Rare ILD/DPLD最新文献_第3页

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

Rare ILD/DPLD 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

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

Rare ILD/DPLD 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

{"title":"Pleuroparenchymal fibroelastosis - clinical analysis of 22 cases","authors":"Joao Pires Parreira, Carina Santos, Marcos Oliveira, N. Melo, P. Mota, H. Bastos, J. Pereira, C. Souto Moura, A. Morais","doi":"10.1183/13993003.congress-2019.pa3678","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3678","url":null,"abstract":"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.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"2016 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":"127267062","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

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

Rare ILD/DPLD 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

引用次数: 1

Cicatricial lung fibrosis: five cases 瘢痕性肺纤维化5例

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1407

M. Samsonova, A. Cherniaev, K. Mikhailichenko

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

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1408

G. Gkrepi, D. Lowe, S. Burns, S. Seneviratne, J. Hurst

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

Rare ILD/DPLD 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

{"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

Rare ILD/DPLD 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

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

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.oa2146

C. Hyldgaard, E. Bendstrup, A. Pedersen, L. Pedersen, T. Ellingsen

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

Rare ILD/DPLD 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

{"title":"Natural history of incidental sporadic or tuberous sclerosis complex associated lymphangioleiomyomatosis","authors":"F. Marco, S. Terraneo, O. M. Dias, G. Imeri, S. Centanni, L. Giuliani, E. Lesma, G. Palumbo, Mark Wanderley, C. R. Carvalho, B. Baldi","doi":"10.1183/13993003.congress-2019.oa2143","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa2143","url":null,"abstract":"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.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"15 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":"129978205","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

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

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3676

M. Baqir, T. Moua, Darin B White, R. Jay

{"title":"Pulmonary light chain deposition disease: a retrospective analysis of 11 cases","authors":"M. Baqir, T. Moua, Darin B White, R. Jay","doi":"10.1183/13993003.congress-2019.pa3676","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3676","url":null,"abstract":"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.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"59 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":"130764401","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