Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG最新文献

{"title":"Postoperative acute exacerbations in patients with idiopathic pulmonary fibrosis undergoing lung cancer surgery.","authors":"Hasibe Çiğdem Erten, Sevda Şener Cömert, Gonca Gül Geçmen, Talha Doğruyol, Cihan Akgül Özmen, Beyzanur Ünver, Recep Demirhan","doi":"10.36141/svdld.2026.18641","DOIUrl":"10.36141/svdld.2026.18641","url":null,"abstract":"<p><strong>Background and aim: </strong>This study evaluated the incidence, risk factors, and mortality associated with postoperative acute exacerbations in IPF patients undergoing lung cancer surgery.</p><p><strong>Methods: </strong>We retrospectively reviewed 1,225 lung cancer surgeries performed between 2018-2024, identifying 40 patients meeting ATS/ERS criteria for IPF. Acute exacerbations were defined as new bilateral ground-glass opacities superimposed on preexisting fibrosis within 30 days postoperatively. Clinical, radiological, functional, and pathological data were analyzed using univariate and multivariate logistic regression to identify risk factors for acute exacerbations and mortality.</p><p><strong>Results: </strong>Postoperative acute exacerbations occurred in 15% (6/40) of patients, with 83.3% mortality within 30 days. Multivariate analysis confirmed lesion size >3cm (OR=3.50, 95%CI: 1.80-6.80, p<0.001), shorter IPF follow-up duration (OR=2.90, 95%CI: 1.50-5.70, p<0.001), and mortality (OR=0.026, 95%CI: 0.001-0.715, P=0.031) as independent risk factors. Overall mortality was 40%, significantly associated with preexisting emphysema (OR=6.998, 95%CI: 1.926-25.449, P=0.003) and long-term oxygen therapy (OR=5.845, 95%CI: 1.475-23.147, P=0.012).</p><p><strong>Conclusions: </strong>Postoperative acute exacerbations are serious complications with high mortality in IPF patients undergoing lung cancer surgery. Tumor characteristics, particularly size and location, should guide preoperative risk assessment and perioperative management strategies.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 3","pages":"18641"},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659545/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083612","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of yoga-based and conventional physiotherapy programs on functional, respiratory, and psychosocial outcomes in stage II sarcoidosis: A randomized controlled trial.","authors":"Şeyda Yıldız, Murat Kavas, Zuhal Didem Takinaci, Selma Aydoğan Eroğlu","doi":"10.36141/svdld.2026.18324","DOIUrl":"10.36141/svdld.2026.18324","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis, a multisystemic granulomatous disease, presents with various clinical symptoms such as deterioration in respiratory functions, decreased exercise capacity, fatigue, sleep disorders, anxiety-depression, and consequently reduced quality of life. A standard rehabilitation protocol has not been defined for sarcoidosis, and due to the nature of the disease, holistic approaches are crucial in this group, which is known to have multisystem involvement. Therefore, this study aims to contribute to the development of future rehabilitation protocols by comparing the effectiveness of yoga and conventional physiotherapy programs delivered via telerehabilitation, in addition to standard aerobic training, in sarcoidosis patients with pulmonary involvement.</p><p><strong>Methods: </strong>Thirty-four Stage II sarcoidosis patients were included in this study, and the participants were randomly divided into two groups (conventional physiotherapy, n=16, yoga, n=18). Before and after the 8-week programs, participants completed the following questionnaires: Fatigue Assessment Scale (FAS), Hospital Anxiety and Depression Scale, Pittsburgh Sleep Quality Index (PSQI), and St. George Respiratory Questionnaire (SGRQ). Furthermore, the 6MWT, Body Oxygen Level Test (BOLT), spirometric measurements, DLCO, Maximum inspiratory pressure (MIP), and dyspnea (mMRC score) measurements.</p><p><strong>Results: </strong>Programs significantly improved exercise capacity, inspiratory muscle strength, fatigue (FAS), dyspnea, PSQI scores, anxiety-depression levels, quality of life (SGRQ), and BOLT time in both groups. While similar improvements were observed overall in intergroup comparisons, the increase in MIP and BOLT time was more pronounced in the conventional physiotherapy group. Neither program resulted in significant changes in spirometric parameters or DLCO values.