Rare ILD/DPLD最新文献_第6页

Prevalence of progressive fibrosing interstitial lung disease 进行性纤维化间质性肺病的患病率

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA3030

A. Olson, Nadine Hartmann, R. Schlenker‐Herceg, L. Wallace

{"title":"Prevalence of progressive fibrosing interstitial lung disease","authors":"A. Olson, Nadine Hartmann, R. Schlenker‐Herceg, L. Wallace","doi":"10.1183/13993003.CONGRESS-2018.PA3030","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA3030","url":null,"abstract":"Background: There is an important group of patients with a progressive fibrosing form of interstitial lung disease (PF-ILD) (Flaherty, K.R. et al. BMJ Open Respir Res 2017;4:e000212) who do not meet idiopathic pulmonary fibrosis (IPF) diagnostic criteria but have a similar natural history and prognosis. Compared to IPF, the relative prevalence of PF-ILD is unknown. Aims and objectives: To estimate the prevalence of PF-ILD in Europe and the USA. Methods: Systematic literature review in Medline and Embase (1990–2017) focused on the prevalence of ILD and the forms of ILD known to be at risk for a progressive fibrosing phenotype. Supplemented by data from physician surveys and interviews, prevalence estimates were generated for each subtype and then combined to estimate overall PF-ILD prevalence. Sensitivity analyses were performed to determine the upper bounds of the estimate. Results: The overall prevalence of ILD (per 10,000 persons) was reported as 0.63–7.6 in 4 studies in Europe and 7.43 in the USA. Prevalence estimates for individual progressive fibrosing ILDs and PF-ILD overall are presented in the Table. PF-ILD prevalence (per 10,000 persons) ranged from 0.22–2.0 in Europe and was 2.80 in the USA. Conclusions: PF-ILD affects fewer than 5 in 10,000 persons in Europe and the USA, a patient population with an unmet need for treatment. Funding: Boehringer Ingelheim. Editorial assistance: Complete HealthVizion.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"302 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":"123398036","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: 8

Effectiveness of inhibitor mTOR in patients with lymphangioleiomyomatosis. mTOR抑制剂治疗淋巴管平滑肌瘤的疗效。

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2240

L. Novikova, Mihail Ilkovich, D. Dzadzua, O. Baranova, M. Vasilyeva

引用次数: 1

Genetic landscape of pulmonary langerhans cell histiocytosis 肺朗格汉斯细胞组织细胞增多症的遗传图谱

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.OA3781

F. Jouenne, G. Lorillon, Carine Laurent-Issartel, A. Sadoux, V. Meignin, C. Leschi, E. Bugnet, S. Mourah, A. Tazi

引用次数: 3

Pulmonary interstitial Glycogenosis – a systematic analysis of new cases 肺间质性糖原症——新病例的系统分析

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.oa3787

E. Seidl, J. Carlens, S. Reu, M. Wetzke, J. Ley-Zaporozhan, F. Brasch, W. Wesselak, A. Schams, Daniela Rauch, Luise A. Schuch, M. Kappler, P. Schelstraete, M. Wolf, F. Stehling, E. Haarmann, D. Borensztajn, M. V. D. Loo, S. Rubak, B. Hinrichs, N. Schwerk, M. Griese

{"title":"Pulmonary interstitial Glycogenosis – a systematic analysis of new cases","authors":"E. Seidl, J. Carlens, S. Reu, M. Wetzke, J. Ley-Zaporozhan, F. Brasch, W. Wesselak, A. Schams, Daniela Rauch, Luise A. Schuch, M. Kappler, P. Schelstraete, M. Wolf, F. Stehling, E. Haarmann, D. Borensztajn, M. V. D. Loo, S. Rubak, B. Hinrichs, N. Schwerk, M. Griese","doi":"10.1183/13993003.congress-2018.oa3787","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.oa3787","url":null,"abstract":"Background: Pulmonary interstitial glycogenosis (PIG) is a rare interstitial lung disease in children. The clinical hallmark is a rapid onset with respiratory distress and hypoxemia shortly after birth in the absence of an infectious cause or a surfactant dysfunction syndrome. The diagnosis can only be made by lung biopsy. The histopathological pattern defining PIG can exist in diffuse or patchy distribution. Only few cases have been reported. Not much is known on clinical features, outcomes, CT imaging and histopathology. Methods: The clinical course, CT-scans and tissue samples of children diagnosed with PIG were collected and systematically re-analysed by clinicians, radiologists and pathologists all specialized in interstitial lung diseases in children. All data were uploaded into the Kidslungregister for follow up. Results: 11 children diagnosed with PIG were included in this study. All presented with respiratory distress shortly after birth. More than half of the children were diagnosed with additional abnormalities, especially congenital heart defects. The CT-scan of the lungs showed mainly groundglass opacities, consolidations and septal thickening. Of interest each tissue sample had signs of reduced alveolarization. The prognosis was favourable in almost all cases. When systemic glucocorticosteroids were given a fast improvement was noticed. Conclusion: PIG is a interstitial lung disease in infants with mostly favourable outcome. As alveolar growth retardation was present in all subjects this can support a theory of an underlying maturation delay. In all infants with congenital heart defects and unexplained respiratory distress an interstitial lung disease due to PIG should be considered.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"54 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":"127894145","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

Chemotherapy in patients with pulmonary Langerhans cell histiocytosis 肺朗格汉斯细胞组织细胞增多症患者的化疗

