Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

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Identifying a core outcome set for pulmonary sarcoidosis research - the Foundation for Sarcoidosis Research - Sarcoidosis Clinical OUtcomes Taskforce (SCOUT). 确定肺结节病研究的核心结果集-结节病研究基金会-结节病临床结果工作组(SCOUT)。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 DOI: 10.36141/svdld.v39i3.12319
Nicola L Harman, Sarah L Gorst, Paula R Williamson, Elliot S Barnathan, Robert P Baughman, Marc A Judson, Heidi Junk, Nynke A Kampstra, Eugene J Sullivan, David E Victorson, Marc K Walton, Tamara Al-Hakim, Hana Nabulsi, Noopur Singh, Jan C Grutters, Daniel A Culver
{"title":"Identifying a core outcome set for pulmonary sarcoidosis research - the Foundation for Sarcoidosis Research - Sarcoidosis Clinical OUtcomes Taskforce (SCOUT).","authors":"Nicola L Harman,&nbsp;Sarah L Gorst,&nbsp;Paula R Williamson,&nbsp;Elliot S Barnathan,&nbsp;Robert P Baughman,&nbsp;Marc A Judson,&nbsp;Heidi Junk,&nbsp;Nynke A Kampstra,&nbsp;Eugene J Sullivan,&nbsp;David E Victorson,&nbsp;Marc K Walton,&nbsp;Tamara Al-Hakim,&nbsp;Hana Nabulsi,&nbsp;Noopur Singh,&nbsp;Jan C Grutters,&nbsp;Daniel A Culver","doi":"10.36141/svdld.v39i3.12319","DOIUrl":"https://doi.org/10.36141/svdld.v39i3.12319","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary sarcoidosis is a rare granulomatous disease of unknown aetiology. Heterogeneity in the outcomes measured in trials of treatment for pulmonary sarcoidosis has impacted on the ability to systematically compare findings, contributing to research inefficiency. The FSR-SCOUT study has aimed to address this heterogeneity by developing a core outcome set that represents a patient and health professional consensus on the most important outcomes to measure in future research for the treatment of pulmonary sarcoidosis.</p><p><strong>Research design and methods: </strong>systematic review of trial registries, narrative synthesis of published qualitative literature on the patient experience and results of a patient survey contributed to the development of a comprehensive list of outcomes that were rated in a two round online Delphi survey. The Delphi survey was completed by patients/carers and health professionals and the results discussed and ratified at an online consensus meeting.</p><p><strong>Results: </strong>259 patients/carers and 51 health professionals completed both rounds of the Delphi survey. A pre-agreed definition of consensus was applied and the results discussed at an online consensus meeting attended by 17 patients and 7 health professionals). Fifteen outcomes, across five domains (physiological/clinical, treatment, resource use, quality of life, and death), reached the definition of consensus and were included in the core outcome set.</p><p><strong>Conclusions: </strong>The core outcome set represents a patient and health professional consensus on the most important outcomes for pulmonary sarcoidosis research. The use of the core outcome set in future trials, and efforts to validate its components, will enhance the relevance of trials to stakeholders and will increase the opportunity for the research to contribute to evidence synthesis.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b7/a8/SVDLD-39-30.PMC9766848.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10739109","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Controversies in the Treatment of Cardiac Sarcoidosis. 心脏结节病治疗的争议。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-06-29 DOI: 10.36141/svdld.v39i2.13136
Ogugua Ndili Obi, Elyse E Lower, Robert P Baughman
{"title":"Controversies in the Treatment of Cardiac Sarcoidosis.","authors":"Ogugua Ndili Obi,&nbsp;Elyse E Lower,&nbsp;Robert P Baughman","doi":"10.36141/svdld.v39i2.13136","DOIUrl":"https://doi.org/10.36141/svdld.v39i2.13136","url":null,"abstract":"<p><p>There are many challenging aspects of the management of cardiac sarcoidosis (CS) with corticosteroids and other immunosuppressive therapy (IST). First, it is not always clear who will benefit from therapy or when to initiate treatment. Secondly, there are no randomized controlled trials or large prospective studies to guide what medications to use, at what doses, and for how long. The European Respiratory Society (ERS) clinical practice guidelines on the treatment of sarcoidosis makes a strong recommendation for the use of immuno-suppressive therapy in CS patients with functional cardiac abnormalities, including heart blocks, dysrhythmias, or cardiomyopathy where patients are considered at-risk of adverse outcomes. Corticosteroids are the first line immunosuppressive therapy in CS however, early initiation of second-line steroid sparing medications has been advocated and there is data to suggest that concomitant initiation of therapy may be more beneficial. The use of anti-tumor necrosis factor (anti-TNF) agents (including infliximab and adalimumab) considered beneficial third-line anti-sarcoidosis treatment agents in other severe refractory manifestations of disease remains controversial.