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BMS-986327 as a novel PET imaging agent for assessment of LPA1 receptors in IPF BMS-986327作为评估IPF中LPA1受体的新型PET显像剂
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1399
R. Smith, Joonyoung Kim, D. Donnelly, Andrea Olga Andrea Olga Shorts, Thomas Petrone, S. Kalinowski, B. Murphy, P. Cheng, Todd A Correll, P. Chow, S. Du, S. Bonacorsi, W. Hayes
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引用次数: 1
Pleuroparenchymal fibroelastosis - clinical analysis of 22 cases 胸膜实质纤维弹性增生22例临床分析
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3678
Joao Pires Parreira, Carina Santos, Marcos Oliveira, N. Melo, P. Mota, H. Bastos, J. Pereira, C. Souto Moura, A. Morais
Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a rare disease, recently described and characterized by fibroelastotic thickening of the pleural and subpleural lung parenchyma, mainly in upper lobes. There is a raising number of diagnosis, but the etiology and pathophysiology are still unknown. Objectives: The objectives of this study were to identify and characterize the patients with PPFE, followed in Hospital de Sao Joao, a hospital university center. Methods: It was made a retrospective analysis of patients with criteria of PPFE. We analyzed demographic, clinical parameters including smoke status, initial symptoms, respiratory functional data and treatment. Results: It was found 22 cases with PPFE diagnostics criteria. The mean age was 62.3 years old. Most patients were woman (n=16) and non-smokers (n=14). Most common symptom at the time of diagnosis was dyspnea, followed by cough, weight loss, repetitive respiratory infections, wheezing and pleuritic pain. In functional evaluation, 12 patients had reduced diffusing capacity; 4 restrictive pattern and 2 obstructive pattern. PPFE was considered idiopathic in 8 patients and was associated with usual interstitial pneumoniae (n=5), chronic hypersensitivity pneumonitis (n=2), exuberant emphysema (n=2), pulmonary cancer (n=2), silicosis (n=1), bronchiectasis (n=1) and amiodarone toxicity (n=1). Only 10 patients were on immunosuppressive treatment. Discussion: The evaluation of this patients confirmed that PPFE has a non-specific and heterogeneous nature, given the different associations and contexts in which occurs. Then, it´s crucial to study the idiopathic form, that can more accurately evidence the natural evolution of this disease and response to treatment.
简介:胸膜实质纤维弹性增生症(PPFE)是一种罕见的疾病,最近被描述并以胸膜和胸膜下肺实质纤维弹性增厚为特征,主要发生在肺上叶。有越来越多的诊断,但病因和病理生理仍是未知的。目的:本研究的目的是识别和描述在圣若昂医院(一家医院大学中心)进行的PPFE患者。方法:对符合PPFE诊断标准的患者进行回顾性分析。我们分析了人口统计学、临床参数(包括吸烟状况、初始症状、呼吸功能数据和治疗)。结果:22例患者符合PPFE诊断标准。平均年龄为62.3岁。大多数患者为女性(n=16)和非吸烟者(n=14)。诊断时最常见的症状是呼吸困难,其次是咳嗽、体重减轻、反复呼吸道感染、喘息和胸膜痛。在功能评估中,12例患者弥散能力降低;4例限制性型,2例阻塞性。8例PPFE患者被认为是特发性的,并伴有通常间质性肺炎(n=5)、慢性超敏性肺炎(n=2)、活跃性肺气肿(n=2)、肺癌(n=2)、矽肺(n=1)、支气管扩张(n=1)和胺碘酮中毒(n=1)。仅有10例患者接受免疫抑制治疗。讨论:对该患者的评估证实,鉴于发生的不同关联和背景,PPFE具有非特异性和异质性。因此,研究特发性形式是至关重要的,这可以更准确地证明这种疾病的自然演变和对治疗的反应。
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引用次数: 0
Macrolides as a potential treatment in organizing pneumonia 大环内酯类药物作为组织性肺炎的潜在治疗方法
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1410
A. Carriço, Patrícia Caetano, N. Melo, H. Bastos, S. Guimarães, C. Moura, J. Pereira, A. Morais
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引用次数: 1
Cicatricial lung fibrosis: five cases 瘢痕性肺纤维化5例
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1407
M. Samsonova, A. Cherniaev, K. Mikhailichenko
{"title":"Cicatricial lung fibrosis: five cases","authors":"M. Samsonova, A. Cherniaev, K. Mikhailichenko","doi":"10.1183/13993003.congress-2019.pa1407","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1407","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"84 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114570048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Assessment of Treatment Response in Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) 肉芽肿性淋巴细胞间质性肺病(GLILD)治疗效果评价
