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2. Biological Age, Chronological Age, and Survival in Pulmonary Fibrosis: A Causal Mediation Analysis

Author

Pugashetti, J.V., primary, Kim, J., additional, Bose, S., additional, Adegunsoye, A.O., additional, Linderholm, A., additional, Chen, C.-H., additional, Strek, M.E., additional, Flaherty, K.R., additional, Murray, S., additional, Newton, C., additional, Bowler, R.P., additional, Han, M.K., additional, Curtis, J.L., additional, Martinez, F.J., additional, Smith, J., additional, and Noth, I., additional

Published
2024
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6. Identification of Molecular Endotypes With Differential Anti-fibrotic Response in Idiopathic Pulmonary Fibrosis: A Latent Class Analysis of Two Multi-center Observational Cohorts

Author

Maddali, M.V., primary, Moore, A.R., additional, Sinha, P., additional, Newton, C., additional, Kim, J., additional, Adegunsoye, A.O., additional, Strek, M., additional, Chen, C.-H., additional, Linderholm, A., additional, Zemans, R.L., additional, Moore, B., additional, Wolters, P.J., additional, Martinez, F.J., additional, Rogers, A., additional, Raj, R., additional, Noth, I., additional, and Oldham, J., additional

Published
2024
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7. Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials

Author

Collard, HR, Yow, E, Richeldi, L, Anstrom, KJ, Glazer, C, Schwarz, M, Zisman, DA, Hunninghake, G, Chapman, J, Olman, M, Lubell, S, Morrison, LD, Steele, MP, Haram, T, Roman, J, Perez, R, Perez, T, Ryu, JH, Utz, JP, Limper, AH, Daniels, CE, Meiras, K, Walsh, S, Brown, KK, Bair, C, Kervitsky, D, Lasky, JA, Ditta, S, De Andrade, J, Thannickal, VJ, Stewart, M, Lynch, J, Calahan, E, Lopez, P, King, TE, Golden, JA, Wolters, PJ, Jeffrey, R, Noth, I, Hogarth, DK, Sandbo, N, Strek, ME, White, SR, Brown, C, Garic, I, Maleckar, S, Martinez, FJ, Flaherty, KR, Han, M, Moore, B, Toews, GB, Dahlgren, D, Raghu, G, Hayes, J, Snyder, M, Loyd, JE, Lancaster, L, Lawson, W, Greer, R, Mason, W, Kaner, RJ, Monroy, V, Wang, M, Lynch, DA, Colby, T, Becker, RC, Eisenstein, EL, MacIntyre, NR, Rochon, J, Sundy, JS, Davidson-Ray, L, Dignacco, P, Edwards, R, Anderson, R, Beci, R, Calvert, S, Cain, K, Gentry-Bumpass, T, Hill, D, Ingham, M, Kagan, E, Kaur, J, Matti, C, McClelland, J, Meredith, A, Nguyen, T, Pesarchick, J, Roberts, RS, Tate, W, Thomas, T, Walker, J, Whelan, D, Winsor, J, Yang, Q, and Reynolds, HY

Abstract

Background: Acute exacerbation of idiopathic pulmonary fibrosis has become an important outcome measure in clinical trials. This study aimed to explore the concept of suspected acute exacerbation as an outcome measure.Methods: Three investigators retrospectively reviewed subjects enrolled in the Sildenafil Trial of Exercise Performance in IPF who experienced a respiratory serious adverse event during the course of the study. Events were classified as definite acute exacerbation, suspected acute exacerbation, or other, according to established criteria.Results: Thirty-five events were identified. Four were classified as definite acute exacerbation, fourteen as suspected acute exacerbation, and seventeen as other. Definite and suspected acute exacerbations were clinically indistinguishable. Both were most common in the winter and spring months and were associated with a high risk of disease progression and short-term mortality.Conclusions: In this study one half of respiratory serious adverse events were attributed to definite or suspected acute exacerbations. Suspected acute exacerbations are clinically indistinguishable from definite acute exacerbations and represent clinically meaningful events. Clinical trialists should consider capturing both definite and suspected acute exacerbations as outcome measures. © 2013 Collard et al.; licensee BioMed Central Ltd.

Published
2013

8. Forced vital capacity trajectories in patients with idiopathic pulmonary fibrosis: a secondary analysis of a multicentre, prospective, observational cohort

Author

Fainberg, HP, Oldham, JM, Molyneau, PL, Allen, RJ, Kraven, LM, Fahy, WA, Porte, J, Braybrooke, R, Saini, G, Karsdal, MA, Leeming, DJ, Sand, JMB, Triguero, I, Oballa, E, Wells, AU, Renzoni, E, Wain, LV, Noth, I, Maher, TM, Stewart, ID, and Jenkins, RG

Subjects
Cohort Studies, Health Information Management, Vital Capacity, Humans, Medicine (miscellaneous), Decision Sciences (miscellaneous), Health Informatics, Prospective Studies, Idiopathic Pulmonary Fibrosis, Biomarkers
Abstract

Background Idiopathic pulmonary fibrosis is a progressive fibrotic lung disease with a variable clinical trajectory. Decline in forced vital capacity (FVC) is the main indicator of progression; however, missingness prevents long-term analysis of patterns in lung function. We aimed to identify distinct clusters of lung function trajectory among patients with idiopathic pulmonary fibrosis using machine learning techniques. Methods We did a secondary analysis of longitudinal data on FVC collected from a cohort of patients with idiopathic pulmonary fibrosis from the PROFILE study; a multicentre, prospective, observational cohort study. We evaluated the imputation performance of conventional and machine learning techniques to impute missing data and then analysed the fully imputed dataset by unsupervised clustering using self-organising maps. We compared anthropometric features, genomic associations, serum biomarkers, and clinical outcomes between clusters. We also performed a replication of the analysis on data from a cohort of patients with idiopathic pulmonary fibrosis from an independent dataset, obtained from the Chicago Consortium. Findings 415 (71%) of 581 participants recruited into the PROFILE study were eligible for further analysis. An unsupervised machine learning algorithm had the lowest imputation error among tested methods, and self-organising maps identified four distinct clusters (1–4), which was confirmed by sensitivity analysis. Cluster 1 comprised 140 (34%) participants and was associated with a disease trajectory showing a linear decline in FVC over 3 years. Cluster 2 comprised 100 (24%) participants and was associated with a trajectory showing an initial improvement in FVC before subsequently decreasing. Cluster 3 comprised 113 (27%) participants and was associated with a trajectory showing an initial decline in FVC before subsequent stabilisation. Cluster 4 comprised 62 (15%) participants and was associated with a trajectory showing stable lung function. Median survival was shortest in cluster 1 (2·87 years [IQR 2·29–3·40]) and cluster 3 (2·23 years [1·75–3·84]), followed by cluster 2 (4·74 years [3·96–5·73]), and was longest in cluster 4 (5·56 years [5·18–6·62]). Baseline FEV1 to FVC ratio and concentrations of the biomarker SP-D were significantly higher in clusters 1 and 3. Similar lung function clusters with some shared anthropometric features were identified in the replication cohort. Interpretation Using a data-driven unsupervised approach, we identified four clusters of lung function trajectory with distinct clinical and biochemical features. Enriching or stratifying longitudinal spirometric data into clusters might optimise evaluation of intervention efficacy during clinical trials and patient management., National Institute for Health and Care Research, Medical Research Council, GlaxoSmithKline