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437759/pdf/SVDLD-39-015.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40366394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Phenotypic clusters and survival analyses in interstitial pneumonia with myositis-specific autoantibodies. 伴有肌炎特异性自身抗体的间质性肺炎的表型聚类和生存分析。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-01-13 DOI: 10.36141/svdld.v38i4.11368
Yihua Lia, Yali Fana, Yuanying Wanga, Shuqiao Yanga, Xuqin Dua, Qiao Yea
{"title":"Phenotypic clusters and survival analyses in interstitial pneumonia with myositis-specific autoantibodies.","authors":"Yihua Lia,&nbsp;Yali Fana,&nbsp;Yuanying Wanga,&nbsp;Shuqiao Yanga,&nbsp;Xuqin Dua,&nbsp;Qiao Yea","doi":"10.36141/svdld.v38i4.11368","DOIUrl":"https://doi.org/10.36141/svdld.v38i4.11368","url":null,"abstract":"<p><strong>Background: </strong>Idiopathic inflammatory myopathy (IIM) is highly combined with interstitial pneumonia (IP), often as the initial or solo presentation with positive myositis-specific autoantibodies (MSAs) but does not fulfill the diagnostic criteria.</p><p><strong>Objectives: </strong>We aimed to explore the phenotypic clusters and prognosis of the patients with IP and positive MSA, which is called MSA-IP in the present study.</p><p><strong>Methods: </strong>A total of 178 patients with MSA-IP were prospectively enrolled for analysis. Serum MSAs were detected using Western blotting. Radiological patterns of IP were determined according to the classification of idiopathic IPs. Clusters of patients with MSA-IP were identified using cluster analysis. Predictors for acute/subacute onset, therapeutic response, IP progression and survival were also analyzed.</p><p><strong>Results: </strong>Patients with MSA-IP were classified into four distinct clusters. Cluster 1 were the elderly with chronic onset, nearly normal oxygenation and good survival. Cluster 2 had dyspnea on exertion and nonspecific IP pattern, with moderate survival. Patients in cluster 3 had chronic onset and were prone to IP progression (OR 2.885). Cluster 4 had multi-systemic involvements, positive anti-melanoma differentiation associated gene 5 antibody, and were prone to acute/subacute onset (OR 3.538) and IP progression (OR 5.472), with poor survival. Corticosteroids combined immunosuppressants showed therapeutic response in MSA-IP (OR 4.303) and had a protective effect on IP progression (OR 0.136).</p><p><strong>Conclusions: </strong>Four clusters of the patients with MSA-IP suggested the distinct clinical, radiological and prognostic features.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8a/37/SVDLD-38-47.PMC8787374.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39762876","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
CT Imaging Characteristics of Nontuberculous Mycobacteria Lung Disease, Active Tuberculosis and Multi-Drug Resistant Tuberculosis. 非结核分枝杆菌肺病、活动性肺结核和耐多药肺结核的 CT 影像特征
IF 1.4 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-06-29 DOI: 10.36141/svdld.v39i2.11829
Liang Xu, Shuangmei Xu
{"title":"CT Imaging Characteristics of Nontuberculous Mycobacteria Lung Disease, Active Tuberculosis and Multi-Drug Resistant Tuberculosis.","authors":"Liang Xu, Shuangmei Xu","doi":"10.36141/svdld.v39i2.11829","DOIUrl":"10.36141/svdld.v39i2.11829","url":null,"abstract":"<p><strong>Background: </strong>The differential diagnosis of nontuberculous mycobacteria (NTM) lung disease, active tuberculosis (ATB) and multi-drug resistant tuberculosis (MDR-TB) remains difficult.</p><p><strong>Objectives: </strong>To explore the CT imaging characteristics of NTM lung disease, ATB and MDR-TB for differential diagnosis.