Pub Date : 2019-09-28DOI: 10.1183/13993003.congress-2019.pa4733
V. Tzilas, A. Tzouvelekis, E. Bouros, Matthew Katsaras, Maria Ntassiou, T. Karampitsakos, Demosthenes Bouros
Introduction: Chronic Hypersensitivity Pneumonitis (c-HP) can exhibit an IPF-like course despite immunosuppressive treatment. In this group of patients there is a need for new therapeutic approaches. Aim: To investigate the effect of antifibrotics in the clinical course of c-HP. Patients and Methods: Retrospective analysis of patients with an initial diagnosis of IPF that were subsequently diagnosed as c-HP in the context of multidisciplinary discussion (Jan2012-Dec2016). HP diagnosis was based in the absence of alternative diagnoses when at least two of the following criteria were present: Recognition of an inciting antigen, compatible HRCT pattern (mainly presence of mosaic pattern), BAL lymphocytosis>20%. Results: We enrolled in the study 96 patients with HP. From this cohort we identified 18 patients, initially diagnosed as IPF (16 males, 88,9%, median age 70 years (95% CI for the median 63 to 73.6) that had received antifibrotics for at least 12 months (10 pirfenidone and 8 nintedanib). Mean (±SE) relative changes in %FVCpred and %DLcopred over one year was -4.19±3.29 and -5.72±5.32, respectively. A decline in FVC was observed in 11 patients (in 4 patients > 10%, and in 5 patients between 5-10%). An increase in relative %FVCpred was observed in 7 patients (mean ΔFVC ± SE = 8.69±3.75). Conclusions: Antifibrotics can stabilize FVC decline in patients with c-HP. Large randomized trials are needed.
{"title":"Antifibrotic treatment in 18 patients with chronic Hypersensitivity Pneumonitis","authors":"V. Tzilas, A. Tzouvelekis, E. Bouros, Matthew Katsaras, Maria Ntassiou, T. Karampitsakos, Demosthenes Bouros","doi":"10.1183/13993003.congress-2019.pa4733","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4733","url":null,"abstract":"Introduction: Chronic Hypersensitivity Pneumonitis (c-HP) can exhibit an IPF-like course despite immunosuppressive treatment. In this group of patients there is a need for new therapeutic approaches. Aim: To investigate the effect of antifibrotics in the clinical course of c-HP. Patients and Methods: Retrospective analysis of patients with an initial diagnosis of IPF that were subsequently diagnosed as c-HP in the context of multidisciplinary discussion (Jan2012-Dec2016). HP diagnosis was based in the absence of alternative diagnoses when at least two of the following criteria were present: Recognition of an inciting antigen, compatible HRCT pattern (mainly presence of mosaic pattern), BAL lymphocytosis>20%. Results: We enrolled in the study 96 patients with HP. From this cohort we identified 18 patients, initially diagnosed as IPF (16 males, 88,9%, median age 70 years (95% CI for the median 63 to 73.6) that had received antifibrotics for at least 12 months (10 pirfenidone and 8 nintedanib). Mean (±SE) relative changes in %FVCpred and %DLcopred over one year was -4.19±3.29 and -5.72±5.32, respectively. A decline in FVC was observed in 11 patients (in 4 patients > 10%, and in 5 patients between 5-10%). An increase in relative %FVCpred was observed in 7 patients (mean ΔFVC ± SE = 8.69±3.75). Conclusions: Antifibrotics can stabilize FVC decline in patients with c-HP. Large randomized trials are needed.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"115 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":"115653027","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 : 2019-09-28DOI: 10.1183/13993003.congress-2019.pa4743
S. Sanna, D. Argnani, M. Mengozzi, A. Parisi, Sara Tommasetti, V. Poletti, F. Stella
{"title":"The role of Surgical Lung Biopsy in Interstitial Lung Diseases. Is it mandatory in this time of multiple diagnostic procedures? Analysis of 186 patients surgically treated","authors":"S. Sanna, D. Argnani, M. Mengozzi, A. Parisi, Sara Tommasetti, V. Poletti, F. Stella","doi":"10.1183/13993003.congress-2019.pa4743","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4743","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"85 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":"122719200","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 : 2019-09-28DOI: 10.1183/13993003.congress-2019.pa1373
M. R. Soares, C. Pereira, A. Botelho, A. Gimenez, Bruno Beraldo, C. Ferraz, E. Mancuzo, F. Machado, F. Toledo, G. Miranda, Maria Auxiliadora Carmo Moreira, M. Taube, M.D.C. Castro, M. Cerezoli, R. Boaventura, Regina Tibana, Tarciane Aline Prata, E. Coletta, R. Ferreira
The relative frequency of different ILD varies in different countries; in Brazil is unknown. The aim of present study was to describe the frequency of ILD in Brazil. Methods: Patients from 5 centers evaluated during 2014-2017 were included. All patients were evaluated by pulmonologists, radiologists and pathologists involved with ILD. IPF was characterized according ATS/ERS/JRS/ALAT 2011 guideline. HP was characterized as definitive, probable and possible. Diagnosis of CTD, including IPAF, followed standard criteria. Familial interstitial pneumonia was the final diagnosis when ILD in first degree relatives was reported. Results: A total of 1,015 patients with ILD were enrolled. Mean age was 61 ± 15 yrs.; 53% were female. FVC was 69.2 ± 19.7% of predicted. Drugs with potential to cause ILD were reported by 12%, GERD symptoms by 42%, occupational exposure by 13% and to possible etiologies for HP by 53%. Autoantibodies were detected in significant levels in 31% (ANA 10.5%). Biopsies contributory to diagnosis were obtained by bronchoscopy in in 97 (9.6%), by surgical lung biopsy in 162 (16.0%) and from diverse sites in 42 (4.1%). Final diagnoses are shown in Figure. Conclusions: CTD-ILD are the most common cause for ILD in Brazil (21.7%), followed by HP (19%). IPF was diagnosed in 10% of cases, familial interstitial pneumonia in 8%, sarcoidosis in 7%. In 9% of diagnosis was undefined.