Published
2022

9. Effects of interferon-gamma 1b on biomarker expression in patients with idiopathic pulmonary fibrosis

Author

Strieter, R M, Starko, K M, Enelow, R I, Noth, I, and Valentine, V G

Subjects
IFN-gamma 1b, proteins, pulmonary fibrosis, RNA
Abstract

In a recent study of IFN-gamma 1b in 330 patients with idiopathic pulmonary fibrosis (IPF), progression-free survival was unchanged; however, a trend toward lower mortality was seen in IFN-gamma 1b-treated patients compared with placebo-treated patients (9.9 vs. 16.7%; p = 0.08). The purpose of this randomized, double-blind, placebo-controlled trial was to characterize molecular effects of subcutaneous IFN-gamma 1b (200 mug) thrice weekly for 6 months versus placebo in 32 patients with IPF. Messenger RNA in transbronchial lung biopsies and bronchoalveolar lavage cell pellet and protein levels in bronchoalveolar lavage fluid (BALF) and plasma were evaluated. After IFN-gamma 1b treatment, IFN-inducible T cell-alpha chemoattractant/CXCL11 (a chemokine with immunomodulatory, antiangiogenic, and defensin-like antimicrobial properties) increased in BALF (p = 0.016) and plasma (p 0.05 and less than or equal to 0.10) associated with IFN-gamma 1b treatment included an increase in IFNI-inducible T cell-alpha chemoattractant/CXCL11, a decrease in elastin, and smaller increases for Type III procollagen and platelet-derived growth factor B. Changes in biomarkers of fibrosis, angiogenesis, proliferation, immunomodulation, and antimicrobial activity suggest that IFN-gamma 1b may affect IPF through multiple pathways.

Published
2004

11. Proteomic Determinants of Idiopathic Pulmonary Fibrosis Survival

Author

Oldham, J., primary, Huang, Y., additional, Ma, S.F., additional, Lee, C.T., additional, Kim, J., additional, Pugashetti, J.V., additional, Mou, K., additional, Schwab, A., additional, Strek, M.E., additional, Adegunsoye, A.O., additional, Linderholm, A., additional, Martinez, F.J., additional, and Noth, I., additional

Published
2023
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14. Machine Learning of Plasma Proteomics for Differential Diagnosis of Interstitial Lung Disease

Author

Huang, Y., primary, Oldham, J., additional, Ma, S.F., additional, Adegunsoye, A.O., additional, Lee, C.T., additional, Murray, S., additional, Kim, J., additional, Bonham, C., additional, Paul, T., additional, Mannem, H., additional, Linderholm, A., additional, Maher, T.M., additional, Molyneaux, P.L., additional, Strek, M.E., additional, Martinez, F.J., additional, and Noth, I., additional

Published
2023
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16. Introduction of Home Spirometry in Tracking Disease Progression in Patients With IPF: Baseline Data From the Prospective Treatment Efficacy in IPF Using Genotype for NAC Selection (PRECISIONS) Clinical Trial

Author

Podolanczuk, A., primary, Kim, J., additional, Taylor, C., additional, Murray, S., additional, Raghu, G., additional, Flaherty, K.R., additional, Spino, C.A., additional, Noth, I., additional, Martinez, F.J., additional, and Cooper, C.B., additional

Published
2023
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18. S109 Genome-wide analysis of longitudinal lung function and gas transfer in individuals with idiopathic pulmonary fibrosis

Author

Allen, RJ, primary, Oldham, JM, additional, Jenkins, DA, additional, Leavy, OC, additional, Guillen-Guio, B, additional, Melbourne, CA, additional, Ma, SF, additional, Jou, J, additional, Kim, JS, additional, Fahy, WA, additional, Oballa, E, additional, Hubbard, RB, additional, Navaratnam, V, additional, Braybrooke, R, additional, Saini, G, additional, Roach, KM, additional, Tobin, MD, additional, Hirani, N, additional, Whyte, MKB, additional, Kaminski, N, additional, Zhang, Y, additional, Martinez, FJ, additional, Linderholm, AL, additional, Adegunsoye, A, additional, Strek, ME, additional, Maher, TM, additional, Molyneaux, PL, additional, Flores, C, additional, Noth, I, additional, Jenkins, RG, additional, and Wain, LV, additional

Published
2022
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20. Longitudinal lung function and gas transfer in individuals with idiopathic pulmonary fibrosis: a genome-wide association study

Author

Allen, RJ, Oldham, JM, Jenkins, DA, Leavy, OC, Guillen-Guio, B, Melbourne, CA, Ma, S-F, Jou, J, Kim, JS, CleanUP-IPF Investigators of the Pulmonary Trials Cooperative, Fahy, WA, Oballa, E, Hubbard, RB, Navaratnam, V, Braybrooke, R, Saini, G, Roach, KM, Tobin, MD, Hirani, N, Whyte, MKB, Kaminski, N, Zhang, Y, Martinez, FJ, Linderholm, AL, Adegunsoye, A, Strek, ME, Maher, TM, Molyneaux, PL, Flores, C, Noth, I, Gisli Jenkins, R, and Wain, LV

Subjects
Pulmonary and Respiratory Medicine
Abstract

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an incurable lung disease characterised by progressive scarring leading to alveolar stiffness, reduced lung capacity, and impeded gas transfer. We aimed to identify genetic variants associated with declining lung capacity or declining gas transfer after diagnosis of IPF. METHODS: We did a genome-wide meta-analysis of longitudinal measures of forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO) in individuals diagnosed with IPF. Individuals were recruited to three studies between June, 1996, and August, 2017, from across centres in the US, UK, and Spain. Suggestively significant variants were investigated further in an additional independent study (CleanUP-IPF). All four studies diagnosed cases following American Thoracic Society/European Respiratory Society guidelines. Variants were defined as significantly associated if they had a meta-analysis p

