BMC Pulmonary Medicine最新文献

{"title":"Pulmonary nocardiosis in patients with underlying pulmonary alveolar proteinosis: a case series and review of the literature.","authors":"Qi Chen, Ruxuan Chen, Zhiyi Li, Yujie Shi, Kai Xu, Chi Shao, Hui Huang","doi":"10.1186/s12890-026-04527-1","DOIUrl":"10.1186/s12890-026-04527-1","url":null,"abstract":"<p><strong>Objectives: </strong>Pulmonary alveolar proteinosis (PAP) and pulmonary nocardiosis are relatively rare, their co-occurrence constitutes a clinically important comorbidity. Nocardia infection exhibits variable clinical manifestations, and carries a substantial risk of mortality in the absence of timely, pathogen-directed antimicrobial therapy. This study aims to summarize the clinical features of Nocardia infection in PAP patients.</p><p><strong>Case presentation: </strong>We present four confirmed cases of PAP complicated by Nocardia infection. These cases illustrate the distinct microbial species, disease courses and clinical outcomes. The duration of Nocardia infection among the four patients ranged from 3 months to over 2 years. All patients received anti-infection treatment guided by antimicrobial susceptibility testing (AST) results. Clinical outcomes included microbiological cure of infection, sustained clinical stability without progression, and all-cause mortality.</p><p><strong>Conclusions: </strong>In patients with PAP, the emergence of fever and new pulmonary opacities warrants strong clinical suspicion for Nocardia infection. Early initiation of pathogen-directed antimicrobial therapy is critical for infection control and enables subsequent PAP stabilization, clinical recovery, and the safe performance of whole-lung lavage.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13616120/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149058188","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of CYFRA 21-1 with quantitative CT-defined fibrotic progression in idiopathic pulmonary fibrosis.","authors":"Ji-Yeon Han, Hyun-Kyung Lee, Hongyeul Lee, Ho Young Lee, SungMin Hong, Yumi Bang","doi":"10.1186/s12890-026-04524-4","DOIUrl":"10.1186/s12890-026-04524-4","url":null,"abstract":"<p><strong>Background and objective: </strong>Although cytokeratin-19 fragment CYFRA 21 - 1 is a potential biomarker for idiopathic pulmonary fibrosis (IPF), its relationship to imaging-based structural progression remains unclear. We evaluated whether baseline CYFRA 21 - 1 is associated with fibrotic progression measured via quantitative computed tomography (QCT) and compared these associations with Krebs von den Lungen-6 (KL-6), including longitudinal relationships between changes in serum biomarker levels and QCT measures.</p><p><strong>Methods: </strong>We retrospectively analyzed 95 patients with IPF who underwent volumetric chest CT, serum biomarker analysis including CYFRA 21 - 1 and KL-6, and pulmonary function tests at baseline and approximately 1 year. QCT measurements, including fibrosis, ground-glass opacity (GGO)-reticulation extent, and total interstitial lung disease (ILD) extent, were measured using deep learning-based software.</p><p><strong>Results: </strong>Baseline CYFRA 21 - 1 was associated with QCT-defined fibrotic progression (odds ratio per 1 ng/mL increase, 1.347; 95% confidence interval [CI], 1.007-1.801). Baseline KL-6 was also associated with fibrotic progression (odds ratio per 100 U/mL increase, 1.085; 95% CI, 1.011-1.164), although the strength of association was lower. Longitudinal increases in CYFRA 21 - 1 were associated with progression of QCT-defined fibrosis (β = 0.943; 95% CI, 0.287-1.600), whereas changes in KL-6 were more closely associated with QCT-derived GGO-reticulation extent (β = 0.429; 95% CI, 0.179-0.680). Neither biomarker was associated with pulmonary function-based progression.</p><p><strong>Conclusions: </strong>CYFRA 21 - 1 was associated with QCT-defined fibrotic progression in IPF, whereas KL-6 showed stronger associations with GGO-reticulation extent and total ILD extent on CT. These findings suggest complementary imaging-based association patterns of the two serum biomarkers.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13647778/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149135052","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association of laboratory and radiological findings during hospitalization with early post-COVID pulmonary function and exercise capacity: a retrospective study.","authors":"Sevil Okan, Fatih Okan, Murat Beyhan","doi":"10.1186/s12890-026-04536-0","DOIUrl":"10.1186/s12890-026-04536-0","url":null,"abstract":"<p><strong>Background: </strong>This study aimed to retrospectively evaluate the relationship between demographic characteristics, chest CT findings, and laboratory parameters during hospitalization and post-COVID pulmonary function and exercise capacity.