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

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Exploring sarcoidosis via nailfold capillaroscopy: A window into the disease's microvascular landscape. 通过甲襞毛细血管镜探查结节病:一扇进入疾病微血管景观的窗口。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-12-15 DOI: 10.36141/svdld.v42i4.17137
Lucrezia Mondini, Mariangela Barbieri, Liliana Trotta, Giorgio Monteleone, Luca Ruggero, Anna De Nes, Chiara Torregiani, Rossella Cifaldi, Caterina Antonaglia, Michael Hughes, Francesco Salton, Marco Confalonieri, Paola Confalonieri, Ilaria Gandin, Barbara Ruaro
{"title":"Exploring sarcoidosis via nailfold capillaroscopy: A window into the disease's microvascular landscape.","authors":"Lucrezia Mondini, Mariangela Barbieri, Liliana Trotta, Giorgio Monteleone, Luca Ruggero, Anna De Nes, Chiara Torregiani, Rossella Cifaldi, Caterina Antonaglia, Michael Hughes, Francesco Salton, Marco Confalonieri, Paola Confalonieri, Ilaria Gandin, Barbara Ruaro","doi":"10.36141/svdld.v42i4.17137","DOIUrl":"10.36141/svdld.v42i4.17137","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is a systemic granulomatous inflammatory disease that can affect virtually any organ, with a predilection for the lungs. It may present vasculitic features involving small, medium, or large vessels. Microvascular alterations in sarcoidosis can be assessed using nailfold videocapillaroscopy (NVC), a tool widely applied in the diagnosis and monitoring of systemic sclerosis, among other conditions. The aim of our study was to detect microcirculatory changes in patients with sarcoidosis and investigate their potential correlation with organ involvement.</p><p><strong>Methods: </strong>We conducted a single-center, retrospective case-control study involving three groups: 51 patients diagnosed with sarcoidosis, 51 healthy volunteers serving as controls, and 51 patients with systemic sclerosis. The groups were similar in sex and age. All participants underwent nailfold videocapillaroscopy (NVC) to assess microvascular changes.</p><p><strong>Results: </strong>This study confirmed the presence of non-specific microcirculatory abnormalities in patients with sarcoidosis, particularly in terms of reduced capillary density, the presence of angiogenesis, and slowed capillary flow. Although these alterations do not currently appear to correlate with specific clinical aspects of the disease, (e.g. the presence of autoantibodies, laboratory parameters and variables of lung function).</p><p><strong>Conclusions: </strong>Our findings confirm the presence of non-specific microvascular abnormalities in sarcoidosis. However, the identification of these capillaroscopic alterations as specific to sarcoidosis requires further confirmation. Ongoing studies aim to explore the potential role of NVC as a diagnostic marker and to investigate its correlation with the clinical manifestations of sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17137"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759104","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}
引用次数: 0
Acute exacerbation of idiopathic pulmonary fibrosis: application of the diagnostic criteria and the role of respiratory intermediate care unit and mechanical ventilation. 特发性肺纤维化急性加重:诊断标准的应用及呼吸中间监护病房和机械通气的作用。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-12-15 DOI: 10.36141/svdld.v42i4.16377
Joana Daniela Rodrigues Barbosa, Sara Moreira da Silva Salgado, Maria Paula Cruz Mata Nazaré Pinheiro Esteves
{"title":"Acute exacerbation of idiopathic pulmonary fibrosis: application of the diagnostic criteria and the role of respiratory intermediate care unit and mechanical ventilation.","authors":"Joana Daniela Rodrigues Barbosa, Sara Moreira da Silva Salgado, Maria Paula Cruz Mata Nazaré Pinheiro Esteves","doi":"10.36141/svdld.v42i4.16377","DOIUrl":"10.36141/svdld.v42i4.16377","url":null,"abstract":"<p><strong>Background and aim: </strong>Acute exacerbations of Idiopathic Pulmonary Fibrosis (AE-IPF) have a poor prognosis, and the majority of evidence supports the lack of benefit of invasive mechanical ventilation. The objective was to evaluate patients with AE-IPF admitted to a Respiratory Intermediate Care Unit (RICU).</p><p><strong>Methods: </strong>A retrospective study was conducted on AE-IPF patients admitted to a Portuguese RICU from 2014 to 2018.</p><p><strong>Results: </strong>Thirty-one admissions (n=20) corresponded to IPF (9 were suspected AE by the diagnostic criteria of IPFnet 2007 and 15 by the International Working Group Report 2016). The mean age was 70±11 years, and 53% were male. FVC was 69.7±29% and DLCO was 38.7±14%. The initial PaO2/FiO2 was 145±71, as opposed to 228±91 in non-AE patients. Bronchoscopy was performed on three patients. Regarding treatment, 60% had non-invasive ventilation, and 40% had high-flow oxygen therapy. Methylprednisolone pulses were used in two patients. Eight patients died during hospitalization (53%); four died within five months, and only one survived.