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

{"title":"IL17 in Sarcoidosis - A level playing field?","authors":"Deepak Nagra, Hassan Choudry, Joy Odia, Akshay Balaji, Katie Bechman, Mark Russell, Maryam Adas, Zijing Yang, Chamith Rosa, Siwalik Banerjee, Tim Blake, Nicola Gullick, James Galloway","doi":"10.36141/svdld.v42i2.16288","DOIUrl":"10.36141/svdld.v42i2.16288","url":null,"abstract":"<p><p></p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16288"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242868/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693020","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":"EBUS/EUS-guided lymph nodal gadolinium injection and MRI of the chest in diagnosis of abnormal lymphatic drainage. A case series.","authors":"Venerino Poletti, Simone Petrarulo, Emanuela Giampalma, Claudia Ravaglia, Sara Piciucchi","doi":"10.36141/svdld.v42i2.16539","DOIUrl":"10.36141/svdld.v42i2.16539","url":null,"abstract":"<p><p>Impaired lymphatic circulation in the mediastinum may result from congenital disorders, leakage secondary to trauma, or infiltration by neoplasia. It manifests as chylothorax, plastic bronchitis, and/or alveolar fatty pneumonitis. Lymphography, involving the injection of contrast medium into peripheral lymph nodes, was previously utilised but posed challenges in execution and image interpretation. We describe two patients admitted to our department with relapsing chylothorax and respiratory failure associated with \"fatty\" pneumonitis and plastic bronchitis. The final diagnosis was confirmed by injecting 2 mL of gadolinium into the mediastinal lymph nodes under endoscopic ultrasonography guidance (EBUS), and magnetic resonance imaging (MRI) visualised its distribution.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16539"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242876/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693017","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":"Mental health burden in progressive pulmonary fibrosis and idiopathic pulmonary fibrosis: Depression and anxiety.","authors":"Jovan Javorac, Dejan Živanović, Miljana Miladinović, Andrijana Mikić, Vesna Dukanac, Miroslav Ilić, Ana Milenković, Sonja Peričević Medić, Tamara Popović, Svetlana Kašiković Lečić","doi":"10.36141/svdld.v42i2.16342","DOIUrl":"10.36141/svdld.v42i2.16342","url":null,"abstract":"<p><strong>Background and aim: </strong>Progressive pulmonary fibrosis (PPF) is an umbrella term for several interstitial lung diseases characterized by the progressive proliferation of fibrous tissue within the lung interstitium. This study aimed to assess the prevalence of depressive and anxiety symptoms among PPF patients and their repercussions on quality of life.</p><p><strong>Methods: </strong>Thirty-seven PPF patients undergoing treatment at the Institute for Pulmonary Diseases of Vojvodina, Serbia were enrolled. The SGRQ questionnaire assessed quality of life, while the DASS-21 questionnaire evaluated anxiety and depression symptoms. Sociodemographic and clinical factors were correlated with questionnaires outcomes, and the influence of anxiety and depression symptoms on quality of life was examined.</p><p><strong>Results: </strong>Anxiety symptoms were detected in 56.75% of PPF patients and were more prevalent in patients previously diagnosed with depression and/or anxiety. Depression symptoms were detected in 45.95% of PPF patients and were more pronounced in the patients with longer duration of PPF, and positive history of malignancies. Stress symptoms were linked to lower PaO2 values and were detected in 37.84% of participants. HRQL was moderately to severely decreased and was lower in patients who, in addition to PPF, also suffered from other respiratory diseases, and was statistically significantly associated with lower DLCO values. A statistically significant correlation has been proven between the presence of anxiety, depression, and stress symptoms and HRQL, as measured through the \"Symptoms\" and \"Impact\" subscales of the SGRQ questionnaire.</p><p><strong>Conclusions: </strong>Anxiety and depression symptoms are highly prevalent among PPF patients, exerting substantial impacts on their quality of life.