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

{"title":"Medication adherence and real-world factors of first line induction treatment selection in ANCA-associated vasculitis.","authors":"Pınar Akyüz Dağlı, Esra Kayacan Erdoğan, Hatice Ecem Konak, Bahar Özdemir Ulusoy, Serdar Can Güven, Berkan Armağan, Rezan Koçak Ulucaköy, Kevser Orhan, Ebru Atalar, İsmail Doğan, Yüksel Maraş, Ahmet Omma, Orhan Küçükşahin, Şükran Erten, Hakan Babaoğlu","doi":"10.36141/svdld.v42i3.16880","DOIUrl":"10.36141/svdld.v42i3.16880","url":null,"abstract":"<p><strong>Background & objective: </strong>This study aimed to explore real-world factors that may influence the selection of first-line remission-induction therapy in ANCA-associated vasculitis(AAV), as well as to evaluate physician compliance with guideline recommendations and patient adherence to maintenance therapies.</p><p><strong>Methods: </strong> A retrospective analysis of 112 patients with AAV, including granulomatosis with polyangiitis(GPA, 67%), microscopic polyangiitis(MPA, 13%),and eosinophilic granulomatosis with polyangiitis (EGPA, 20%), was conducted at a single tertiary care center using electronic health records from 2018 to 2023. Treatment regimens, patient demographics, organ involvement, and adherence to EULAR guidelines were analyzed.Patients receiving RTX or CYC as first-line remission-induction therapy were compared.Compliance was defined as alignment with guideline-recommended dosing and timing of induction therapy, while adherence during maintenance was evaluated based on consistency with prescribed regimens, assessed via prescription refill data.</p><p><strong>Results: </strong>Of the 102 patients included in the study, 85 received CYC and 17 received RTX as first-line remission induction therapy.Compared to the RTX group, those receiving CYC were significantly older (median age 57 vs. 44 years, p <0.05), had higher BVAS scores (median 12 vs. 10, p = 0.02), and exhibited more comorbidities (74% vs. 35%). Organ involvement rates were similar in both groups. No significant differences in major organ involvement were observed between the two groups. RTX adherence was 100% in both the induction and maintenance phases, whereas adherence to oral maintenance therapies was notably lower, at 66% for methotrexate, 36% for mycophenolate mofetil, and 28% for azathioprine.</p><p><strong>Conclusion: </strong>In real-world practice, older age, higher BVAS, and a greater comorbidity burden appear to influence clinicians' preference for CYC over RTX as first-line induction,despite similar organ involvement between groups.Overall compliance with induction guidelines was high,but adherence to oral maintenance regimens remained suboptimal.These findings underscore the need for personalized treatment strategies and targeted measures to enhance long-term medication adherence in AAV.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16880"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536833/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202749","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":"WASOG Clinical Trial Endpoint Task Force: Executive Summary.","authors":"Robert P Baughman, Elyse E Lower, Daniel A Culver, Marc A Judson, Athol U Wells","doi":"10.36141/svdld.v42i3.17859","DOIUrl":"10.36141/svdld.v42i3.17859","url":null,"abstract":"<p><p>Over the past few years, an increased number of clinical trials have been performed evaluating specific therapies for pulmonary and cardiac sarcoidosis. However, a lack of consensus remains for appropriate clinical trial endpoints. In 2024, the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) established a clinical trial endpoint Task Force to update the 2011 WASOG Task Force. This initiative was spearheaded by five sarcoidosis specialists (RPB, EEL, DAC, MAJ, AUW) and enlisted a total of 55 stakeholders, including 37 health care providers, 14 industry representatives, and 4 patients. In March 2025, 46 stakeholders participated in a one-day meeting which included twenty focused talks regarding clinical trial endpoints for pulmonary and cardiac sarcoidosis trials. A compilation of individual talk summaries was prepared and distributed to all stakeholders, including those unable to attend the meeting. Based on feedback from all stakeholders, the team leaders developed a series of statements reflecting the presentations and discussions with subsequent anonymous voting by all stakeholders. The majority of the voters endorsed thirteen specific clinical trial endpoint statements: two evaluating overall trial design, seven regarding pulmonary sarcoidosis, and