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1408
G. Gkrepi, D. Lowe, S. Burns, S. Seneviratne, J. Hurst
{"title":"Assessment of Treatment Response in Granulomatous Lymphocytic Interstitial Lung Disease (GLILD)","authors":"G. Gkrepi, D. Lowe, S. Burns, S. Seneviratne, J. Hurst","doi":"10.1183/13993003.congress-2019.pa1408","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1408","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"11 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127787686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Clinical impact of BRAFV600E mutation in adult pulmonary Langerhans cell histiocytosis BRAFV600E突变对成人肺朗格汉斯细胞组织细胞增多症的临床影响
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.oa2141
A. Tazi, F. Jouenne, E. Bugnet, G. Lorillon, V. Meignin, A. Sadoux, S. Chevret, S. Mourah
Background: The clinical significance of the BRAFV600E mutation in tissue lesions from patients with pulmonary Langerhans cell histiocytosis (PLCH) has not been evaluated. Objective: To search for an association between BRAFV600E mutation and PLCH presentation and outcome. Methods:BRAFV600E genotyping was performed in biopsies from 83 patients with PLCH (43 males, median age 36 years, 65 current smokers). The outcome was based on variations of lung function tests and the occurrence of a new pneumothorax during the study [1]. Cox models were used to estimate the strength of association of baseline characteristics on the hazard of PLCH progression. P-values ≤0.05 denoted statistical significance. Results: A BRAFV600E mutation was detected in 31(37%) cases. No difference was identified in PLCH presentation, including smoking status (p=0.42), pneumothorax (p=0.29), or lung function (p>0.05), according to BRAF status. Patients were followed for a median time of 5 years. Thirty-eight (46%) patients experienced lung progression. BRAF status was not associated with PLCH outcome (Figure). In multivariable analysis, airflow obstruction at diagnosis was associated with increased risk of lung progression (p=0.028). Conclusions:BRAFV600E mutation was not associated with clinical features or outcome in adult PLCH patients. Airflow obstruction at diagnosis was the main factor associated with the risk of lung progression overtime.
背景:BRAFV600E突变在肺朗格汉斯细胞组织细胞增多症(PLCH)患者组织病变中的临床意义尚未得到评估。目的:探讨BRAFV600E突变与PLCH的表现和预后之间的关系。方法:对83例PLCH患者(男性43例,中位年龄36岁,当前吸烟者65例)的活检进行BRAFV600E基因分型。结果是基于研究期间肺功能测试的变化和新气胸的发生。Cox模型用于估计基线特征与PLCH进展危险的关联强度。p值≤0.05为有统计学意义。结果:在31例(37%)病例中检测到BRAFV600E突变。根据BRAF状态,PLCH的表现没有差异,包括吸烟状况(p=0.42)、气胸(p=0.29)或肺功能(p>0.05)。患者随访时间中位数为5年。38例(46%)患者出现肺部进展。BRAF状态与PLCH结果无关(图)。在多变量分析中,诊断时气流阻塞与肺部进展风险增加相关(p=0.028)。结论:BRAFV600E突变与成年PLCH患者的临床特征或预后无关。诊断时的气流阻塞是肺部进展危险的主要因素。
{"title":"Clinical impact of BRAFV600E mutation in adult pulmonary Langerhans cell histiocytosis","authors":"A. Tazi, F. Jouenne, E. Bugnet, G. Lorillon, V. Meignin, A. Sadoux, S. Chevret, S. Mourah","doi":"10.1183/13993003.congress-2019.oa2141","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa2141","url":null,"abstract":"Background: The clinical significance of the BRAFV600E mutation in tissue lesions from patients with pulmonary Langerhans cell histiocytosis (PLCH) has not been evaluated. Objective: To search for an association between BRAFV600E mutation and PLCH presentation and outcome. Methods:BRAFV600E genotyping was performed in biopsies from 83 patients with PLCH (43 males, median age 36 years, 65 current smokers). The outcome was based on variations of lung function tests and the occurrence of a new pneumothorax during the study [1]. Cox models were used to estimate the strength of association of baseline characteristics on the hazard of PLCH progression. P-values ≤0.05 denoted statistical significance. Results: A BRAFV600E mutation was detected in 31(37%) cases. No difference was identified in PLCH presentation, including smoking status (p=0.42), pneumothorax (p=0.29), or lung function (p>0.05), according to BRAF status. Patients were followed for a median time of 5 years. Thirty-eight (46%) patients experienced lung progression. BRAF status was not associated with PLCH outcome (Figure). In multivariable analysis, airflow obstruction at diagnosis was associated with increased risk of lung progression (p=0.028). Conclusions:BRAFV600E mutation was not associated with clinical features or outcome in adult PLCH patients. Airflow obstruction at diagnosis was the main factor associated with the risk of lung progression overtime.