</p><p><strong>Methods: </strong>The retrospective study included 101 patients who were hospitalized with COVID-19, had CT findings consistent with COVID-19, and completed at least two months after discharge. In the study, Six-Minute Walk Test (6MWT), Pulmonary Function Tests (PFT), thorax CT and laboratory findings were evaluated. Statistical analyses were conducted using SPSS (v25.0); normality and variance homogeneity were assessed by Shapiro-Wilk and Levene tests, respectively, while group comparisons were performed using Student's t, Mann-Whitney U, Chi-square, and one-way ANOVA with Tukey post-hoc tests, and correlations were evaluated by Spearman analysis.</p><p><strong>Results: </strong>Patients with severe/critical COVID-19 had significantly higher age, BMI, length of hospital stay, time from diagnosis to admission, ferritin, CRP, D-dimer, neutrophil %, N/L ratio, and Charlson Comorbidity Index, and lower lymphocyte % (p < 0.005). Severe/critical pneumonia was observed in 66.7% of CO-RADS 4 and 67.9% of CO-RADS 5 cases (p = 0.002). Restrictive respiratory patterns were found in 32.7% of the severe/critical group (p = 0.018). ICU patients had significantly shorter 6MWT distances, more frequent restrictive PFT results, and higher inflammatory markers at admission compared to non-ICU patients (p < 0.05). No significant correlation was found between disease severity and 6MWT distance.</p><p><strong>Conclusion: </strong>Elevated inflammatory markers and ICU admission during hospitalization were associated with impaired pulmonary function and reduced exercise capacity in the early post-COVID period. In contrast, disease severity and CO-RADS classification were not predictive of functional outcomes. These findings highlight the role of inflammatory burden and critical illness as key predictors of post-COVID impairment.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13412122/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148599185","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Building and externally validating a prediction model for long COVID in severe and critical COVID-19 patients: a multi-center cohort study.","authors":"Zhang Haojing, Kan Lin, Pan Dianzhu","doi":"10.1186/s12890-026-04409-6","DOIUrl":"10.1186/s12890-026-04409-6","url":null,"abstract":"<p><strong>Objective: </strong>To explore the risk factors of Long COVID and to construct a nomogram to predict the occurrence of Long COVID.</p><p><strong>Method: </strong>This study was an observational study. Clinical data were collected from patients diagnosed with COVID-19 and hospitalized at the First Affiliated Hospital of Jinzhou Medical University Hospital from December 7, 2022, to February 1, 2023. The prediction model was constructed using a nomogram. The clinical data of patients in Panjin Central Hospital Hospital from December 7, 2022 to December 7, 2023 were used for external validation.</p><p><strong>Results: </strong>In the development cohort and the validation cohort of this study, 60.3% and 62.3% of the patients developed Long COVID, respectively. After Least absolute shrinkage and selection operator regression, the final variables included in the prediction model were percentage of lymphocyte, the Charlson Comorbidity Index, Computed Tomography score, and oxygen requirement. The Area Under the Receiver Operating Characteristic for external validation of the model is 0.786, and the p value of Spiegelhalter test was 0.126. The p value of the Hosmer and Lemeshow chi-square statistic was 0.098. The decision curve analysis indicates that the model performs well.</p><p><strong>Conclusion: </strong>The prediction model developed in this study is useful for assessing the likelihood of developing Long COVID in hospitalized patients.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13602745/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149029844","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary epithelioid hemangioendothelioma with pleural dissemination: a case report of prolonged diagnostic delay and the role of surgical biopsy.","authors":"Rui Yan, Weiwei Feng, Lu Lu, Yi Gong, Guifang Wang","doi":"10.1186/s12890-026-04510-w","DOIUrl":"10.1186/s12890-026-04510-w","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor with low incidence and nonspecific clinical and radiological features, frequently leading to misdiagnosis and delayed diagnosis.</p><p><strong>Case presentation: </strong>We report a 32-year-old man with bilateral pulmonary nodules that were followed for 6 years before the development of a new left lower lobe mass, pleuritic chest pain, dyspnea, and massive pleural effusion in 2025. Initial imaging revealed diffuse miliary nodules that remained relatively indolent for several years. Two transbronchial lung biopsies (TBLB) and repeated pleural fluid cytology failed to establish a diagnosis. Definitive diagnosis was achieved only after video-assisted thoracoscopic surgery (VATS), with immunohistochemical support for endothelial differentiation and molecular confirmation of the WWTR1-CAMTA1 fusion gene. The patient underwent surgery, systemic therapy, and intrapleural treatment, followed by maintenance sirolimus. Disease remained clinically and radiologically stable during 6 months of follow-up.