</p><p><strong>Conclusions: </strong>This study found that both 8-week telerehabilitation-based yoga and conventional physiotherapy programs were effective for patients with Stage II sarcoidosis. The similar effects observed between yoga and conventional physiotherapy suggest that yoga, a holistic mind-body approach, may be an effective alternative in the treatment of sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 3","pages":"18324"},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659556/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083660","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy and safety of extended-release oral opioid for cough in idiopathic pulmonary fibrosis: A systematic review and meta-analysis of randomized controlled trials.","authors":"Shun Nakahara, Ana Alicia Velarde-Pineda, Maria Júlia Moura, Brent Matsuda","doi":"10.36141/svdld.2026.18930","DOIUrl":"10.36141/svdld.2026.18930","url":null,"abstract":"<p><strong>Background and aim: </strong>Patients with idiopathic pulmonary fibrosis (IPF) experience a high burden of cough. Currently, no established effective therapy exists for cough in IPF. The overall evidence base of oral extended-release (ER) opioid therapies in this population remains limited. This study aimed to compare the efficacy and safety of ER oral opioids versus placebo for cough in patients with IPF.</p><p><strong>Methods: </strong>PubMed, Embase, Cochrane, and ClinicalTrial.gov databases were reviewed for randomized controlled trials (RCTs). The primary outcome was 24-hour objective cough frequency. Secondary outcomes included Evaluating Respiratory Symptoms-Idiopathic Pulmonary Fibrosis (E-RS IPF) cough subscale score, Cough Severity-Numeric Rating Scale (CS-NRS) score. Safety outcomes included serious adverse events and adverse events leading to discontinuation.</p><p><strong>Results: </strong>Three RCTs, including 250 patients, were analyzed. Opioid interventions included ER nalbuphine and morphine. ER opioid statistically significantly improved 24-hour objective cough frequency (GMR 0.46; 95% CI 0.23 to 0.92; p = 0.029), CS-NRS score (MD -1.69; 95% CI -2.55 to -0.84; p < 0.001), and E-RS IPF cough subscale score (MD -0.49; 95% CI -0.89 to -0.09; p = 0.016), compared with placebo. No statistically significant differences were observed for serious adverse events or adverse events leading to discontinuation.</p><p><strong>Conclusions: </strong>ER oral opioids significantly improved cough frequency and patient-reported cough severity and discomfort in patients with IPF, without a significant increase in serious adverse events or treatment discontinuation. Extended-release opioids may be cautiously considered as a therapeutic option for cough in patients with IPF.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 3","pages":"18930"},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659551/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of home-based pulmonary rehabilitation in patients with interstitial lung disease in a tertiary care centre - A randomized controlled trial.","authors":"Christie George Joseph, Manju Rajaram, Madhusmita Mohanty Mohapatra, Subathra Adithan, Chengappa Kavadichanda, Jayalakshmy Ramakrishnan, Moushumi Purkayastha Mukherjee, Bhanumathy Mohanakrishnanan, Vijayalakshmi Krishnamoothi, Sivaselvi Chellamuthu","doi":"10.36141/svdld.2026.16799","DOIUrl":"10.36141/svdld.2026.16799","url":null,"abstract":"<p><strong>Background and aim: </strong>Interstitial lung disease causes functional limitation and exercise intolerance. Although pulmonary rehabilitation in ILD can improve physical function and quality of life, multiple factors hinder its utilisation including lack of facilities, mobility, caregivers and cost. Therefore, alternative models such as home-based pulmonary rehabilitation are required to bridge the gap between guidelines and practice. This study explored whether a 12-week home-based pulmonary rehabilitation program could improve the functional capacity of ILD patients as measured by 6MWD when compared to conventional drug therapy alone.