{"title":"Multicenter Registry of Interstitial Lung Diseases in Brazil","authors":"M. R. Soares, C. Pereira, A. Botelho, A. Gimenez, Bruno Beraldo, C. Ferraz, E. Mancuzo, F. Machado, F. Toledo, G. Miranda, Maria Auxiliadora Carmo Moreira, M. Taube, M.D.C. Castro, M. Cerezoli, R. Boaventura, Regina Tibana, Tarciane Aline Prata, E. Coletta, R. Ferreira","doi":"10.1183/13993003.congress-2019.pa1373","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1373","url":null,"abstract":"The relative frequency of different ILD varies in different countries; in Brazil is unknown. The aim of present study was to describe the frequency of ILD in Brazil. Methods: Patients from 5 centers evaluated during 2014-2017 were included. All patients were evaluated by pulmonologists, radiologists and pathologists involved with ILD. IPF was characterized according ATS/ERS/JRS/ALAT 2011 guideline. HP was characterized as definitive, probable and possible. Diagnosis of CTD, including IPAF, followed standard criteria. Familial interstitial pneumonia was the final diagnosis when ILD in first degree relatives was reported. Results: A total of 1,015 patients with ILD were enrolled. Mean age was 61 ± 15 yrs.; 53% were female. FVC was 69.2 ± 19.7% of predicted. Drugs with potential to cause ILD were reported by 12%, GERD symptoms by 42%, occupational exposure by 13% and to possible etiologies for HP by 53%. Autoantibodies were detected in significant levels in 31% (ANA 10.5%). Biopsies contributory to diagnosis were obtained by bronchoscopy in in 97 (9.6%), by surgical lung biopsy in 162 (16.0%) and from diverse sites in 42 (4.1%). Final diagnoses are shown in Figure. Conclusions: CTD-ILD are the most common cause for ILD in Brazil (21.7%), followed by HP (19%). IPF was diagnosed in 10% of cases, familial interstitial pneumonia in 8%, sarcoidosis in 7%. In 9% of diagnosis was undefined.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"8 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":"128881674","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 : 2019-09-28DOI: 10.1183/13993003.CONGRESS-2019.OA1607
Fernanda Ines Hernandez Gonzalez, M. A. Calvo, Leonardo Arosemena Angulo, Marcelo Sánchez, M. Benegas, J. Ramírez, C. Lucena, C. Agustí, M. Boada, R. Castellanos, S. Rodriguez-García, I. Bobolea, E. Arismendi, M. Pascal, O. Viñas, E. Ruiz, Sergio Prieto Gonzalez, G. Espinosa, S. Cuerpo, J. Francesqui, J. Sellarés
Background: The diagnosis of hypersensitivity pneumonitis (HP) onset after fungal antigens inhalation at home can be challenging. Although an early identification of the inhaled antigen and its complete avoidance are recommended, current strategies for identifying the causal antigen are controversial. Objective: To determine the usefulness of this diagnostic strategy in detecting potentially causative fungal agents in the patient’s home environment. Methods: We selected 8 patients with a multidisciplinary diagnosis of HP in our Interstitial Lung Disease Program at a tertiary referral center. All patients had their serum tested against the standard HP screening antigen panel with a positive antibody response, but without an identified antigen based on patient interviews. A standardized environmental sampling took place at each patient’s home. Results: Up to 64 air and swabs samples (median=40; Interquartile range=17) were collected from each patient’s environment. High numbers of colony-forming units (CFU) (more than 100 CFU/plate or more than 100 CFU/ml) of different species of fungi were identificated in each patient’s home. The most prevalent species were Penicillium spp and Cladosporium herbarum. All the individuals had a positive response on the standard antigens panel to more than 1 antigen from the environmental sample. After identifying the possible causative factor, an exhaustive cleaning and remediation of the affected areas were made. Conclusion: An indoor environmental study may be crucial to avoid the continuation of unrecognized exposure to the causative fungal antigen. This might contribute to the correct diagnosis and appropriate management of patients with HP.