</p><p><strong>Conclusions: </strong>PEH presents substantial diagnostic challenges because of its indolent course and nonspecific clinical and radiological features. For patients with unexplained multiple pulmonary nodules and pleural involvement, especially when minimally invasive investigations remain repeatedly nondiagnostic, early surgical biopsy combined with endothelial immunophenotyping, and molecular testing is essential for timely diagnosis and optimal management.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Metabolomic signatures of early pathway disruptions and clinical outcomes in community-acquired pneumonia: a prospective case-control study.","authors":"Souvik Chaudhuri, Varashree Bolar Suryakanth, Shruthi Rao, Ashritha A Udupa, Monalisa Biswas, Y S Phaneedra Mallimoggala, Thejesh Srinivas, Bharatkumar Appasaheb Patil, Ranajit Das, Nitin Gupta","doi":"10.1186/s12890-026-04503-9","DOIUrl":"10.1186/s12890-026-04503-9","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;The disruption of metabolic pathways in patients with community-acquired pneumonia (CAP) may provide insights into disease progression. We aim to identify early disruption in the serum metabolome of CAP patients using untargeted metabolic approaches.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;We conducted a single-centre, prospective case-control study involving 105 participants (70 with CAP and 35 healthy controls), between January 2025 and November 2025. We also performed a subgroup exploratory metabolomic analysis among the 70 CAP patients, comparing 60 patients with severe CAP requiring ICU admission with the 10 non-severe CAP patients who did not require ICU admission. Untargeted serum metabolomics was performed using gas chromatography-mass spectrometry (GC-MS) to investigate metabolic dysregulation in patients with CAP. The analysis of the clinical data was performed using SPSS version 25.0. The data of metabolomics was analyzed using MetaboAnalyst version 6.0. After the preprocessing, log&lt;sub&gt;10&lt;/sub&gt; transformation, normalization and scaling, 93 GC-MS metabolites were retained from the initial 300 metabolites. Principal component analysis was done for unsupervised clustering, which was then followed by OPLS-DA for supervised group discrimination. Model performance was assessed using R²Y and cross-validation-derived Q² values, and model robustness and overfitting exclusion was further evaluated using permutation testing. Discriminatory metabolites were identified using VIP scores, fold change, log₂ fold change and univariate testing with false discovery rate correction where applicable. ROC analysis, heatmap-based clustering and pathway enrichment analysis were performed.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;The variable importance in projection (VIP) scores showed that out of 93 untargeted metabolites studied using GC-MS, the greatest differences between the CAP patients and healthy controls were about malic acid (VIP:3.374, fold change 2.591), 4-hydroxybenzeneacetic acid (VIP:2.459, fold change 27.97), 2-hydroxybutyric acid (VIP:2.059, fold change 4.779), and 4-hydroxyphenyllactic acid (VIP:1.882, fold change 20.67). Key disrupted pathways in CAP patients included propanoate, glyoxylate, and dicarboxylate; tricarboxylic acid cycle; nitrogen; tyrosine, cysteine, and methionine; and amino acid pathways. Comparison between the severe CAP patients (ICU admission required) and non-severe CAP patients (no-ICU admission), VIP analysis identified boric acid, ethylene glycol, ethanimidic acid, linoleic acid, oleic acid, cholesterol, 3-hydroxybutyrate, and α-tocopherol as the principal discriminating metabolites, while pathway analysis revealed perturbations in glutathione metabolism, amino acid metabolism, the tricarboxylic acid cycle, pyruvate metabolism, and arachidonic acid metabolism.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;CAP patients demonstrated early disruptions of key metabolic pathways compared with healthy controls, spann","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13625439/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149079758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Longitudinal recovery of physical function after lung transplantation: a comparative analysis of ECMO and non-ECMO recipients.","authors":"Woo Chul Son, Jong Yoon Chang, Ji Yeon Park, Jun Hyeok Lee, Geon Woo Kim, Jung Hwa Do, Sang-Bum Hong, Ho Cheol Kim, Won Kim","doi":"10.1186/s12890-026-04506-6","DOIUrl":"https://doi.org/10.1186/s12890-026-04506-6","url":null,"abstract":"<p><strong>Introduction: </strong>Lung transplantation (LT) improves survival in patients with end-stage lung disease; however, recovery of physical function after transplantation remains insufficiently characterized, particularly in critically ill recipients bridged with extracorporeal membrane oxygenation (ECMO). Understanding longitudinal functional recovery and potential differences between ECMO and non-ECMO recipients is essential for optimizing post-transplant rehabilitation strategies.