SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES结节病、血管炎与弥漫性肺疾病
SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES(英文缩写 SARCOIDOSIS VASC DIF),ISSN 1124-0490,中文译名:结节病、血管炎与弥漫性肺疾病 是一本学术期刊。本页汇总该期刊的最新影响因子、分区信息以及最新收录于 PubMed 的文献,帮助您快速了解期刊全貌。
发文量统计区间：2025-10-01 至 2026-09-30，按本站收录文献的发表日期统计。
期刊介绍
历年影响因子趋势
| JCR 数据年份 | 影响因子 | JCR 分区 |
|---|---|---|
| 2021 | 1.803 | Q4 |
| 2022 | 1.600 | Q4 |
| 2023 | 1.400 | Q4 |
| 2024 | 1.800 | Q3 |
| 2025 | 1.800 | Q3 |
SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES 最新收录文献
-
1. Bilateral corneal keratolysis (corneal melt) in sarcoidosis.
PMID:日期:2026-09-30Corneal involvement in sarcoidosis is rare, and central corneal keratolysis has not been previously described. We present the first reported case of bilateral central corneal keratolysis in a patient with multi-organ sarcoidosis. Case report Results: A 37-year-old woman with pulmonary, cutaneous, and ocular sarcoidosis presented with severe bilateral ocular pain, photophobia, and rapidly progressive vision loss. Examination revealed severe uveitis with bilateral hypopyons and central corneal keratolysis. Results from infectious, rheumatological, and vasculitis work-up were negative. She was treated with high-dose intravenous corticosteroids, methotrexate, and infliximab but developed significant stromal scarring with persistent visual impairment. Conclusions: This case highlights the need to consider sarcoidosis in the differential diagnosis of rapidly progressive keratolysis, particularly in patients with known systemic disease or anterior sarcoid uveitis.
-
2. Peripheral immune checkpoint gene expression as diagnostic biomarkers in idiopathic pulmonary fibrosis.
PMID:日期:2026-09-30Idiopathic pulmonary fibrosis (IPF) is a relatively rare and progressive fibrotic interstitial lung disease with limited annual incidence and poor prognosis. Increasing evidence suggests that immune dysregulation, including altered immune checkpoint and innate regulatory signaling pathways, may contribute to disease pathogenesis. This study aimed to investigate peripheral blood expression levels of PD-1, PD-L1, TIM-3, and TOLLIP genes and to evaluate their diagnostic potential in patients with IPF. This case-control study included 20 IPF patients and 20 age-matched healthy controls. Gene expression levels were quantified using real-time quantitative polymerase chain reaction (RT-qPCR) and calculated using the ΔCt method. Diagnostic discrimination was assessed by receiver operating characteristic (ROC) analysis. PD-L1 (p < 0.001) and TOLLIP (p = 0.002) ΔCt values were significantly higher, indicating reduced relative gene expression. ROC analysis demonstrated strong diagnostic performance for PD-L1 (AUC = 0.883) and TOLLIP (AUC = 0.865). The combined PD-L1 + TOLLIP biomarker panel demonstrated superior diagnostic accuracy (AUC = 0.948). Internal validation using bootstrap resampling confirmed model stability (mean AUC = 0.950; 95% CI: 0.85-1.00). In conclusion, peripheral blood PD-L1 and TOLLIP expression levels are significantly downregulated in patients with idiopathic pulmonary fibrosis. The combined PD-L1 + TOLLIP biomarker panel demonstrated strong diagnostic discrimination (AUC = 0.948), supporting the potential clinical utility of peripheral immune regulatory gene signatures as minimally invasive biomarkers in IPF. Larger prospective studies are warranted to validate these findings and to explore their potential role in clinical decision-making.
-
3. Fibroblast proliferative capacity predicts prognosis in idiopathic pulmonary fibrosis and identifies PITX1 as a growth-associated gene.