</p><p><strong>Methods: </strong>Patients with NTM lung disease (n=200), ATB (n=200) and MDR-TB (n=200) who were examined and treated from August 2013 to May 2021 were included. Their chest CT imaging results were retrospectively analyzed, and the imaging characteristics were compared.</p><p><strong>Results: </strong>The proportion of cases complicated with underlying lung disease, cough and hemoptysis was significantly higher in NTM group than those in ATB and MDR-TB groups (P<0.05). Compared with ATB and MDR-TB groups, NTM group had significantly more cases of nodule-bronchus dilation type, but significantly fewer cases of nodule-mass type and other types (P<0.05). In NTM group, the cases of thin-wall cavity, bronchiectasis and centrilobular nodules increased, but the detection rate of thick-wall cavity, lung consolidation, atelectasis, lung damage, lung volume reduction, intrapulmonary calcification, hilar and mediastinal lymph node calcification, acinar nodules, pleural thickening and pleural effusion declined compared with ATB and MDR-TB groups (P<0.05). The detection rates of lesions, cavities and bronchiectasis in the lingual lobe of left lung and middle lobe of right lung were significantly higher in NTM group than those in ATB and MDR-TB groups (P<0.05).</p><p><strong>Conclusions: </strong>The imaging characteristics of NTM lung disease are quite similar to those of ATB and MDR-TB, but they can be differentially diagnosed through the types of cavities and nodules, incidence rate of bronchiectasis, and differences in lung consolidation, lung damage, calcification, pleural thickening and pleural effusion.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437753/pdf/SVDLD-39-008.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40366387","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Tackling Health Care Disparities: How to Build a Sarcoidosis Center. 解决医疗保健差距:如何建立一个结节病中心。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 DOI: 10.36141/svdld.v39i3.13423
Jordana Kron, Aamer Syed, Thomas Iden, Kelly Gwathmey, Kelly Polly, Jessica Randolph, Vikram Brar, Patrick Nana-Sinkam, Kenneth Ellenbogen, Huzaefah Syed
{"title":"Tackling Health Care Disparities: How to Build a Sarcoidosis Center.","authors":"Jordana Kron,&nbsp;Aamer Syed,&nbsp;Thomas Iden,&nbsp;Kelly Gwathmey,&nbsp;Kelly Polly,&nbsp;Jessica Randolph,&nbsp;Vikram Brar,&nbsp;Patrick Nana-Sinkam,&nbsp;Kenneth Ellenbogen,&nbsp;Huzaefah Syed","doi":"10.36141/svdld.v39i3.13423","DOIUrl":"https://doi.org/10.36141/svdld.v39i3.13423","url":null,"abstract":"<p><p>Sarcoidosis is a multi-organ system inflammatory disease of unknown etiology that disproportionately affects women and black patients in the United States. In addition, woman and minority patients have worse outcomes. In 2015, sarcoidosis physicians in cardiology, pulmonary medicine and rheumatology joined forces to create a multidisciplinary sarcoidosis at Virginia Commonwealth University. In 2019, the clinic was recognized as a World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) Center of Excellence. We identify four pillars of a patient-centered sarcoidosis clinic: clinical care, research, teaching, and community outreach. We detail how each of these facets plays a critical role in improving the health of individual patients, creating a strong infrastructure to improve the future of sarcoidosis treatment, and developing community-based resources that can empower patients. Most importantly, we highlight how a multidisciplinary clinic can help identify and combat healthcare disparities.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/3c/64/SVDLD-39-24.PMC9766846.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10727470","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Rituximab experience from a single centre for patients with rheumatoid arthritis-related interstitial lung disease. 来自单一中心的利妥昔单抗治疗类风湿关节炎相关间质性肺疾病的经验。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 DOI: 10.36141/svdld.v39i3.12337
Didem Sahin Eroglu, Anil Colaklar, Serdar Baysal, Murat Torgutalp, Asaf Baygul, Mucteba Enes Yayla, Serdar Sezer, Caglar Uzun, Ozlem Ozdemir Kumbasar, Tahsin Murat Turgay, Gulay Kinikli, Askin Ates