</p><p><strong>Methods: </strong>We retrospectively analyzed 84 adult patients who underwent lung or heart-lung transplantation between 2018 and 2023. Physical function was assessed at 6 and 12 months using the 6-minute walk test, gait speed, and strength measures. Changes over time and group differences were evaluated using longitudinal statistical models.</p><p><strong>Results: </strong>Fifty patients (59.5%) required ECMO support. Overall physical function significantly improved from 6 to 12 months, with 93.4% achieving independent ambulation by 12 months. ECMO recipients had a lower proportion of independent ambulation at 6 months compared with non-ECMO recipients. At 12 months, no statistically significant between-group difference was observed in the proportion of patients achieving independent ambulation.</p><p><strong>Conclusions: </strong>LT recipients showed significant improvement in physical function between 6 and 12 months after transplantation. ECMO-bridged recipients had lower independent ambulation rates at 6 months, while no statistically significant between-group difference was observed at 12 months. Further studies incorporating pretransplant and early postoperative assessments are needed to better characterize functional recovery after lung transplantation.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparison of HALP and DECAF in predicting 30 day in hospital mortality in a retrospective cohort study of acute exacerbation of COPD.","authors":"Ridvan Gode, Behcet Demir, Ertan Sonmez","doi":"10.1186/s12890-026-04532-4","DOIUrl":"https://doi.org/10.1186/s12890-026-04532-4","url":null,"abstract":"<p><strong>Background: </strong>Acute exacerbations of chronic obstructive pulmonary disease (AECOPD) are associated with substantial short term mortality and healthcare utilization, yet the prognostic utility of the immunonutrition based hemoglobin, albumin, lymphocyte, and platelet (HALP) score in this setting remains unclear, particularly in comparison with the well validated dyspnoea, eosinopenia, consolidation, acidaemia, atrial fibrillation (DECAF) score.</p><p><strong>Methods: </strong>We conducted a retrospective, single centre observational cohort study of adults presenting to the emergency department (ED) with AECOPD between June 2022 and June 2025. DECAF and HALP scores were calculated at admission using routinely collected clinical and laboratory data, and the primary outcome was 30 day in hospital mortality. Discrimination was assessed using receiver operating characteristic (ROC) analysis, with Youden-derived, data-driven cut-off values, and we further evaluated previously validated DECAF thresholds and a categorical DECAF model. Associations with 30 day mortality were examined using univariable and multivariable logistic regression models adjusted for baseline clinical variables. Model calibration was evaluated using Brier scores and the Hosmer-Lemeshow test, and 30 day survival was analysed with Kaplan-Meier.</p><p><strong>Results: </strong>A total of 499 patients were included in the study, 63 (12.6%) died within 30 days, and 76% of the cohort were male. The HALP score showed acceptable predictive performance (area under the curve (AUC) 0.724), at a cut-off of 16.84, sensitivity was 57.1%, specificity 75.0% and negative predictive value (NPV) 92.4% for 30 day in hospital mortality. The DECAF score demonstrated excellent discrimination for 30 day in hospital mortality (AUC 0.932), and showed significantly better discrimination than HALP (DeLong p < 0.001), using a cut-off of ≥ 4, sensitivity was 76.2%, specificity 95.6% and NPV 96.5%.</p><p><strong>Conclusion: </strong>In ED patients with AECOPD, the HALP score provides only limited prognostic information for 30 day in hospital mortality, whereas the DECAF score exhibits excellent discriminative performance and retains its value as a robust, clinically applicable predictor of short term outcomes in acute care.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149223338","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk factors for idiopathic pulmonary fibrosis: an umbrella review of meta-analyses and mendelian randomization analyses.","authors":"Huanrong Ruan, Siyuan Lei, Jun Wang, Hulei Zhao, Xuezhong Zhou, Jiansheng Li","doi":"10.1186/s12890-026-04519-1","DOIUrl":"10.1186/s12890-026-04519-1","url":null,"abstract":"<p><strong>Background: </strong>The incidence of idiopathic pulmonary fibrosis (IPF) is rising globally. Existing meta-analyses and Mendelian randomization (MR) studies have yielded inconsistent findings with uncertain evidence quality.