Rare ILD/DPLD最新文献

Hyaluronan (HA) accumulation following SARS-CoV-2 infection and in non-resolvable COVID-19 fibrosis SARS-CoV-2感染后和不可解决的COVID-19纤维化中透明质酸(HA)的积累

Rare ILD/DPLD Pub Date : 2022-03-10 DOI: 10.1183/23120541.lsc-2022.175

Andrew Peters, S. Collum, X. Yuan, Bihong Zhao, P. Shivshankar, W. Bi, L. Buja, R. Thandavarayan, Howard Huang, H. Eltzschig, Ivan O. Rosas, F. Poli de Frias, B. Akkanti, S. Jyothula, N. Wareing, H. Karmouty-quintana

引用次数: 2

Analysis of hospitalizations by lymphangioleiomyomatosis in Spain 西班牙淋巴管平滑肌瘤病住院分析

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1411

José Rafael Terán Tinedo, F. Serrano, C. C. Riera, Z. Ji, A. L. Andrés, R. García, V. H. Barrera, L. P. Maestú, J. D. M. Díez

引用次数: 0

Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis: prevalence and the role of Cardiopulmonary Exercise Testing and echocardiogram in predicting it 肺朗格汉斯细胞组织细胞增多症的肺动脉高压:患病率和心肺运动试验和超声心动图在预测它的作用

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3684

G. I. Heiden, Juliana Sobral, J. Alves, J. M. Salge, A. Albuquerque, C. Fernandes, R. Kairalla, C. R. Carvalho, Rogério Souza, B. Baldi

{"title":"Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis: prevalence and the role of Cardiopulmonary Exercise Testing and echocardiogram in predicting it","authors":"G. I. Heiden, Juliana Sobral, J. Alves, J. M. Salge, A. Albuquerque, C. Fernandes, R. Kairalla, C. R. Carvalho, Rogério Souza, B. Baldi","doi":"10.1183/13993003.congress-2019.pa3684","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3684","url":null,"abstract":"Pulmonary Langerhans Cell Histiocytosis (PLCH) is often associated with pulmonary hypertension (PH). But the prevalence of PH in different severities of PLCH isn’t completely defined. Findings in Cardiopulmonary Exercise Testing (CPET) and echocardiogram may predict PH. Objectives: to evaluate the prevalence of PH in PLCH and the role of CPET and echocardiogram in predicting PH. Methods: Thirty-five patients (32% men, 47 ± 11 years) underwent CPET on treadmill, echocardiogram and lung function tests. Patients with tricuspid regurgitation velocity (TRV) ≥ 2,5 m/s and/or indirect signs of PH and/or DLCO Results: Peak oxygen consumption (VO2) was 73 ± 19 %predicted, reduced in 70% of cases. Oxygen pulse was 85 ± 18 %predicted, PetCO2 at anaerobic threshold was 34 ± 6, and VE/VCO2 slope was 31 ± 10. Ventilatory and cardiocirculatory limitations, CPET suggestive of PH and dynamic hyperinflation were identified in 58%, 41%, 33% and 68%, respectively. TRV was 2.9 (2.4 – 3.1) m/s and DLCO was 56 ± 21 %predicted. Twenty-one (60%) patients met criteria for RHC and 18 underwent it. The prevalence of PH was 41% (85% pre- capillary). The mPAP was 32 ± 12 mmHg and was associated with VO2 (r = - 0.54, p = 0.02), Oxygen pulse (r = - 0.55, p = 0.02); TRV (r = 0.69, p = 0.004), and DLCO (r = - 0.56, p = 0.01). However, there wasn’t association with PetCO2 or VE/VCO2. Conclusion: The prevalence of PH in PLCH was 41%, mainly pre- capillary. Classical CPET variables didn’t predict PH, possibly because of multifactorial involvement and a high prevalence of ventilatory limitation.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"17 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":"122952933","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

The natural history of Pulmonary Alveolar Proteinosis (PAP): data from the Italian National Reference Center 肺泡蛋白沉积症(PAP)的自然史:来自意大利国家参考中心的数据