Published
2022

21. Leveraging global multi-ancestry meta-analysis in the study of idiopathic pulmonary fibrosis genetics

Author

Partanen, J. J. (Juulia J.), Häppölä, P. (Paavo), Zhou, W. (Wei), Lehisto, A. A. (Arto A.), Ainola, M. (Mari), Sutinen, E. (Eva), Allen, R. J. (Richard J.), Stockwell, A. D. (Amy D.), Leavy, O. C. (Olivia C.), Oldham, J. M. (Justin M.), Guillen-Guio, B. (Beatriz), Cox, N. J. (Nancy J.), Hirbo, J. B. (Jibril B.), Schwartz, D. A. (David A.), Fingerlin, T. E. (Tasha E.), Flores, C. (Carlos), Noth, I. (Imre), Yaspan, B. L. (Brian L.), Jenkins, R. G. (R. Gisli), Wain, L. V. (Louise V.), Ripatti, S. (Samuli), Pirinen, M. (Matti), I. I. (International IPF Genetics Consortium), G. B. (Global Biobank Meta-Analysis Initiative (GBMI)), Laitinen, T. (Tarja), Kaarteenaho, R. (Riitta), Myllärniemi, M. (Marjukka), Daly, M. J. (Mark J.), Koskela, J. T. (Jukka T.), Partanen, J. J. (Juulia J.), Häppölä, P. (Paavo), Zhou, W. (Wei), Lehisto, A. A. (Arto A.), Ainola, M. (Mari), Sutinen, E. (Eva), Allen, R. J. (Richard J.), Stockwell, A. D. (Amy D.), Leavy, O. C. (Olivia C.), Oldham, J. M. (Justin M.), Guillen-Guio, B. (Beatriz), Cox, N. J. (Nancy J.), Hirbo, J. B. (Jibril B.), Schwartz, D. A. (David A.), Fingerlin, T. E. (Tasha E.), Flores, C. (Carlos), Noth, I. (Imre), Yaspan, B. L. (Brian L.), Jenkins, R. G. (R. Gisli), Wain, L. V. (Louise V.), Ripatti, S. (Samuli), Pirinen, M. (Matti), I. I. (International IPF Genetics Consortium), G. B. (Global Biobank Meta-Analysis Initiative (GBMI)), Laitinen, T. (Tarja), Kaarteenaho, R. (Riitta), Myllärniemi, M. (Marjukka), Daly, M. J. (Mark J.), and Koskela, J. T. (Jukka T.)

Abstract

Summary The research of rare and devastating orphan diseases, such as idiopathic pulmonary fibrosis (IPF) has been limited by the rarity of the disease itself. The prognosis is poor—the prevalence of IPF is only approximately four times the incidence, limiting the recruitment of patients to trials and studies of the underlying biology. Global biobanking efforts can dramatically alter the future of IPF research. We describe a large-scale meta-analysis of IPF, with 8,492 patients and 1,355,819 population controls from 13 biobanks around the globe. Finally, we combine this meta-analysis with the largest available meta-analysis of IPF, reaching 11,160 patients and 1,364,410 population controls. We identify seven novel genome-wide significant loci, only one of which would have been identified if the analysis had been limited to European ancestry individuals. We observe notable pleiotropy across IPF susceptibility and severe COVID-19 infection and note an unexplained sex-heterogeneity effect at the strongest IPF locus MUC5B.

Published
2022

23. Integrating Gene Expression with Genome-Wide Association Summary Statistics to Identify Genes Associated with Idiopathic Pulmonary Fibrosis Survival

Author

Hu, X., primary, Kim, J., additional, Ma, S.F., additional, Huang, Y., additional, Oldham, J., additional, Allen, R., additional, Molyneaux, P.L., additional, Joseph, C., additional, Guillen Guio, B., additional, Hernández Beeftink, T., additional, Kropski, J., additional, Lee, C.T., additional, Adegunsoye, A.O., additional, Pugashetti, J.V., additional, Linderholm, A., additional, Strek, M.E., additional, Hubbard, R.B., additional, Hart, S.P., additional, Nicholson, A., additional, Lancaster, L., additional, Lorenzo-Salazar, J.M., additional, Vo, V., additional, Hirani, N., additional, Whyte, M.K., additional, Parfrey, H., additional, Rassl, D., additional, Wallace, W., additional, Valenzi, E., additional, Zhang, Y., additional, Stockwell, A., additional, Kaminski, N., additional, Wolters, P.J., additional, Molina-Molina, M., additional, Martinez, F.J., additional, Hall, I.P., additional, Tobin, M.D., additional, Maher, T.M., additional, Blackwell, T.S., additional, Yaspan, B.L., additional, Jenkins, R.G., additional, Wain, L.V., additional, Flores, C., additional, Noth, I., additional, and Manichaikul, A., additional

Published
2022
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24. Analysis of Forced Vital Capacity (FVC) Trajectories in Idiopathic Pulmonary Fibrosis (IPF) Identifies Four Distinct Clusters of Disease Behaviour

Author

Fainberg, H., primary, Allen, R., additional, Kraven, L., additional, Molyneaux, P.L., additional, Oldham, J., additional, Fahy, W., additional, Porte, J., additional, Saini, G., additional, Karsdal, M., additional, Leeming, D.J., additional, Triguero, I., additional, Oballa, E., additional, Noth, I., additional, Wells, A.U., additional, Renzoni, E., additional, Maher, T., additional, Wain, L.V., additional, Stewart, I., additional, and Jenkins, J., additional

Published
2022
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25. Phage Immunoprecipitation Sequencing Defines CDHR5 and GIMAP1 as Novel Biomarkers for a Subset of Fibrotic Pulmonary Disorders

Author

Upadhyay, V., primary, Yoon, Y.M., additional, Vazquez, S.E., additional, Velez, T.E., additional, Jones, K.D., additional, Lee, C.T., additional, Law, C.S., additional, Noth, I., additional, Strek, M.E., additional, Anderson, M., additional, DeRisi, J.L., additional, Sperling, A.I., additional, and Shum, A., additional

Published
2022
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26. S63 Genome-wide sex-by-SNP interaction analysis of susceptibility to idiopathic pulmonary fibrosis

Author

Leavy, OC, primary, Allen, RJ, additional, Oldham, JM, additional, Guillen-Guio, B, additional, Stockwell, A, additional, Braybrooke, R, additional, Hubbard, RB, additional, Ma, S, additional, Fingerlin, TE, additional, Kaminski, N, additional, Zhang, Y, additional, Schwartz, DA, additional, Yaspan, B, additional, Maher, TM, additional, Molyneaux, PL, additional, Flores, C, additional, Noth, I, additional, Jenkins, RG, additional, and Wain, LV, additional

Published
2021
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27. S65 Genome-wide association study of survival times after diagnosis of idiopathic pulmonary fibrosis

Author

Allen, RJ, primary, Oldham, JM, additional, Lorenzo-Salazar, JM, additional, Molyneaux, PL, additional, Ma, SF, additional, Stockwell, A, additional, Joseph, C, additional, Kim, JS, additional, Guillen-Guio, B, additional, Hernandez-Beeftink, T, additional, Kropski, J, additional, Huang, Y, additional, Lee, CT, additional, Adegunsoye, A, additional, Pugashetti, JV, additional, Linderholm, A, additional, Vo, V, additional, Strek, M, additional, Hubbard, R, additional, Hirani, N, additional, Whyte, MKB, additional, Hart, S, additional, Nicholson, A, additional, Parfrey, H, additional, Rassl, D, additional, Wallace, W, additional, Fahy, WA, additional, Valenzi, E, additional, Zhang, Y, additional, Kaminski, N, additional, Wolters, P, additional, Molina-Molina, M, additional, Martinez, FJ, additional, Hall, I, additional, Tobin, MD, additional, Maher, TM, additional, Blackwell, T, additional, Yaspan, B, additional, Jenkins, RG, additional, Flores, C, additional, Wain, LV, additional, and Noth, I, additional