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127804959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
Expectations of patients suffering from idiopathic pulmonary fibrosis about their treatment: results of the French national survey, RESPIR 特发性肺纤维化患者对其治疗的期望:法国国家调查RESPIR的结果
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1405
A. Bourdin, E. Bergot, H. Nunes, G. Prévôt, B. Wallaert, S. Marchand-Adam, V. Cottin
{"title":"Expectations of patients suffering from idiopathic pulmonary fibrosis about their treatment: results of the French national survey, RESPIR","authors":"A. Bourdin, E. Bergot, H. Nunes, G. Prévôt, B. Wallaert, S. Marchand-Adam, V. Cottin","doi":"10.1183/13993003.congress-2019.pa1405","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1405","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126928170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Disease patterns in connective tissue disease-associated interstitial lung disease: a population based study 结缔组织病相关间质性肺病的疾病模式:一项基于人群的研究
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.oa2146
C. Hyldgaard, E. Bendstrup, A. Pedersen, L. Pedersen, T. Ellingsen
{"title":"Disease patterns in connective tissue disease-associated interstitial lung disease: a population based study","authors":"C. Hyldgaard, E. Bendstrup, A. Pedersen, L. Pedersen, T. Ellingsen","doi":"10.1183/13993003.congress-2019.oa2146","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa2146","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"28 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129404524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Natural history of incidental sporadic or tuberous sclerosis complex associated lymphangioleiomyomatosis 偶发或结节性硬化症合并淋巴管平滑肌瘤病的自然病史
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.oa2143
F. Marco, S. Terraneo, O. M. Dias, G. Imeri, S. Centanni, L. Giuliani, E. Lesma, G. Palumbo, Mark Wanderley, C. R. Carvalho, B. Baldi
The majority of patients who require medical intervention for lymphangioleiomyomatosis have the sporadic form of the disease (S-LAM). Some data suggested that TSC associated LAM could be a milder disease compared to S-LAM. Systematic screening for LAM using chest CT scan in TSC patients, could lead to a pre-clinic identification of LAM. To investigate whether the different disease behaviour is real or due to overdiagnosis of screened TSC women, we compared the natural history of S-LAM and TSC-LAM in patients with incidental diagnosis. A retrospective study involving outpatients with S-LAM and TSC-LAM followed in two hospitals in Milan, Italy and Sao Paolo, Brazil from 1995 to2017 was conduced. Incidental LAM diagnosis was defined by the finding of lung cysts in the abdominal (upper slices) or chest CT scans performed for reasons other than symptoms due to LAM. Clinical and functional data from 52 patients (23 with S-LAM and 29 with TSC-LAM) were analysed. There was no difference in yearly rate of functional decline between TSC-LAM, and S-LAM patients: At diagnosis functional impairment was mild without differences between groups. Patients with S-LAM had a trend to more severe cystic involvement but less renal angiomyolipoma, and lower VEGF-D serum levels than TSC-LAM. The natural history of TSC-LAM and S-LAM, when a potential selection bias due to screening in the latter group is balanced, is similar.
