":"The parent cohort included a paediatric department (Hôpital Necker), but the reported median age of 56 years and ILD cohort demographics suggest predominant adult inclusion; age-group tagging reflects this, though a small number of younger patients may be included.","value_numeric":null},{"paper_doi":"10.1136/rmdopen-2026-006943","tag_type":"warning","value":"The study predates routine use of antifibrotic agents—only 3 patients received them—so conclusions about antifibrotic efficacy in this population cannot be drawn.","value_numeric":null}],"summaries":{"what_studied":"This retrospective, multicentre cohort study across 10 French university hospitals (2010–2025) described the characteristics and outcomes of ILD in 60 anti-Ku antibody–positive patients with systemic autoimmune diseases (from a parent cohort of 154), with a median follow-up of 6 years.","key_result":"ILD occurred in 39% (60/154) of anti-Ku–positive patients; 75% (45/60) progressed over a median of 4 years. Male sex was independently associated with faster progression (aHR 2.7; 95% CI 1.4–5.2). Baseline pulmonary fibrosis (present in 57%) independently predicted death (aHR 6.5; 95% CI 1.5–28.2). Inaugural ILD also worsened overall survival (aHR 3.1; 95% CI 1.3–7.3). The most common CT pattern was fibrotic NSIP (27%).","limitations":"- Retrospective design with non-uniform follow-up intervals; progression criteria were applied over the entire observation period rather than within the standard 12-month window, likely inflating the progressor rate compared to studies using strict ATS/ERS/JRS/ALAT criteria.\n- Not all anti-Ku–positive patients (17%) underwent chest CT, risking underestimation of true ILD prevalence.\n- Treatment heterogeneity and lack of longitudinal treatment-timing data precluded reliable adjustment for therapy as a time-dependent covariate.","practice_takeaway":"Screen every anti-Ku–positive patient for ILD at diagnosis and monitor regularly with PFTs and chest CT, as ILD is present in ~40% and progresses in ~75%. Male sex and baseline pulmonary fibrosis flag higher-risk patients who may benefit from earlier consideration of antifibrotic therapy."}},{"doi":"10.1016/j.jid.2026.06.1269","pmid":"42470402","title":"Updates on endothelial injury in dermatomyositis: Clinical correlations and potential mechanisms","journal_title":"Journal of Investigative Dermatology","journal_issn":"0022-202X","published_at":"2026-07-17T00:00:00.000Z","url":"https://doi.org/10.1016/j.jid.2026.06.1269","source_score":1,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-25T13:57:50.405Z","updated_at":"2026-07-25T13:57:50.405Z","tags":[{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"category","value":"Dermatology","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"condition","value":"Cardiovascular Disease","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"condition","value":"Dermatomyositis","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"warning","value":"Age group tagged as 'mixed' as the review does not specify a single studied population age range.","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"warning","value":"No original patient data or sample size reported — this is a narrative review; level of evidence is estimated at 5.","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"warning","value":"No specific therapeutic agents or modalities are discussed in the abstract; drug/modality tags could not be assigned.","value_numeric":null},{"paper_doi":"10.1016/j.jid.2026.06.1269","tag_type":"warning","value":"The paper is dated July 17, 2026, which is 8 days before today's date; it may be a recent ahead-of-print publication and details may be subject to change upon final publication.","value_numeric":null}],"summaries":{"what_studied":"This review evaluates the role of vascular endothelial cell (EC) injury in dermatomyositis (DM), covering its clinical correlations (edema, ulcers, capillary disturbances, muscle ischemia, cardiovascular risk) and proposed mechanisms linking EC damage to skin, muscle, and pulmonary involvement.","key_result":"EC injury appears central to vascular damage in DM; clinical features tied to vascular damage (edema, ulcers, severe microcirculatory disturbances) correlate with more severe disease, and cardiovascular disease risk in DM patients is significantly elevated compared to healthy individuals.","limitations":"Review article without original patient data — no sample size, effect sizes, or direct clinical trial results are reported. Mechanistic evidence is largely experimental, and many proposed pathways require further validation in humans.","practice_takeaway":"Clinicians should recognize vascular features like edema, ulcers, and capillary abnormalities in DM as markers of more severe disease. Monitoring cardiovascular risk in DM patients is warranted, and EC-related biomarkers may emerge as useful disease activity indicators."