Published
2021
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28. Einleitung - Gleichgeschlechtliche Liebe und die Kirchen : Zum Umgang mit homosexuellen Partnerschaften

Author

Braunschweig, Michael Ulrich; https://orcid.org/0000-0001-9771-9115, Noth, Isabelle, Tanner, Mathias, Braunschweig, M U ( Michael Ulrich ), Noth, I ( Isabelle ), Tanner, M ( Mathias ), Braunschweig, Michael Ulrich; https://orcid.org/0000-0001-9771-9115, Noth, Isabelle, Tanner, Mathias, Braunschweig, M U ( Michael Ulrich ), Noth, I ( Isabelle ), and Tanner, M ( Mathias )

Published
2021

29. Ehe und Familie im Wandel – Entwicklungen in Recht und Politik

Author

Braunschweig, Michael Ulrich; https://orcid.org/0000-0001-9771-9115, Noth, Isabelle, Tanner, Mathias, Braunschweig, M U ( Michael Ulrich ), Noth, I ( Isabelle ), Tanner, M ( Mathias ), Braunschweig, Michael Ulrich; https://orcid.org/0000-0001-9771-9115, Noth, Isabelle, Tanner, Mathias, Braunschweig, M U ( Michael Ulrich ), Noth, I ( Isabelle ), and Tanner, M ( Mathias )

Published
2021

30. The MUC5B rs35705950 Allele Associates with Increased Honeycombing Risk in Individuals of Diverse Ancestry with Pulmonary Fibrosis

Author

Adegunsoye, A.O., primary, Garcia, N.M., additional, Tyker, A., additional, Lee, C.T., additional, Strykowski, R., additional, Bauer Ventura, I., additional, Guzy, R., additional, Jablonski, R., additional, Vij, R., additional, Chung, J.H., additional, Flores, C., additional, Ma, S.F., additional, Oldham, J., additional, Strek, M.E., additional, and Noth, I., additional

Published
2021
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35. Detection and Early Referral of Patients With Interstitial Lung Abnormalities: An Expert Survey Initiative

Author

Hunninghake, G. M., Goldin, J. G., Kadoch, M. A., Kropski, J. A., Rosas, I. O., Wells, A. U., Yadav, R., Lazarus, H. M., Abtin, F. G., Corte, T. J., de Andrade, J. A., Johannson, K. A., Kolb, M. R., Lynch, D. A., Oldham, J. M., Spagnolo, P., Strek, M. E., Tomassetti, S., Washko, G. R., White, E. S., Abtin, F., Antoniou, K., Blackwell, T., Brown, K., Chung, J., Corte, T., Crestani, B., Crossno, P., Culver, D., de Andrade, J., Deveraj, A., Flaherty, K., Gudmundsson, G., Hatabu, H., Jacob, J., Johansson, K., Kanne, J., Kazerooni, E., Kolb, M., Lynch, D., Maher, T., Martinez, F., Morais, A., Nathan, S. D., Noth, I., Oldham, J., Podolanczuk, A., Poletti, V., Ravaglia, C., Renzoni, E., Richeldi, L., Rubin, G., Ryerson, C., Sahoo, D., Suh, R., Sverzellati, N., Valeyre, D., Walsh, S., and Washko, G.

Subjects
Lung Diseases, interstitial lung disease, Male, fibrosis, Settore MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, X-Ray Computed, Respiratory Function Tests, CT, interstitial lung abnormalities, survey, Early Diagnosis, Pulmonologists, Surveys and Questionnaires, Radiologists, Disease Progression, Humans, Female, Interstitial, Lung Diseases, Interstitial, Tomography, X-Ray Computed, Tomography, Referral and Consultation
Abstract

Interstitial lung abnormalities (ILA) may represent undiagnosed early-stage or subclinical interstitial lung disease (ILD). ILA are often observed incidentally in patients who subsequently develop clinically overt ILD. There is limited information on consensus definitions for, and the appropriate evaluation of, ILA. Early recognition of patients with ILD remains challenging, yet critically important. Expert consensus could inform early recognition and referral.Can consensus-based expert recommendations be identified to guide clinicians in the recognition, referral, and follow-up of patients with or at risk of developing early ILDs?Pulmonologists and radiologists with expertise in ILD participated in two iterative rounds of surveys. The surveys aimed to establish consensus regarding ILA reporting, identification of patients with ILA, and identification of populations that might benefit from screening for ILD. Recommended referral criteria and follow-up processes were also addressed. Threshold for consensus was defined a priori as ≥ 75% agreement or disagreement.Fifty-five experts were invited and 44 participated; consensus was reached on 39 of 85 questions. The following clinically important statements achieved consensus: honeycombing and traction bronchiectasis or bronchiolectasis indicate potentially progressive ILD; honeycombing detected during lung cancer screening should be reported as potentially significant (eg, with the Lung CT Screening Reporting and Data System "S-modifier" [Lung-RADS; which indicates clinically significant or potentially significant noncancer findings]), recommending referral to a pulmonologist in the radiology report; high-resolution CT imaging and full pulmonary function tests should be ordered if nondependent subpleural reticulation, traction bronchiectasis, honeycombing, centrilobular ground-glass nodules, or patchy ground-glass opacity are observed on CT imaging; patients with honeycombing or traction bronchiectasis should be referred to a pulmonologist irrespective of diffusion capacity values; and patients with systemic sclerosis should be screened with pulmonary function tests for early-stage ILD.Guidance was established for identifying clinically relevant ILA, subsequent referral, and follow-up. These results lay the foundation for developing practical guidance on managing patients with ILA.

Published
2020

36. Proportion of idiopathic pulmonary fibrosis risk explained by known genetic loci

Author

Leavy, OC, Ma, S-F, Molyneaux, PL, Maher, TM, Oldham, JM, Flores, C, Noth, I, Jenkins, RG, Dudbridge, F, Wain, LV, and Allen, RJ

Subjects
respiratory system, respiratory tract diseases
Abstract

Genome-wide association studies have identified 14 genetic loci associated with susceptibility to idiopathic pulmonary fibrosis (IPF), a devastating lung disease with poor prognosis. Of these, the variant with the strongest association, rs35705950, is located in the promoter region of the MUC5B gene and has a risk allele (T) frequency of 30-35% in IPF cases. Here we present estimates of the proportion of disease liability explained by each of the 14 IPF risk variants as well as estimates of the proportion of cases that can be attributed to each variant. We estimate that rs35705950 explains 5.9-9.4% of disease liability, which is much lower than previously reported estimates. Of every 100,000 individuals with the rs35705950_GG genotype we estimate 30 will have IPF, whereas for every 100,000 individuals with the rs35705950_GT genotype 152 will have IPF. Quantifying the impact of genetic risk factors on disease liability improves our understanding of the underlying genetic architecture of IPF and provides insight into the impact of genetic factors in risk prediction modelling.

