Pub Date : 2018-09-15DOI: 10.1183/13993003.congress-2018.pa3021
J. Bermudez, Benjamin Coulon, Y. Trigui, Carine Gomez, J. Mege, N. Bardin, M. Reynaud‐Gaubert
{"title":"Lung involvement associated with anti-NXP2 autoantibodies: a monocentre observational French study","authors":"J. Bermudez, Benjamin Coulon, Y. Trigui, Carine Gomez, J. Mege, N. Bardin, M. Reynaud‐Gaubert","doi":"10.1183/13993003.congress-2018.pa3021","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.pa3021","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"38 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":"123341494","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.PA3025
N. Takeuchi, T. Arai, Y. Sasaki, Y. Matsuda, K. Katayama, C. Sugimoto, K. Tachibana, S. Hayashi, M. Akira, T. Kasai, M. Kitaichi, Y. Inoue
Objectives: We aim to clarify relapse predictive factors of chronic eosinophilic pneumonia (CEP). Subjects and Methods: We extracted 61 consecutive cases of CEP in our institution between 1999 and 2016. 5 cases were excluded because they did not undergo bronchoalveolar lavage (BAL) and/or high-resolution (HR) CT at diagnosis. We retrospectively reviewed 56 cases with CEP, and examined relapse of CEP by December 2017. Relapse was defined as increased dose of corticosteroid or deterioration of radiological findings consistent with CEP during the clinical course after the start of the treatment. We investigated the predictive factors of relapse of CEP by univariate and multivariate Cox proportional hazard regression analysis. Results: The median (IQR) age at diagnosis was 61.5 (40.3-70.8) years old. There were 25 males and 31 females. 31 patients (55.3%) were never smokers, and 19 patients (33.9%) had asthma. The median (IQR) laboratory findings at diagnosis revealed peripheral blood eosinophilia: 22.8 (10.7-43.7)%, BAL eosinophilia: 28.5 (10.7-65.5)%. 32 patients received corticosteroid therapy. Radiologic findings on HRCT showed centrilobular opacities in 26 patients (46.4%). The relapse of CEP was seen in 20 patients (35.7%) during the observation period (median: 504 days, IQR: 139.8-1171.8 days). The patients with centrilobular opacities show significantly earlier relapse than that without centrilobular opacities (p=0.0224) by univariate analysis. However, the percentages of eosinophils of BAL and peripheral blood, and serum immunoglobulin-E were not significant predictive factors of relapse of CEP. Conclusion: Relapse of CEP could be predicted by centrilobular opacities on HRCT at the diagnosis of CEP.
{"title":"Relapse predictive factors of chronic eosinophilic pneumonia","authors":"N. Takeuchi, T. Arai, Y. Sasaki, Y. Matsuda, K. Katayama, C. Sugimoto, K. Tachibana, S. Hayashi, M. Akira, T. Kasai, M. Kitaichi, Y. Inoue","doi":"10.1183/13993003.CONGRESS-2018.PA3025","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA3025","url":null,"abstract":"Objectives: We aim to clarify relapse predictive factors of chronic eosinophilic pneumonia (CEP). Subjects and Methods: We extracted 61 consecutive cases of CEP in our institution between 1999 and 2016. 5 cases were excluded because they did not undergo bronchoalveolar lavage (BAL) and/or high-resolution (HR) CT at diagnosis. We retrospectively reviewed 56 cases with CEP, and examined relapse of CEP by December 2017. Relapse was defined as increased dose of corticosteroid or deterioration of radiological findings consistent with CEP during the clinical course after the start of the treatment. We investigated the predictive factors of relapse of CEP by univariate and multivariate Cox proportional hazard regression analysis. Results: The median (IQR) age at diagnosis was 61.5 (40.3-70.8) years old. There were 25 males and 31 females. 31 patients (55.3%) were never smokers, and 19 patients (33.9%) had asthma. The median (IQR) laboratory findings at diagnosis revealed peripheral blood eosinophilia: 22.8 (10.7-43.7)%, BAL eosinophilia: 28.5 (10.7-65.5)%. 