}},{"doi":"10.1152/ajplung.00008.2026","pmid":"42284110","title":"Macrophage in the crosshairs: chemical inhalation and lung immune defense","journal_title":"American Journal of Physiology-Lung Cellular and Molecular Physiology","journal_issn":"1040-0605","published_at":"2026-06-12T00:00:00.000Z","url":"https://doi.org/10.1152/ajplung.00008.2026","source_score":3,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-22T13:37:49.891Z","updated_at":"2026-07-22T13:37:49.891Z","tags":[{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"condition","value":"Acute Inhalation Injury","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"condition","value":"Chemical-Induced Lung Injury","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"condition","value":"Sterile Lung Inflammation","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"modality","value":"Macrophage-Targeted Therapy","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"warning","value":"No original patient or experimental data are presented; all mechanistic conclusions are synthesized from prior literature across heterogeneous animal models.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"warning","value":"Sex of subjects in cited studies is not reported in the abstract, so the 'sex' tag was omitted.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00008.2026","tag_type":"warning","value":"The 'mixed' age group tag reflects the likely mix of model systems (animal and human data) referenced across cited studies, not a specific study population.","value_numeric":null}],"summaries":{"what_studied":"This review examines the role of pulmonary macrophages — including alveolar and interstitial subtypes — in lung injury and repair following acute inhalation of toxic chemicals (vesicants, particulates, volatile organic compounds, microbial toxins, and toxic gases), with a focus on macrophage ontogeny, functional specialization, macrophage-epithelial cross talk, and emerging therapeutic targets.","key_result":"Early depletion of resident alveolar macrophages and disproportionate recruitment of monocyte-derived macrophages is identified as a conserved pathogenic feature of severe chemical-induced lung injury across multiple experimental models; macrophage transcriptional, metabolic, and functional reprogramming drives the trajectory toward either resolution or fibrosis.","limitations":"As a narrative review, no original experimental data are reported; conclusions rely on heterogeneous animal models, and translation to human chemical inhalation injury remains unvalidated.","practice_takeaway":"Clinicians managing acute toxic inhalation injury should be aware that macrophage dysregulation — not just direct epithelial damage — may drive progression to fibrosis, making macrophage-targeted strategies (e.g., restoring resident alveolar macrophage populations) an active area of therapeutic development."}},{"doi":"10.1002/art.70228","pmid":"42206826","title":"The Nuclear Receptor Testicular Receptor 4 Orchestrates Cytoskeletal Organization in a G Protein Subunit Alpha 12/Rho‐Associated Protein Kinase–Dependent Manner to Promote Myofibroblast Differentiation and Tissue Fibrosis in Systemic Sclerosis","journal_title":"Arthritis & Rheumatology","journal_issn":"2326-5191","published_at":"2026-06-12T00:00:00.000Z","url":"https://doi.org/10.1002/art.70228","source_score":3,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-09T13:35:49.090Z","updated_at":"2026-07-09T13:35:49.090Z","tags":[{"paper_doi":"10.1002/art.70228","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"condition","value":"Dermal Fibrosis","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"condition","value":"Systemic Sclerosis","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"generic","value":"bleomycin","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"modality","value":"Nuclear Receptor Inhibition","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"modality","value":"ROCK Inhibition","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"study_design","value":"Translational/Preclinical Study","value_numeric":null},{"paper_doi":"10.1002/art.70228","tag_type":"warning","value":"No clinical trial or patient-level outcome data are reported; the 'practice_changing' impact flag reflects the novelty of the TR4 target mechanism rather than a change in current clinical practice. The study design is best classified as translational/preclinical, combining in vitro, murine knockout, and ex vivo human tissue models without a randomized clinical