{"title":"Rituximab experience from a single centre for patients with rheumatoid arthritis-related interstitial lung disease.","authors":"Didem Sahin Eroglu,&nbsp;Anil Colaklar,&nbsp;Serdar Baysal,&nbsp;Murat Torgutalp,&nbsp;Asaf Baygul,&nbsp;Mucteba Enes Yayla,&nbsp;Serdar Sezer,&nbsp;Caglar Uzun,&nbsp;Ozlem Ozdemir Kumbasar,&nbsp;Tahsin Murat Turgay,&nbsp;Gulay Kinikli,&nbsp;Askin Ates","doi":"10.36141/svdld.v39i3.12337","DOIUrl":"https://doi.org/10.36141/svdld.v39i3.12337","url":null,"abstract":"<p><strong>Objective: </strong>To demonstrate the effects of rituximab (RTX) in patients with rheumatoid arthritis-related interstitial lung disease (RA-ILD).</p><p><strong>Methods: </strong>A total of 165 patients who used RTX for the management of rheumatoid arthritis were retrospectively scrutinised. Among these, 26 patients diagnosed with RA-ILD were analysed (61.5% male, mean age at RTX infusion 61.4 ± 6.5 years). To evaluate the efficacy of RTX on lung response, patients with pulmonary function test results and/or thorax computed tomography (chest-CT) of pre- and post-RTX were compared. Disease progression was defined as either a decline of ≥10% in forced vital capacity (FVC) and/or a decline of ≥15% in diffusion capacity of carbon monoxide (DLCO), or an increase of parenchymal involvement on chest-CT images according to the radiologists' assessment.</p><p><strong>Results: </strong>Among 26 patients, the most common radiologic pattern was usual interstitial pneumonia (42.3%), followed by non-specific interstitial pneumonia (38.5%). Data for lung response was available in 20 patients. Median pre- and post- RTX DLCO values were 71.0% (60.0-77.0) and 63.0% (47.0-74.0), respectively (p= 0.06). Median pre- and post-RTX FVC values were 74.0% (61.0-99.0) and 84.0% (63.0-100.0), respectively (p= 0.28). Overall, stabilization or regression of RA-ILD was provided in 13 (65.0%) patients, whereas 7 patients had progressive RA-ILD. Post-RTX, 5 patients were diagnosed with RA-ILD.</p><p><strong>Conclusion: </strong>Our results suggest that RTX is effective in achieving stabilization or even improvement of RA-ILD. However, considering that it does not cause regression in every patient and some develop RA-ILD under RTX, we still need more effective treatment options.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/48/44/SVDLD-39-29.PMC9766850.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10727472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Radiographic Progression and Survival of the Different HRCT Patterns of Idiopathic Pulmonary Fibrosis. 特发性肺纤维化不同HRCT表现的影像学进展和生存期。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-06-29 DOI: 10.36141/svdld.v39i2.12534
Marco Mura, Carlotta Rellini, Nada Taha, Francesco Paolo Sbordone, Flavia Rufi, Francesca Montesanto, Roberto Floris, Maurizio Zompatori, Gianluigi Sergiacomi
{"title":"Radiographic Progression and Survival of the Different HRCT Patterns of Idiopathic Pulmonary Fibrosis.","authors":"Marco Mura,&nbsp;Carlotta Rellini,&nbsp;Nada Taha,&nbsp;Francesco Paolo Sbordone,&nbsp;Flavia Rufi,&nbsp;Francesca Montesanto,&nbsp;Roberto Floris,&nbsp;Maurizio Zompatori,&nbsp;Gianluigi Sergiacomi","doi":"10.36141/svdld.v39i2.12534","DOIUrl":"https://doi.org/10.36141/svdld.v39i2.12534","url":null,"abstract":"<p><strong>Introduction: </strong>Idiopathic pulmonary fibrosis (IPF) is a chronic disease with a peculiar (typical) HRCT pattern, but biopsy can demonstrate usual interstitial pneumonia in patients with atypical patterns. It is unknown how progression pattern varies among different radiographic presentations of IPF. We sought to investigate the longitudinal radiographic evolution and survival of typical and non-typical patterns.</p><p><strong>Materials and methods: </strong>One-hundred-twenty-three patients diagnosed with IPF in 2 tertiary referral hospitals were included in the study. Longitudinal evolution of non-typical patterns was considered. The HRCT visual fibrosis score was used as a reliable evaluation tool of disease progression. HRCTs were scored by 2 senior chest radiologists with ILD expertise. The primary endpoint was the evolution of the presentation pattern to probable or typical. The secondary endpoint was lung transplant (LTx)-free survival from the time of diagnosis.</p><p><strong>Results: </strong>Average interval between HRCTs was 16±5 months; average follow-up after the 2<sup>nd</sup> HRCT was 17±11 months. Four out of 45 (8.9%) patients with probable pattern \"evolved\" to a typical pattern of IPF, while 5 out of 31 (16.1%) with indeterminate/alternative pattern \"evolved\" to probable pattern. An average HRCT fibrosis score increase of 9±11% was observed with typical (n=49), 6±5% with probable (n=43) and 7±8% (n=31) with indeterminate/alternative presentation pattern. LTx-free survival and lung function declines did not show any difference related to presentation HRCT patterns.