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16342"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242862/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693022","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":"Advanced interstitial lung disease: Evidence-based management and clinical approach.","authors":"Zein Kattih, Zachary Greenstein, Priyanka Makkar, Rohit Gupta","doi":"10.36141/svdld.v42i2.16206","DOIUrl":"10.36141/svdld.v42i2.16206","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease (ILD) is a heterogeneous group of diseases characterized by clinical, physiologic, and radiologic abnormalities. Advanced ILD (sometimes referred to as end-stage interstitial lung disease) is characterized by either gradual or rapid progressive decline of pre-existing or newly diagnosed ILD with worsening of symptoms, pulmonary function tests, six-minute walk test (6MWT) distance, and/or imaging findings, specifically high-resolution computed tomography (HRCT) imaging of the chest affecting patient's quality of life. Special considerations are required for the management of patients with these advanced ILDs, particularly those who will ultimately require lung transplantation. Though several society and expert panels provide guidance on ILD management, there are none focused specifically on the approach to patients with advanced ILD.</p><p><strong>Objectives: </strong>In this review, we attempt to define advanced ILD and identify the clinical considerations required for diagnosis and management of patients with advanced ILD.</p><p><strong>Discussion: </strong>Pathophysiologic mechanisms of ILD and complications are not completely understood but include inflammation, fibrosis, pulmonary hypertension, venous thromboembolism, recurrent infections, and aspiration. Management of these patients includes consideration of anti-fibrotic medications, anti-inflammatory/immunosuppressive medications and treatment of concurrent pulmonary hypertension as indicated on a case-by-case basis. Non-pharmacologic management with supplemental oxygen, pulmonary rehabilitation, and palliative care are tailored to each patient's clinical status. Finally, early referral to lung transplantation is critical, as advanced ILD can be fatal, and sometimes rapidly so.</p><p><strong>Conclusion: </strong>Advanced ILD is an oftentimes fatal group of heterogeneous diseases that have varying managements depending on underlying subtype. Identification of progressive disease and complications are the first steps to management of these patients to optimize lung function and quality of life and improve outcomes before lung transplantation.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16206"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242865/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693014","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":"Chest radiography in interstitial lung disease: Accuracy and radiological features from a systematic review and meta-analysis.","authors":"Ruaa Mustafa Qafesha, Mahmoud Diaa Hindawi, Menna Elbadry, Israa Sharabati, Doaa Mashaly, Muhiddin Dervis, Qasi Najah, Muataz Kashbour","doi":"10.36141/svdld.v42i2.16108","DOIUrl":"10.36141/svdld.v42i2.16108","url":null,"abstract":"<p><strong>Background: </strong>This systematic review aims to summarize and evaluate the diagnostic performance of chest X-ray (CXR) compared with high-resolution computed tomography (HRCT) for detecting interstitial lung disease (ILD).</p><p><strong>Research question: </strong>what is the diagnostic accuracy and radiological finding of CXR in ILD, using HRCT as a gold standard.</p><p><strong>Study design and method: </strong>We systematically searched electronic databases to find studies evaluating the diagnostic accuracy of CXR and HRCT for detecting ILD. We used StataMP/17 and R statistical software for the quantitative analysis. Values like pooled sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, diagnostic odds ratio with their 95% confidence interval, and the SROC curve was performed.</p><p><strong>Results: </strong>We included a total of 18 studies involving 1917 patients. Compared to HRCT, CXR had sensitivity of 0.62 (95% CI: 0.47-0.74), specificity of 0.90 (95% CI: 0.85 - 0.93), positive likelihood ratio of 5.9 (95% CI 4.5-7.7), negative likelihood ratio of 0.43 (95% CI 0.31, 0.59), diagnostic odds ratio of 14 (95% CI 9 - 21), and area under the ROC curve of 0.88 (95% CI 0.85-0.91). Deek's plot showed no publication bias (p= 0.44). CXR had lower sensitivity compared with HRCT in detecting specific radiologic findings. Subgroup analysis revealed that a patient sample surpassing 100 indicated significantly higher specificity.</p><p><strong>Conclusion: </strong>Chest radiography exhibits moderate sensitivity and high specificity for detecting ILD when HRCT is regarded as the gold standard test. Although CXR is recommended as an initial diagnostic tool, it should not be solely relied upon for a definitive diagnosis, as it might miss some cases.