Published
2020

37. Overlap of Genetic Risk between Interstitial Lung Abnormalities and Idiopathic Pulmonary Fibrosis

Author

Oldham, J.M., Hunninghake, G.M., Rosas, I.O., Rotter, J.I., Fingerlin, T.E., Bleecker, E.R., Hobbs, B.D., Eiriksdottir, G., Wain, L.V., Cho, M.H., Zilhao Nogueira, N.R., Lederer, D.J., Schwartz, D.A., Podolanczuk, A.J., Ortega, V.E., Jenkins, R.G., Newell, J.D., Jr, Silverman, E.K., Araki, T., Dupuis, J., Meyers, D.A., Allen, R.J., Nguyen, J., O'Neal, W.K., Maher, T.M., Barr, R.G., Hubbard, R.B., Nishino, M., Gudnason, V., Xu, H., Ampleford, E.J., Manichaikul, A., Ma, S.-F., Hatabu, H., Rich, S.S., Peters, S.P., Putman, R.K., Noth, I., Madahar, P., Washko, G.R., Gudmundsson, G., and O'Connor, G.T.

Subjects
Lung Diseases, Male, Aging, Chronic Obstructive Pulmonary Disease, Respiratory System, SNP, Autoimmune Disease, Medical and Health Sciences, Promoter Regions, Rare Diseases, Genetic, interstitial lung abnormalities, Clinical Research, Genetics, Humans, 2.1 Biological and endogenous factors, Genetic Predisposition to Disease, Polymorphism, Aetiology, Lung, Aged, genome-wide association study, Prevention, Human Genome, Single Nucleotide, Middle Aged, beta Karyopherins, idiopathic pulmonary fibrosis, Mucin-5B, respiratory tract diseases, Genetic Loci, Case-Control Studies, Respiratory, TATA Box Binding Protein-Like Proteins, Female, Interstitial
Abstract

Rationale: Interstitial lung abnormalities (ILAs) are associated with the highest genetic risk locus for idiopathic pulmonary fibrosis (IPF); however, the extent to which there are unique associations among individuals with ILAs or additional overlap with IPF is not known.Objectives: To perform a genome-wide association study (GWAS) of ILAs.Methods: ILAs and a subpleural-predominant subtype were assessed on chest computed tomography (CT) scans in the AGES (Age Gene/Environment Susceptibility), COPDGene (Genetic Epidemiology of Chronic Obstructive Pulmonary Disease [COPD]), Framingham Heart, ECLIPSE (Evaluation of COPD Longitudinally to Identify Predictive Surrogate End-points), MESA (Multi-Ethnic Study of Atherosclerosis), and SPIROMICS (Subpopulations and Intermediate Outcome Measures in COPD Study) studies. We performed a GWAS of ILAs in each cohort and combined the results using a meta-analysis. We assessed for overlapping associations in independent GWASs of IPF.Measurements and Main Results: Genome-wide genotyping data were available for 1,699 individuals with ILAs and 10,274 control subjects. The MUC5B (mucin 5B) promoter variant rs35705950 was significantly associated with both ILAs (P = 2.6 × 10-27) and subpleural ILAs (P = 1.6 × 10-29). We discovered novel genome-wide associations near IPO11 (rs6886640, P = 3.8 × 10-8) and FCF1P3 (rs73199442, P = 4.8 × 10-8) with ILAs, and near HTRE1 (rs7744971, P = 4.2 × 10-8) with subpleural-predominant ILAs. These novel associations were not associated with IPF. Among 12 previously reported IPF GWAS loci, five (DPP9, DSP, FAM13A, IVD, and MUC5B) were significantly associated (P