</p><p><strong>Conclusions: </strong>Recent diagnostic criteria of AE-IPF help identify these patients, contrary to old criteria. Bronchoscopy is not always possible to perform, and a small number of patients receive methylprednisolone pulses. No statistically significant differences were observed between the discharged and deceased groups, but higher levels of LDH at admission and lower DLCO could help identify risk groups. AE-IPF has a poor prognosis, and admission at RICU could be helpful in the management of these patients, reducing the need for admission to intensive care units, and consequently costs and risks of invasive mechanical ventilation.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"16377"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815297/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759138","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}
引用次数: 0
Survival in familial idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis. 家族性特发性肺纤维化和纤维化超敏性肺炎的生存率。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-12-15 DOI: 10.36141/svdld.v42i4.16938
Deborah Reis Estrella, Eliane Mancuzo, Ricardo De Amorim Correa, Regina Tibana, Maria Raquel Soares, Carlos Alberto De Castro Pereira
{"title":"Survival in familial idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis.","authors":"Deborah Reis Estrella, Eliane Mancuzo, Ricardo De Amorim Correa, Regina Tibana, Maria Raquel Soares, Carlos Alberto De Castro Pereira","doi":"10.36141/svdld.v42i4.16938","DOIUrl":"10.36141/svdld.v42i4.16938","url":null,"abstract":"<p><strong>Background: </strong>Familial pulmonary fibrosis (FPF) is defined as the occurrence of any interstitial lung disease (ILD) in two or more first- or second-degree relatives. Although the most frequently reported phenotype is idiopathic pulmonary fibrosis (IPF), other forms of ILD have been identified, such as fibrotic hypersensitivity pneumonitis (fHP). This study aims to explore survival between patients with IPF and fHP substratified by presence or absence of self-reported familial pulmonary fibrosis.</p><p><strong>Methods: </strong>Family history was acquired systematically, and patients were stratified by IPF or fHP according to specific guidelines.  Clinical data on pulmonary function and imaging were collected during the first follow-up evaluation of patients. All cases were reviewed in multidisciplinary meetings. Other ILD diagnosis was excluded.   RESULTS: A total of 198 participants were included: 88 with IPF and 110 with fHP. In the IPF group, 36 had familial forms (F-IPF), and in the fHP group, 30 had familial fHP (F-fHP). The median follow-up time was 37 (3 - 1200) months. The variables age (hazard ratio [HR]: 1.03; 95% CI: 1.01 - 1.06; p = 0.01), FVC% (HR: 0.96; 95% CI: 0.94 - 0.98; p < 0.01), SpO2% (HR: 0.86; 95% CI: 0.76 - 0.97; p < 0.02), and family history of pulmonary fibrosis (HR: 1.79; 95% CI: 1.09 - 2.93; p = 0.02) were associated with mortality and were controlled before performing the survival curves. The median survival for the FPF group was 46 months compared to 75 months for the non-familial group (p = 0.009).</p><p><strong>Conclusion: </strong>patients with familial presentations of IPF and fHP have significantly lower survival compared to patients with non-familial presentations of the same diseases. In clinical practice across many countries, genetic testing is not widely available, so family history should always be investigated.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"16938"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815290/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759096","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}
引用次数: 0
Effect of exercise training in patients with cardiac sarcoidosis after induction of oral corticosteroid therapy. 运动训练对心脏结节病患者口服皮质类固醇治疗诱导后的影响。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.15628
Hidetoshi Yanagi, Harumi Konishi, Yukihiro Shimada, Hiroyuki Miura, Tatsuo Aoki, Teruo Noguchi