大多数患者需要医疗干预的淋巴管平滑肌瘤病有散发形式的疾病(S-LAM)。一些数据表明,与S-LAM相比,TSC相关的LAM可能是一种较轻的疾病。在TSC患者中使用胸部CT扫描系统筛查LAM,可以在临床前识别LAM。为了研究不同的疾病行为是真实的还是由于筛查的TSC妇女的过度诊断,我们比较了偶然诊断的患者的S-LAM和TSC- lam的自然史。回顾性研究1995 - 2017年在意大利米兰和巴西圣保罗两家医院随访的S-LAM和TSC-LAM门诊患者。偶然的LAM诊断定义为在腹部(上片)或胸部CT扫描中发现肺囊肿,而不是由于LAM引起的症状。分析52例患者(23例为S-LAM, 29例为TSC-LAM)的临床和功能资料。TSC-LAM和S-LAM患者的年功能下降率无差异:诊断时功能损害轻微,组间无差异。S-LAM患者囊性受累更严重,但肾血管平滑肌脂肪瘤较少,血清VEGF-D水平低于TSC-LAM。TSC-LAM和S-LAM的自然历史是相似的,当后者筛选的潜在选择偏倚是平衡的。
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引用次数: 1
Pulmonary light chain deposition disease: a retrospective analysis of 11 cases 肺部轻链沉积病11例回顾性分析
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3676
M. Baqir, T. Moua, Darin B White, R. Jay
Introduction: Light Chain Deposition Disease (LCDD) is a rarely affects the lungs. We aimed to explore the clinical and radiologic manifestations including positron emission tomography (PET) scan, of pulmonary LCDD. Methods: A retrospective, computer-assisted search was performed to identify patients with biopsy-proven pulmonary LCDD seen at Mayo Clinic, Rochester from 1997-2018. Demographic, clinical, and imaging features were analyzed. Results: Among 11 patients with pulmonary LCDD; the median age at the time of diagnosis was 55.1(39.1-77.2) years, 72% were females, and 64% were never smokers. Dyspnea (27%) and chest pain (27%) were the most common respiratory symptoms. Six patients had Sjogren’s syndrome and 2 had sarcoidosis. Eight patients had lymphoma involving the mucosa-associated lymphoid tissue and 1 had multiple myeloma. CT scan of the chest was available for 10 patients; nine (90%) had cysts mostly of lower lung distribution. Round/oval was the predominant shape of the cysts. Fifty-five percent had 1-5 cysts present, 44% had more than 21 cysts. The median diameter of the cyst was 22 mm (range, 5-68);67% of patients had cyst wall thickness of 1 mm. All 10 patients had solid nodules with 40% having >10 nodules. The median diameter of the nodule was 13 mm (range, 6-26). PET scan was available for 9 patients; in 7 patients lung nodules manifested FDG uptake with a median maximum standardized uptake value of 2.0 (range, 1.0-5.9). In the median follow up of 2.4 yr(range, 0.5-9.9);3 patients died. Conclusions: LCDD can affect the lungs in the form of cysts and nodules, especially in the setting of Sjogren’s syndrome.
轻链沉积病(LCDD)是一种少见的肺部疾病。我们的目的是探讨肺LCDD的临床和放射学表现,包括正电子发射断层扫描(PET)。方法:对1997-2018年在罗切斯特梅奥诊所就诊的经活检证实的肺部LCDD患者进行回顾性计算机辅助检索。分析了人口统计学、临床和影像学特征。结果:11例肺部LCDD患者中;诊断时的中位年龄为55.1(39.1-77.2)岁,72%为女性,64%为从不吸烟者。呼吸困难(27%)和胸痛(27%)是最常见的呼吸道症状。6例有干燥综合征,2例有结节病。8例淋巴瘤累及粘膜相关淋巴组织,1例多发性骨髓瘤。胸部CT扫描10例;9例(90%)囊肿多分布于肺下部。圆形/卵圆形是囊肿的主要形状。55%有1-5个囊肿,44%有超过21个囊肿。囊肿中位直径为22 mm(范围5-68),67%的患者囊壁厚度为1 mm。10例患者均为实性结节,其中40%为bbb10结节。结节的中位直径为13 mm(范围6-26)。PET扫描9例;7例肺结节表现为FDG摄取,最大标准化摄取值中位数为2.0(范围1.0-5.9)。中位随访时间为2.4年(0.5-9.9),3例患者死亡。结论:LCDD可以以囊肿和结节的形式影响肺部,特别是在干燥综合征的情况下。
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Rare ILD/DPLD
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