component. Sample size for human SSc precision-cut skin slice experiments is not specified in the abstract.","value_numeric":null}],"summaries":{"what_studied":"This study examined the role of the nuclear receptor TR4 (Nr2c2) in fibroblast activation and fibrosis in systemic sclerosis (SSc), using human SSc skin, precision-cut skin slices, and multiple murine fibrosis models (bleomycin, constitutively active TGFβRI, and chronic graft-versus-host disease) with fibroblast-specific Tr4 knockout.","key_result":"TR4 was upregulated in SSc fibroblasts via TGFβ/SMAD3 signaling; fibroblast-specific Tr4 knockout ameliorated dermal and pulmonary fibrosis in all murine models tested. Mechanistically, TR4 drove myofibroblast differentiation through Gα12/ROCK-dependent cytoskeletal remodeling, and TR4 knockdown in SSc precision-cut skin slices reduced ROCK activity and profibrotic gene expression.","limitations":"All intervention data are preclinical (murine knockout models and ex vivo human tissue slices); no clinical trial data are available. Study does not characterize potential off-target effects of TR4 inhibition in non-fibroblast cell types. The precise transcriptional targets through which TR4 activates Gα12 remain incompletely defined.","practice_takeaway":"TR4 is not yet a clinically actionable target, but preclinical evidence positions it as a promising therapeutic candidate in SSc-related fibrosis. Clinicians should watch for TR4/ROCK pathway inhibitors entering early-phase trials in SSc and related fibrosing diseases."}},{"doi":"10.1016/j.ajt.2026.05.2303","pmid":"42252026","title":"Myeloid-avid mTOR-inhibiting nanobiologic attenuates allograft fibrosis after lung transplantation","journal_title":"American Journal of Transplantation","journal_issn":"1600-6135","published_at":"2026-06-06T00:00:00.000Z","url":"https://doi.org/10.1016/j.ajt.2026.05.2303","source_score":16,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-10T14:24:10.183Z","updated_at":"2026-06-10T14:24:10.183Z","tags":[{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"category","value":"Surgery","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"condition","value":"Chronic Lung Allograft Dysfunction","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"condition","value":"Lung Transplant Rejection","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"generic","value":"mTOR-inhibiting nanobiologic","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"level_of_evidence","value":"6","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"modality","value":"mTOR Inhibitor","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"modality","value":"Nanobiologic","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"study_design","value":"Preclinical Animal Study","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"warning","value":"Sample size not reported in the abstract; no N provided for tagging.","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"warning","value":"Sex of animals not reported in the abstract.","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"warning","value":"Study is entirely preclinical (mouse model); clinical applicability is speculative at this stage.","value_numeric":null},{"paper_doi":"10.1016/j.ajt.2026.05.2303","tag_type":"warning","value":"The mTORi-NB is described as a novel investigational agent with no established brand or generic drug name; tagged generically.","value_numeric":null}],"summaries":{"what_studied":"A myeloid-avid mTOR-inhibiting nanobiologic (mTORi-NB) was evaluated in a semi-allogeneic mouse lung transplant model of chronic lung allograft dysfunction (CLAD), focusing on its effects on macrophage infiltration, fibrotic gene expression, and allograft fibrosis after brief perioperative therapy.","key_result":"Perioperative mTORi-NB preferentially targeted myeloid and endothelial cells in allografts, reduced early macrophage infiltration, downregulated pro-fibrotic genes in macrophages and alveolar epithelial cells (AT1/AT2), upregulated anti-fibrotic genes in macrophages, and reduced allograft fibrosis with preservation of club and AT2 cells.","limitations":"Preclinical mouse model only (semi-allogeneic); no human or large-animal data; no direct comparison to current standard immunosuppression regimens.","practice_takeaway":"This is early-stage preclinical work; no immediate clinical changes are warranted. However, myeloid-targeted nanobiologics represent a novel mechanistic approach to CLAD prevention that warrants further translational study."