32 patients received corticosteroid therapy. Radiologic findings on HRCT showed centrilobular opacities in 26 patients (46.4%). The relapse of CEP was seen in 20 patients (35.7%) during the observation period (median: 504 days, IQR: 139.8-1171.8 days). The patients with centrilobular opacities show significantly earlier relapse than that without centrilobular opacities (p=0.0224) by univariate analysis. However, the percentages of eosinophils of BAL and peripheral blood, and serum immunoglobulin-E were not significant predictive factors of relapse of CEP. Conclusion: Relapse of CEP could be predicted by centrilobular opacities on HRCT at the diagnosis of CEP.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"53 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":"121079636","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.PA2238
D. Marangu, K. Pillay, E. Banderker, A. Vanker, D. Gray, M. Zampoli
{"title":"Exogenous lipoid pneumonia in African children: a mixed-methods case series","authors":"D. Marangu, K. Pillay, E. Banderker, A. Vanker, D. Gray, M. Zampoli","doi":"10.1183/13993003.CONGRESS-2018.PA2238","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2238","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"7 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":"115130273","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.congress-2018.pa3023
G. Dabar, Z. Aoun, M. Riachi, J. Boutros, G. Khayat
{"title":"Effect of MUC5B gene polymorphism on survival in a Lebanese population of patients with idiopathic pulmonary fibrosis","authors":"G. Dabar, Z. Aoun, M. Riachi, J. Boutros, G. Khayat","doi":"10.1183/13993003.congress-2018.pa3023","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.pa3023","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"1960 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":"129405597","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.PA3017
E. Yamaguchi, Kenshi Kosaka, Toshiyuki Yonezawa, H. Shibata, A. Matsubara, Toshi Kato, Hiroyuki Tanaka, Norihito Yokoe, S. Ito, A. Kubo
{"title":"Neutralizing capacity of autoantibody against GM-CSF in patients with autoimmune pulmonary alveolar proteinosis","authors":"E. Yamaguchi, Kenshi Kosaka, Toshiyuki Yonezawa, H. Shibata, A. Matsubara, Toshi Kato, Hiroyuki Tanaka, Norihito Yokoe, S. Ito, A. Kubo","doi":"10.1183/13993003.CONGRESS-2018.PA3017","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA3017","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"55 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":"115288649","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.PA3019
M. Kokosi, P. Saunders, P. Molyneaux, P. George, G. Margaritopoulos, V. Kouranos, T. Pickworth, F. Chua, T. Maher, E. Renzoni, A. Wells, C. Morgan
{"title":"Lung cysts in pulmonary alveolar proteinosis.","authors":"M. Kokosi, P. Saunders, P. Molyneaux, P. George, G. Margaritopoulos, V. Kouranos, T. Pickworth, F. Chua, T. Maher, E. Renzoni, A. Wells, C. Morgan","doi":"10.1183/13993003.CONGRESS-2018.PA3019","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA3019","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"67 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":"132384020","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.OA3783
Y. Inoue, E. Yamaguchi, Y. Setoguchi, T. Ichiwata, M. Ebina, Kazutoshi Cho, R. Tazawa, H. Ishii, T. Kasai, M. Akira, T. Arai, K. Uchida, H. Kida, K. Morimoto, M. Kitaichi, C. Sugimoto, K. Nakata, K. Katayama, N. Takeuchi, A. Matsumuro
{"title":"Fatal cases of pulmonary alveolar proteinosis: a nationwide surveillance in Japan","authors":"Y. Inoue, E. Yamaguchi, Y. Setoguchi, T. Ichiwata, M. Ebina, Kazutoshi Cho, R. Tazawa, H. Ishii, T. Kasai, M. Akira, T. Arai, K. Uchida, H. Kida, K. Morimoto, M. Kitaichi, C. Sugimoto, K. Nakata, K. Katayama, N. Takeuchi, A. Matsumuro","doi":"10.1183/13993003.CONGRESS-2018.OA3783","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.OA3783","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"29 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":"122724520","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.PA2234
Hiroyuki Ito, H. Nakaoka, Y. Yamashita, Masahiro Takaki, Takeshi Tanaka, M. Ishida, K. Morimoto
