M. L. Alberti, V. Wolff, F. Reyes, Ernesto Juarez Leon, V. Leiva, G. Carballo, J. Serrano, M. Mejía, L. Fassola, F. Caro, M. Florenzano, F. Paulin
{"title":"Variables associated with functional improvement in patients with interstitial lung disease and myositis related antibodies: Results from a multicentric Latin American study","authors":"M. L. Alberti, V. Wolff, F. Reyes, Ernesto Juarez Leon, V. Leiva, G. Carballo, J. Serrano, M. Mejía, L. Fassola, F. Caro, M. Florenzano, F. Paulin","doi":"10.1183/13993003.congress-2019.pa5194","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5194","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"28 25 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":"125365621","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}
Nuno Pereira, D. Machado, I. Sucena, D. Coutinho, C. Nogueira, I. Marques, A. Sanches, Ana Oliveira, J. Almeida, S. Campainha, S. Neves
{"title":"Should we biopsy one or two lobes? – diagnostic yield and risks of transbronchial lung cryobiopsy","authors":"Nuno Pereira, D. Machado, I. Sucena, D. Coutinho, C. Nogueira, I. Marques, A. Sanches, Ana Oliveira, J. Almeida, S. Campainha, S. Neves","doi":"10.1183/13993003.congress-2019.oa1612","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa1612","url":null,"abstract":"","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":"122260420","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}
M. Florenzano, Juan Carlos Maya, F. Reyes, Carolina Cuéllar, Alexis Peralta, V. Balboa, L. Toro, V. Wolff
{"title":"Utility of myositis related antibodies in interstitial lung disease and suspected autoimmunity","authors":"M. Florenzano, Juan Carlos Maya, F. Reyes, Carolina Cuéllar, Alexis Peralta, V. Balboa, L. Toro, V. Wolff","doi":"10.1183/13993003.congress-2019.pa5189","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5189","url":null,"abstract":"Introduction: Myositis related antibodies (MRA) are helpful in the study of patients with idiopathic inflammatory myopathies (IIM), but their role in patients with interstitial lung disease (ILD) is unclear. The aim of this study is to assess whether MRA contribute to define diagnosis and / or determine change of treatment in a cohort of patients with ILD and to describe the clinical characteristics associated with a positive MRA. Methods: A panel of 16 MRA was performed on 111 patients with ILD who did not have a clear underlying diagnosis. The clinical baseline characteristics associated with a positive MRA were compared. Results: Half of patients had at least 1 positive MRA. There was a change in diagnosis in 27.9% of the patients and in the treatment of 62.3%. Ro-52 and anti-synthetase antibodies were the most frequent. The main rheumatological diagnoses were Anti-Synthetase syndrome, other IIM and ILD with autoimmune features (IPAF). The clinical characteristics that were associated with having a positive MRA were younger age, Raynaud´s, mechanical hands, anti Ro, and anti La. HRCT pattern of NSIP/OP and the absence of pattern of hypersensitivity showed a tendency to predict a positive MRA (Table 1). Conclusions: In the study of patients with ILD, testing MRA is specially useful in younger patients with some specific clinical features and specific HRCT patterns, and they contribute to change diagnosis and treatment.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"427 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":"122800718","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}
R. Okuda, T. Katano, M. Asaoka, Y. Uchida, S. Ikeda, R. Ootoshi, E. Tabata, R. Shintani, H. Okabayashi, T. Niwa, T. Oda, A. Sekine, H. Kitamura, T. Baba, S. Komatsu, E. Hagiwara, T. Ogura
{"title":"Utility of inhalation challenge test using avian egg for hypersensitivity pneumonia","authors":"R. Okuda, T. Katano, M. Asaoka, Y. Uchida, S. Ikeda, R. Ootoshi, E. Tabata, R. Shintani, H. Okabayashi, T. Niwa, T. Oda, A. Sekine, H. Kitamura, T. Baba, S. Komatsu, E. Hagiwara, T. Ogura","doi":"10.1183/13993003.congress-2019.pa5191","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5191","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"3 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":"114360390","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":"An observational study on effectiveness of azathioprine in treatment of interstitial lung disease secondary to connective tissue disease","authors":"M. Saeidinejad, Sandosh Gnanalingam, A. Ashish","doi":"10.1183/13993003.congress-2019.pa4740","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4740","url":null,"abstract":"Azathioprine (AZA) is common treatment for connective tissue disease (CTD) related interstitial lung disease (ILD) which is reflected in the British Thoracic Society 2008 ILD guideline [1]. However, this is only a grade C evidence and no studies have reported the real-life effectives in this patient subgroup. An observational study of the effectiveness of AZA in CTD ILD was carried out by retrospective data collection of 30 patients seen in CTD – ILD clinic between January 2010 and December 2018. The mean age of patients was 62.79 (+/- 13 SD) years of which 16 (53%) were females with underlying CTDs (table 1). 16 (53%) patients continued AZA for a mean duration of 52 (+/- 18 SD) months. The FVC and DLCO were preserved in this group compared to those who did not [Mean FVC change: -1.630% (CI 0.95 = 0.284) vs -15.767% (CI 0.95 = 0.555), Mean DLCO change: -4.478% (CI 0.95 = 0.302) vs -14.233% (CI 0.95 = 0.689) respectively]. AZA was stopped in 16 patients due to toxicity (16%) or intolerability (27%). 2 were later re-started on AZA with good tolerance. Our single centre experience showed that FVC of patients with CTD related ILD, over a mean 50-month duration of therapy with of AZA, remained stable. This leads us to conclude that AZA is effective in treatment of CTD related ILD but prone to poor tolerability and systemic toxicity. Reference: [1] Wells AU, Hirani N. Interstitial lung disease guideline. Thorax. 2008 Sep 1;63(Suppl 5):v1-58.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"69 3 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":"123105009","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, D. Abraham, C. Denton, V. Ong, A. Wells, E. Renzoni
