首页 > 最新文献

Rare ILD/DPLD最新文献

英文 中文
Prevalence of progressive fibrosing interstitial lung disease 进行性纤维化间质性肺病的患病率
Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA3030
A. Olson, Nadine Hartmann, R. Schlenker‐Herceg, L. Wallace
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.
背景:有一个重要的患者群体患有进行性纤维化形式的间质性肺病(PF-ILD) (Flaherty, K.R.等)。不符合特发性肺纤维化(IPF)诊断标准,但具有相似的自然病史和预后的患者。与IPF相比,PF-ILD的相对患病率尚不清楚。目的和目的:估计PF-ILD在欧洲和美国的患病率。方法:Medline和Embase(1990-2017)的系统文献综述,重点关注ILD的患病率以及已知具有进行性纤维化表型风险的ILD形式。补充来自医生调查和访谈的数据,得出每个亚型的患病率估计,然后结合估计总体PF-ILD患病率。进行敏感性分析以确定估计的上界。结果:在欧洲的4项研究中,ILD的总患病率(每10,000人)为0.63-7.6,在美国为7.43。个别进行性纤维化性ild和整体PF-ILD的患病率估计见表。PF-ILD患病率(每10,000人)在欧洲为0.22-2.0,在美国为2.80。结论:在欧洲和美国,PF-ILD的发生率低于5 / 10000,这是一个治疗需求未得到满足的患者群体。融资:勃林格殷格翰公司。编辑协助:完整的健康视野。
{"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。
Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2240
L. Novikova, Mihail Ilkovich, D. Dzadzua, O. Baranova, M. Vasilyeva
{"title":"Effectiveness of inhibitor mTOR in patients with lymphangioleiomyomatosis.","authors":"L. Novikova, Mihail Ilkovich, D. Dzadzua, O. Baranova, M. Vasilyeva","doi":"10.1183/13993003.CONGRESS-2018.PA2240","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2240","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"211 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":"121220062","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
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
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
{"title":"Genetic landscape of pulmonary langerhans cell histiocytosis","authors":"F. Jouenne, G. Lorillon, Carine Laurent-Issartel, A. Sadoux, V. Meignin, C. Leschi, E. Bugnet, S. Mourah, A. Tazi","doi":"10.1183/13993003.CONGRESS-2018.OA3781","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.OA3781","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"16 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":"126118817","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: 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
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
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.
背景:肺间质性糖原病是一种罕见的儿童间质性肺疾病。临床特征是在没有感染原因或表面活性剂功能障碍综合征的情况下,出生后不久迅速发作呼吸窘迫和低氧血症。只能通过肺活检来诊断。定义PIG的组织病理学模式可以呈弥漫性或斑片状分布。只有少数病例被报道。对临床特征、结果、CT成像和组织病理学知之甚少。方法:由儿童间质性肺疾病的临床医师、放射科医师和病理学家对诊断为PIG的儿童的临床病程、ct扫描和组织样本进行系统的再分析。所有数据都被上传到kidslunregister以供后续跟进。结果:本研究纳入了11名确诊为PIG的儿童。都在出生后不久出现呼吸窘迫。超过一半的儿童被诊断出有其他异常,尤其是先天性心脏缺陷。肺部ct扫描主要显示磨玻璃影、实变及间隔增厚。有趣的是,每个组织样本都有肺泡化减少的迹象。几乎所有病例的预后都很好。当给予全身糖皮质激素后,病情迅速好转。结论:猪肺是一种婴幼儿间质性肺疾病,预后良好。由于肺泡生长迟缓存在于所有受试者中,这可以支持潜在成熟延迟的理论。在所有有先天性心脏缺陷和不明原因呼吸窘迫的婴儿中,应考虑由PIG引起的间质性肺疾病。
{"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
Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.oa3782
E. Radzikowska, E. Wiatr, Katarzyna Blasnska-Przerwa, K. Roszkowski-Śliż
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.
肺朗格汉斯细胞组织细胞增多症(PLCH)是一种罕见的疾病,主要影响年轻的成年吸烟者。本研究的目的是介绍氯德里滨(2-CdA)治疗PLCH患者的结果。患者和方法:回顾性分析12例患者(女7例,男5例;2010 ~ 2017年2-CDa治疗LCH患者平均年龄(40.08±6.7岁)。多系统LCH 8例,孤立性肺LCH 4例。患者接受2- cda治疗2- 6个疗程,单药剂量为0.15 mg/kg / d / iv,以每月为间隔连续5天。结果:治疗使5例(36%)和7例(64%)患者的肺功能参数改善或稳定。2例骨病变患者、1例腹部病变患者和1例椎体及主动脉周围间隙浸润患者出现部分消退,病情不活跃。治疗相关的毒性为:上呼吸道感染2级7例(64%),3级2例(18%),白细胞减少1级3例(25%),2级4例(33%),3级1例(9%),淋巴细胞减少1级3例(25%),2级7例(64%),3级2例(18%),血小板减少1级2例(17%),2级1例(9%),4级1例(9%),贫血4级和2级1例(9%)。无LCH进展病例。在观察期间(平均111.5±59.22个月),1例患者突然死亡原因不明,1例发展为慢性髓性白血病。没有发现这种疾病的复发。结论:克拉德滨单药治疗成人进行性PLCH是一种有效的治疗方法,但主要以稳定肺功能为主。
{"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
Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2245
M. Nasser, P. Rigaud, K. Ahmad, J. Traclet, V. Cottin
{"title":"Unclassifiable interstitial lung disease: a distinct entity with heterogeneous progression","authors":"M. Nasser, P. Rigaud, K. Ahmad, J. Traclet, V. Cottin","doi":"10.1183/13993003.CONGRESS-2018.PA2245","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2245","url":null,"abstract":"","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":"115181650","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 Lymphangiomatosis – insights into an ultra-rare disease 肺淋巴管瘤病——一种极为罕见的疾病
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
{"title":"Pulmonary Lymphangiomatosis – insights into an ultra-rare disease","authors":"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","doi":"10.1183/13993003.CONGRESS-2018.PA2246","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2246","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"35 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":"133308123","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
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
Pub Date : 2018-09-15 DOI: 10.1183/13993003.CONGRESS-2018.PA2230
S. Geleff, Jasmin Gmeiner, P. Jaksch, H. Prosch
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.