{"title":"Identification of indoor fungal antigens in assessment of hypersensitivity pneumonitis – an alternate approach","authors":"Fernanda Ines Hernandez Gonzalez, M. A. Calvo, Leonardo Arosemena Angulo, Marcelo Sánchez, M. Benegas, J. Ramírez, C. Lucena, C. Agustí, M. Boada, R. Castellanos, S. Rodriguez-García, I. Bobolea, E. Arismendi, M. Pascal, O. Viñas, E. Ruiz, Sergio Prieto Gonzalez, G. Espinosa, S. Cuerpo, J. Francesqui, J. Sellarés","doi":"10.1183/13993003.CONGRESS-2019.OA1607","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2019.OA1607","url":null,"abstract":"Background: The diagnosis of hypersensitivity pneumonitis (HP) onset after fungal antigens inhalation at home can be challenging. Although an early identification of the inhaled antigen and its complete avoidance are recommended, current strategies for identifying the causal antigen are controversial. Objective: To determine the usefulness of this diagnostic strategy in detecting potentially causative fungal agents in the patient’s home environment. Methods: We selected 8 patients with a multidisciplinary diagnosis of HP in our Interstitial Lung Disease Program at a tertiary referral center. All patients had their serum tested against the standard HP screening antigen panel with a positive antibody response, but without an identified antigen based on patient interviews. A standardized environmental sampling took place at each patient’s home. Results: Up to 64 air and swabs samples (median=40; Interquartile range=17) were collected from each patient’s environment. High numbers of colony-forming units (CFU) (more than 100 CFU/plate or more than 100 CFU/ml) of different species of fungi were identificated in each patient’s home. The most prevalent species were Penicillium spp and Cladosporium herbarum. All the individuals had a positive response on the standard antigens panel to more than 1 antigen from the environmental sample. After identifying the possible causative factor, an exhaustive cleaning and remediation of the affected areas were made. Conclusion: An indoor environmental study may be crucial to avoid the continuation of unrecognized exposure to the causative fungal antigen. This might contribute to the correct diagnosis and appropriate management of patients with HP.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"1 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":"130403390","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 : 2019-09-28DOI: 10.1183/13993003.congress-2019.pa1370
S. Mittal, U. Kumar, R. Guleria, A. Mohan, K. Madan, A. Bhalla, S. Mathur
{"title":"Bronchoalveolar lavage fluid cytokines in the assessment of interstitial lung disease in systemic sclerosis: a prospective study","authors":"S. Mittal, U. Kumar, R. Guleria, A. Mohan, K. Madan, A. Bhalla, S. Mathur","doi":"10.1183/13993003.congress-2019.pa1370","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1370","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"22 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":"121102710","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.PA2974
Emil Lind Flensborg
{"title":"Impulse oscillometry compared to standard lung function testing in patients with idiopathic pulmonary fibrosis","authors":"Emil Lind Flensborg","doi":"10.1183/13993003.CONGRESS-2018.PA2974","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2974","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"79 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":"117129769","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.pa2988
A. Mondal, Poulami Banerjee
{"title":"An audio-imaging approach to measure the degree of severity of patients suffering from DPLD disease","authors":"A. Mondal, Poulami Banerjee","doi":"10.1183/13993003.congress-2018.pa2988","DOIUrl":"https://doi.org/10.1183/13993003.congress-2018.pa2988","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"42 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":"127492368","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.PA2975
S. Uh, B. Lee, S. Koo, Yang-Ki Kim, K. Kim, Do Jin Kim
{"title":"Change of primary Sjören’s syndrome-associated interstitial lung disease in computerized tomogram of chest during two years.","authors":"S. Uh, B. Lee, S. Koo, Yang-Ki Kim, K. Kim, Do Jin Kim","doi":"10.1183/13993003.CONGRESS-2018.PA2975","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2975","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"96 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":"114253960","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.PA3664
C. Stock, R. Hoyles, C. Daccord, M. Kokosi, V. Alfieri, C. Campochiaro, J. Donovan, L. Mori, T. Maher, V. Kouranos, G. Margaritopoulos, P. George, P. Molyneaux, F. Chua, V. Ong, C. Denton, A. Wells, E. Renzoni
{"title":"Serum KL-6 as a marker of disease progression in SSc-ILD","authors":"C. Stock, R. Hoyles, C. Daccord, M. Kokosi, V. Alfieri, C. Campochiaro, J. Donovan, L. Mori, T. Maher, V. Kouranos, G. Margaritopoulos, P. George, P. Molyneaux, F. Chua, V. Ong, C. Denton, A. Wells, E. Renzoni","doi":"10.1183/13993003.CONGRESS-2018.PA3664","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA3664","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"13 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":"122229865","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.PA2966
Michelle Li Wei Kam, Hui Hua Li, Yi Hern Tan, S. Low
{"title":"Validation of the ILD-GAP model in a Singapore population","authors":"Michelle Li Wei Kam, Hui Hua Li, Yi Hern Tan, S. Low","doi":"10.1183/13993003.CONGRESS-2018.PA2966","DOIUrl":"https://doi.org/10.1183/13993003.CONGRESS-2018.PA2966","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"75 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":"127639897","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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