</p><p><strong>Objectives: </strong>This study aimed to synthesize available evidence and assess its credibility for potential risk factors of IPF.</p><p><strong>Methods: </strong>We systematically searched 8 databases and selected meta-analyses and MR studies related to IPF risk factors from inception to March 11, 2025. We assessed the quality of meta-analyses using the AMSTAR-2 tool and evaluated MR studies using the STROBE-MR guidelines. We categorized the evidence into four levels and performed pooled analysis for risk factors reported in at least two MR studies.</p><p><strong>Results: </strong>A total of 8 meta-analyses were included, reporting 22 associations, mainly involving genetics, infections, occupational and environmental exposures, lifestyle, and clinical variables. According to the grading of evidence credibility, 12 were rated as suggestive associations, and 10 as weak associations. The overall credibility of the evidence is relatively low. The 36 MR studies investigated a total of 124 associations, with evidence grading showed 13 as robust, 90 as possible, 17 as suggestive, and 4 as insufficient. The pooled analysis identified 108 associations covering lifestyle, diet and nutrition, anthropometric indices, biomarkers, and clinical variables. The quality level of the included meta-analyses was low and critically low, while the MR studies strictly adhered to STROBE-MR, demonstrating good reporting quality.</p><p><strong>Conclusions: </strong>This umbrella review uncovers the evidence regarding the associations between IPF risk and factors such as genetics, viral infections, occupational and environmental exposures, lifestyle, and clinical variables through a comprehensive analysis of meta-analyses and MR studies. These findings provide an evidence base for IPF risk factor research and support clinical decisions and public health strategies.</p><p><strong>Prospero registration number: </strong>CRD42024575341.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617888/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cystic pulmonary amyloidosis with lymphoid interstitial pneumonia in Sjögren's disease: a case report.","authors":"Jinzhou Chen, Songtao Ren, Jing Huang, Ting Yang, Guangbing Lu, Hua Fang, Xiaojing Deng, Bo Zhang, Sixian Liu, Liangdong Tian","doi":"10.1186/s12890-026-04528-0","DOIUrl":"10.1186/s12890-026-04528-0","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung diseases are commonly seen in association with Sjögren's disease, especially lymphoproliferative disorders, such as lymphoid interstitial pneumonia. In occasional and rare cases, pulmonary amyloidosis with cysts, nodules, and/or ground-glass opacites as radiological features, can also become a complication of Sjögren's disease. On the other hand, diffuse cystic lung disease, without nodules, concomitant with chylous fluid accumulation in female leads to initial suspicion of lymphangioleiomyomatosis. Interestingly, it's challenging to differentiate if diffuse cystic lung lesion absent of nodules, chylous fluid accumulation and Sjögren's disease happen simultaneously.</p><p><strong>Case presentation: </strong>A 34-year-old female, with diffuse cystic lung disease, chylous reflux lymphedema and Sjögren's disease, was eventually diagnosed with cystic pulmonary amyloidosis with lymphoid interstitial pneumonia, secondary to Sjögren's disease, and chylous reflux lymphedema as comorbidity. The case presented with atypical pulmonary amyloidosis radiological features of lung lesion, where diffuse cysts existed without nodules or ground-glass opacites, apart from the scarcely seen size of the biggest cyst. The patient was diagnosed with CPA with LIP, secondary to SjD. Clinical symptoms were relieved after thoracoscopic lobectomy of the right lower lobe, where located the biggest cyst. The immunosuppressants that she had been taking were recommended again for disease stabilization.</p><p><strong>Conclusion: </strong>Cystic pulmonary amyloidosis is a rare complication of Sjögren's disease, while lymphoid interstitial pneumonia is comparatively well-known. There can be cystic pulmonary amyloidosis cases where the lung lesion only consists of diffuse, thin-walled, and variably-sized cysts, with the absence of nodules or ground-glass opacities, which warrants a lung biopsy. Clinicians should be alerted to the cyst-only radiological features of the cystic pulmonary amyloidosis with Sjögren's disease.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":""},"PeriodicalIF":3.1,"publicationDate":"2026-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149219714","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}