}},{"doi":"10.1002/art.70238","pmid":"42223224","title":"Human Fibroblast‐Myeloid cell tissue atlas across lung, synovium, skin and heart","journal_title":"Arthritis & Rheumatology","journal_issn":"2326-5191","published_at":"2026-06-01T00:00:00.000Z","url":"https://doi.org/10.1002/art.70238","source_score":4,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-10T14:23:53.511Z","updated_at":"2026-06-10T14:23:53.511Z","tags":[{"paper_doi":"10.1002/art.70238","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"condition","value":"Inflammatory Heart Disease","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"condition","value":"Inflammatory Skin Disease","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"condition","value":"Rheumatoid Arthritis","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"modality","value":"Immunofluorescence","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"modality","value":"Single-Cell RNA Sequencing","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"sample_size","value":"n=253843","value_numeric":253843},{"paper_doi":"10.1002/art.70238","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"study_design","value":"Cross-Sectional Transcriptomic Atlas (Secondary Analysis of Public scRNA-seq Datasets)","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"warning","value":"Impact flag 'practice_changing' is applied cautiously — findings are discovery-level and not yet validated in clinical trials, but the cross-tissue pathogenic subset identification (SPP1+ macrophages, POSTN+ fibroblasts) has clear translational implications.","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"warning","value":"Sample size tag reflects total cells analyzed, not human subjects; subject-level n ranged from 41 (heart) to 48 (synovium) across tissues.","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"warning","value":"Specific diseases within each organ category (e.g., specific ILD subtypes, exact skin diseases) are not detailed in the abstract; condition tags reflect likely represented diseases based on context.","value_numeric":null},{"paper_doi":"10.1002/art.70238","tag_type":"warning","value":"Study design tag is non-standard — this is a secondary integrative analysis of 14 public scRNA-seq datasets, not a traditional clinical study design; level of evidence reflects this indirect, observational nature.","value_numeric":null}],"summaries":{"what_studied":"Cross-tissue single-cell RNA sequencing atlas of fibroblasts and myeloid cells across lung, synovium, skin, and heart in healthy and diseased states, using 14 public datasets (n=148 total samples; 136,741 myeloid cells and 117,102 fibroblasts), with immunofluorescence validation of key subsets.","key_result":"Myeloid populations were broadly similar across organs; shared fibroblast subsets (POSTN+, APOE+, MFAP5+) were most evident in skin and synovium. **SPP1+ macrophages** and **POSTN+ fibroblasts** emerged as common pathogenic subsets in diseased tissues across multiple organs.","limitations":"- Heavy reliance on public datasets introduces significant inter-individual variability and batch effects.\n- Integration methods (Seurat, Harmony, BBKNN) were compared but cross-dataset harmonization remains an inherent limitation for atlas-level studies.\n- Immunofluorescence validation was performed for select subsets only; functional causality of identified pathogenic populations was not established.","practice_takeaway":"SPP1+ macrophages and POSTN+ fibroblasts appear as shared pathogenic cell subsets across inflammatory diseases of the lung, skin, synovium, and heart — making them candidate therapeutic targets worth monitoring in future trials across rheumatologic and fibrotic conditions."}},{"doi":"10.1002/resp.70196","pmid":"41507105","title":"Likelihood of Usual Interstitial Pneumonia: A Novel Approach to Prognosis for Pulmonary Fibrosis","journal_title":"Respirology","journal_issn":"1323-7799","published_at":"2026-01-09T00:00:00.000Z","url":"https://doi.org/10.1002/resp.70196","source_score":3,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2026-01-14","doi":"10.1002/resp.70196","days":"7","issn":"1323-7799","rank":"8","field":"Clinical Medicine","score":"3","category":"Respiratory System","categories":["Respiratory System"],"_categories":["Respiratory System"]},"pubmed":{"doi":"10.1002/resp.70196","title":"Likelihood of Usual Interstitial Pneumonia: A Novel Approach to Prognosis for Pulmonary Fibrosis.","journal":"Respirology (Carlton, Vic.)","methods":"","results":"HRCT was available for analysis in 515 patients. Median follow-up of the cohort was 4.1 years (IQR 2.0-6.2 years). Median total ILD extent was 30% (IQR20-45%), with traction bronchiectasis and honeycombing present in 96.3% and 45.4% of cases respectively, and all three variables were associated with worse transplant-free survival. A pattern of definite or probable UIP was favoured in 26.4% and 32.4% of