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.oa3782

E. Radzikowska, E. Wiatr, Katarzyna Blasnska-Przerwa, K. Roszkowski-Śliż

{"title":"Chemotherapy in patients with pulmonary Langerhans cell histiocytosis","authors":"E. Radzikowska, E. Wiatr, Katarzyna Blasnska-Przerwa, K. Roszkowski-Śliż","doi":"10.1183/13993003.congress-2018.oa3782","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.oa3782","url":null,"abstract":"Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder, affecting mainly young adult smoker. The aim of this study was to present the results of cladribine (2-CdA) treatment in patients with PLCH. Patients and method: This retrospective analysis included 12 patients (7 females and 5 males; aged mean 40.08 ± 6.7 years) with LCH treated with 2-CDa in years 2010 to 2017. Eight patients had multi system and 4 isolated pulmonary LCH. Patients received 2 to 6 courses of 2-CdA, as a single agent in a dose of 0.15 mg/kg per day/ iv. for 5 consecutive days at monthly intervals. Results: Treatment resulted in improvement or stabilisation of pulmonary function parameters in 5(36%) and 7(64%) patients respectively. Two patients with bone lesions, one with abdominal changes and one woman with infiltration in vertebra and in periaortic space experienced partial regression and disease was not active. Treatment related toxicities were: upper respiratory tract infections grade 2 in 7(64%) patients, and grade 3 in 2(18%), leukopenia grade 1 in 3(25%), grade 2 in 4 (33%), grade 3 in 1(9%) patients, lymphopenia grade 1 in 3(25%), grade 2 in 7(64%), and grade 3 in 2(18%) patients, thrombocytopenia grade 1 in 2(17%), grade 2 in 1(9%), grade 4 in one (9 %) patient, anaemia grade 4 and 2 in one (9%) patient respectively. There was no case of LCH progression. During observation (mean 111.5 ± 59.22 months), one patient suddenly died with unknown cause, other one developed chronic myelogenic leukaemia. No reactivation of the disease has been noticed. Conclusion: Cladribine as a single agent is an effective therapy in adult patients with progressive PLCH but was mainly resulted in stabilization of pulmonary function.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"132 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":"127414866","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

Unclassifiable interstitial lung disease: a distinct entity with heterogeneous progression 无法分类的间质性肺疾病:具有异质性进展的独特实体

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2245

M. Nasser, P. Rigaud, K. Ahmad, J. Traclet, V. Cottin

引用次数: 1

Pulmonary Lymphangiomatosis – insights into an ultra-rare disease 肺淋巴管瘤病——一种极为罕见的疾病

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2246

Nilab Sarmand, S. Piel, Eva Brunnemer, J. Waelscher, M. Polke, A. Warth, C. Heussel, M. Eichinger, Felix Lasistschka, Benjamin Hoegerle, L. Frankenstein, H. Zabeck, S. Rieken, F. Herth, M. Kreuter

引用次数: 1

Prevalence of pleuroparenchymal fibroelastosis (PPFE): A retrospective single-centre case study 胸膜实质纤维弹性增生(PPFE)的患病率:一项回顾性单中心病例研究

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2230

S. Geleff, Jasmin Gmeiner, P. Jaksch, H. Prosch

{"title":"Prevalence of pleuroparenchymal fibroelastosis (PPFE): A retrospective single-centre case study","authors":"S. Geleff, Jasmin Gmeiner, P. Jaksch, H. Prosch","doi":"10.1183/13993003.CONGRESS-2018.PA2230","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2230","url":null,"abstract":"PPFE is a rare, idiopathic interstitial pneumonia with specific clinical, radiological, and pathological features. It affects the visceral pleura and the subpleural parenchyma with an upper lobe predilection and can be associated with a separate interstitial lung disease (ILD). The aim of this study was to evaluate the prevalence of pluridisciplinary diagnosis of PPFE in a cohort of 82 explanted fibrotic lungs, between 2014 and 2016. The inclusion criteria were availability of imaging data before transplantation, and histological diagnosis of fibrosis. The radiological evaluation followed the ATS/ERS 2003 classification of PPFE, and the histological classification of ILD conformed to the 2013 ATS/ERS guidelines. We further classified PPFE as definite or probable and as idiopathic (iPPFE) or PPFE associated with ILD. Pre-transplant CT-imaging was available in 62 of 82 lungs, with 15 classified as definite PPFE based on the radiologic pattern, i.e., bilateral pleural thickening in the upper lobes with subpleural dense areas of airspace consolidation. An additional CT pattern of chronic hypersensitivity pneumonitis was diagnosed in 8 cases, which had histological UIP /NSIP patterns. In 2 cases, IPF was the underlying ILD. 5 cases were classified as iPPFE. All 15 cases had histological upper lobe pleural and subpleural fibroelastosis. A sharp demarcation from the unaffected parenchyma was prominent in iPPFE. One case was radiologically classified as probable PPFE with a histological UIP-pattern, but no proof of PPFE. As both iPPFE and PPFE associated with ILD carry a poor prognosis, the importance of diagnosing this clinicopathologic entity cannot be overemphasized.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"14 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":"125124822","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 toxicity to mesalazine 对美沙拉嗪的肺毒性

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA3027

Maria Raquel Araújo Pereira, M. J. Araújo, F. Aguiar, J. Lages, Ana Luísa Vieira, J. Cruz

引用次数: 2

Case of lymphangioleiomyomatosis complicated by severe alveolar hemorrhagic syndrome 淋巴管平滑肌瘤病合并严重肺泡出血性综合征1例

Rare ILD/DPLD Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.pa3033

O. Fesenko, Svetlana Shwaiko, Z. Sheykh, O. Paklina, E. Kulaeva

引用次数: 1