Published
2019

38. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study

Author

Walsh S. L. F., Maher T. M., Kolb M., Poletti V., Nusser R., Richeldi L., Vancheri C., Wilsher M. L., Antoniou K. M., Behr J., Bendstrup E., Brown K., Calandriello L., Corte T. J., Cottin V., Crestani B., Flaherty K., Glaspole I., Grutters J., Inoue Y., Kokosi M., Kondoh Y., Kouranos V., Kreuter M., Johannson K., Judge E., Ley B., Margaritopoulos G., Martinez F. J., Molina-Molina M., Morais A., Nunes H., Raghu G., Ryerson C. J., Selman M., Spagnolo P., Taniguchi H., Tomassetti S., Valeyre D., Wijsenbeek M., Wuyts W., Hansell D., Wells A., Zhu P. S., Yuan Y., Yoshito Fukuda C., Yoshimatsu Y., Xaubet A., Wong A. M., White P., Westney G., West A., Wessendorf T., Waseda Y., Wang C., Vienna J. M., Videnovic Ivanov J., Vicens Zygmunt V., Venero Caceres M. C., Velasquez Pinto G., Veitch E., Vasakova M., Varone F., Varela B. E., Van Hal P., Van De Ven M., Van Der Lee I., Van Den Toorn L., Urrutia Gajate A., Urban J., Ugarte Fornell L. G., Tzouvelekis A., Twohig K., Turner A., Trujillo S., Triani A., Traila D., Torres V., Tomioka H., Tomii K., Tomic R., Toma C., Tokgoz Akyil F., Tobino K., Tobar R., Tiwari A., Tibana R., Tian X., Thillai M., Tham W., Teo F., Tekavec Trkanjec J., Teixeira P., Tarpey D., Tapias L., Tanizawa K., Tanino Y., Takada T., Tabaj G., Szolnoki E., Swarnakar R., Strambu I., Sterclova M., Spinks K., Soo C. I., Soltani A., Solanki S., Sobh E., Soares M. R., Smith J., Smith B., Slocum P., Slabbynck H., Sivokozov I., Shifren A., Shen S. M., Sharp C., Shanmuganathan A., Sebastiani A., Scarlata S., Savas R., Sasaki S., Santeliz J., Santana ANC., Sanchez R., Salinas M., Saito S., Ryan F., Royo Prats J. A., Rosi E., Rokadia H., Robles Perez A., Rivera Ortega P., Rio Ramirez M., Righetti S., Reichner C., Ravaglia C., Ratanawatkul P., Ramalingam V., Rajasekaran A., Radzikowska E., Ra S. W., Quadrelli S., Precerutti J., Prasad J., Popa D., Pizzalato S., Piotrowski W., Pineiro A., Piloni D., Peros Golubicic T., Perez R., Pereira C., Pereira B., Perch M., Patel N., Patel D., Papanikolaou I., Papakosta D., Panselinas E., Pang Y. K., Pandya P., Padrao E., Ozdemir Kumbasar O., Overbeek M. J., Otto Minasian A., O'Riordan D., Ora J., Oldham J., Okutan O., Ohshimo S., Oguzulgen I. K., Ogura T., O'Donnell T., O'Dochartaigh C., O'Beirne S., Novikova L., Novelli L., Noth I., Nogueira Mendes Neto N., Niroumand M., Nieto A., Neves A., Nambiar A., Nair S., Nadama R., Murtagh E., Mura M., Muller Quernheim J., Mukhopadhyay A., Mukherjee S., Morisset J., Moran O., Mooney J., Moller J., Mogulkoc N., Miyamoto A., Milenkovic B., Mette S., Mejia M., Mei F., Mazzei M., Matsuda T., Mason C., Martinez Frances M., Mannarino S., Mancuzo E., Malli F., Malhotra P., Maillo M., Maia J., Mahdavian M., Madsen F., Luckhardt T., Lucht W., Low S. Y., Lopez Miguel C. P., Lipchik R., Levy S., Levin K., Lee K. L., Lederer D., Lammi M. R., Kwan H. Y., Kukreja S., Kruavit A., Kotecki M., Kolilekas L., Knoop H., Kiyan E., Kishaba T., King Biggs M., Khor Y. H., Khan A., Khalil N., Kedia R., Kebba N., Kawano Dourado L., Kapitan K., Kan C. D., Kalyoncu A. F., Kalluri M., Kabasakal Y., Jyothula S., Juretschke M. A., Jovanovic D., Jonkers R., Jo H., Izumi S., Ishii H., Ikeda S., Ibrahim A., Hyldgaard C., Hunninghake G., Huie T., Hufton A., Hu X., Hseih W. C., Hoyos R., Hoyles R., Holguin Rodriguez O., Hogan M. P., Hodgson U., Hilkin Sogoloff H., Herrera E., Henry B. M., Hellemons M., Hecimovic A., Hayashi R., Hart S., Harari S., Haney S., Hambly N., Hakkim R., Gutierrez M., Gripaldo R., Gomez A., Goh N., Godoy R., Gilbert C., Giannarakis I., Gasparini S., Garcha P., Furtado S., Fois A., Flood Page P., Fletcher S., Fiss E., Figueroa Casas J., Figueroa Casas M., Fiddler C. A., Ferrara G., Fernandez Casares M., Felton C., Faverio P., Fabro A. T., Estrada A., Errhalt P., Enomoto N., Enghelmayer J. I., El Kersh K., Eiger G., Dubaniewicz A., Drakopanagiotakis F., Disayabutr S., Dijkstra A., Diaz Patino J. C., Diaz Castanon J. J., Dhooria S., Dhasmana D. J., De Rosa M., De Luca S., Delobbe A., Delgado D., Delgado C., De La Fuente I., De Kruif M., De Gier M., De Andrade J., Davidsen J. R., Daoud B., Dalhoff K., Cotera Solano J. V., Costa A. N., Coronel S., Confalonieri M., Conemans L., Comellas A., Colella S., Clemente S., Clark J., Ciuffreda M., Chung C. L., Chong S. G., Chirita D., Chen P. L., Chaudhuri N., Chambers D., Chalmers G., Chairman D., Chai G. T., Chacon Chaves R., Cetinsu V., Ceruti M., Ceballos Zuniga C. O., Castillo D., Carbone R. G., Caminati A., Callejas Gonzalez F. J., Butler M., Bustos C., Bukowczan M., Buendia I., Brunetti G., Brockway B., Bresser P., Breseghello J., Bouros D., Botero Zaccour J. A., Borzone G., Borie R., Blum H. C., Blank J., Biswas A., Bennett D., Benjamin M., Belaconi I. N., Beirne P., Beckert L., Bastiampillai S., Bascom R., Bartholmai B., Barros M., Ban AYL., Balestro E., Baldi B., Baddini Martinez J., Baburao A., Babu S., Averyanov A., Avdeev S., Athanazio R., Atahan E., Asuquo B., Assayag D., Antuni J., Antillon S., Anderson K. C., Anderson A., Alwani F., Altinisik G., Alsouofi N., Allam J. S., Al Jahdali H., Al Farttoosi A., Alfaro T., Al Busaidi N., Alavi Foumani A., Agreda Vedia M. G., Agarwal A., Afridi F., Adeyeye O. O., Adegunsoye A., Adamali H., Abedini A., Walsh, S. L. F., Maher, T. M., Kolb, M., Poletti, V., Nusser, R., Richeldi, L., Vancheri, C., Wilsher, M. L., Antoniou, K. M., Behr, J., Bendstrup, E., Brown, K., Calandriello, L., Corte, T. J., Cottin, V., Crestani, B., Flaherty, K., Glaspole, I., Grutters, J., Inoue, Y., Kokosi, M., Kondoh, Y., Kouranos, V., Kreuter, M., Johannson, K., Judge, E., Ley, B., Margaritopoulos, G., Martinez, F. J., Molina-Molina, M., Morais, A., Nunes, H., Raghu, G., Ryerson, C. J., Selman, M., Spagnolo, P., Taniguchi, H., Tomassetti, S., Valeyre, D., Wijsenbeek, M., Wuyts, W., Hansell, D., Wells, A., Zhu, P. S., Yuan, Y., Yoshito