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3680

I. Campo, S. Lettieri, D. Piloni, A. Silvestri, E. Salvaterra, M. Zorzetto, A. Corino, F. Meloni, F. Mariani

{"title":"The natural history of Pulmonary Alveolar Proteinosis (PAP): data from the Italian National Reference Center","authors":"I. Campo, S. Lettieri, D. Piloni, A. Silvestri, E. Salvaterra, M. Zorzetto, A. Corino, F. Meloni, F. Mariani","doi":"10.1183/13993003.congress-2019.pa3680","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3680","url":null,"abstract":"Background: PAP is a ultra-rare syndrome characterized by the accumulation of surfactant material within the alveolar spaces. The current therapy with whole lung lavage (WLL) is still the gold standard even if clinical trials with inhaled rGM-CSF supplementation are ongoing. Aims: To prospectively study the Italian PAP population. Methods: We carried out a descriptive study of the baseline characteristics of the PAP patients enrolled in the Italian registry from 1989 until 2019. Results: We enrolled 126 PAP, among these 92.8% were affected by autoimmune (aPAP), while the remaining patients were divided in: hereditary (0.8%), secondary (3.2%) and PAP-like (3.2%). Considering the aPAP patients, the mean age at diagnosis was 43±14, with a male/female ratio = 2. Smoking history was reported in 66.7% of cases; 37.2% of them are currently smokers. 49 patients did not necessitate WLL treatment in the long-term follow up. The baseline pulmonary function test (PFT) (% of predicted value) of aPAP patients is shown in tab. 1, as mean±SD. Differences are significant also in a multivariate regression analysis, taking into account age, sex and smoking habit. Conclusions: The establishment of a national registry for PAP has enabled a detailed characterization of the natural course of the disease, moreover our results indicate that also PFT could recommend on when to treat, along with persistent or progressive respiratory failure and exercise desaturation.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"37 46","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"113934283","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

A rare diagnosis: Hermansky-Pudlak syndrome in a patient with pulmonary fibrosis, oculocutaneous albinism and thrombocytopathy 一个罕见的诊断:Hermansky-Pudlak综合征患者肺纤维化，皮肤白化病和血小板病

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1400

M. Doubková, J. Trizuljak, A. Hrazdirová, Z. Vrzalova, I. Blaháková, L. Radová, Š. Pospíšilová, M. Doubek

引用次数: 0

Gender perspective in Interstitial Lung Diseases (ILD) 肺间质性疾病(ILD)的性别视角

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1413

Elizabeth María De Freitas González, J. García, R. Cabrera, Carlos Mier Bango, Carmen López Garay, E. Miguel, I. H. Ortega, Nieves Alonso Orcajo, L. S. Herranz

引用次数: 0

Subclinical right ventricular dysfunction in fibrotic interstitial lung diseases: a combined assessment by speckle tracking and three-dimensional echocardiography 纤维化间质性肺疾病的亚临床右室功能障碍:斑点追踪和三维超声心动图的联合评估

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3688

A. Canora, A. Buonauro, P. Dolce, C. Nicoletta, Giacomo Ghinassi, Emanuele Ciasullo, A. Zamparelli, M. Galderisi, M. Bocchino

{"title":"Subclinical right ventricular dysfunction in fibrotic interstitial lung diseases: a combined assessment by speckle tracking and three-dimensional echocardiography","authors":"A. Canora, A. Buonauro, P. Dolce, C. Nicoletta, Giacomo Ghinassi, Emanuele Ciasullo, A. Zamparelli, M. Galderisi, M. Bocchino","doi":"10.1183/13993003.congress-2019.pa3688","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3688","url":null,"abstract":"Background: Little is known about subclinical right ventricle(RV) involvement in interstitial lung disease (ILD)patients. Our study aimed at evaluating early RV dysfunction by standard and advanced echocardiography in this clinical scenario. Methods: Sixty fibrotic ILD patients, including 30 cases affected by idiopathic pulmonary fibrosis(IPF), without heart failure and 20 age- and sex-matched healthy subjects underwent standard, speckle tracking and real time 3D echocardiography of the right ventricle. All patients also performed complete lung function testing. Results: Systolic pulmonary arterial pressure (sPAP) was significantly higher in ILD patients (with no differences between IPF and no-IPF) than in controls (p Conclusions: A subclinical RV dysfunction is detectable by speckle tracking in ILD patients. The impairment of RV GLS, RV SLS and RV LLS is more prominent in IPF patients and inversely correlates with gas transfer, despite comparable sPAP and 3D RVEF in no-IPF cases.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"43 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":"116051160","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

Radiological and clinical specific features of anti-synthetase syndrome : a retrospective analytic study 抗合成酶综合征的影像学和临床特征:回顾性分析研究

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3673

Q. Maloir, F. Gester, J. Corhay, R. Louis, J. Guiot

{"title":"Radiological and clinical specific features of anti-synthetase syndrome : a retrospective analytic study","authors":"Q. Maloir, F. Gester, J. Corhay, R. Louis, J. Guiot","doi":"10.1183/13993003.congress-2019.pa3673","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3673","url":null,"abstract":"Introduction: Anti-synthetase syndrome (ASS) is a rare auto-immune disorder combining autoantibodies and specifics clinical manifestations. One of the particularities of the ASS is the pleiomorphic radiological presentation seen at the initial work-up. Evaluating treatment response can also be challenging and requires a specific clinical, functional, biological and radiological monitoring. Aim: To identify specific radiological and clinical features of ASS. Methods: We retrospectively studied all patients suffering from ASS in CHU of Liege until now. The diagnosis of ASS was made according to ERS’s criteria. We analyzed clinical features, pulmonary function test (PFT), computed tomography (CT), and longitudinal evolution with regard to their treatments. Results: In the whole cohort of 31 patients, we identified 19 anti-JO1, 5 anti-PL12 & 7 anti-PL-7. The sex-ratio is slightly in favor of male. Interestingly PL-12 syndrome was present in younger patients than those with others antibodies (mean age 39,8 vs 53,1 (JO1) & 73,3 (PL7) (p Conclusion: In our single center retrospective study, we found different profile of auto-antibodies according to age and radiological apparence.","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":"124966106","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