cases respectively. Likelihood of definite UIP was independently associated with both reduced transplant-free survival (HR1.12, 95% CI 1.04-1.20; p = 0.002) and 12-month progression (HR1.19, 95% CI 1.04-1.35; p = 0.009) and was superior to the ATS/ERS/JRS/ALAT 2018 guideline-based categories for these associations.","abstract":"$30","keywords":["idiopathic pulmonary fibrosis","radiology","usual interstitial pneumonia"],"pubmed_id":"41507105","conclusions":"","publication_date":"2026-01-09T00:00:00"},"crossref":{"DOI":"10.1002/resp.70196","URL":"https://doi.org/10.1002/resp.70196","ISSN":["1323-7799","1440-1843"],"link":[{"URL":"https://onlinelibrary.wiley.com/doi/pdf/10.1002/resp.70196","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":null,"type":"journal-article","issue":null,"score":1,"title":["Likelihood of Usual Interstitial Pneumonia: A Novel Approach to Prognosis for Pulmonary Fibrosis"],"author":[{"given":"John A.","family":"Mackintosh","sequence":"first","affiliation":[{"name":"Department of Thoracic Medicine The Prince Charles Hospital Brisbane Queensland Australia"},{"name":"Faculty of Medicine University of Queensland Brisbane Queensland Australia"},{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"}]},{"given":"Simon L. F.","family":"Walsh","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute Imperial College London London UK"}]},{"given":"Mario","family":"Silva","sequence":"additional","affiliation":[{"name":"Scienze Radiologiche, Department of Medicine and Surgery (DiMeC) University of Parma Parma Italy"}]},{"given":"Lucio","family":"Calandriello","sequence":"additional","affiliation":[{"name":"Dipartimento di Diagnostica per immagini, Radioterapia oncologica ed Ematologia Fondazione Policlinico Universitario A. Gemelli, IRCCS Rome Italy"}]},{"given":"Stephen M.","family":"Humphries","sequence":"additional","affiliation":[{"name":"Department of Radiology National Jewish Health Denver Colorado USA"}]},{"given":"David A.","family":"Lynch","sequence":"additional","affiliation":[{"name":"Department of Radiology National Jewish Health Denver Colorado USA"}]},{"given":"Helen E.","family":"Jo","sequence":"additional","affiliation":[{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"},{"name":"Respiratory Medicine Royal Prince Alfred Hospital Sydney New South Wales Australia"}]},{"given":"Ian","family":"Glaspole","sequence":"additional","affiliation":[{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"},{"name":"Department of Allergy and Respiratory Medicine Alfred Hospital Melbourne Victoria Australia"}]},{"given":"Christopher","family":"Grainge","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine John Hunter Hospital Newcastle New South Wales Australia"}]},{"given":"Nicole","family":"Goh","sequence":"additional","affiliation":[{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"},{"name":"Department of Respiratory and Sleep Medicine Austin Health Melbourne Victoria Australia"},{"name":"Institute for Breathing and Sleep Melbourne Victoria Australia"},{"name":"University of Melbourne Melbourne Victoria Australia"}]},{"given":"Peter M. A.","family":"Hopkins","sequence":"additional","affiliation":[{"name":"Department of Thoracic Medicine The Prince Charles Hospital Brisbane Queensland Australia"},{"name":"Faculty of Medicine University of Queensland Brisbane Queensland Australia"}]},{"given":"Yuben","family":"Moodley","sequence":"additional","affiliation":[{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"},{"name":"School of Medicine & Pharmacology University of Western Australia Perth Western Australia Australia"}]},{"given":"Paul N.","family":"Reynolds","sequence":"additional","affiliation":[{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"},{"name":"Royal Adelaide Hospital Chest Clinic Adelaide South Australia Australia"}]},{"given":"Christopher","family":"Zappala","sequence":"additional","affiliation":[{"name":"Royal Brisbane and Women‘s Hospital Brisbane Queensland Australia"}]},{"given":"Gregory","family":"Keir","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Princess Alexandra Hospital Brisbane Queensland Australia"}]},{"given":"Wendy A.","family":"Cooper","sequence":"additional","affiliation":[{"name":"Tissue Pathology and Diagnostic Oncology, New South Wales Health Pathology Royal Prince Alfred Hospital Sydney New South Wales Australia"},{"name":"School of Medicine University of Sydney Sydney New South Wales Australia"}]},{"given":"Annabelle M.","family":"Mahar","sequence":"additional","affiliation":[{"name":"Tissue Pathology and Diagnostic Oncology, New South Wales Health Pathology Royal Prince Alfred Hospital Sydney New South Wales Australia"}]},{"given":"Samantha","family":"Ellis","sequence":"additional","affiliation":[{"name":"Department