</p><p><strong>Conclusions: </strong>The evolution of a non-typical UIP pattern to a typical one is infrequent. All presentation HRCT patterns of IPF evolve in similar way and are associated with comparable survival time.[/sc].</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437754/pdf/SVDLD-39-021.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40364482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Idiopathic Chronic Eosinophilic Pneumonia Evolving to Pulmonary Fibrosis: A Retrospective Analysis. 特发性慢性嗜酸性粒细胞性肺炎发展为肺纤维化:回顾性分析。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-06-29 DOI: 10.36141/svdld.v39i2.12656
Misbah Baqir, Tobias Peikert, Tucker F Johnson, Yasmeen K Tandon, Eunhee S Yi, Darrell R Schroeder, Jay H Ryu
{"title":"Idiopathic Chronic Eosinophilic Pneumonia Evolving to Pulmonary Fibrosis: A Retrospective Analysis.","authors":"Misbah Baqir,&nbsp;Tobias Peikert,&nbsp;Tucker F Johnson,&nbsp;Yasmeen K Tandon,&nbsp;Eunhee S Yi,&nbsp;Darrell R Schroeder,&nbsp;Jay H Ryu","doi":"10.36141/svdld.v39i2.12656","DOIUrl":"https://doi.org/10.36141/svdld.v39i2.12656","url":null,"abstract":"<p><strong>Background: </strong>Patients with idiopathic chronic eosinophilic pneumonia (ICEP) may have pulmonary fibrosis.</p><p><strong>Objectives: </strong>To investigate the predictors of pulmonary fibrosis in ICEP, to describe the timeline of pulmonary fibrosis after ICEP diagnosis, and to detail the radiologic pattern of fibrosis.</p><p><strong>Methods: </strong>A retrospective computer-assisted search was performed to identify patients with ICEP seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1997, through September 1, 2019. Patients with follow-up chest computed tomography (CT) beyond 12 months after the ICEP diagnosis were included in the study. Demographic, clinical, radiologic, and histopathologic characteristics were analyzed. Proportional hazards regression was used to assess the predictors of pulmonary fibrosis.</p><p><strong>Results: </strong>We identified 62 patients (mean [SD] age at ICEP diagnosis, 60 [13] years; female sex, 37 [60%]). Cough (87%) and shortness of breath (85%) were the most common presenting symptoms. Of patients, 27 (44%) had a history of smoking and 27 (44%) had a history of asthma. During follow-up, 23 patients (37%) had CT evidence of pulmonary fibrosis, of whom 16 patients (70%) had a CT pattern inconsistent with usual interstitial pneumonia. In 29% of the patients, the CT evidence of pulmonary fibrosis developed within 2 years after ICEP. Age and male sex were predictors of pulmonary fibrosis. Of note, a history of asthma decreased the likelihood of pulmonary fibrosis.</p><p><strong>Conclusions: </strong>Development of pulmonary fibro-sis is not uncommon in patients with ICEP, especially older men, and is associated with increased risk of death.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437755/pdf/SVDLD-39-020.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40364483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","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 clinical course of interstitial lung disease in an adult patient with an ABCA3 homozygous complex allele under hydroxychloroquine and a review of the literature. 羟氯喹治疗下ABCA3纯合复合体等位基因成人间质性肺病的临床病程及文献综述
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-06-29 DOI: 10.36141/svdld.v39i2.12730
Marie Legendre, Xavier Darde, Marion Ferreira, Sandra Chantot-Bastaraud, Marion Campana, Laurent Plantier, Nadia Nathan, Serge Amselem, Annick Toutain, Patrice Diot, Sylvain Marchand-Adam
{"title":"The clinical course of interstitial lung disease in an adult patient with an ABCA3 homozygous complex allele under hydroxychloroquine and a review of the literature.","authors":"Marie Legendre,&nbsp;Xavier Darde,&nbsp;Marion Ferreira,&nbsp;Sandra Chantot-Bastaraud,&nbsp;Marion Campana,&nbsp;Laurent Plantier,&nbsp;Nadia Nathan,&nbsp;Serge Amselem,&nbsp;Annick Toutain,&nbsp;Patrice Diot,&nbsp;Sylvain Marchand-Adam","doi":"10.36141/svdld.v39i2.12730","DOIUrl":"https://doi.org/10.36141/svdld.v39i2.12730","url":null,"abstract":"<p><strong>Objective: </strong>The gene mutations responsible for ABCA3 protein deficiency are involved in respiratory distress of the newborn and much more rarely in adult interstitial lung diseases (ILD). An adult patient homozygous for a complex allele encompassing the p.Ala1027Pro likely pathogenic mutation and the p.Gly974Asp variation was followed for a late-onset and fibrotic ILD. The evolution was marked by progressive clinical and functional degradation despite corticosteroid pulses. The patient, who was first registered on the list for lung transplantation, was improved quickly and persistently for at least 6.5 years with hydroxychloroquine treatment, allowing removal from the transplant list.