Fukuda, C., Yoshimatsu, Y., Xaubet, A., Wong, A. M., White, P., Westney, G., West, A., Wessendorf, T., Waseda, Y., Wang, C., Vienna, J. M., Videnovic Ivanov, J., Vicens Zygmunt, V., Venero Caceres, M. C., Velasquez Pinto, G., Veitch, E., Vasakova, M., Varone, F., Varela, B. E., Van Hal, P., Van De Ven, M., Van Der Lee, I., Van Den Toorn, L., Urrutia Gajate, A., Urban, J., Ugarte Fornell, L. G., Tzouvelekis, A., Twohig, K., Turner, A., Trujillo, S., Triani, A., Traila, D., Torres, V., Tomioka, H., Tomii, K., Tomic, R., Toma, C., Tokgoz Akyil, F., Tobino, K., Tobar, R., Tiwari, A., Tibana, R., Tian, X., Thillai, M., Tham, W., Teo, F., Tekavec Trkanjec, J., Teixeira, P., Tarpey, D., Tapias, L., Tanizawa, K., Tanino, Y., Takada, T., Tabaj, G., Szolnoki, E., Swarnakar, R., Strambu, I., Sterclova, M., Spinks, K., Soo, C. I., Soltani, A., Solanki, S., Sobh, E., Soares, M. R., Smith, J., Smith, B., Slocum, P., Slabbynck, H., Sivokozov, I., Shifren, A., Shen, S. M., Sharp, C., Shanmuganathan, A., Sebastiani, A., Scarlata, S., Savas, R., Sasaki, S., Santeliz, J., Santana, Anc., Sanchez, R., Salinas, M., Saito, S., Ryan, F., Royo Prats, J. A., Rosi, E., Rokadia, H., Robles Perez, A., Rivera Ortega, P., Rio Ramirez, M., Righetti, S., Reichner, C., Ravaglia, C., Ratanawatkul, P., Ramalingam, V., Rajasekaran, A., Radzikowska, E., Ra, S. W., Quadrelli, S., Precerutti, J., Prasad, J., Popa, D., Pizzalato, S., Piotrowski, W., Pineiro, A., Piloni, D., Peros Golubicic, T., Perez, R., Pereira, C., Pereira, B., Perch, M., Patel, N., Patel, D., Papanikolaou, I., Papakosta, D., Panselinas, E., Pang, Y. K., Pandya, P., Padrao, E., Ozdemir Kumbasar, O., Overbeek, M. J., Otto Minasian, A., O'Riordan, D., Ora, J., Oldham, J., Okutan, O., Ohshimo, S., Oguzulgen, I. K., Ogura, T., O'Donnell, T., O'Dochartaigh, C., O'Beirne, S., Novikova, L., Novelli, L., Noth, I., Nogueira Mendes Neto, N., Niroumand, M., Nieto, A., Neves, A., Nambiar, A., Nair, S., Nadama, R., Murtagh, E., Mura, M., Muller Quernheim, J., Mukhopadhyay, A., Mukherjee, S., Morisset, J., Moran, O., Mooney, J., Moller, J., Mogulkoc, N., Miyamoto, A., Milenkovic, B., Mette, S., Mejia, M., Mei, F., Mazzei, M., Matsuda, T., Mason, C., Martinez Frances, M., Mannarino, S., Mancuzo, E., Malli, F., Malhotra, P., Maillo, M., Maia, J., Mahdavian, M., Madsen, F., Luckhardt, T., Lucht, W., Low, S. Y., Lopez Miguel, C. P., Lipchik, R., Levy, S., Levin, K., Lee, K. L., Lederer, D., Lammi, M. R., Kwan, H. Y., Kukreja, S., Kruavit, A., Kotecki, M., Kolilekas, L., Knoop, H., Kiyan, E., Kishaba, T., King Biggs, M., Khor, Y. H., Khan, A., Khalil, N., Kedia, R., Kebba, N., Kawano Dourado, L., Kapitan, K., Kan, C. D., Kalyoncu, A. F., Kalluri, M., Kabasakal, Y., Jyothula, S., Juretschke, M. A., Jovanovic, D., Jonkers, R., Jo, H., Izumi, S., Ishii, H., Ikeda, S., Ibrahim, A., Hyldgaard, C., Hunninghake, G., Huie, T., Hufton, A., Hu, X., Hseih, W. C., Hoyos, R., Hoyles, R., Holguin Rodriguez, O., Hogan, M. P., Hodgson, U., Hilkin Sogoloff, H., Herrera, E., Henry, B. M., Hellemons, M., Hecimovic, A., Hayashi, R., Hart, S., Harari, S., Haney, S., Hambly, N., Hakkim, R., Gutierrez, M., Gripaldo, R., Gomez, A., Goh, N., Godoy, R., Gilbert, C., Giannarakis, I., Gasparini, S., Garcha, P., Furtado, S., Fois, A., Flood Page, P., Fletcher, S., Fiss, E., Figueroa Casas, J., Figueroa Casas, M., Fiddler, C. A., Ferrara, G., Fernandez Casares, M., Felton, C., Faverio, P., Fabro, A. T., Estrada, A., Errhalt, P., Enomoto, N., Enghelmayer, J. I., El Kersh, K., Eiger, G., Dubaniewicz, A., Drakopanagiotakis, F., Disayabutr, S., Dijkstra, A., Diaz Patino, J. C., Diaz Castanon, J. J., Dhooria, S., Dhasmana, D. J., De Rosa, M., De Luca, S., Delobbe, A., Delgado, D., Delgado, C., De La Fuente, I., De Kruif, M., De Gier, M., De Andrade, J., Davidsen, J. R., Daoud, B., Dalhoff, K., Cotera Solano, J. V., Costa, A. N., Coronel, S., Confalonieri, M., Conemans, L., Comellas, A., Colella, S., Clemente, S., Clark, J., Ciuffreda, M., Chung, C. L., Chong, S. G., Chirita, D., Chen, P. L., Chaudhuri, N., Chambers, D., Chalmers, G., Chairman, D., Chai, G. T., Chacon Chaves, R., Cetinsu, V., Ceruti, M., Ceballos Zuniga, C. O., Castillo, D., Carbone, R. G., Caminati, A., Callejas Gonzalez, F. J., Butler, M., Bustos, C., Bukowczan, M., Buendia, I., Brunetti, G., Brockway, B., Bresser, P., Breseghello, J., Bouros, D., Botero Zaccour, J. A., Borzone, G., Borie, R., Blum, H. C., Blank, J., Biswas, A., Bennett, D., Benjamin, M., Belaconi, I. N., Beirne, P., Beckert, L., Bastiampillai, S., Bascom, R., Bartholmai, B., Barros, M., Ban, Ayl., Balestro, E., Baldi, B., Baddini Martinez, J., Baburao, A., Babu, S., Averyanov, A., Avdeev, S., Athanazio, R., Atahan, E., Asuquo, B., Assayag, D., Antuni, J., Antillon, S., Anderson, K. C., Anderson, A., Alwani, F., Altinisik, G., Alsouofi, N., Allam, J. S., Al Jahdali, H., Al Farttoosi, A., Alfaro, T., Al Busaidi, N., Alavi Foumani, A., Agreda Vedia, M. G., Agarwal, A., Afridi, F., Adeyeye, O. O., Adegunsoye, A., Adamali, H., Abedini, A., National Institute for Health Research, British Lung Foundation, Walsh, S, Maher, T, Kolb, M, Poletti, V, Nusser, R, Richeldi, L, Vancheri, C, Wilsher, M, Antoniou, K, Behr, J, Bendstrup, E, Brown, K, Calandriello, L, Corte, T, Cottin, V, Crestani, B, Flaherty, K, Glaspole, I, Grutters, J, Inoue, Y, Kokosi, M, Kondoh, Y, Kouranos, V, Kreuter, M, Johannson, K, Judge, E, Ley, B, Margaritopoulos, G, Martinez, F, Molina-Molina, M, Morais, A, Nunes, H, Raghu, G, Ryerson, C, Selman, M, Spagnolo, P, Taniguchi, H, Tomassetti, S, Valeyre, D, Wijsenbeek, M, Wuyts, W, Hansell, D, Wells, A, Zhu, P, Yuan, Y, Yoshito