of Radiology Alfred Health Melbourne Victoria Australia"}]},{"given":"Athol U.","family":"Wells","sequence":"additional","affiliation":[{"name":"National Heart and Lung Institute Imperial College London London UK"},{"name":"Interstitial Lung Disease Unit Royal Brompton Hospital London UK"}]},{"given":"Tamera J.","family":"Corte","sequence":"additional","affiliation":[{"name":"NHMRC Centre of Research Excellence in Pulmonary Fibrosis Sydney Australia"},{"name":"Respiratory Medicine Royal Prince Alfred Hospital Sydney New South Wales Australia"},{"name":"The University of Sydney School of Medicine (Central Clinical School) Sydney New South Wales Australia"}]}],"funder":[{"id":[{"id":"10.13039/501100000925","id-type":"DOI","asserted-by":"publisher"}],"DOI":"10.13039/501100000925","name":"National Health and Medical Research Council","doi-asserted-by":"publisher"},{"id":[{"id":"10.13039/100000002","id-type":"DOI","asserted-by":"publisher"}],"DOI":"10.13039/100000002","name":"National Institutes of Health","award":["CS‐2018‐18‐ST2‐004"],"award-info":[{"award-number":["CS‐2018‐18‐ST2‐004"]}],"doi-asserted-by":"publisher"},{"id":[{"id":"10.13039/501100006630","id-type":"DOI","asserted-by":"publisher"}],"DOI":"10.13039/501100006630","name":"Lung Foundation Australia","doi-asserted-by":"publisher"}],"issued":{"date-parts":[[2026,1,8]]},"member":"311","prefix":"10.1002","source":"Crossref","volume":null,"archive":["Portico"],"created":{"date-time":"2026-01-09T03:49:50Z","timestamp":1767930590000,"date-parts":[[2026,1,9]]},"indexed":{"date-time":"2026-01-09T05:40:16Z","timestamp":1767937216034,"date-parts":[[2026,1,9]]},"license":[{"URL":"http://onlinelibrary.wiley.com/termsAndConditions#vor","start":{"date-time":"2026-01-08T00:00:00Z","timestamp":1767830400000,"date-parts":[[2026,1,8]]},"delay-in-days":0,"content-version":"vor"}],"subject":[],"abstract":"$31","language":"en","relation":{},"resource":{"primary":{"URL":"https://onlinelibrary.wiley.com/doi/10.1002/resp.70196"}},"subtitle":[],"assertion":[{"name":"received","group":{"name":"publication_history","label":"Publication History"},"label":"Received","order":0,"value":"2025-09-16"},{"name":"accepted","group":{"name":"publication_history","label":"Publication History"},"label":"Accepted","order":2,"value":"2025-12-16"},{"name":"published","group":{"name":"publication_history","label":"Publication History"},"label":"Published","order":3,"value":"2026-01-08"}],"deposited":{"date-time":"2026-01-09T03:50:09Z","timestamp":1767930609000,"date-parts":[[2026,1,9]]},"issn-type":[{"type":"print","value":"1323-7799"},{"type":"electronic","value":"1440-1843"}],"published":{"date-parts":[[2026,1,8]]},"publisher":"Wiley","reference":[{"DOI":"10.1164/rccm.201807-1255ST","key":"e_1_2_10_2_1","year":"2018","issue":"5","author":"Raghu G.","volume":"198","first-page":"e44","unstructured":null,"article-title":"Diagnosis of Idiopathic Pulmonary Fibrosis. 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U.","volume":"167","first-page":"962","unstructured":null,"article-title":"Idiopathic Pulmonary Fibrosis: A Composite Physiologic Index Derived From Disease Extent Observed by Computed Tomography","journal-title":"American Journal of Respiratory and Critical Care Medicine","doi-asserted-by":"crossref"}],"short-title":[],"update-policy":"https://doi.org/10.1002/crossmark_policy","alternative-id":["10.1002/resp.70196"],"article-number":"resp.70196","content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"original-title":[],"container-title":["Respirology"],"reference-count":18,"published-online":{"date-time":null,"timestamp":null,"date-parts":[[2026,1,8]]},"references-count":18,"short-container-title":["Respirology"],"is-referenced-by-count":0},"unpaywall":{"doi":"10.1002/resp.70196","is_oa":true,"title":"Likelihood of Usual Interstitial Pneumonia: A Novel Approach to Prognosis for Pulmonary Fibrosis","updated":"2026-01-11T01:49:12+00:00","published_date":"2026-01-08","best_oa_location":{"url":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/resp.70196","pmh_id":null,"url_for_pdf":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/resp.70196","url_for_landing_page":"https://doi.org/10.1002/resp.70196"}}},"created_at":"2026-01-16T11:56:46.634Z","updated_at":"2026-01-21T12:05:26.220Z","tags":[{"paper_doi":"10.1002/resp.70196","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"sample_size","value":"n=515","value_numeric":515},{"paper_doi":"10.1002/resp.70196","tag_type":"study_design","value":"Retrospective Cohort Study","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"warning","value":"Abstract does not detail covariate adjustments or the definition of 12‑month