G. Margaritopoulos, A. Proklou, D. Bonet, F. Chua, P. George, M. Kokosi, V. Kouranos, E. Renzoni, K. Antoniou, A. Wells
{"title":"Nocturnal desaturation predicts mortality in immune-mediated ILDs","authors":"G. Margaritopoulos, A. Proklou, D. Bonet, F. Chua, P. George, M. Kokosi, V. Kouranos, E. Renzoni, K. Antoniou, A. Wells","doi":"10.1183/13993003.congress-2019.pa5196","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5196","url":null,"abstract":"Introduction: Nocturnal desaturation may contribute to long-term pulmonary vascular stress in interstitial lung disease (ILD). We studied the prevalence and prognostic utility of nocturnal desaturation across ILDs. Methods: ILD patients with overnight oximetry (January 2010-December 2013) were included (n=437; Idiopathic Pulmonary Fibrosis, n=122; Connective Tissue Disease-ILD, n=48; Hypersensitivity Pneumonitis, n=60; Non-specific Interstitial Pneumonia, n=56; unclassifiable, n=27; Sarcoidosis, n=66; other, n=58). Desaturation index (DI) was defined as the number of desaturation events >4%/hr. Results: DI was higher in NSIP and CTD-ILD than in IPF (p=0.03 and p=0.02 respectively). In the whole cohort, DLco and serum BNP predicted mortality (HR 0.95; 95% CI 0.94, 0.96; p Conclusion: DI is more prevalent and it is a stronger predictor of mortality in immune-mediated ILDs than in IPF and may be an important manifestation of these disorders.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"9 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":"132484951","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}
C. Stock, A. Lauretis, D. Visca, C. Daccord, M. Kokosi, V. Alfieri, V. Kouranos, G. Margaritopoulos, P. George, P. Molyneaux, F. Chua, T. Maher, V. Ong, D. Abraham, C. Denton, A. Wells, E. Renzoni
{"title":"Serum biomarkers in SSc-ILD: association with presence, severity and prognosis","authors":"C. Stock, A. Lauretis, D. Visca, C. Daccord, M. Kokosi, V. Alfieri, V. Kouranos, G. Margaritopoulos, P. George, P. Molyneaux, F. Chua, T. Maher, V. Ong, D. Abraham, C. Denton, A. Wells, E. Renzoni","doi":"10.1183/13993003.congress-2019.pa5198","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5198","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"26 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":"134430358","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}
F. Reyes, V. Wolff, L. Alberti, Ernesto Juárez, V. Leiva, L. Fassola, M. Mejía, I. Buendía, F. Caro, J. Serrano, F. Paulin, M. Florenzano
{"title":"Multicentric Latin American study of 211 patients with Interstitial lung disease and myositis related antibodies","authors":"F. Reyes, V. Wolff, L. Alberti, Ernesto Juárez, V. Leiva, L. Fassola, M. Mejía, I. Buendía, F. Caro, J. Serrano, F. Paulin, M. Florenzano","doi":"10.1183/13993003.congress-2019.pa4744","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4744","url":null,"abstract":"Introduction: ILD is a common manifestation of patients with myositis related antibodies (MRA). The aim of our study is to describe the clinical and radiological features of a group of patients with ILD and MRA, and their association with the initial pulmonary function (PF). Methods: Descriptive study of a multicentric cohort of patients evaluated between 2016-2018 in 3 ILD clinics in Argentina, Chile and Mexico. Descriptive statistics, univariate and multivariate analysis were performed. Results: 184 patients presented initial ILD diagnosis or simultaneous with connective tissue disease (CTD). The majority were women, with a mean age of 57±12 years. Anti-Synthetase (AS) antibodies were the most frequent (Jo-1, PL-12, PL-7). Main CTD diagnoses were AS syndrome and ILD with autoimmune features (IPAF). Main extra-thoracic symptoms, more frequent HRCT patterns and PFTs are described in Table 1. Worse PF was defined as FVC Conclusions: AS antibodies, NSIP and NSIP/OP patterns were the most frequent data, as reported in other cohorts. Worse PF could be related to the absence of extra-thoracic symptoms and “classic” antibodies of CTD, causing a delay in ILD diagnosis.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"78 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":"128502976","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}
{"title":"Modes of Disease Progression in Different Forms of Interstitial Lung Disease with Usual Interstitial Pneumonia Histology in Patients with Idiopathic and Non-idiopathic Etiologies - A Single Center Experience","authors":"Florian Rampp, H. Popper, J. Meyer","doi":"10.1183/13993003.congress-2019.pa1366","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1366","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"51 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":"115938336","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}
N. Lanzarone, Valerio Alonzi, L. Bergantini, F. Bianchi, M. d’Alessandro, P. Rottoli, R. M. Refini, M. Pieroni, E. Bargagli, M. Mazzei, F. Gentili, P. Sestini
{"title":"Serum and BAL levels of KL6 in chronic hypersensitivity pneumonitis Serum and BAL levels of KL6 in chronic hypersensitivity pneumonitis","authors":"N. Lanzarone, Valerio Alonzi, L. Bergantini, F. Bianchi, M. d’Alessandro, P. Rottoli, R. M. Refini, M. Pieroni, E. Bargagli, M. Mazzei, F. Gentili, P. Sestini","doi":"10.1183/13993003.congress-2019.pa1359","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1359","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"204 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":"127403700","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}
L. Coelho, K. S. Simon, C. Melo-Silva, Paulo Henrique de Holanda Veloso Junior, J. P. Longo, L. Vianna, V. Amado, M. Mortari, A. Bocca