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16108"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242875/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693016","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":"Homocysteine and risk of interstitial lung disease: A mendelian randomization approach to causal inference.","authors":"Shimin Li, Xiaochen Hu, Xin Hu, Hang Liu, Shanshan Meng","doi":"10.36141/svdld.v42i2.16386","DOIUrl":"10.36141/svdld.v42i2.16386","url":null,"abstract":"<p><strong>Background and aim: </strong>Homocysteine (Hcy) has been implicated in inflammatory, oxidative stress (OS), and endoplasmic reticulum (ER) stress mechanisms, which are hypothesized to contribute to the pathogenesis of interstitial lung disease (ILD). Given the paucity of evidence regarding Hcy's role in ILD, a two-sample Mendelian randomization study was performed to investigate the causal association between Hcy and ILD.</p><p><strong>Methods: </strong>We sourced data for total plasma Hcy from genome-wide association studies (GWAS) involving 44,147 European individuals. Data for ILD, idiopathic pulmonary fibrosis (IPF), IPF-related respiratory insufficiency, and systemic autoimmune disease-associated IPF were derived from the FinnGen consortium. To evaluate the causal association of reduced total plasma Hcy with ILD and related conditions, a range of Mendelian randomization (MR) analytical techniques were utilized to analyze the data. The results are reported as odds ratios (ORs) with corresponding 95% confidence intervals (CIs). We conducted sensitivity analyses through leave-one-out procedures and Radial MR plots.</p><p><strong>Results: </strong>Our IVW estimates suggested that total plasma Hcy had a potential causal association with IPF (OR=0.649, 95%CI: 0.495-0.851), indicating that along with total plasma Hcy depressed 1 µmol/L, odds of IPF decreased 0.351. Although it seemed that decreased total plasma Hcy level is associated with lower odds of IPF-related respiratory insufficiency (OR=0.672, 95%CI: 0.489-0.924), due to the existence of horizontal pleiotropy, this causal association was not robust. In addition, MR leave-one-out and Radial MR sensitivity analyses showed there is no outlier among the selected IVs that could affect the potential causal relationship between Hcy and IPF.</p><p><strong>Conclusions: </strong>The levels of total plasma Hcy may bear a significant association with the risk of developing IPF, a specific form of ILD. However, to definitively establish a causal relationship between elevated Hcy levels and the pathogenesis of ILD, additional well-controlled, prospective studies are indispensable.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16386"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242866/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693018","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":"Time and frequency domain assessment of heart rate variability in patients with pulmonary sarcoidosis.","authors":"Pier-Valerio Mari, Veronica Melita, Angelo Coppola, Matteo Siciliano, Francesco Macagno, Giuliana Pasciuto","doi":"10.36141/svdld.v42i2.16408","DOIUrl":"10.36141/svdld.v42i2.16408","url":null,"abstract":"<p><strong>Background and aim: </strong>The association between sarcoidosis and autonomic dysfunction is demonstrated but poorly known. Heart rate variability (HRV) studies can provide a simple, non-invasive analysis of sympathetic and vagal tone in sarcoidosis. To date, the burden of steroid treatment on HRV has not been investigated. We aim to compare the HRV in sarcoidosis with general population and to explorate the burden of steroid treatment in HRV.</p><p><strong>Methods: </strong>Prospective analysis of 30 patients enrolled in Sarcoidosis Clinic of Policlinico Gemelli Hospital compared to 72 healthy control subjects. Continuous EKG recording over 24 hours has been performed and HRV was assessed using time and frequency domain analysis. Results were evaluated using a propensity score matching 1:1.</p><p><strong>Results: </strong>Patients affected by sarcoidosis presented a mean age of 59.7±10.1 y and female gender predominance (n=20, 71.4%) %) while the control group was 54.6±16.0 y. The HRV analysis outlined a remarkable difference when both time / frequency domain were compared to the general, healthy population (ΔSDNN: 89.4±6.7 p=0.0001; ΔrMSSD: 5.7±2.6 p=0.03; ΔLF: 497.0±142.3 p=0.0007; ΔHF: 325.0±159.5 p=0.0442) suggesting a significant impact of sarcoidosis in autonomic activation. Remarkably the lung function tests and scadding stage did not show any relationship with the HRV (p=NS). At the same time, steroid treatment of ≥10 mg/day of prednisone for the previous 3 months was associated with a significant reduction of HRV in time (SDNN: Δ17.2±6.9, p=0.020) and frequency domain (LF: Δ355.2±125.7, p=0.009 and HF: Δ116.2±65.2, p=0.087). Lastly, the propensity score matching did confirm the previous results regarding time/frequency domain analysis.