</p><p><strong>Methods: </strong>Patients suffering from ILD attending a tertiary hospital in South-India were stratified according to disease sub-type and randomised. A structured 12-weeks home-based pulmonary rehabilitation consisting of exercise training, health education, nutritional and psychological support was provided to the intervention arm. Conventional drug therapy was continued in both arms. Change in 6MWD and FVC, K-BILD and HADS scores were measured at the end of 12 weeks. The results were analysed for determining factors responsible for the change in health status indicators.</p><p><strong>Results: </strong>At the end of 12 weeks, 6MWD and K-BILD scores alone showed statistically significant improvement in intervention arm when compared to controls. However, it did not reach the MCID for either parameter. Spirometry and HADS score had no significant improvement in either arm. A significantly higher proportion of participants in the intervention group achieved the MCID for 6MWD (RR 2.32 ,95% CI 1.47-3.68) and K-BILD score (RR 1.94, 95% CI 1.28-2.96) compared with controls.   Conclusion: Home based pulmonary rehabilitation was found to effect modest improvement in 6MWD and K-BILD scores: measures of functional capacity and QoL respectively, among ILD patients. A substantially higher proportion of patients in the intervention arm experienced clinically meaningful benefits.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 3","pages":"16799"},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659543/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk and characteristics of interstitial lung disease in patients with inflammatory bowel disease: A retrospective cohort study.","authors":"Amarilys Alarcon-Calderon, Modi Pranav, Felix Wireko, Salma Abdulla, Livia F Kruger, Emily Liu, Phillip J Schulte, Jay H Ryu, Misbah Baqir","doi":"10.36141/svdld.2026.18654","DOIUrl":"10.36141/svdld.2026.18654","url":null,"abstract":"<p><strong>Background: </strong>Inflammatory bowel disease (IBD) is a disorder with extraintestinal manifestations that may involve multiple organ systems. Interstitial lung disease (ILD) is an extraintestinal manifestation that remains underrecognized, and its prevalence, clinical characteristics, and risk factors in IBD are poorly defined.</p><p><strong>Methods: </strong>We conducted a retrospective study of adults (≥18 years) with IBD who had at least 1 clinical encounter at our hospital between January 1, 2013, and December 31, 2023. IBD and ILD diagnoses were identified by using International Classification of Diseases, Ninth [or Tenth] Revision, Clinical Modification codes, and ILD diagnoses were confirmed by a thoracic radiologist. Demographic and clinical characteristics were abstracted from the electronic health record. Associations between patient characteristics and ILD were evaluated with 2 analyses: cross-sectional cohort and matched case-control. Risk of death was assessed by using a Cox proportional hazards model with ILD as a time-dependent covariate.</p><p><strong>Results: </strong>Among 875 patients with IBD, 109 (12%) had ILD. Radiographic patterns of ILD most commonly showed nonspecific interstitial pneumonia (27%), indeterminate (26%), and usual interstitial pneumonia (24%). Pulmonary function testing most commonly showed restriction (59%) and impaired diffusing capacity (61%). The cohort analysis showed that higher BMI, bronchiectasis, history of smoking, and use of adalimumab or infliximab were independently associated with ILD. The case-control analysis confirmed these findings and identified older age as another significant factor. ILD was associated with a higher risk of death (hazard ratio, 3.78; 95% CI, 2.50-5.72; P<.001).</p><p><strong>Conclusion: </strong>ILD in patients with IBD was associated with various clinical characteristics and a higher risk of death. These findings highlight the need for early identification, routine respiratory assessment, and multidisciplinary management.