PMID:日期:2026-09-30Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with poor prognosis. Lung fibroblasts isolated from patients with IPF exhibit enhanced proliferative capacity; however, the molecular determinants linking fibroblast proliferation to clinical outcomes remain incompletely understood. Fibroblast growth slopes were measured in primary lung fibroblast derived from patients with IPF and control subjects. Transcriptome microarray data were integrated with fibroblast growth kinetics to identify growth slope-associated genes. Differential expression analysis was performed between IPF and control fibroblasts, and candidate genes were further evaluated at the mRNA and protein levels in lung fibroblasts and bronchoalveolar lavage (BAL) fluids. Associations with clinical outcomes were assessed. Fibroblasts derived from patients with IPF showed significantly higher growth slope than control fibroblasts, and increased fibroblast growth slope were associated with reduced survival in IPF. Integration of fibroblast growth kinetics with transcriptomic analysis identified a fibroblast growth slope-associated gene signature, from which PITX1 emerged as a disease-relevant candidate gene. PITX1 mRNA and protein levels were elevated in IPF fibroblasts compared with controls. In BAL fluids, PITX1 protein levels were significantly higher in patients with IPF and were associated with clinical outcomes, although they did not correlate with pulmonary function parameters. Enhanced fibroblast proliferative capacity is associated with poor prognosis in IPF, and PITX1 represents a fibroblast growth slope-associated gene reflecting fibroblast heterogeneity and prognostic relevance. These findings suggest that assessment of fibroblast growth behavior and PITX1 expression may provide insight into disease progression in IPF, although further validation studies are required.
-
4. Efficacy and safety of extended-release oral opioid for cough in idiopathic pulmonary fibrosis: A systematic review and meta-analysis of randomized controlled trials.
PMID:日期:2026-09-30Patients 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. 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. 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. 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.
-
5. Trajectories of progressive pulmonary fibrosis and outcomes in connective tissue disease associated interstitial lung disease (CTD-ILD).
PMID:日期:2026-09-30Progressive pulmonary fibrosis (PPF) can develop in various interstitial lung diseases (ILD), including connective tissue disease-associated ILD (CTD-ILD). This study aimed to examine the longitudinal trajectory and transition of PPF status in patients with CTD-ILD. In this single-center study at a tertiary referral hospital, we analyzed patients with CTD-ILD who underwent consecutive chest computed tomography (CT) scans and pulmonary function tests over two-year period. Patients were categorized into four groups based on annual PPF status. Among the 144 patients, the majority (n = 75) maintained a non-PPF status throughout follow-up (Group 1), while 25 patients developed PPF at year 2 (Group 2), 12 showed PPF improvement (Group 3), and 31 had persistent PPF (Group 4). The persistent PPF group showed significantly higher mortality risk (p = 0.007). Based on PPF status at 2 years, annual changes in forced vital capacity (FVC) and diffusing capacity of carbon monoxide (DLCO) were more pronounced in PPF groups than in non-PPF groups (FVC: -3.72 ± 0.52 %/year vs. 2.82 ± 0.41 %/year; DLCO: -4.30 ± 0.75 %/year vs. 3.64 ± 0.60 %/year). Patients treated with immunomodulating agents showed a reduced risk of progression to PPF, although the association was not statistically significant (Group 2, adjusted odds ratio: 0.26, 95% CI: 0.02-4.43, p = 0.351). Regular pulmonary function monitoring and continuous assessment of PPF progression are essential for patients with CTD-ILD. Disease-directed treatment may reduce the risk of PPF development.
-
6. Reflections of upper extremity performance in idiopathic pulmonary fibrosis on exercise capacity, clinical status, and quality of life.
PMID:日期:2026-09-30Clinical assessments in idiopathic pulmonary fibrosis (IPF) typically emphasize lower extremities; however, upper extremity performance may also influence clinical status, daily functioning, and quality of life. This study aimed to examine the relationship between upper extremity performance and exercise capacity, clinical status, and quality of life. IPF patients underwent 6-minute pegboard and ring test (6PBRT), Unsupported Upper Extremity Exercise Test (UULEX), 6-Minute Walk Test (6MWT), 1-Minute Sit-to-Stand Test (1MSTST), Modified Medical Research Council Dyspnea Scale (mMRC), Fatigue Severity Scale (FSS), London Chest Activities of Daily Living Scale (LCADL), and St. George Respiratory Questionnaire (SGRQ). Moderate correlation was found between 6PBRT and 6MWT (r=0.516, p=0.017), 1MSTST (r=0.612, p=0.003), and UULEX (r=0.614, p=0.003). Negative correlations were observed between 6PBRT and mMRC (r=-0.482, p=0.027), GAP Index (r=-0.453, p=0.039), and LCADL-Leisure (r=-0.519, p=0.016). In multiple linear regression analysis, 6MWT distance and GAP Index explained 30% of the variance in 6PBRT (R²=0.303, p=0.039). Additionally, UULEX was negatively correlated with mMRC, FSS, LCADL, and SGRQ scores. The 6PBRT shows moderate associations with lower extremity performance and cardiopulmonary capacity, supporting its use in assessing upper extremity function in IPF patients. Clinically, 6PBRT and UULEX may complement lower extremity tests in comprehensive IPF assessment.