</p><p><strong>Conclusions: </strong>HRV is an effective tool for the autonomic evaluation in patients affected by sarcoidosis. The time (r-MSSD, SDNN, pNN50%) and frequency domain (HF/LF mean) analysis in sarcoidosis suggested that autonomic dysfunction is not related to the lung function or scadding stage though steroid treatment may impact.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16408"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242870/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693042","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":"Interstitial lung disease hospitalization patterns between U.S. states from 2016-2020.","authors":"Eva Ma, Niranjan Jeganathan","doi":"10.36141/svdld.v42i2.16312","DOIUrl":"10.36141/svdld.v42i2.16312","url":null,"abstract":"","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16312"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693021","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":"Pulmonary sarcoidosis with dendriform ossification: Diagnostic and pathological insights.","authors":"Hannah Smith, Konstantin Shilo, Arindam Singha","doi":"10.36141/svdld.v42i2.16126","DOIUrl":"10.36141/svdld.v42i2.16126","url":null,"abstract":"<p><p>Pulmonary ossification is the histologic finding of mature bone with or without marrow elements within pulmonary tissue. The mechanism of formation is incompletely understood but thought to include metaplastic transformation of pulmonary fibroblasts to osteoblasts secondary to some form of chronic insult and the resulting inflammation from this insult. There are two classifications of pulmonary ossification, nodular and diffuse, based on histologic features. Management of pulmonary ossification is typically supportive, with treatment of the underlying process if one is identified. Here, we describe a man with known pulmonary sarcoidosis previously considered stable who presented with worsening dyspnea and was found to have pulmonary ossification and active cardiac and pulmonary sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16126"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242873/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693037","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":"Response to corticosteroids and alternative therapies in sarcoidosis-related hypercalcemia: A guideline-lacking retrospective analysis.","authors":"Drew Robinson, Denver Crumpler, Guihua Zhai, Joseph Barney","doi":"10.36141/svdld.v42i2.16239","DOIUrl":"10.36141/svdld.v42i2.16239","url":null,"abstract":"<p><strong>Background and aim: </strong> Sarcoidosis is a systemic inflammatory disease of unknown etiology that can affect multiple organs including a known complication of hypercalcemia. Many therapies, both steroidal and nonsteroidal, have been employed. However, the optimal therapy for hypercalcemia in sarcoidosis is unknown, with most data from case reports and small case series. More information is needed to determine which nonsteroidal agents control hypercalcemia in sarcoidosis patients, particularly for populations in whom steroids should be avoided. We sought to answer the question, which pharmacological agents control hypercalcemia in patients diagnosed with sarcoidosis?</p><p><strong>Methods: </strong> We performed a retrospective chart review on all adult patients at our hospital a diagnosis of Sarcoidosis and Hypercalcemia and a recorded calcium level of greater than 10.4 mg/dL. We then determined which agents were able to control their calcium to a level of 10.4 mg/dL or less for at least 6 months and 12 months.</p><p><strong>Results: </strong> The most common efficacious treatments were prednisone, methotrexate, hydroxychloroquine, and prednisone + methotrexate. A positive linear relationship was found between ACE and highest calcium levels. No significant relationship between organ involvement or race and highest calcium levels were noted.</p><p><strong>Conclusions: </strong> There is currently little data or guidelines to best guide the treatment of hypercalcemia in sarcoidosis. This study represents the first with the primary aim to compare treatment options for hypercalcemia in sarcoidosis across a large cohort. Several non-steroidal options were identified that controlled hypercalcemia as a single agent. In addition, this study further investigated potential biomarkers for abnormal calcium metabolism as well as the relationship between calcium levels and organ involvement/race. Further work is needed to determine the most effective steroid sparing therapy to control hypercalcemia in sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 2","pages":"16239"},"PeriodicalIF":0.0,"publicationDate":"2025-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12242867/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144693038","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}