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 3","pages":"18654"},"PeriodicalIF":0.0,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13659546/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149083707","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes of Lung Transplantation for Pulmonary Sarcoidosis Across Multiple Eras: A 25-Year Experience from a US Center.","authors":"Zein Kattih, Abhimanyu Chandel, Vikramjit Khangoora, Jared Wilkinson, Alan Nyquist, Anju Singhal, Christopher Thomas, Silpa Kilaru, Christopher King, Oksana Shlobin, Steven Nathan, Shambhu Aryal","doi":"10.36141/svdld.2026.18338","DOIUrl":"10.36141/svdld.2026.18338","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is a systemic condition with various clinical manifestations including end-stage lung disease. The aim of this study was to evaluate the characteristics and outcomes of patients with sarcoidosis undergoing lung transplantation.</p><p><strong>Methods: </strong>A single center retrospective review was performed of 63 sarcoidosis patients who underwent lung transplantation between January 1, 2000 and April 30, 2025. Patients were divided into arbitrary eras by year (Era 1 2000-2007; Era 2 2007-2013; Era 3 2013-2025).</p><p><strong>Results: </strong>The median age of the cohort was 53 years, 51% were female, 79% were black, 33% had blood type A, and the median body mass index was 24.4 kg/m2 (21.3-28.2). The majority (93%) of patients had pulmonary sarcoidosis stage IV by Scadding staging, and 89% had pulmonary hypertension. Baseline median six-minute walk test distance (6MWD) was 235 m (133-306). The median FVC% was 45% (34-50), median FEV1 was 37% (26-45), median- FEV1/FVC was 73% (62-83) and median DLCO% was 26% (18-32). The median mean pulmonary artery pressure (mPAP) was 35 (26-44) mmHg, and the median pulmonary vascular resistance (PVR) was 4 (3-6) Wood Units (WU). Chronic lung allograft dysfunction (CLAD) occurred in 38% of patients at a median 85 months post-transplant. Overall survival was 87.6%, 66% and 50.6% at 1, 3, and 5 years, respectively. There was no statistically significant difference in mortality across eras: era 1 vs era 3 (HR 0.888, 95% CI 0.407-1.94, p=0.766).</p><p><strong>Conclusions: </strong>Outcomes of sarcoidosis patients who undergo lung transplantation are similar to patients with other underlying diagnoses. Timely referral should be made to specialized centers.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 2","pages":"18338"},"PeriodicalIF":0.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13359002/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148298310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unveiling novel susceptibility genes for sarcoidosis by a cross-tissue transcriptome-wide association study.","authors":"Xiqiao Sun","doi":"10.36141/svdld.2026.18029","DOIUrl":"10.36141/svdld.2026.18029","url":null,"abstract":"<p><strong>Background: </strong>Sarcoidosis is a systemic granulomatous disease with heterogeneous clinical manifestations and unclear pathogenesis. Although genome-wide association studies (GWAS) have identified several immune-related loci, the functional interpretation of these signals remains limited. Integrative transcriptome-wide approaches may uncover novel susceptibility genes and provide mechanistic insights.</p><p><strong>Objectives: </strong>The primary objective of this study was to identify novel susceptibility genes for sarcoidosis and provide mechanistic insights into its pathogenesis through a comprehensive, cross-tissue transcriptome-wide approach.</p><p><strong>Methods: </strong>We conducted a cross-tissue transcriptome-wide association study (TWAS) using the UTMOST framework, followed by single-tissue TWAS via FUSION. Candidate genes identified in both analyses were further evaluated using Multi-marker Analysis of Genomic Annotation (MAGMA), Mendelian randomization (MR), and Bayesian colocalization. Functional characterization was explored through gene-chemical-disease associations from the Comparative Toxicogenomics Database (CTD) and phenome-wide association studies (PheWAS) in the UK Biobank.</p><p><strong>Results: </strong>Cross-tissue TWAS identified 48 genes. Integrative analyses prioritized four novel susceptibility genes: RNF215, PLCL1, FAM117B, and RFTN2. MR and colocalization supported causal effects of RNF215 (risk-increasing), FAM117B and RFTN2 (protective), and tissue-dependent effects for PLCL1. CTD analyses revealed interactions of these genes with environmental chemicals including bisphenol A and tetrachlorodibenzodioxin, while PheWAS demonstrated pleiotropic associations with immune, respiratory, hematological, and cardiovascular traits.