Rare ILD/DPLD最新文献_第5页

Clinical features and outcome of adult patients with pulmonary Langerhans cell histiocytosis 成人肺朗格汉斯细胞组织细胞增多症的临床特点及预后

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3683

E. Radzikowska, E. Wiatr, K. Błasińska-Przerwa, M. Jeśkiewicz, R. Langfort, B. Maksymiuk, Katarzyna Modrzewska, I. Bestry, M. Załęska, J. Szopiński, Agnieszka Jerzemska, M. Ochman, W. Naumnik, D. Jastrzębski, W. Piotrowski, K. Roszkowski-Śliż

{"title":"Clinical features and outcome of adult patients with pulmonary Langerhans cell histiocytosis","authors":"E. Radzikowska, E. Wiatr, K. Błasińska-Przerwa, M. Jeśkiewicz, R. Langfort, B. Maksymiuk, Katarzyna Modrzewska, I. Bestry, M. Załęska, J. Szopiński, Agnieszka Jerzemska, M. Ochman, W. Naumnik, D. Jastrzębski, W. Piotrowski, K. Roszkowski-Śliż","doi":"10.1183/13993003.congress-2019.pa3683","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3683","url":null,"abstract":"Pulmonary Langerhans’ cell histiocytosis (PLCH) is a neoplastic disorder with strong inflammatory component. The clinical manifestations, features, and outcome of the PLCH vary widely, and clinical course of the disease is unpredictable. Material and methods: 124 adults (61 women and 63 men in age 15 to 69 years) with LCH, have been presented in our Department for the last 21 years. The median follow-up period was 146 months (range 3 to 338 months). Results: Isolated PLCH was diagnosed in 90(72%) cases, multisystem disease in 30(26%) patients. Two (1.6%) patients had multifocal bone disease, one (0.8%) had isolated mucosal, and one isolated bone lesion. Out of whole group only 6(5%) patients were nonsmokers. Incidentally the disease was diagnosed in 20% of patients. Pneumothorax as a first symptom of the disease was observed in 26% of patients. The most common findings in the pulmonary function tests were obstructive ventilatory defect (57%), and decreasing of transfer factor for carbon monoxide (80%). During the time of observation 70(56%) patients did not require immunosuppressive therapy. Only in 3(14%) patients steroid treatment was sufficient, other 19(86%) patients required chemotherapy. Chemotherapy with vinblastine, prednisone and mercaptopurine was administered in 11 patients, but only in 4 the regression with non-active disease was observed. Sixteen (13%) patients were treated successfully with cladribine, and till now no relapse was noticed. Conclusions: PLCH is a rare, cystic lung disease, affects mainly young adult smokers, without gender predominance; early diagnosis, smoking cessation, and adequate treatment are critical in its management.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"13 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":"116749324","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

Interstitial lung disease in CVID (GLILD): clinical presentation and comparison to CVID without ILD 间质性肺疾病CVID (GLILD):临床表现和与无ILD的CVID的比较

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1409

V. Somogyi, M. Eichinger, F. Lasitschka, J. Kappes, M. Kreuter

{"title":"Interstitial lung disease in CVID (GLILD): clinical presentation and comparison to CVID without ILD","authors":"V. Somogyi, M. Eichinger, F. Lasitschka, J. Kappes, M. Kreuter","doi":"10.1183/13993003.congress-2019.pa1409","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1409","url":null,"abstract":"Introduction: Common variable immunodeficiency (CVID) patients are susceptible to respiratory diseases including bronchiectasis and granulomatous lymphocytic interstitial lung disease (GLILD). As data on evaluating CVID with and without GLILD are sparse, we aimed to compare these groups. Methods: 21 CVID patients were analyzed retrospectively for characteristics and outcomes and grouped into CVID with (ILD,n=9) and without GLILD (noILD,n=12). CT scoring (CTS) assessed 11 patterns (ground glass opacities, reticulations, traction bronchiectasis, honeycombing, consolidations, air-trapping, nodules, bronchial wall thickening, bronchiectasis, mucus plugging, effusion). Histology scoring (HS) evaluated changes of the bronchial wall and of the interstitium (fibrosis, inflammation, anthracosis, granuloma, tumor, infection, metaplasia, mucoid impactation, microhoneycombing, eosinophilia). Results: Baseline characteristics were similar between groups. Most CVID patients received immunoglobulins (Ig) and all ILD patients were treated immunosuppressive (IS). Under Ig and IS lung function remained stable in ILD. CTS were worse for ILD than noILD (ILD:16.5±8.7 vs. noILD:9.4±4.9, p=0.02) while HS (ILD:5.8±2.7, noILD 6.0±2.6) were similar. CTS deteriorated in ILD but not in noILD with pattern changes in ILD, from initial mainly GGO to fibrosis (p Conclusion: CVID with and without GLILD has similar clinical characteristics but differs mainly in CT scoring. Future work has to assess clinical predictors of GLILD.","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":"114931156","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