{"title":"Verification of genetic associations with Scleroderma associated Interstitial Lung Disease","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, D. Abraham, C. Denton, V. Ong, A. Wells, E. Renzoni","doi":"10.1183/13993003.congress-2019.pa5197","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5197","url":null,"abstract":"","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":"123889315","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}
G. Raghu, O. Distler, A. Azuma, Aryeh Fischer, Kristin B. Highland, Masataka Kuwana, Maureen D. Mayes, D. Wachtlin, S. Stowasser, M. Alves, M. Gahlemann, Toby M. Maher
{"title":"Effects of nintedanib in patients with systemic sclerosis-associated ILD (SSc-ILD) and differing extents of lung fibrosis: the SENSCIS trial","authors":"G. Raghu, O. Distler, A. Azuma, Aryeh Fischer, Kristin B. Highland, Masataka Kuwana, Maureen D. Mayes, D. Wachtlin, S. Stowasser, M. Alves, M. Gahlemann, Toby M. Maher","doi":"10.1183/13993003.congress-2019.pa5193","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5193","url":null,"abstract":"Background: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the annual rate of FVC decline (mL/year) vs placebo (primary endpoint). There was no significant difference between groups in change in modified Rodnan skin score (mRSS) or St George’s Respiratory Questionnaire (SGRQ) total score (key secondary endpoints) at week 52. Aim: Assess whether extent of lung fibrosis influenced the efficacy of nintedanib. Methods: Subjects with SSc-ILD, ≥10% fibrosis of the lungs on HRCT and FVC ≥40% predicted were randomised to nintedanib 150 mg bid or placebo. We analysed primary and key secondary endpoints in subgroups with extent of lung fibrosis Results: Mean±SD extent of fibrosis at baseline was 36.8±21.8% in the nintedanib group (n=288) and 35.2±20.7% in the placebo group (n=288); 80.2% and 74.3% of subjects in these groups, respectively, had ≥20% fibrosis. The effect of nintedanib on FVC decline was numerically more pronounced in subjects with ≥20% fibrosis, but the treatment-by-time-by-subgroup interaction did not reach statistical significance. A more pronounced increase in SGRQ total score with nintedanib vs placebo was observed in patients with Conclusion: Nintedanib reduced ILD progression in patients with SSc-ILD irrespective of extent of lung fibrosis at baseline.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"4 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":"128914716","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}
T. Sjåheim, Anna-Maria Hoffman-Vold, T. Eagan, Arnt‐Ove Hovden, M. Lund, G. Bjerke, T. Jonassen, O. Johansen, M. Durheim
{"title":"A comparison of heath-related quality of life in IPF and systemic sclerosis-related ILD","authors":"T. Sjåheim, Anna-Maria Hoffman-Vold, T. Eagan, Arnt‐Ove Hovden, M. Lund, G. Bjerke, T. Jonassen, O. Johansen, M. Durheim","doi":"10.1183/13993003.congress-2019.pa4735","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4735","url":null,"abstract":"Introduction: IPF and systemic sclerosis (SSc)-related interstitial lung disease (ILD) are fibrotic ILDs with a poor prognosis. Assessment of health-related quality of life (HRQL) is important to understand disease trajectory and response to therapy in ILD; however, few studies have compared HRQL in these conditions. Objective: To compare HRQL in SSc-ILD and IPF, independent of lung function. Methods: The Kings Brief Interstitial Lung Disease Questionnaire (K-BILD), validated to assess HRQL in IPF and scored 0-100 with higher scores indicating better HRQL, was used to compare HRQL between patients with IPF (n=58) and SSc-ILD (n=29) in a multi-center cross-sectional study. ANCOVA was used to compare mean K-BILD score between the IPF and SSc-ILD groups, with adjustment for age, gender, FVC and DLCO. Values are presented as mean (SD). Results: Demographics and lung function are listed in table 1. K-BILD score was 69.4 (17.9) among patients with IPF and 80.5 (21.2) among those with SSc-ILD (p =0.014), indicating slightly better HRQL in SSc-ILD. After covariate adjustment, however, no statistical difference in K-BILD was observed between patients with IPF (70.5 [19.3]) and SSc-ILD (79.8 [21.2], p = 0.096). Conclusions: K-BILD scores appeared to be similar between patients with IPF and SSc-ILD after adjusting for age, gender and lung function. This new observation underscores the impact of lung function impairment on HRQL in ILD and the need for treatments that positively impact HRQL by preserving lung function.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"44 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":"122451906","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":"Hypersensitivity Pneumonitis in North Indian population","authors":"A. Sinha","doi":"10.1183/13993003.congress-2019.pa4737","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4737","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"38 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":"121133613","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}
M. Sterclova, P. Sojka, N. Kaspříková, M. Vašáková
{"title":"Specific serum immunoglobulins G (IgGs) may serve as pointers towards inhalation antigen exposure in patients with hypersensitivity pneumonitis (HP) and uncertain exposure source","authors":"M. Sterclova, P. Sojka, N. Kaspříková, M. Vašáková","doi":"10.1183/13993003.congress-2019.pa1360","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1360","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"45 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":"127625255","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}