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9437752/pdf/SVDLD-39-019.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40366392","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Diagnosis distribution in cases with granulomatous inflammation in lung, pleura, and lymph node biopsies: an experience from a tertiary level single center chest diseases and thoracic surgery hospital. 肺、胸膜、淋巴结活检肉芽肿性炎症的诊断分布:来自三级单中心胸外科医院的经验。
IF 1.6 4区 医学
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases Pub Date : 2022-01-01 Epub Date: 2022-01-13 DOI: 10.36141/svdld.v38i4.11914
Selma Aydogan Eroglu, Tekin Yildiz, Esin Sonkaya, Murat Kavas, Fatma Ozbaki, Lale Sertçelik, Aycim Sen, Tulin Sevim
{"title":"Diagnosis distribution in cases with granulomatous inflammation in lung, pleura, and lymph node biopsies: an experience from a tertiary level single center chest diseases and thoracic surgery hospital.","authors":"Selma Aydogan Eroglu,&nbsp;Tekin Yildiz,&nbsp;Esin Sonkaya,&nbsp;Murat Kavas,&nbsp;Fatma Ozbaki,&nbsp;Lale Sertçelik,&nbsp;Aycim Sen,&nbsp;Tulin Sevim","doi":"10.36141/svdld.v38i4.11914","DOIUrl":"https://doi.org/10.36141/svdld.v38i4.11914","url":null,"abstract":"<p><strong>Background: </strong>Granulomatous inflammation is found in a wide range of diseases, and most commonly associated with sarcoidosis and tuberculosis. Granulomas are pathologically classified into two main groups; necrotic and non-necrotic.</p><p><strong>Objectives: </strong>The aim of this study was to evaluate the radiological, laboratory, and pathological findings of a large patient population with granuloma in biopsy samples, to determine the final diagnostic distribution.</p><p><strong>Methods: </strong>This study was designed as a retrospective, descriptive, observational, cross-sectional study. It was conducted in patients with granulomatous inflammation detected in lung, pleural, mediastinal, hilar, and/or peripheral lymph node biopsies. Demographic information, radiological, microbiological, and laboratory results of the patients were obtained via the information processing system of the hospital. The diagnoses recorded were re-evaluated by at least two experienced clinicians and the final diagnosis distributions were made.</p><p><strong>Results: </strong>A total of 392 patients were included in the study. Non-necrotizing inflammation was detected in 268 patients, and necrotizing granulomatous inflammation was found in 124 patients. The most common cause of non-necrotizing inflammation was sarcoidosis, and tuberculosis in the case of necrotizing inflammation. A total of 77.2% of sarcoidosis patients had non-necrotizing inflammation and 54.3% of the tuberculosis patients had necrotizing inflammation. In the diagnosis distribution of granulomatous inflammation sarcoidosis, mycobacterium infections (especially tuberculosis), sarcoid reaction due to malignancy, pneumoconiosis, granulomatosis with polyangiitis and hypersensitivity pneumonitis were detected, respectively. A total of 392 patients were diagnosed with 13 different diseases. In 15 patients (3.8%) no specific diagnosis could be made.</p><p><strong>Conclusions: </strong>The diagnosis of granulomatous inflammation detected in biopsy samples is common for clinicians and a differential diagnosis is difficult in many cases. A patient's clinical findings, laboratory results, and radiological appearance, should be evaluated in detail and a final diagnosis only made following a multidisciplinary discussion. The presence of necrosis in tissue samples alone is not a reliable finding for a final diagnosis.</p>","PeriodicalId":21394,"journal":{"name":"Sarcoidosis, Vasculitis, and Diffuse Lung Diseases","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/15/67/SVDLD-38-48.PMC8787379.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39762877","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
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