Psychological abnormalities are commonly present in adult patients with pulmonary Langerhans cell histiocytosis 心理异常常见于肺朗格汉斯细胞组织细胞增多症的成年患者

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1404

A. Tazi, E. Bugnet, N. Gupta, G. Lorillon, S. Arbabzadeh-Bouchez, J. Lépine, S. Chevret

{"title":"Psychological abnormalities are commonly present in adult patients with pulmonary Langerhans cell histiocytosis","authors":"A. Tazi, E. Bugnet, N. Gupta, G. Lorillon, S. Arbabzadeh-Bouchez, J. Lépine, S. Chevret","doi":"10.1183/13993003.congress-2019.pa1404","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1404","url":null,"abstract":"Background: The prevalence of neuropsychiatric symptoms and substance abuse disorders, in adult patients with pulmonary Langerhans cell histiocytosis (PLCH) has not been explored. Objectives: The aim of this study was to examine the psychological healthcare burden faced by PLCH patients. Methods: All PLCH patients seen during one year completed the Hospital Anxiety and Depression scale (HAD), the Barratt Impulsiveness Scale, Version 10 (BIS-10) and the Cannabis Use Disorders Identification Test (CUDIT) questionnaires. Scores were used to determine the prevalence of psychological and substance abuse problems, and were associated with clinical assessment of the disease. The primary endpoint of the study was the proportion of patients having definite or doubtful anxious state (HAD-A scores of 11 or higher or between 8 and 10, respectively). Logistic regression models were used to quantify the strength of association of various disease-related parameters and the occurrence of anxiety, depression or impulsivity. Results: Sixty-eight patients were included. Definite anxious and depressive states were identified in 29% and 6% patients, respectively, and doubtful anxious and depressive states in 34% and 6%, respectively. Impulsive behaviour was detected in 13% of the cohort. Eleven patients (16%) consumed cannabis on a regular basis, and in 55% of them CUDIT scores suggested cannabis abuse or dependence. Increasing time from diagnosis and successful cessation of cigarette smoke exposure were associated with decreased risk of anxiety. Conclusions: Psychological abnormalities commonly co-exist in adult PLCH patients and should be considered while making their management decisions.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"107 1 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":"122643892","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

Correlation between imaging fibrosis severity and bronchoalveolar lavage pattern in interstitial lung disease 肺间质性疾病影像学纤维化严重程度与支气管肺泡灌洗模式的相关性

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3682

Besma Dhahri-Ourari, R. Fessi, H. Zaibi, M. Ferchichi, N. Guediri, A. Jarrar, J. Amar, H. Aouina

{"title":"Correlation between imaging fibrosis severity and bronchoalveolar lavage pattern in interstitial lung disease","authors":"Besma Dhahri-Ourari, R. Fessi, H. Zaibi, M. Ferchichi, N. Guediri, A. Jarrar, J. Amar, H. Aouina","doi":"10.1183/13993003.congress-2019.pa3682","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3682","url":null,"abstract":"Aim: To assess correlation between fibrosis severity and bronchoalveolar lavage (BAL) profile in interstitial lung disease (ILD). Methods: A retrospective study was conducted including 111 patients with confirmed diagnosis of ILD by HRCT. Semi-quantitative scale as proposed by Warrick was used. Total score (TS), indices for alveolitis (AI) and fibrosis (FI) were assessed. According to the total score, fibrosis was classified as mild ( 15. BAL was performed in 82% of cases. Results: Mean age was 59.3 years. Sex ratio was 0.76. Main etiologies were idiopathic ILD (38.7%), sarcoidosis (28.8%) and connectivite tissue diseases (13.5%). Most frequent Warrick elementary lesions were septal and no septal lines (77.5%) and ground glass opacities (48.6%). TS, AI and FI were 1.67±1.73; 9 ± 5.8 and 10.2 ±6.3 respectively. Fibrosis was mild (31.5%), moderate (51.4%) and severe (17.1%). Patients with mild fibrosis had greater BAL lymphocyte counts and CD4/CD8 ratio (p=0.043 and 0.031) and lower eosinophil rates (p=0.04). There were no correlations between BAL profiles and AI. Whereas significant correlations were found between TS and FI and lymphocyte (p=0.001; r=-0.33 and p Conclusion: Fibrosis extent and severity in ILD correlate with neutrophil rates and inversely with lymphocyte counts in BAL.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"1 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":"130038139","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