Transbronchial cryobiopsy in interstitial lung disease 间质性肺疾病的经支气管低温活检

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA3029

L. Hagmeyer, D. Theegarten, M. Treml, A. Pietzke-Calcagnile, S. Herkenrath, W. Randerath

引用次数: 10

Atypical presentation of a rare lung condition: axillary and paraspinal lymphadenopathy in a patient with clinical and radiological features of lymphangioleomatosis (LAM) 罕见肺部疾病的不典型表现:以淋巴血管油瘤病(LAM)为临床和影像学表现的腋窝和棘旁淋巴结病患者

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.pa3032

A. Kavidasan, Vicky Taylor, S. Chaudhry, R. Siva, Y. Raste

引用次数: 0

Eosinophilic Pneumonia Due to Sulphasalazine Use 磺胺嘧啶引起的嗜酸性肺炎

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.pa3026

S. Sökücü, C. Özdemir, Özlem Kahya, S. ÖnÜr

{"title":"Eosinophilic Pneumonia Due to Sulphasalazine Use","authors":"S. Sökücü, C. Özdemir, Özlem Kahya, S. ÖnÜr","doi":"10.1183/13993003.congress-2018.pa3026","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.pa3026","url":null,"abstract":"Sulfasalazine is a compound of 5-aminosalicylic acid and sulfapyridine joined by an azo bond. There are only case reports in the literature about pulmonary toxicity caused by this drug. A 23-year old man diagnosed as ulcerative colitis 3 months ago admitted to our hospital with a 1 month history of fever, cough, dyspnoea. He didn9t respond to antibiotics. He was a nonsmoker and had been on sulfazalazine and mesalazine treatment for 8 weeks. All of his medications were held on admission. He had fever (38.6 °C) with respiratory rate of 20/min with normal oxygen saturations in room air Physical examination revealed crepitation over right lung on auscultation. Chest radiograph showed multilobar pulmonary infiltrates dominated right lung peripherally. He has peripheral eosinophilia. For differential diagnosis, an autoimmune screen, Ig E level and parasite search in his feces was negative. Sputum culture was negative for pathogenic bacteria and acid-fast bacilli. HRCT revealed patchy consolidations at all zones of the right lung and upper zone of left lung, peribronchial and interlobuler septal thickenings, ground glass opasities. No endobronchial pathology was detected. Bronchoalveolar lavage done from medial segment of right middle lobe was negative for viral, tuberculous and fungal infections. The differential cell count showed 20% eosinophils. After withdrawal of sulfazalazine, pulmonary infiltrates started to regress in the control chest radiograph but respiratory symptoms were not resolved completely so oral prednisone treatment was started. 2 weeks later, his chest radiograph was dramatically improved with near complete resolution of the pulmonary infiltrates. The patient is in our follow up for 6 months without any symptoms.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130106725","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

Traction bronchiectasis and platythorax on computed tomography are determinants of progression and mortality in pleuro-parenchymal fibroelastosis 在计算机断层扫描上，牵引性支气管扩张和平胸是胸膜实质纤维弹性增生的进展和死亡率的决定因素

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2232

P. George, D. Armstrong-James, A. Devaraj, E. Renzoni, T. Maher, A. Wells, I. Pulzato, P. Molyneaux, M. Kokosi, V. Kouranos, G. Margaritopoulos, S. Desai, F. Chua

{"title":"Traction bronchiectasis and platythorax on computed tomography are determinants of progression and mortality in pleuro-parenchymal fibroelastosis","authors":"P. George, D. Armstrong-James, A. Devaraj, E. Renzoni, T. Maher, A. Wells, I. Pulzato, P. Molyneaux, M. Kokosi, V. Kouranos, G. Margaritopoulos, S. Desai, F. Chua","doi":"10.1183/13993003.CONGRESS-2018.PA2232","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2232","url":null,"abstract":"Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial disease characterised by accumulation of elastin-rich fibrosis and predominant upper lobe shrinkage. The pathogenesis is poorly understood but idiopathic and secondary forms are recognised; an association with Aspergillus infection has been reported. We evaluated computed tomography (CT) and clinical/serological indices to identify factors influencing progression and mortality in patients with PPFE. CT studies in 72 patients, with a multidisciplinary diagnosis of PPFE, were consensus scored by two thoracic radiologists. The presence and severity of traction bronchiectasis in areas of PPFE correlated with the extent, severity and progression of PPFE (all p 40 mgA/L) was seen in 18/72 (25%) patients and was higher than in reference cohorts of idiopathic pulmonary fibrosis (20/105; 19%) and chronic hypersensitivity pneumonitis (12/111; 11%) (p=0.04). These data highlight the importance of platythorax as a clinico-radiological marker of volume loss and, more importantly, an index of poor outcome in PPFE, potentially reflecting inexorable physiological decline. The possibility that fungal sensitisation may form part of a pathogenetic or progressive PPFE profile warrants further investigation.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"57 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126688480","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

Pneumothorax in pulmonary langerhans cell histiocytosis (PLCH) 肺朗格汉斯细胞组织细胞增多症(PLCH)并发气胸

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2241

P. Guen, S. Chevret, E. Bugnet, C. Margerie-Mellon, F. Jouenne, G. Lorillon, A. Seguin-Givelet, S. Mourah, D. Gossot, R. Vassallo, A. Tazi

