{"title":"Prevalence and associated risk factors of post covid-19 interstitial lung disease; An emerging challenge to pulmonologists","authors":"D. Yasaratne, A. Shantha, Safa Shafee, Nuwani Nissanka, A. Thilakarathne, A. Medagama","doi":"10.1183/23120541.lsc-2022.170","DOIUrl":"https://doi.org/10.1183/23120541.lsc-2022.170","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123034530","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":"Interstitial pneumonia with autoimmune features associated with myositis autoantibodies: clinical characteristics from a multicenter Latin-American cohort","authors":"José Ernesto Juárez León, M. L. Alberti, V. Wolff, F. Reyes, F. Paulin, L. Fassola, F. Caro, G. Carballo, Tamara Palavecino, Juan Carlos Rodríguez Díaz, I. Roldán, M. Mejía, M. Florenzano, J. Serrano","doi":"10.1183/13993003.congress-2019.pa1371","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1371","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"143 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":"124885505","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":"Pneumotox metrics: Drugs, patterns, users and countries. Foreground and quiet zones","authors":"P. Camus, C. Camus, G. Beltramo, P. Bonniaud","doi":"10.1183/13993003.congress-2019.oa1613","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa1613","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"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":"125960070","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}
Background: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the annual rate of decline in FVC (mL/year) vs placebo (primary endpoint). There was no significant difference between treatment groups in change from baseline in modified Rodnan skin score (mRSS) or St George’s Respiratory Questionnaire (SGRQ) total score (key secondary endpoints) at week 52. Aim: To assess the efficacy of nintedanib in subgroups by FVC % predicted at baseline. Methods: Subjects with SSc-ILD with ≥10% fibrosis of the lungs on HRCT and FVC ≥40% predicted were randomised to receive nintedanib 150 mg bid or placebo. We analysed the primary and key secondary endpoints in subgroups by baseline FVC Results: 201 (69.8%) subjects in the nintedanib group and 196 (68.1%) in the placebo group had FVC Conclusion: In patients with SSc-ILD, nintedanib was observed to reduce ILD progression irrespective of FVC % predicted at baseline. Table.
{"title":"Effects of nintedanib in patients with systemic sclerosis-associated ILD (SSc-ILD) and differing FVC at baseline: the SENSCIS trial","authors":"Toby M. Maher, O. Distler, A. Azuma, Aryeh Fischer, Kristin B. Highland, Masataka Kuwana, Maureen D. Mayes, D. Wachtlin, M. Alves, M. Gahlemann, S. Stowasser, G. Raghu","doi":"10.1183/13993003.congress-2019.oa3599","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa3599","url":null,"abstract":"Background: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the annual rate of decline in FVC (mL/year) vs placebo (primary endpoint). There was no significant difference between treatment groups in change from baseline in modified Rodnan skin score (mRSS) or St George’s Respiratory Questionnaire (SGRQ) total score (key secondary endpoints) at week 52. Aim: To assess the efficacy of nintedanib in subgroups by FVC % predicted at baseline. Methods: Subjects with SSc-ILD with ≥10% fibrosis of the lungs on HRCT and FVC ≥40% predicted were randomised to receive nintedanib 150 mg bid or placebo. We analysed the primary and key secondary endpoints in subgroups by baseline FVC Results: 201 (69.8%) subjects in the nintedanib group and 196 (68.1%) in the placebo group had FVC Conclusion: In patients with SSc-ILD, nintedanib was observed to reduce ILD progression irrespective of FVC % predicted at baseline. Table.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"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":"115094938","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}
Background: Systemic sclerosis (SSc) is a rare connective tissue disease associated with potential rapid evolving interstitial lung disease (SSc-ILD), driving the mortality of these patients. Therefore, a specific biomarker associated with the evolution of that disease is highly needed to identify patients with an increased risk of death. Aim of the Study: Identify specific biomarkers of SSc-ILD to predict the evolution of the disease. Methods: In this prospective longitudinal study, we compared serum levels of biomarkers assumed to be associated with lung fibrosis (TGF-β, IGF-1, IGFBP-1, IGFBP-2, IGFBP-3, IL-8, MMP-7, MMP-9, YKL-40 and TNF-α) among three groups: SSc-ILD (n=39), SSc without ILD (n=63) and healthy subjects (HS)(n=39). We also prospectively analyzed variations of biomarkers and correlated them to pulmonary function tests (n=28). Then, we realized an in vitro analysis to study the potential anti-fibrotic effect of IGFBP-2 (n=3). Results: IGFBP-2, IL-8 and MMP-9 are increased in SSc patients compared to HS (p Conclusion: IGFBP-1 has a potential interest to identify early SSc-ILD whereas IGFBP-2 would rather predict the risk of a rapid evolution of lung fibrosis. Moreover, in vitro studies confirmed the anti-fibrotic effect of IGFBP-2 underlying its potential interest in further mechanical studies and therapeutic aspects.