four discussing cardiac sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"17859"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536850/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202752","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":"Comparison of the effectiveness of pirfenidone and nintedanib in patients with advanced stage idiopathic pulmonary fibrosis.","authors":"Guliz Degirmenci, Celal Satici, Damla Azakli Yazici, Nazim Cetinkaya, Umut Erdem, Baris Demirkol, Aysegul Erinc","doi":"10.36141/svdld.v42i3.16849","DOIUrl":"10.36141/svdld.v42i3.16849","url":null,"abstract":"<p><strong>Background and aim: </strong>Idiopathic Pulmonary Fibrosis (IPF) is a progressive, fatal lung disease with high mortality, especially in advanced stages, defined as forced vital capacity (FVC) ≤50% and/or diffusing capacity of the lungs for carbon monoxide (DLCO) ≤30% of predicted values. While antifibrotic therapies, such as nintedanib and pirfenidone, are effective in mild to moderate IPF, their efficacy in advanced-stage IPF remains unclear due to the exclusion of these patients from major clinical trials.  This study aimed to compare the effectiveness of nintedanib and pirfenidone on mortality and to identify predictors of mortality in patients with advanced-stage IPF.</p><p><strong>Methods: </strong>This retrospective cohort study included 198 advanced-stage IPF patients treated with nintedanib (n=91) or pirfenidone (n=107) between 2017 and 2023. Demographic, clinical, and radiological data were collected. Mortality predictors were analyzed using multivariate Cox regression, and survival outcomes were compared using adjusted Kaplan-Meier curves.</p><p><strong>Results: </strong>Independent predictors of mortality were found to be six-minute walk distance (HR=0.99, 95% CI: 0.996-0.999, p=0.049), pulmonary fibrosis score ≥150 (HR=1.86, 95% CI: 1.07-3.22, p=0.026), and heart failure (HR=2.24, 95% CI: 1.18-4.26, p=0.014). After adjusting for these factors, no significant differences in mortality were observed between the nintedanib and pirfenidone groups.</p><p><strong>Conclusions: </strong>Nintedanib and pirfenidone showed comparable effectiveness in reducing mortality among advanced-stage IPF patients. Future clinical trials should include advanced-stage patients to better evaluate treatment efficacy in this population.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16849"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536837/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202674","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":"Association of non-linear dynamics of heart rate variability with all-cause mortality in patients with sarcoidosis.","authors":"Elias Giallafos, Ioannis Ilias, Spiros Katsanos, Likourgos Kolilekas, Efrosini Manali, Aglaia Vakrakou, Theodore Papaioannou, Kostas Zisimos, Gethsimani Seitaridi, Giannis Parisis, Nikos Koulouris, Spiros Papiris","doi":"10.36141/svdld.v42i3.16332","DOIUrl":"10.36141/svdld.v42i3.16332","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis (Sarc) is linked to increased morbidity and mortality. While 24-hour ambulatory ECG recording is essential for evaluating patients with cardiac symptoms, the complementary role of heart rate variability (HRV) for risk stratification in Sarc patients remains undefined. The aim of this observational study was the assessment of non-linear indices of HRV as independent predictors of mortality in Sarc.</p><p><strong>Methods: </strong>317 patients with confirmed diagnosis of Sarc, underwent comprehensive conventional cardiac testing, including Cardiac Magnetic Resonance (CMR) with late gadolinium enhancement (LGE). These patients were followed up for all-cause mortality as endpoint. Each patient underwent twenty-four-hour Holter monitoring, from which approximate entropy (APEN), short and long-term detrended fluctuation analysis (DFAα1 and DFAα2, respectively), as well as other HRV parameters, were calculated.</p><p><strong>Results: </strong>The study recruited 317 patients (137 men, 180 women) with a mean age of 48.22 ± 11.88 years. Over a median follow-up period of approximately 48 months, 17 deaths were recorded. Competing risk analysis revealed that decreased DFAα1 was a robust prognostic indicator for increased total mortality. ROC analysis demonstrated that the area under the curve (AUC) of DFAα1 (< 0.95) for predicting mortality was 0.761 (95% confidence interval (CI) = 0.617-0.905). DFAα1 ≥ 0.95 was associated with total mortality (HR = 0.109, 95% CI = 0.033-0.362, P= 0.0003).