{"title":"Pneumothorax in pulmonary langerhans cell histiocytosis (PLCH)","authors":"P. Guen, S. Chevret, E. Bugnet, C. Margerie-Mellon, F. Jouenne, G. Lorillon, A. Seguin-Givelet, S. Mourah, D. Gossot, R. Vassallo, A. Tazi","doi":"10.1183/13993003.CONGRESS-2018.PA2241","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2241","url":null,"abstract":"Background: Pneumothorax (PNO) is a salient complication of PLCH, which management is not standardized. The factors associated with PNO recurrence are poorly understood. Objectives: To determine PNO outcome after thoracic surgery and search for factors associated with the risk of recurrence. Methods: All PLCH patients ≥18 years with ≥1PNO between 11/2003 and 12/2015 of the database of the National Registry for histiocytoses and followed ≥6 months were retrospectively studied. Kaplan Meier method and univariate Cox models were used for statistical analyses. Results: Among the 43 patients included (39 smokers), 53% presented at least 1 PNO recurrence during a 49 months median time (53 additional episodes, 75% ipsilateral, all within 2 years). Thoracic surgery did not modify the risk of PNO recurrence, as compared to conservative treatment (p=0.96). However, the rate of recurrences was lower after thoracotomy (TCT), as compared to video-assisted thoracoscopy (VATS) (p=0.03). In the univariate analyses, lung function air trapping at diagnosis was associated with increased hazard of PNO recurrence (p=0.03). Patients whose LCH lesion harboured the BRAFV600E mutation had a lower rate of ipsilateral recurrences (p=0.019). When incorporating all ipsilateral recurrences, VATS was associated with increased hazard of subsequent recurrence (p=0.05). Conclusions: Surgery did not decrease the risk of PNO recurrence in PLCH. Thus, no particular treatment can be recommended for the first PNO in PLCH patients. In case of recurrence, TCT which appears more effective than VATS to prevent subsequent recurrence, should be privileged. The reduced risk of PNO recurrence associated with the presence of BRAFV600E mutation needs to be confirmed.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"119 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121591912","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

Cytokine profiling in Pulmonary Langerhans Cell Histiocytosis: novel insights into pathogenesis. 肺朗格汉斯细胞组织细胞增多症的细胞因子谱分析:对发病机制的新见解。

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2242

Silvia Barril Farre, S. Hortelano, A. Luque, M. Higueras, C. Rodríguez-Martín, C. Robledo, A. Feliu, P. Millán, O. Sibila, F. J. Alonso, D. Castillo

{"title":"Cytokine profiling in Pulmonary Langerhans Cell Histiocytosis: novel insights into pathogenesis.","authors":"Silvia Barril Farre, S. Hortelano, A. Luque, M. Higueras, C. Rodríguez-Martín, C. Robledo, A. Feliu, P. Millán, O. Sibila, F. J. Alonso, D. Castillo","doi":"10.1183/13993003.CONGRESS-2018.PA2242","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2242","url":null,"abstract":"Introduction: Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon disorder link with tobacco smoking. Although an abnormal inflammatory response to tobacco particles has been consider the hallmark of PLCH pathogenesis, there is limited knowledge about the mediators of inflammation in PLCH. Objectives: To investigate inflammatory serum profile in PLCH patients. Methods: Blood samples and clinical data were obtained from adult patients with PLCH. Cytokine profile in serum of these patients and 5 healthy adult volunteers were analysed using the Cytokine Human Membrane Antibody Array. Results: Twelve patients with PLCH were included. Median age (±SD) was 40±14 years old. All were current smokers and only 1 (8,3%) had multisystemic disease. Mean FVC (% predicted) was 85±20% and DLCO (% predicted) was 65±11%. Regarding the 80 cytokine evaluated, there were significant differences between healthy and PLCH patients in levels of MCP-1 (CCL2), MCP-2 (CCL8), SCF, TARC (CCL17), IGF-I, Oncostatin M, Thrombopoietin, FDF-7, Fractalkine, LIF and TIMP-2 (see figure 1). Conclusions: Cytokine profile in patients with PLCH has a distinctive pattern compare to health subjects. Our data supports that IL-6 family could have a meaningful role in the pathogenesis of PLCH.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"23 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2018-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122879758","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

Vascular endothelial growth factors and matrix metalloproteinases serum levels for LAM diagnosis in patients with sporadic LAM and tuberous sclerosis 血管内皮生长因子和基质金属蛋白酶血清水平对散发性LAM和结节性硬化症患者LAM诊断的意义

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.OA3785

S. Terraneo, F. Marco, G. Imeri, L. Giuliani, S. Ancona, P. Cetrangolo, E. Grasso, S. Centanni, E. Lesma

引用次数: 1

Disease characteristics and outcomes in a Swedish cohort of patients with pulmonary fibrosis (PF) 瑞典肺纤维化(PF)患者队列的疾病特征和结局

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA3028

C. Sköld, K. Bartley, A. Levine, L. Arnheim‐Dahlström, K. Kirchgaessler, R. Linder, C. Janson, G. Ferrara

引用次数: 0