{"title":"IGFBP-2: a new pathway in systemic sclerosis associated interstitial lung disease","authors":"F. Gester, M. Henket, Dominique Deseny, C. Moermans, B. André, M. Malaise, R. Louis, J. Guiot","doi":"10.1183/13993003.congress-2019.oa3597","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa3597","url":null,"abstract":"Background: Systemic sclerosis (SSc) is a rare connective tissue disease associated with potential rapid evolving interstitial lung disease (SSc-ILD), driving the mortality of these patients. Therefore, a specific biomarker associated with the evolution of that disease is highly needed to identify patients with an increased risk of death. Aim of the Study: Identify specific biomarkers of SSc-ILD to predict the evolution of the disease. Methods: In this prospective longitudinal study, we compared serum levels of biomarkers assumed to be associated with lung fibrosis (TGF-β, IGF-1, IGFBP-1, IGFBP-2, IGFBP-3, IL-8, MMP-7, MMP-9, YKL-40 and TNF-α) among three groups: SSc-ILD (n=39), SSc without ILD (n=63) and healthy subjects (HS)(n=39). We also prospectively analyzed variations of biomarkers and correlated them to pulmonary function tests (n=28). Then, we realized an in vitro analysis to study the potential anti-fibrotic effect of IGFBP-2 (n=3). Results: IGFBP-2, IL-8 and MMP-9 are increased in SSc patients compared to HS (p Conclusion: IGFBP-1 has a potential interest to identify early SSc-ILD whereas IGFBP-2 would rather predict the risk of a rapid evolution of lung fibrosis. Moreover, in vitro studies confirmed the anti-fibrotic effect of IGFBP-2 underlying its potential interest in further mechanical studies and therapeutic aspects.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"87 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":"122951019","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":"A single center experience of rituximab for anti synthetase syndrome associated interstitial lung disease (ASS-ILD)","authors":"A. Rathnapala, L. Wing, R. Benamore, J. David, R. Hoyles","doi":"10.1183/13993003.congress-2019.pa1367","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1367","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"61 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":"128293898","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}
Purpose: To describe the characteristics of ILD-RA patients and to asess their inicence rate of functional respiratory impairment Methods: This is a longitudinal prospective study. A cohort of RA patients diagnosed of ILD since 02/2007 until 03/2018 and followed till 05/2018, in a ILD unit, carried by a pneumologist and a rheumatologist. The main variable was FVC decline ≥ 10 % pred value since the previous visit. Covariables: sociodemographic, basal comorbidities, radiological pattern (UIP, NSIP), laboratory tests, therapy (corticoids, Azathioprine [AZA], Mycophenolate [MMF], Leflunomide [LEF], anti-TNF, Abatacept [ABA], Rituximab [RTX]. Survival techniques were used to estimate the incidence rate (IR) of functional impairment, expressed per 100 patient-years [95 % CI]. Results: We included 43 patients, 63% women, mean age 70±9.6 years. 42% never smoked. HTN and vascular disease were frecuent comorbidities, RF was positive in 85% and anti-CCP in 82%, ESR was 43±26. UIP pattern in 63%, NSIP in 32 %. Median FVC were 103%. 33 patients used Corticoids, 4 MMF, 18 AZA, 16 LEF, 6 TNFs, 20 RTX and 5 ABA. 37% sufered functional deterioration, IR of 17 [11-25] (141 patients-years). 50% achieved funtional deterioration at 4.6 years since the ILD diagnosis. IR for UIP was 18 [11-30] and for NSIP 16 [8-29]. Corticoids IR was 15[10-24], LEF 13[6-29], AZA and MMF 17[8-36], RTX 11 [4-29] Conclusion: RA patients had active disease at ILD diagnosis. The most common radiological pattern was UIP. 37% of patients suffered functional deterioration with IR of 17% patient-years, being 4.5 years the median time free of impairment. The crude incidence of functional impairment was less for RTX.