{"title":"The potential role of antimicrobial peptides from wasp as regulators of the fibrotic process in idiopathic pulmonary fibrosis","authors":"L. Coelho, K. S. Simon, C. Melo-Silva, Paulo Henrique de Holanda Veloso Junior, J. P. Longo, L. Vianna, V. Amado, M. Mortari, A. Bocca","doi":"10.1183/13993003.congress-2019.pa4741","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4741","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"19 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":"125406281","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}
{"title":"Longitudinal cost of care in individuals with different subtypes of interstitial lung diseases","authors":"L. Schwarzkopf, S. Witt, J. Wälscher, M. Kreuter","doi":"10.1183/13993003.congress-2019.pa4732","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4732","url":null,"abstract":"Background: Recently, several studies emphasized the high economic burden of interstitial lung diseases (ILDs). Most research focussed on idiopathic pulmonary fibrosis (IPF) in a cross-sectional perspective and did not disentangle disease-related and non-disease-related costs. We therefore aimed by analysing claims data to a) display the share of ILD-related costs in all-cause costs in the longitudinal view, to b) disentangle the structure of ILD-related costs and corresponding shifts over time, to c) contrast subtype specific cost profiles. Methods: We assessed quarterwise health care spending for individuals with six ILD subtypes from the year prior to diagnosis up to five years post. ILD-related expenditures were identified by ATC-Codes (medication) respectively OPS-Codes and ICD-10 diagnoses (in- and outpatient care). Mean expenditures per quarter were examined via Generalized Estimation Equations adjusted by age, gender, ILD-subtype and proximity to death. Results: Costs peaked in the quarter of diagnosis (~€4,700) with a 1/3 share of ILD-related costs. Then, costs stabilized at ~€2,000 with a quite stable 20% share of ILD-related costs. Hospital care was the main contributor to ILD-related costs (>90% in quarter of diagnosis, >50% in post diagnosis period) followed by drug-expenditures (~1/3 in post diagnosis period). Longitudinal profiles were similar across ILD-subtypes with substantial differences in level. As only exception Connective Drug associated ILDs presented an increasing share of ILD-related costs over time. Conclusion: Subtype level cost profiles mirror the mainly hospital-based diagnostic process for 1st diagnosis. Declining relevance of hospital care reflects a primarily outpatient-management during follow up.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"83 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":"126994276","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}
Željka Anzulović, Maja Antolić, Anja Ognjenović, S. Čužić, I. Glojnarić, B. Hrvačić
{"title":"A translational value of pulmonary function tests in a mouse model of bleomycin-induced pulmonary fibrosis: effects of approved therapies Nintedanib and Pirfenidone","authors":"Željka Anzulović, Maja Antolić, Anja Ognjenović, S. Čužić, I. Glojnarić, B. Hrvačić","doi":"10.1183/13993003.congress-2019.pa4727","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4727","url":null,"abstract":"Pulmonary function tests (PFT’s) routinely implemented in clinics are the first step in the diagnosis of idiopathic pulmonary fibrosis. Evaluation of PFT’s in the mouse model of pulmonary fibrosis accompanied by histological readouts may improve clinical predictability of new therapeutic candidates. Forced expiration (FE) parameters are considered as the most predictive for restrictive pulmonary disorders. The aim of the study was to estimate the translational value of the PFT technique in the established mouse model by evaluating the effects of two approved therapies, pirfenidone and nintedanib. C57BL/6 mice were administered with bleomycin (BLM) intranasally and treated with pirfenidone or nintedanib from day 0 to day 14. Fourteen days after BLM challenge, PFT’s were assessed by using in vivo invasive lung function measurements. Histological evaluation was performed as modified Ashcroft score and digital analysis of de novo collagen deposition (CO1A1) and alpha-smooth muscle actin (αSMA) expression. Bleomycin challenge induced a significant decrease of forced vital capacity (FVC) and forced expiratory volume (FEV). Nintedanib treatment induced significant improvement of FE parameters, specifically 30% improvement of FVC and FEV100 in comparison to the vehicle control. Pirfenidone treatment showed no effect. These findings were confirmed with histological analysis. In conclusion, a good correlation of functional test results and clinical effect of nintedanib and pirfenidone was shown. Based on our findings, implementation of PFT could be a good platform to increase the translational value of the model.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"34 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":"121612383","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}
E. Klester, K. Klester, Y. Shoykhet, V. Elykomov, V. Yarkova, A. Berdyugina, E. Mukhtarova
{"title":"Risk factors of interstitial lung diseases in patients with rheumatoid arthritis","authors":"E. Klester, K. Klester, Y. Shoykhet, V. Elykomov, V. Yarkova, A. Berdyugina, E. Mukhtarova","doi":"10.1183/13993003.congress-2019.pa1365","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1365","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"46 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":"132532542","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}
E. Diamanti, C. Daccord, S. Ahmetovic, Nathalie Bacco, R. Lazor
{"title":"Squeaks in hypersensitivity pneumonitis: prevalence and clinical correlates","authors":"E. Diamanti, C. Daccord, S. Ahmetovic, Nathalie Bacco, R. Lazor","doi":"10.1183/13993003.congress-2019.oa1611","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa1611","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"4 2","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114032033","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}