{"title":"Effect of exercise training in patients with cardiac sarcoidosis after induction of oral corticosteroid therapy.","authors":"Hidetoshi Yanagi, Harumi Konishi, Yukihiro Shimada, Hiroyuki Miura, Tatsuo Aoki, Teruo Noguchi","doi":"10.36141/svdld.v42i3.15628","DOIUrl":"10.36141/svdld.v42i3.15628","url":null,"abstract":"<p><strong>Background and aim: </strong>To our knowledge, no study has investigated altered muscle strength in patients with cardiac sarcoidosis (CS). This retrospective observational study investigated the relationship between quadriceps isometric strength (QIS) and exercise training (ET) in patients with CS.</p><p><strong>Methods: </strong>Eighteen patients who participated in a 4-month ET programme immediately after initiating oral corticosteroids were studied. They underwent 3-5 ET sessions per week during hospitalisation and 1-2 sessions per week after discharge. We measured their QIS at the beginning of the ET programme and at 1 and 4 months after programme enrolment. The patients received 29±4 mg/day prednisolone during the corticosteroid induction period and 15±4 mg/day prednisolone during the corticosteroid tapering period.</p><p><strong>Results: </strong>They continued ET and showed significant QIS weakness in the early corticosteroid induction period (0.52±0.12 to 0.48±0.11 kgf/kg, P=.048) and no significant improvement during the corticosteroid tapering period (0.53±0.08 to 0.58±0.06 kgf/kg, P=.099). Patients who received ≤12.5 mg/day prednisolone reported an improved QIS-to-body weight ratio.</p><p><strong>Conclusions: </strong>Patients with CS treated with 30 mg/day prednisolone showed poor QIS early in the corticosteroid induction period, even with ET. Patients with CS who receive corticosteroids may be more susceptible to developing corticosteroid myopathy.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"15628"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536840/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202733","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}
引用次数: 0
Evaluation of patients diagnosed with primary Sjogren's syndrome with and without pulmonary involvement. 原发性干燥综合征伴及不伴肺受累患者的评价。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.17364
Zehra Ozsoy, Tuğçe Şahin Ozdemırel, Kerem Ensarıoğlu, Hakan Erturk, Zeynep Ozturk, Hasret Yıldırım, Esma Sevil Akkurt, Esin Beyan, Umut Kalyoncu, Berna Akıncı Ozyurek
{"title":"Evaluation of patients diagnosed with primary Sjogren's syndrome with and without pulmonary involvement.","authors":"Zehra Ozsoy, Tuğçe Şahin Ozdemırel, Kerem Ensarıoğlu, Hakan Erturk, Zeynep Ozturk, Hasret Yıldırım, Esma Sevil Akkurt, Esin Beyan, Umut Kalyoncu, Berna Akıncı Ozyurek","doi":"10.36141/svdld.v42i3.17364","DOIUrl":"10.36141/svdld.v42i3.17364","url":null,"abstract":"<p><strong>Background and aim: </strong>Interstitial lung disease (ILD) is the most severe pulmonary complicationin Primary Sjögren's Syndrome (pSS). We aimed to evaluate and compare the pulmonary involvement patterns, respiratory parameters, clinical, radiological, pathological, and laboratory features, disease activity scores, treatment choices, and the relationships between these findings at diagnosis in pSS patients with and without pulmonary involvement.</p><p><strong>Methods: </strong>Patients at Ankara Ataturk Sanatorium Training and Research Hospital were included in the analysis. Patients with ILD who met the classification criteria were included as the study group. Patients who met the SS classification criteria but had no findings in the lungs were included as the control group.</p><p><strong>Results: </strong>The median disease duration of ILD patients included in the study was 9.98 months. In pSS patients, patients with ILD had demographic (older age, male gender, more frequent smoking), symptomatology (frequent dry eyes), auto-antibody positivity (more frequent antinuclear antibodies (ANA), anti-Sjögren's Syndrome-related antigen A (SS-A), anti-Sjögren's Syndrome-related antigen B (SS-B)), higher disease activity and more frequent immunosuppressive use. When patients with pSS and ILD were compared according to high resolution computed tomography (HRCT) uptake pattern as nonspecific interstitial pneumonia (NSIP) (fibrotic or not) and unclassified, there were more male gender, lower forced vital capacity (FVC) values, and more frequent immunosuppressive and anti-fibrotic use in the NSIP group. When patients with ILD were classified according to gender, males had more smoking, SSB positivity, fibrotic NSIP, and lower FVC and diffusing lung capacity for carbon monoxide (DLCO) values.</p><p><strong>Conclusion: </strong>A multidisciplinary approach involving pulmonologists, radiologists, and rheumatologists who are experts in ILD is important to increase diagnostic reliability. Pulmonary involvement in pSS is an important cause of morbidity and mortality and should be managed more carefully in male patients.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"17364"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536849/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202754","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}
引用次数: 0
Global disease burden of interstitial lung disease and pulmonary sarcoidosis, 1990-2021, and forecast analysis over the next decade. 1990-2021年间质性肺病和肺结节病的全球疾病负担,以及未来十年的预测分析