</p><p><strong>Conclusion: </strong>Evaluation of cardiac autonomic dysfunction by DFAα1 serves as an independent predictor for all-cause mortality in patients with Sarc.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16332"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536836/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202721","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":"Classic imaging in cardiac sarcoidosis.","authors":"Apoorwa Thati, Erika Hutt, Christine Jellis, Manuel Ribeiro Neto","doi":"10.36141/svdld.v42i3.16548","DOIUrl":"10.36141/svdld.v42i3.16548","url":null,"abstract":"","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16548"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536838/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202689","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":"Cardiac sarcoidosis recurrence post-heart transplant: A critical literature and case report.","authors":"Andrea Salotti, Lucrezia Mondini, Paola Confalonieri, Francesco Salton, Chiara Nalli, Nicolò Reccardini, Chiara Torregiani, Caterina Antonaglia, Michael Hughes, Marco Confalonieri, Rossella Cifaldi, Barbara Ruaro","doi":"10.36141/svdld.v42i3.16865","DOIUrl":"10.36141/svdld.v42i3.16865","url":null,"abstract":"<p><strong>Background and aim: </strong>Recurrence of cardiac sarcoidosis (CS) following heart transplantation (HT) is a rare but clinically significant complication that influences management strategies and prognosis. This review examines existing evidence on the diagnosis and treatment of post-transplant CS recurrence. Additionally, we present a case report of CS recurrence in a patient after HT.</p><p><strong>Methods: </strong>We analyzed clinical studies, case reports, and systematic reviews published up to January 2025, focusing on the recurrence of CS after transplantation.</p><p><strong>Results: </strong>Limited data suggest that immunosuppressive therapies are crucial in managing post-transplant CS recurrence. Diagnosis relies on imaging modalities such as Positron Emission Tomography (PET-CT), Cardiac Magnetic Resonance (CMR), and endomyocardial biopsies. Treatment generally involves intensifying immunosuppressive therapy.</p><p><strong>Conclusions: </strong>Standardized guidelines are essential to improve the management of this rare and complex condition and to enhance long-term patient outcomes.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16865"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536832/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202723","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":"Clinical and radiological factors affecting progression in sarcoidosis.","authors":"Olcay Aycicek, Ensar Cihat Emiroglu, Kadir Coban, Muge Erbay, Tevfik Ozlu, Funda Oztuna, Yılmaz Bulbul","doi":"10.36141/svdld.v42i3.16820","DOIUrl":"10.36141/svdld.v42i3.16820","url":null,"abstract":"<p><strong>Background and aim: </strong>To determine the clinical and radiological indicators of disease progression in a newly diagnosed sarcoidosis patient.</p><p><strong>Methods: </strong>The data of patients who were diagnosed with sarcoidosis in our department between January 2014 and June 2022 were analyzed. The patients were divided into two groups: progression and non-progression. Groups were compared according to symptoms at the time of diagnosis, comorbidities, foci of extrapulmonary involvement, shape, size, density, and localization of mediastinal lymph nodes, parenchymal findings, disease stage  and whether or not treatment was received at the beginning.</p><p><strong>Results: </strong>The study included 292 sarcoidosis patients. The number of patients who progressed was 46 (15.8%). It was observed that stage II patients progressed more than stage I patients (p<0,001). The mean time to progression was 38.05±30.45 months in stage I and 28±58 months in stage II patients. Progression rate was higher in patients with right upper paratracheal, subaortic-paraaortic, subcarinal LAP (p= 0.010, p= 0.012, p= 0.020, respectively).  The higher number of stations with LAP was associated with the presence of progression (p=0.017). The presence of parenchymal nodules (29/64.4%) and the number of lobes with nodules was also associated with progression (p= 0.027, 0.022, respectively). Progression rate was 76.1% in patients with treatment indication at the time of diagnosis (p<0.001).</p><p><strong>Conclusions: </strong>Disease stage is a prognostically important factor in the course of sarcoidosis, which is supported by our study results. Accordingly, it is important to follow patients with high-stage sarcoidosis more closely and to identify patients with treatment indications in time.