progression.","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"warning","value":"Design classification inferred from the abstract as a retrospective cohort study.","value_numeric":null},{"paper_doi":"10.1002/resp.70196","tag_type":"warning","value":"Findings are from the Australian IPF Registry; applicability to other settings is uncertain.","value_numeric":null}],"summaries":{"what_studied":"Retrospective analysis of 515 Australian IPF Registry patients’ HRCTs, where two radiologists assigned a percentage likelihood of definite UIP within the 2018 ATS/ERS/JRS/ALAT categories, to test prognostic value for transplant-free survival and 12‑month progression over a median 4.1 years.","key_result":"Higher likelihood of definite UIP independently predicted reduced transplant‑free survival (HR 1.12, 95% CI 1.04–1.20; p=0.002) and 12‑month progression (HR 1.19, 95% CI 1.04–1.35; p=0.009), outperforming the 2018 guideline categories; median ILD extent was 30% (IQR 20–45%), traction bronchiectasis was present in 96.3% and honeycombing in 45.4%, and each was associated with worse transplant‑free survival.","limitations":"Retrospective, single‑registry cohort from the Australian IPF Registry; imaging likelihood scores were assigned by two radiologists.","practice_takeaway":"When interpreting HRCT in pulmonary fibrosis, reporting a percentage likelihood of definite UIP can better stratify risk for transplant‑free survival and 12‑month progression than assigning a single 2018 guideline category."}},{"doi":"10.1002/acr.25696","pmid":"41236136","title":"Validation of a Genetic Risk Score Combined with Clinical Variables for Predicting Pulmonary Fibrosis in early Rheumatoid Arthritis","journal_title":"Arthritis Care & Research","journal_issn":"2151-464X","published_at":"2025-11-14T00:00:00.000Z","url":"https://doi.org/10.1002/acr.25696","source_score":34,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2025-11-16","doi":"10.1002/acr.25696","days":"30","issn":"2151-464X","rank":"5","field":"Clinical Medicine","score":"34","category":"Rheumatology","categories":["Rheumatology"],"_categories":["Rheumatology"]},"pubmed":{"doi":"10.1002/acr.25696","title":"Validation of a Genetic Risk Score Combined with Clinical Variables for Predicting Pulmonary Fibrosis in early Rheumatoid Arthritis.","journal":"Arthritis care & research","methods":"","results":"Of the 1115 patients with complete data, 60 (5.6%) were diagnosed with PF. PF was significantly associated with age, rheumatoid factor positivity, disease activity, and MUC5B (rs35705950) and FAM13A(rs2609255) SNPs. The GRS demonstrated a significant association with RA-PF (odds ratio 2.6, (95%CI 1.6, 4.5), while the combined risk score exhibited superior performance (AUC 0.75, p<0.001) compared to the GRS alone (AUC 0.62). The combined risk score outperformed the GRS in discriminating RA-PF, indicating its potential utility in clinical practice.","abstract":"$32","keywords":[],"pubmed_id":"41236136","conclusions":"This study provides external validation of the VARA-ILD-GRS and VARA-ILD combined risk score in an RA cohort, demonstrating their generalizability and effectiveness in identifying high-risk individuals for RA-ILD. The findings support the integration of genetic and clinical data in risk stratification models, which could significantly improve screening strategies for RA patients at risk of developing PF.","publication_date":"2025-11-14T00:00:00"},"crossref":{"DOI":"10.1002/acr.25696","URL":"https://doi.org/10.1002/acr.25696","ISSN":["2151-464X","2151-4658"],"link":[{"URL":"https://acrjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/acr.25696","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"page":null,"type":"journal-article","issue":null,"score":1,"title":["Validation of a Genetic Risk Score Combined with Clinical Variables for Predicting Pulmonary Fibrosis in early Rheumatoid Arthritis"],"author":[{"given":"Mikael","family":"Brink","sequence":"first","affiliation":[{"name":"Umeå University Umeå Sweden"}]},{"given":"Austin","family":"Wheeler","sequence":"additional","affiliation":[{"name":"University of Nebraska Medical Center & VA Nebraska‐Western Iowa Health Care System Omaha NE USA"}]},{"given":"Bryant R.","family":"England","sequence":"additional","affiliation":[{"name":"University of Nebraska Medical Center & VA Nebraska‐Western Iowa Health Care System Omaha NE USA"}]},{"given":"Solbritt","family":"Rantapää‐Dahlqvist","sequence":"additional","affiliation":[{"name":"Umeå University Umeå