PPFE是一种罕见的特发性间质性肺炎,具有特殊的临床、放射学和病理特征。它影响内脏胸膜和胸膜下实质,并以上肺叶为主,可与单独的间质性肺疾病(ILD)相关。本研究的目的是评估2014年至2016年期间82例外植纤维化肺中PPFE多学科诊断的患病率。纳入标准是移植前影像学资料的可用性和纤维化的组织学诊断。放射学评估遵循ATS/ERS 2003对PPFE的分类,ILD的组织学分类符合2013 ATS/ERS指南。我们进一步将PPFE分为明确或可能、特发性(iPPFE)或与ILD相关的PPFE。82个肺中有62个肺移植前ct成像,其中15个肺根据放射学模式分类为明确的PPFE,即双侧胸膜上叶增厚伴胸膜下致密空域实变。8例CT表现为慢性超敏性肺炎,组织学表现为UIP /NSIP。2例IPF为潜在ILD。5例为iPPFE。15例均有组织学胸膜上叶及胸膜下纤维弹性增生。iPPFE与未受影响的实质有明显的界限。1例放射学分类为可能的PPFE,组织学上为upp型,但没有PPFE的证据。由于iPPFE和与ILD相关的PPFE预后都很差,诊断这种临床病理实体的重要性怎么强调都不过分。
{"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
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
{"title":"Pulmonary toxicity to mesalazine","authors":"Maria Raquel Araújo Pereira, M. J. Araújo, F. Aguiar, J. Lages, Ana Luísa Vieira, J. Cruz","doi":"10.1183/13993003.CONGRESS-2018.PA3027","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA3027","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"120 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":"128065631","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
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
Pub Date : 2018-09-15 DOI: 10.1183/13993003.congress-2018.pa3033
O. Fesenko, Svetlana Shwaiko, Z. Sheykh, O. Paklina, E. Kulaeva
{"title":"Case of lymphangioleiomyomatosis complicated by severe alveolar hemorrhagic syndrome","authors":"O. Fesenko, Svetlana Shwaiko, Z. Sheykh, O. Paklina, E. Kulaeva","doi":"10.1183/13993003.congress-2018.pa3033","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.pa3033","url":null,"abstract":"","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":"126253075","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
期刊
Rare ILD/DPLD
全部 Acc. Chem. Res. ACS Applied Bio Materials ACS Appl. Electron. Mater. ACS Appl. Energy Mater. ACS Appl. Mater. Interfaces ACS Appl. Nano Mater. ACS Appl. Polym. Mater. ACS BIOMATER-SCI ENG ACS Catal. ACS Cent. Sci. ACS Chem. Biol. ACS Chemical Health & Safety ACS Chem. Neurosci. ACS Comb. Sci. ACS Earth Space Chem. ACS Energy Lett. ACS Infect. Dis. ACS Macro Lett. ACS Mater. Lett. ACS Med. Chem. Lett. ACS Nano ACS Omega ACS Photonics ACS Sens. ACS Sustainable Chem. Eng. ACS Synth. Biol. Anal. Chem. BIOCHEMISTRY-US Bioconjugate Chem. BIOMACROMOLECULES Chem. Res. Toxicol. Chem. Rev. Chem. Mater. CRYST GROWTH DES ENERG FUEL Environ. Sci. Technol. Environ. Sci. Technol. Lett. Eur. J. Inorg. Chem. IND ENG CHEM RES Inorg. Chem. J. Agric. Food. Chem. J. Chem. Eng. Data J. Chem. Educ. J. Chem. Inf. Model. J. Chem. Theory Comput. J. Med. Chem. J. Nat. Prod. J PROTEOME RES J. Am. Chem. Soc. LANGMUIR MACROMOLECULES Mol. Pharmaceutics Nano Lett. Org. Lett. ORG PROCESS RES DEV ORGANOMETALLICS J. Org. Chem. J. Phys. Chem. J. Phys. Chem. A J. Phys. Chem. B J. Phys. Chem. C J. Phys. Chem. Lett. Analyst Anal. Methods Biomater. Sci. Catal. Sci. Technol. Chem. Commun. Chem. Soc. Rev. CHEM EDUC RES PRACT CRYSTENGCOMM Dalton Trans. Energy Environ. Sci. ENVIRON SCI-NANO ENVIRON SCI-PROC IMP ENVIRON SCI-WAT RES Faraday Discuss. Food Funct. Green Chem. Inorg. Chem. Front. Integr. Biol. J. Anal. At. Spectrom. J. Mater. Chem. A J. Mater. Chem. B J. Mater. Chem. C Lab Chip Mater. Chem. Front. Mater. Horiz. MEDCHEMCOMM Metallomics Mol. Biosyst. Mol. Syst. Des. Eng. Nanoscale Nanoscale Horiz. Nat. Prod. Rep. New J. Chem. Org. Biomol. Chem. Org. Chem. Front. PHOTOCH PHOTOBIO SCI PCCP Polym. Chem.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1