-
7. The prognostic value of the prognostic nutritional index in patients with sarcoidosis.
PMID:日期:2026-09-30Sarcoidosis is a heterogeneous multisystem granulomatous disease with an unpredictable clinical course. Reliable biomarkers for predicting relapse remain limited. The Prognostic Nutritional Index (PNI), reflecting systemic inflammation and immunonutritional status, has emerged as a prognostic marker in various inflammatory and pulmonary diseases. However, its role in sarcoidosis has not been sufficiently clarified. This study aimed to investigate the association between baseline PNI levels and relapse in patients with sarcoidosis. This retrospective single-center study included 302 adult patients diagnosed with sarcoidosis between January 2015 and January 2025. Baseline laboratory parameters, including serum albumin, lymphocyte count, C-reactive protein (CRP), neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR), were recorded. PNI was calculated as: 10 × serum albumin (g/dL) + 0.005 × total lymphocyte count (/mm³). The primary outcome was disease relapse during follow-up. Receiver operating characteristic (ROC) curve analysis was performed to evaluate the predictive performance of baseline PNI. Relapse occurred in 52 patients (17.2%) during follow-up. Patients who developed relapse had significantly lower baseline albumin levels, lymphocyte counts, and PNI values, and significantly higher CRP, NLR, and PLR levels (all p<0.01). Baseline PNI demonstrated excellent discriminative ability for predicting relapse, with an area under the curve (AUC) of 0.922 (95% CI: 0.887-0.957, p<0.001). A cutoff value of ≤39.25 yielded a sensitivity of 94.2% and a specificity of 82.8%. Lower baseline PNI values are strongly associated with relapse in patients with sarcoidosis and show excellent predictive performance. As a simple, inexpensive, and widely available biomarker reflecting both inflammatory burden and immune competence, PNI may serve as a practical tool for risk stratification and individualized follow-up in clinical practice. Prospective multicenter studies are warranted to validate these findings.
-
8. Risk and characteristics of interstitial lung disease in patients with inflammatory bowel disease: A retrospective cohort study.
PMID:日期:2026-09-30Inflammatory 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. 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. 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). 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.
-
9. Postoperative acute exacerbations in patients with idiopathic pulmonary fibrosis undergoing lung cancer surgery.
PMID:日期:2026-09-30This study evaluated the incidence, risk factors, and mortality associated with postoperative acute exacerbations in IPF patients undergoing lung cancer surgery. 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. 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). 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.
-
10. Sarcoidosis in hospitalised patients in Western Australia: A long-term study on prevalence, comorbidity and mortality.
PMID:日期:2026-09-30To determine disease prevalence, comorbidity and mortality for hospitalised patients with sarcoidosis in Western Australia (WA). A retrospective statewide cohort study of patients over 15 years of age hospitalised with sarcoidosis between 1980 and 2014. Sarcoidosis was identified based on validated ICD -9CM/ICD10-AM based algorithms with index admission treated as baseline. Period and point prevalence per 100.000 adult population and mortality rate (MR) per 100 person years were estimated with 95% confidence intervals (CI). Kaplan Meyer survival curves and other outcomes were compared with a comparator group of Rheumatoid Arthritis patients with interstitial lung disease (RA-ILD). Median values are given with interquartile range (IQR). We included 276 patients with sarcoidosis (index age 58 yrs IQR 46-69, 51.4 % females, invasive assessment in 68%) for a period prevalence of 21.1 (CI 18.7-23.7) and a 2015-point prevalence of 12.1 (CI 10.6-13.8). No specific risk factors were identified and extrapulmonary manifestations were recorded in 15%. During follow-up (3428-person years; median 11 years, IQR 5-19), patients with sarcoidosis were more likely to develop hypercalcemia (10.9 vs 4. 1%, p<0.01), new onset autoimmune rheumatic disease (9.3 vs 2.3%, p<0.01) and incident cancer (21.5 vs15%, p=0.07) and less likely to develop heart failure (18.5 vs 40%, p<0.01) than RA-ILD patients. In contrast to RA-ILD patients, age adjusted standardised MR was not increased for patients with sarcoidosis and respiratory causes of death significantly less frequent (p<0.01) Conclusion: The hospital-based prevalence of sarcoidosis in WA is higher than previously reported from Australia. Due to lower burden of comorbidity, the overall and respiratory prognosis for sarcoidosis is much better than for RA associated lung disease.