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16820"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536830/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202710","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":"A randomized trial of narrow band imaging for performing airway mucosal biopsy in pulmonary sarcoidosis (NABS).","authors":"Kritarth Rathi, Pooja Dogra, Ritesh Agarwal, Inderpaul Singh Sehgal, Valliappan Muthu, Kuruswamy Thurai Prasad, Ashutosh Nath Aggarwal, Amanjit Bal, Nalini Gupta, Sahajal Dhooria","doi":"10.36141/svdld.v42i3.16418","DOIUrl":"10.36141/svdld.v42i3.16418","url":null,"abstract":"<p><strong>Background: </strong>Data suggest that narrow band imaging (NBI) highlights airway mucosal nodules better than white light bronchoscopy (WLB) in sarcoidosis. No randomized trial has compared the diagnostic yield of endobronchial biopsy (EBB) performed using NBI versus WLB.</p><p><strong>Methods: </strong>We performed an investigator-initiated single-center, parallel-arm, randomized trial. Consecutive subjects ≥18 years of age with clinico-radiologic presentation consistent with sarcoidosis were randomised 1:1 to undergo EBB using NBI plus WLB (intervention) or WLB (control).</p><p><strong>Outcomes: </strong>The primary outcome was the diagnostic yield of EBB defined by finding of granulomas on pathologic examination in subjects with final diagnosed with sarcoidosis. The secondary outcomes included procedure duration and complications.</p><p><strong>Results: </strong>We included 150 (mean age, 43.1 years; 53.3% men) subjects; 75 each were assigned to either study group. Sarcoidosis was diagnosed in 126/150 subjects (66 and 60 in the NBI plus WLB and WLB groups, respectively). There was no difference (p=0.53) in the diagnostic yield of EBB between the NBI plus WLB (25/66, 37.9%) and the WLB (26/60, 43.3%) groups. Transbronchial lung biopsy and endobronchial ultrasound-guided transbronchial needle aspiration yielded granulomas in 69 of 115 (60%) and 68 of 98 (69.4%) subjects, who underwent these additional procedures, respectively. In 11 of the 14 (78.6%) cases, where WLB demonstrated airway nodules, NBI enhanced visualization. Moreover, it identified nodules in five additional cases. There were no between-group differences in the procedure duration or complications.</p><p><strong>Conclusions: </strong>The use of NBI did not improve the yield of EBB in patients with sarcoidosis. Additional research is warranted. (Clinicaltrials.gov: NCT05311150).</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16418"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536843/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202699","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":"Eosinophil-associated disease after SARS-CoV-2 infection or vaccination: EGPA and related entities.","authors":"Ghadah A Alrehaili, Karina A Keogh, Misbah Baqir","doi":"10.36141/svdld.v42i3.16598","DOIUrl":"10.36141/svdld.v42i3.16598","url":null,"abstract":"<p><p>N/A.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"16598"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536829/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202712","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":"Americas Association of Sarcoidosis and other granulomatous disorders 2024 conference highlights.","authors":"Kristen Mathias, Anjali Wagle, Susana Dominguez-Peñuela, Carlos Pardo, Michelle Sharp, Nisha Gilotra","doi":"10.36141/svdld.v42i3.17197","DOIUrl":"10.36141/svdld.v42i3.17197","url":null,"abstract":"<p><p>The Americas Association of Sarcoidosis and Other Granulomatous Disorders (AASOG) 2024 conference, held in Baltimore, Maryland, leveraged a multidisciplinary approach to disseminating and addressing the latest updates, challenges and opportunities in multisystemic sarcoidosis. The conference, aptly titled \"The Art of Working Together for Progress,\" featured insights from diverse perspectives in sarcoidosis both nationally and internationally. This review summarizes the key takeaways from the six conference sessions: I. Sarcoidosis Multidisciplinary Care, II. Health Disparities in Sarcoidosis, III. The Search for Precision in Sarcoidosis, IV. Clinical Outcomes in Sarcoidosis, V. Clinical Trials in Sarcoidosis, and VI. Advanced Disease in Sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 3","pages":"17197"},"PeriodicalIF":0.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12536846/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145202668","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}