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.16462
Xinlian Li, Zhihong Zhang
{"title":"Global disease burden of interstitial lung disease and pulmonary sarcoidosis, 1990-2021, and forecast analysis over the next decade.","authors":"Xinlian Li, Zhihong Zhang","doi":"10.36141/svdld.v42i3.16462","DOIUrl":"10.36141/svdld.v42i3.16462","url":null,"abstract":"<p><strong>Background and aim: </strong>Interstitial lung disease is a major class of disease that affect lung health, however, there is a lack of epidemiological studies related to the global burden of this disease. Data from the Global Burden of Disease Study 2021 (GBD 2021) were utilized to evaluate the global burden of interstitial lung disease and pulmonary sarcoidosis comprehensively in 21 global regions, 204 countries and territories from 1990 to 2021, examining their association with the sociodemographic index, and forecasting trends for the forthcoming decade.</p><p><strong>Methods: </strong> Using GBD 2021 data, we analyzed interstitial lung disease and pulmonary sarcoidosis prevalence, incidence, mortality, and disability-adjusted life years. We calculated age-standardized rate trends (1990-2021) through generalized linear models and assessed disease burden inequalities using the sociodemographic index, slope index, and concentration index. Global maps and regional comparisons visualized burden distribution. Decomposition analysis quantified impacts of population growth, aging, and epidemiological changes. Future burden trends (next decade) were projected using Bayesian Age-Period-Cohort modeling. Results: From 1990 to 2021, the global burden of interstitial lung disease and pulmonary sarcoidosis has been on the rise. Generally, Higher age-standardized rates of interstitial lung disease and pulmonary sarcoidosis were observed in higher age groups and areas with a high socio-demographic index. Moreover, population growth and ageing are the main causes of the increased burden of disease. Projections indicate that by 2034, the number of cases, illnesses, deaths, and disability-adjusted life years associated with interstitial lung disease and pulmonary sarcoidosis will continue to rise, while age-standardized rates are expected to plateau.</p><p><strong>Conclusions: </strong>The global disease burden of interstitial lung disease and pulmonary sarcoidosis remains significant and deserves our attention. People aged 70-84 and high sociodemographic index group should be the focus of attention.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16462"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536834/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202725","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}
引用次数: 0
The value of endobronchial ultrasound-guided transbronchial needle aspiration for the diagnosis of pulmonary sarcoidosis real life-settings. 超声引导下经支气管穿刺诊断肺结节病的价值。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.16693
Ibrahim Guven Cosgun, Aydin Balci, Sule Cilekar
{"title":"The value of endobronchial ultrasound-guided transbronchial needle aspiration for the diagnosis of pulmonary sarcoidosis real life-settings.","authors":"Ibrahim Guven Cosgun, Aydin Balci, Sule Cilekar","doi":"10.36141/svdld.v42i3.16693","DOIUrl":"10.36141/svdld.v42i3.16693","url":null,"abstract":"<p><strong>Background: </strong>Endobronchial ultrasonography-assisted transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive technique that is used in the diagnosis of mediastinal and hilar lymphadenopathy. The aim of this study was to investigate the diagnostic value of EBUS-TBNA in the diagnosis of sarcoidosis.</p><p><strong>Methods: </strong>The study included 42 patients who underwent EBUS-TBNA because of suspected sarcoidosis (Grade I and II) on pulmonary radiography and thorax tomography. A total of 104 lymph node stations were sampled from 42 patients.</p><p><strong>Results: </strong>A diagnosis of sarcoidosis was made histologically in 38 of the 42 patients from whom lymph node sampling was performed with EBUS-TBNA.</p><p><strong>Conclusion: </strong>EBUS-TBNA was seen to be an extremely successful method in the diagnosis of Grade I and Grade II sarcoidosis. Therefore, EBUS-TBNA should be considered first in the histopathological diagnosis of Grade I and Grade II sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16693"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536835/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202744","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}
引用次数: 0
Survival impact and safety comparison of pirfenidone and nintedanib for idiopathic pulmonary fibrosis: A meta-analysis. 吡非尼酮和尼达尼布治疗特发性肺纤维化的生存影响和安全性比较:一项荟萃分析。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.16432