Sweden"}]}],"issued":{"date-parts":[[2025,11,14]]},"member":"311","prefix":"10.1002","source":"Crossref","volume":null,"archive":["Portico"],"created":{"date-time":"2025-11-14T14:27:39Z","timestamp":1763130459000,"date-parts":[[2025,11,14]]},"indexed":{"date-time":"2025-11-14T14:32:13Z","timestamp":1763130733276,"date-parts":[[2025,11,14]]},"license":[{"URL":"http://creativecommons.org/licenses/by-nc/4.0/","start":{"date-time":"2025-11-14T00:00:00Z","timestamp":1763078400000,"date-parts":[[2025,11,14]]},"delay-in-days":0,"content-version":"vor"}],"subject":[],"abstract":"$33","language":"en","relation":{},"resource":{"primary":{"URL":"https://acrjournals.onlinelibrary.wiley.com/doi/10.1002/acr.25696"}},"subtitle":[],"assertion":[{"name":"received","group":{"name":"publication_history","label":"Publication History"},"label":"Received","order":0,"value":"2025-06-10"},{"name":"accepted","group":{"name":"publication_history","label":"Publication History"},"label":"Accepted","order":2,"value":"2025-10-21"},{"name":"published","group":{"name":"publication_history","label":"Publication History"},"label":"Published","order":3,"value":"2025-11-14"}],"deposited":{"date-time":"2025-11-14T14:27:39Z","timestamp":1763130459000,"date-parts":[[2025,11,14]]},"issn-type":[{"type":"print","value":"2151-464X"},{"type":"electronic","value":"2151-4658"}],"published":{"date-parts":[[2025,11,14]]},"publisher":"Wiley","reference":null,"short-title":[],"update-policy":"https://doi.org/10.1002/crossmark_policy","alternative-id":["10.1002/acr.25696"],"article-number":"acr.25696","content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"original-title":[],"container-title":["Arthritis Care & Research"],"reference-count":0,"published-online":{"date-time":null,"timestamp":null,"date-parts":[[2025,11,14]]},"references-count":0,"short-container-title":["Arthritis Care & Research"],"is-referenced-by-count":0},"unpaywall":{"doi":"10.1002/acr.25696","is_oa":true,"title":"Validation of a Genetic Risk Score Combined with Clinical Variables for Predicting Pulmonary Fibrosis in early Rheumatoid Arthritis","updated":"2025-11-16T00:15:47+00:00","published_date":"2025-11-14","best_oa_location":{"url":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/acr.25696","pmh_id":null,"url_for_pdf":"https://onlinelibrary.wiley.com/doi/pdfdirect/10.1002/acr.25696","url_for_landing_page":"https://doi.org/10.1002/acr.25696"}}},"created_at":"2025-12-04T03:42:26.941Z","updated_at":"2025-12-08T12:24:58.509Z","tags":[{"paper_doi":"10.1002/acr.25696","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"condition","value":"Pulmonary Fibrosis","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"condition","value":"Rheumatoid Arthritis","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"sample_size","value":"n=1115","value_numeric":1115},{"paper_doi":"10.1002/acr.25696","tag_type":"study_design","value":"Cohort","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"warning","value":"Design classification approximated as a cohort study; this is an external validation of a prognostic model.","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"warning","value":"Only abstract‑level details are available; full model specification and calibration are not described.","value_numeric":null},{"paper_doi":"10.1002/acr.25696","tag_type":"warning","value":"The cohort is from northern Sweden (1996–2016); applicability to other populations is not addressed in the abstract.","value_numeric":null}],"summaries":{"what_studied":"An inception cohort of early rheumatoid arthritis patients from northern Sweden (1996–2016) was used to externally validate a 12‑SNP genetic risk score and a combined clinical‑genetic risk score for predicting RA‑associated pulmonary fibrosis (n=1115 with complete data). Discrimination was assessed with logistic regression and AUC, comparing the genetic score alone versus the combined score.","key_result":"Among 1,115 patients, 60 (5.6%) developed pulmonary fibrosis. The genetic risk score was associated with RA‑PF (OR 2.6; 95% CI 1.6–4.5), and the combined score showed better discrimination (AUC 0.75, p<0.001) than the genetic score alone (AUC 0.62); PF was also associated with age, rheumatoid factor positivity, disease activity, and MUC5B (rs35705950) and FAM13A (rs2609255).","practice_takeaway":"A combined clinical‑genetic risk score (VARA‑ILD combined) identified patients at higher risk of pulmonary fibrosis better than genetics alone (AUC 0.75 vs 0.62). Clinicians can consider such combined scores to guide risk stratification and targeted screening in early RA."}}],"initialOffset":9,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"condition","value":"Pulmonary Fibrosis"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Conditions","href":"/hub/tag/condition"},{"name":"Pulmonary Fibrosis"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"condition","hub_tag_value":"Pulmonary Fibrosis"}}}]]

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