</p><p><strong>Conclusions: </strong>This comprehensive integrative study identifies four biologically plausible susceptibility genes for sarcoidosis, expanding the genetic architecture of sarcoidosis and suggest potential targets for mechanistic and therapeutic investigation.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 2","pages":"18029"},"PeriodicalIF":0.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13358998/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148298349","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Advances in management of sarcoidosis-associated fatigue: A narrative review.","authors":"Hunter Scott","doi":"10.36141/svdld.2026.18501","DOIUrl":"10.36141/svdld.2026.18501","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis-associated fatigue (SAF) occurs in approximately 30% to 90% of patients. While previous reviews examined select medications, these omitted nonpharmacological and emerging treatments that could significantly improve fatigue for individuals with sarcoidosis. Limited guidance exists for excluding alternative causes of fatigue and selecting between treatment options for these patients. This narrative review synthesized pharmacological and nonpharmacological interventions for SAF and interpreted evidence strength to guide clinical decision-making.</p><p><strong>Methods: </strong>PubMed was queried in December 2025 for clinical trials, reviews, and meta-analyses published within the last 10 years. This yielded 41 results which were supplemented by manual review of bibliographies, clinical guidelines, and a search of the Cochrane Library. Evidence was interpreted and analyzed for quality using a modified GRADE-informed approach.</p><p><strong>Results: </strong>Treatment of active disease with prednisone or methotrexate improves systemic inflammation but may paradoxically worsen fatigue with chronic use. Stimulants (e.g., dexmethylphenidate, armodafinil) display low-quality evidence for improving SAF and side effects may be prohibitive for some patients. TNF-α inhibitors (e.g., infliximab) have very low-quality evidence with few studies and negative findings. Pulmonary rehabilitation and exercise training show low-to-moderate quality evidence with few adverse effects but lack specific implementation protocols.</p><p><strong>Conclusions: </strong>Diagnosis of SAF requires exclusion of alternative causes which may be challenging. Active disease should be treated appropriately while monitoring for adverse effects. Despite low-to-moderate quality evidence for most interventions, nonpharmacological approaches (e.g., pulmonary rehabilitation, exercise) are recommended as first-line therapy given favorable risk-benefit profiles. Larger, well-designed RCTs are needed given the substantial disease burden.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 2","pages":"18501"},"PeriodicalIF":0.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13358997/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148298324","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Transcriptomic profiling of collagen gene expression in lung fibroblasts from patients with idiopathic pulmonary fibrosis.","authors":"Eun-Jeong Seo, Seung-Lee Park, Min-Kyung Kim, Jae-Sung Choi, Hun-Gyu Hwang, Jung-Hyun Kim, Hun-Soo Chang, Choon-Sik Park, Jong-Uk Lee","doi":"10.36141/svdld.2026.18053","DOIUrl":"10.36141/svdld.2026.18053","url":null,"abstract":"<p><strong>Background and aim: </strong>Idiopathic Pulmonary Fibrosis (IPF) is a progressive interstitial lung disease characterized by aberrant extracellular matrix remodeling. While type I and III collagens are known contributors, the broader transcriptional landscape of diverse collagen genes in IPF fibroblasts and their potential associations with clinical outcomes remain incompletely understood. This study aimed to comprehensively characterize gene expression of collagen genes in IPF fibroblasts and to explore its relationship with patient survival.</p><p><strong>Methods: </strong>RNA sequencing was conducted on primary lung fibroblasts from 33 IPF patients and 10 non-fibrotic controls. Differential expression of collagen genes was analyzed using EdgeR's exact test, with significance defined as false discovery rate-adjusted p < 0.05. Association with mortality was assessed using Cox regression analysis.