{"title":"Mid-term clinical course of the three Japanese SFTPC mutated familial interstitial pneumonia patients.","authors":"Hiroyuki Ito, H. Nakaoka, Y. Yamashita, Masahiro Takaki, Takeshi Tanaka, M. Ishida, K. Morimoto","doi":"10.1183/13993003.CONGRESS-2018.PA2234","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2234","url":null,"abstract":"","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"381 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":"116001120","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}
Pub Date : 2018-09-15DOI: 10.1183/13993003.CONGRESS-2018.OA3786
L. Young, R. Nevel, A. Casey, M. Fishman, Sebastian K Welsh, D. Liptzin, J. Hagood, G. Kurland, D. Craven, E. Fiorino, Jane B Taylor, S. Goldfarb, C. Conrad, Christin S. Kuo, G. Deutsch, A. De, M. Powers, R. Deterding
Introduction: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare pulmonary disorders. Our objectives are to advance knowledge on clinical features, management, and outcomes of this population. Methods: The Children’s Interstitial and Diffuse Lung Disease Research Network (ChILDRN) established a longitudinal observational study in 2016 using a national platform for single IRB reliance agreements with 13 participating sites across the United States. Results: 254 subjects have been enrolled to date. Specific chILD diagnoses and clinical characteristics are summarized in Table 1. Overall mean age at study enrollment was 101±73 months. Identified morbidity included home oxygen supplementation in 71% at any time and 44% with ongoing requirement. Failure to thrive was noted in 53%. 46% of subjects had undergone lung biopsy; genetic studies were used in diagnosis for 23%. Pulmonary function abnormalities varied based on disease subgroup. Conclusions: The first multicenter prospective study of chILD in the U.S. indicates substantial morbidity with variable phenotypes in different forms of chILD. This cohort provides a framework for future longitudinal studies focused on elucidation of the genetic and molecular underpinnings of these disorders, development of targeted therapies, and optimization of supportive care.
{"title":"A national registry for childhood interstitial and diffuse lung diseases in the United States.","authors":"L. Young, R. Nevel, A. Casey, M. Fishman, Sebastian K Welsh, D. Liptzin, J. Hagood, G. Kurland, D. Craven, E. Fiorino, Jane B Taylor, S. Goldfarb, C. Conrad, Christin S. Kuo, G. Deutsch, A. De, M. Powers, R. Deterding","doi":"10.1183/13993003.CONGRESS-2018.OA3786","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.OA3786","url":null,"abstract":"Introduction: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare pulmonary disorders. Our objectives are to advance knowledge on clinical features, management, and outcomes of this population. Methods: The Children’s Interstitial and Diffuse Lung Disease Research Network (ChILDRN) established a longitudinal observational study in 2016 using a national platform for single IRB reliance agreements with 13 participating sites across the United States. Results: 254 subjects have been enrolled to date. Specific chILD diagnoses and clinical characteristics are summarized in Table 1. Overall mean age at study enrollment was 101±73 months. Identified morbidity included home oxygen supplementation in 71% at any time and 44% with ongoing requirement. Failure to thrive was noted in 53%. 46% of subjects had undergone lung biopsy; genetic studies were used in diagnosis for 23%. Pulmonary function abnormalities varied based on disease subgroup. Conclusions: The first multicenter prospective study of chILD in the U.S. indicates substantial morbidity with variable phenotypes in different forms of chILD. This cohort provides a framework for future longitudinal studies focused on elucidation of the genetic and molecular underpinnings of these disorders, development of targeted therapies, and optimization of supportive care.","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":"130284066","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}
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