Hafiz Muhammad Ehsan Arshad, Faaz Ali, Anas Babar, Muhammad Zain Raza, Musab Maqsood, Akmal Ameer
{"title":"Survival impact and safety comparison of pirfenidone and nintedanib for idiopathic pulmonary fibrosis: A meta-analysis.","authors":"Hafiz Muhammad Ehsan Arshad, Faaz Ali, Anas Babar, Muhammad Zain Raza, Musab Maqsood, Akmal Ameer","doi":"10.36141/svdld.v42i3.16432","DOIUrl":"10.36141/svdld.v42i3.16432","url":null,"abstract":"<p><strong>Background and aim: </strong>Idiopathic pulmonary fibrosis (IPF) is a severe restrictive lung disease affecting approximately 3 million people worldwide with two approved antifibrotics, nintedanib and pirfenidone, available for use. This review aims to compare their survival impact and safety profile.</p><p><strong>Methods: </strong>Two databases and two trial registers along with additional sources were searched for cohorts reporting IPF patients of any age or stage, receiving either pirfenidone or nintedanib. The Inverse-Variance and Mantel-Haenszel method along with either a fixed- or random-effects model was used for analysing survival and other dichotomous outcomes, respectively.</p><p><strong>Results: </strong>23 cohorts were included. The pooled analysis showed that compared to pirfenidone, nintedanib group had similar survival (HR=1.12; 95%-CI:0.99-1.27; P=0.07), all-cause mortality (OR=1.11; 95%-CI:0.94-1.31; P=0.22), drug switches (OR=1.82; 95%-CI:0.69-4.78; P=0.22), and treatment discontinuations (OR=0.92; 95%-CI:0.60-1.41; P=0.70), higher odds of diarrhoea (OR=12.39; 95%-CI: 5.67-27.07; P<0.00001) and abnormal liver-function tests (OR=2.98; 95%-CI:1.92-4.61; P<0.00001), and lower odds of photosensitivity (OR=0.06; 95%-CI:0.01-0.25; P=0.0001), and skin-rash (OR=0.17; 95%-CI:0.08-0.34; P<0.00001).</p><p><strong>Conclusions: </strong>While both treatment groups had similar overall survival and all-cause mortality, the safety profiles of nintedanib and pirfenidone differed significantly, with nintedanib being associated with greater odds of liver toxicity and diarrhoea, and pirfenidone with photosensitivity and skin rash, suggesting they could be favoured in slightly distinct population groups. Further research is necessary to refine the current comprehension of these drugs and their optimal utilization in IPF treatment, particularly taking into account factors such as disease stage and sequential therapy.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16432"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536848/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202747","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}
引用次数: 0
Particles exposures and sarcoidosis. 颗粒暴露和结节病。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.16384
Andrew J Ghio, Spencer A Scott, Stephen L Tilley
{"title":"Particles exposures and sarcoidosis.","authors":"Andrew J Ghio, Spencer A Scott, Stephen L Tilley","doi":"10.36141/svdld.v42i3.16384","DOIUrl":"10.36141/svdld.v42i3.16384","url":null,"abstract":"<p><strong>Background: </strong>Sarcoidosis is a chronic disease characterized by the formation of non-caseating granulomas with the lung being the most frequently involved organ. The current diagnosis of sarcoidosis requires that the etiology be idiopathic with alternative causes of granulomatous disease excluded. The assessment that sarcoidosis has no known cause has been challenged by numerous case reports, case series, and epidemiologic studies supporting associations of this disease with recognized exposures to particles.</p><p><strong>Objective: </strong>The literature is reviewed to test for an association between particle exposures and a diagnosis of pulmonary sarcoidosis.</p><p><strong>Methods: </strong>A systematic review of the literature was performed using PubMed/MEDLINE, Web of Science, and TOXNET.</p><p><strong>Results: </strong>Particles can impact a development of granulomas comparable or identical to those in sarcoidosis patients. Prominent among these particles which participate in a pathogenesis of sarcoidosis are silicas and silicates, metals and metal oxides, and World Trade Center dust.</p><p><strong>Conclusions: </strong>Particle exposure is frequently associated with pulmonary sarcoidosis and should not preclude the diagnosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16384"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536831/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202687","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}
引用次数: 0
Impact of nutritional status in sarcoidosis patients. 营养状况对结节病患者的影响。
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG Pub Date : 2025-09-30 DOI: 10.36141/svdld.v42i3.16968
Şeyma Tunç, Pınar Yıldız, Mehmet Ali Sungur
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