</p><p><strong>Results: </strong>Among 17 collagen genes, 14 genes - including COL1A1, COL1A2, COL3A1, COL4A4, COL4A5, COL4A6, COL5A2, COL6A6, COL8A1, COL11A1, COL14A1, COL18A1, COL24A1, and COL27A1 - were significantly upregulated in IPF fibroblasts, while COL5A3, COL13A1, and COL16A1 were downregulated. COL3A1, COL4A4, and COL24A1 were associated with reduced survival in both Cox regression analysis and Kaplan-Meier plots. Among them, COL4A4 remained association with mortality in multivariable Cox model.</p><p><strong>Conclusions: </strong>This study delineates fibroblast-specific dysregulation across multiple collagen families in IPF, providing mechanistic insight into extracellular matrix remodeling. Although COL4A4 remained associated with mortality in multivariable Cox models, this finding should be interpreted as exploratory, given the limited number of events and the absence of experimental validation. Overall, our results highlight collagen transcript signatures as hypothesis-generating markers that warrant further validation to clarify their potential clinical relevance in IPF.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 2","pages":"18053"},"PeriodicalIF":0.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13358995/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148298336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Diagnostic Course of Sarcoidosis: A Population-Based Study Highlighting Risk Factors for a Delay in Diagnosis.","authors":"Miles D Hagner, Aaron Miller, Solanus De la Serna, Alan Arakkal, Joseph Cavanaugh, Alejandro Pezzulo, Nabeel Hamzeh, Philip Polgreen, Alicia Gerke","doi":"10.36141/svdld.2026.18607","DOIUrl":"10.36141/svdld.2026.18607","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is a systemic inflammatory syndrome of unknown cause characterized by granulomas, heterogeneous presentation, and variable clinical course. Diagnosis is often delayed, contributing to patient distress, increased healthcare costs, and potentially worse outcomes. Prior estimates of diagnostic delays rely largely on case-based studies, which may overestimate delays by failing to account for baseline care practices and common alternative diagnoses that may resemble sarcoidosis.</p><p><strong>Methods: </strong>We conducted a retrospective population-based cohort study to characterize healthcare utilization before a sarcoidosis diagnosis and to identify risk factors associated with a diagnostic delay. Using longitudinal commercial, Medicare, and Medicaid healthcare insurance claims data from 2001-2022, we identified patients with sarcoidosis and evaluated diagnostic delay frequency, time to diagnosis, and potential missed opportunities for diagnosis. Secondary analysis compared diagnostic differences between pulmonary and cutaneous sarcoidosis.</p><p><strong>Results: </strong>87,092 sarcoidosis cases were identified, of which 56% experienced at least one healthcare visit with a symptomatically similar diagnosis before sarcoidosis was diagnosed. The mean time to diagnosis was 44 days, defined as the interval between increased baseline healthcare utilization and a sarcoidosis diagnosis. Patients had an average of 2.5 visits prior to sarcoidosis diagnosis that represented potential missed diagnostic opportunities. Pulmonary involvement was associated with longer time to diagnosis and more missed opportunities compared to cutaneous sarcoidosis. Risk factors for delays included obesity, outpatient evaluation, weekend visits, Medicaid insurance, and treatment for symptoms commonly attributable to sarcoidosis. Diagnosis was unaffected by age, season, or rural vs urban setting. Within Medicaid, white individuals had the highest risk for a missed diagnostic opportunity, while Black individuals had the lowest.</p><p><strong>Conclusions: </strong>In this large population-based study, diagnostic delays in sarcoidosis were shorter than previously reported, yet substantial missed opportunities remain. Identifying patient- and system-level risk factors may help reduce delays, prevent disease progression, and improve outcomes in sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 2","pages":"18607"},"PeriodicalIF":0.0,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13359006/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148298313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}