{"title":"Features and functional deterioration in interstitial lung disease (ILD) asociated to rheumatoid arthritis (RA)","authors":"M. A. N. Barbero, C. Vadillo, F. Pelaez, Ana Palomar, L. Abasolo","doi":"10.1183/13993003.congress-2019.pa4742","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4742","url":null,"abstract":"Purpose: To describe the characteristics of ILD-RA patients and to asess their inicence rate of functional respiratory impairment Methods: This is a longitudinal prospective study. A cohort of RA patients diagnosed of ILD since 02/2007 until 03/2018 and followed till 05/2018, in a ILD unit, carried by a pneumologist and a rheumatologist. The main variable was FVC decline ≥ 10 % pred value since the previous visit. Covariables: sociodemographic, basal comorbidities, radiological pattern (UIP, NSIP), laboratory tests, therapy (corticoids, Azathioprine [AZA], Mycophenolate [MMF], Leflunomide [LEF], anti-TNF, Abatacept [ABA], Rituximab [RTX]. Survival techniques were used to estimate the incidence rate (IR) of functional impairment, expressed per 100 patient-years [95 % CI]. Results: We included 43 patients, 63% women, mean age 70±9.6 years. 42% never smoked. HTN and vascular disease were frecuent comorbidities, RF was positive in 85% and anti-CCP in 82%, ESR was 43±26. UIP pattern in 63%, NSIP in 32 %. Median FVC were 103%. 33 patients used Corticoids, 4 MMF, 18 AZA, 16 LEF, 6 TNFs, 20 RTX and 5 ABA. 37% sufered functional deterioration, IR of 17 [11-25] (141 patients-years). 50% achieved funtional deterioration at 4.6 years since the ILD diagnosis. IR for UIP was 18 [11-30] and for NSIP 16 [8-29]. Corticoids IR was 15[10-24], LEF 13[6-29], AZA and MMF 17[8-36], RTX 11 [4-29] Conclusion: RA patients had active disease at ILD diagnosis. The most common radiological pattern was UIP. 37% of patients suffered functional deterioration with IR of 17% patient-years, being 4.5 years the median time free of impairment. The crude incidence of functional impairment was less for RTX.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"116 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":"126706594","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":"Interstitial lung disease associated with ANCA positivity: a retrospective analysis","authors":"S. Juman, S. Haque, N. Chaudhuri","doi":"10.1183/13993003.congress-2019.pa1362","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa1362","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"12 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":"126471375","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}
Background: There is growing appreciation for early palliative care (PC) input in the management of patients with interstitial lung disease (ILD). Patients receiving PC experience improved quality of life, but it is our anecdotal experience that PC input may not always be introduced promptly. Aim: The aim was to increase the regularity with which the ILD MDT discussed end of life (EOL) care and to improve the quality of patient care. Methods: 21 ILD Clinicians working in a tertiary unit were surveyed and ILD inpatients were interviewed. The results of the initial survey led to three interventions:(a)Regular attendance of the PC team at inpatient ward reviews,(b) Routine implementation of the surprise question; “Would you be surprised if this patient died within the next 12 months” and (c)Written documentation identifying patients appropriate for PC. Results: 81% of clinicians surveyed felt that EOL conversations with patients could be timelier. Reasons cited for delayed discussion included ambiguity of patient wishes, unpredictability of disease behaviour and uncertainty of prognosis. Prior to the interventions, only 40% of ILD inpatients had PC discussed; within 14 weeks this increased to 100%. There was an average increase of 3.21 minutes per patient in discussion time. The number of referrals to PC increased, as did patient awareness of advanced care planning. The intervention also prompted 2 new transplant referrals. Patient experience interviews were conducted and responses were universally positive. Conclusion: Implementation of PC conversations in this group can be challenging due to the difficulty in predicting trajectory of disease. Routine involvement of the PC team is valued by inpatients with ILD and may improve outcomes.
{"title":"Death in ILD - Why arent we talking about it?","authors":"A. Hudson, A. Hare, L. Berry, Linda Freeman, P. George","doi":"10.1183/13993003.congress-2019.pa5186","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5186","url":null,"abstract":"Background: There is growing appreciation for early palliative care (PC) input in the management of patients with interstitial lung disease (ILD). Patients receiving PC experience improved quality of life, but it is our anecdotal experience that PC input may not always be introduced promptly. Aim: The aim was to increase the regularity with which the ILD MDT discussed end of life (EOL) care and to improve the quality of patient care. Methods: 21 ILD Clinicians working in a tertiary unit were surveyed and ILD inpatients were interviewed. The results of the initial survey led to three interventions:(a)Regular attendance of the PC team at inpatient ward reviews,(b) Routine implementation of the surprise question; “Would you be surprised if this patient died within the next 12 months” and (c)Written documentation identifying patients appropriate for PC. Results: 81% of clinicians surveyed felt that EOL conversations with patients could be timelier. Reasons cited for delayed discussion included ambiguity of patient wishes, unpredictability of disease behaviour and uncertainty of prognosis. Prior to the interventions, only 40% of ILD inpatients had PC discussed; within 14 weeks this increased to 100%. There was an average increase of 3.21 minutes per patient in discussion time. The number of referrals to PC increased, as did patient awareness of advanced care planning. The intervention also prompted 2 new transplant referrals. Patient experience interviews were conducted and responses were universally positive. Conclusion: Implementation of PC conversations in this group can be challenging due to the difficulty in predicting trajectory of disease. Routine involvement of the PC team is valued by inpatients with ILD and may improve outcomes.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"92 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":"126161052","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":"Cellular consequences of uncharacterized surfactant protein-A2 (SFTPA2)-gene mutations associated with familial IPF and lung cancer","authors":"K. Guenther, W. Seeger, A. Guenther, M. Korfei","doi":"10.1183/13993003.congress-2019.oa1608","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa1608","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"79 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":"127236890","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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