Fukuda, C, Yoshimatsu, Y, Xaubet, A, Wong, A, White, P, Westney, G, West, A, Wessendorf, T, Waseda, Y, Wang, C, Vienna, J, Videnovic Ivanov, J, Vicens Zygmunt, V, Venero Caceres, M, Velasquez Pinto, G, Veitch, E, Vasakova, M, Varone, F, Varela, B, Van Hal, P, Van De Ven, M, Van Der Lee, I, Van Den Toorn, L, Urrutia Gajate, A, Urban, J, Ugarte Fornell, L, Tzouvelekis, A, Twohig, K, Turner, A, Trujillo, S, Triani, A, Traila, D, Torres, V, Tomioka, H, Tomii, K, Tomic, R, Toma, C, Tokgoz Akyil, F, Tobino, K, Tobar, R, Tiwari, A, Tibana, R, Tian, X, Thillai, M, Tham, W, Teo, F, Tekavec Trkanjec, J, Teixeira, P, Tarpey, D, Tapias, L, Tanizawa, K, Tanino, Y, Takada, T, Tabaj, G, Szolnoki, E, Swarnakar, R, Strambu, I, Sterclova, M, Spinks, K, Soo, C, Soltani, A, Solanki, S, Sobh, E, Soares, M, Smith, J, Smith, B, Slocum, P, Slabbynck, H, Sivokozov, I, Shifren, A, Shen, S, Sharp, C, Shanmuganathan, A, Sebastiani, A, Scarlata, S, Savas, R, Sasaki, S, Santeliz, J, Santana, A, Sanchez, R, Salinas, M, Saito, S, Ryan, F, Royo Prats, J, Rosi, E, Rokadia, H, Robles Perez, A, Rivera Ortega, P, Rio Ramirez, M, Righetti, S, Reichner, C, Ravaglia, C, Ratanawatkul, P, Ramalingam, V, Rajasekaran, A, Radzikowska, E, Ra, S, Quadrelli, S, Precerutti, J, Prasad, J, Popa, D, Pizzalato, S, Piotrowski, W, Pineiro, A, Piloni, D, Peros Golubicic, T, Perez, R, Pereira, C, Pereira, B, Perch, M, Patel, N, Patel, D, Papanikolaou, I, Papakosta, D, Panselinas, E, Pang, Y, Pandya, P, Padrao, E, Ozdemir Kumbasar, O, Overbeek, M, Otto Minasian, A, O'Riordan, D, Ora, J, Oldham, J, Okutan, O, Ohshimo, S, Oguzulgen, I, Ogura, T, O'Donnell, T, O'Dochartaigh, C, O'Beirne, S, Novikova, L, Novelli, L, Noth, I, Nogueira Mendes Neto, N, Niroumand, M, Nieto, A, Neves, A, Nambiar, A, Nair, S, Nadama, R, Murtagh, E, Mura, M, Muller Quernheim, J, Mukhopadhyay, A, Mukherjee, S, Morisset, J, Moran, O, Mooney, J, Moller, J, Mogulkoc, N, Miyamoto, A, Milenkovic, B, Mette, S, Mejia, M, Mei, F, Mazzei, M, Matsuda, T, Mason, C, Martinez Frances, M, Mannarino, S, Mancuzo, E, Malli, F, Malhotra, P, Maillo, M, Maia, J, Mahdavian, M, Madsen, F, Luckhardt, T, Lucht, W, Low, S, Lopez Miguel, C, Lipchik, R, Levy, S, Levin, K, Lee, K, Lederer, D, Lammi, M, Kwan, H, Kukreja, S, Kruavit, A, Kotecki, M, Kolilekas, L, Knoop, H, Kiyan, E, Kishaba, T, King Biggs, M, Khor, Y, Khan, A, Khalil, N, Kedia, R, Kebba, N, Kawano Dourado, L, Kapitan, K, Kan, C, Kalyoncu, A, Kalluri, M, Kabasakal, Y, Jyothula, S, Juretschke, M, Jovanovic, D, Jonkers, R, Jo, H, Izumi, S, Ishii, H, Ikeda, S, Ibrahim, A, Hyldgaard, C, Hunninghake, G, Huie, T, Hufton, A, Hu, X, Hseih, W, Hoyos, R, Hoyles, R, Holguin Rodriguez, O, Hogan, M, Hodgson, U, Hilkin Sogoloff, H, Herrera, E, Henry, B, Hellemons, M, Hecimovic, A, Hayashi, R, Hart, S, Harari, S, Haney, S, Hambly, N, Hakkim, R, Gutierrez, M, Gripaldo, R, Gomez, A, Goh, N, Godoy, R, Gilbert, C, Giannarakis, I, Gasparini, S, Garcha, P, Furtado, S, Fois, A, Flood Page, P, Fletcher, S, Fiss, E, Figueroa Casas, J, Figueroa Casas, M, Fiddler, C, Ferrara, G, Fernandez Casares, M, Felton, C, Faverio, P, Fabro, A, Estrada, A, Errhalt, P, Enomoto, N, Enghelmayer, J, El Kersh, K, Eiger, G, Dubaniewicz, A, Drakopanagiotakis, F, Disayabutr, S, Dijkstra, A, Diaz Patino, J, Diaz Castanon, J, Dhooria, S, Dhasmana, D, De Rosa, M, De Luca, S, Delobbe, A, Delgado, D, Delgado, C, De La Fuente, I, De Kruif, M, De Gier, M, De Andrade, J, Davidsen, J, Daoud, B, Dalhoff, K, Cotera Solano, J, Costa, A, Coronel, S, Confalonieri, M, Conemans, L, Comellas, A, Colella, S, Clemente, S, Clark, J, Ciuffreda, M, Chung, C, Chong, S, Chirita, D, Chen, P, Chaudhuri, N, Chambers, D, Chalmers, G, Chairman, D, Chai, G, Chacon Chaves, R, Cetinsu, V, Ceruti, M, Ceballos Zuniga, C, Castillo, D, Carbone, R, Caminati, A, Callejas Gonzalez, F, Butler, M, Bustos, C, Bukowczan, M, Buendia, I, Brunetti, G, Brockway, B, Bresser, P, Breseghello, J, Bouros, D, Botero Zaccour, J, Borzone, G, Borie, R, Blum, H, Blank, J, Biswas, A, Bennett, D, Benjamin, M, Belaconi, I, Beirne, P, Beckert, L, Bastiampillai, S, Bascom, R, Bartholmai, B, Barros, M, Ban, A, Balestro, E, Baldi, B, Baddini Martinez, J, Baburao, A, Babu, S, Averyanov, A, Avdeev, S, Athanazio, R, Atahan, E, Asuquo, B, Assayag, D, Antuni, J, Antillon, S, Anderson, K, Anderson, A, Alwani, F, Altinisik, G, Alsouofi, N, Allam, J, Al Jahdali, H, Al Farttoosi, A, Alfaro, T, Al Busaidi, N, Alavi Foumani, A, Agreda Vedia, M, Agarwal, A, Afridi, F, Adeyeye, O, Adegunsoye, A, Adamali, H, Abedini, A, and Pulmonary Medicine

Subjects
Male, Pediatrics, International Cooperation, Respiratory System, Hospitals, University, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Cohen's kappa, Diagnosis, UK, 030212 general & internal medicine, Medical diagnosis, Referral and Consultation, Pulmonologists, Idiopathic Pulmonary Fibrosi, Interstitial lung disease, 11 Medical And Health Sciences, Middle Aged, respiratory system, Prognosis, Hospitals, humanities, Dimensional Measurement Accuracy, Clinical Competence, Diagnosis, Differential, Diagnostic Techniques, Respiratory System, Female, Humans, Idiopathic Pulmonary Fibrosis, Quality of Health Care, Reproducibility of Results, Human, Cohort study, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Prognosi, education, MEDLINE, Reproducibility of Result, INTERSTITIAL PNEUMONIA, Interstitial Lung Diseases, 03 medical and health sciences, Internal medicine, PARENCHYMAL LUNG-DISEASE, MANAGEMENT, medicine, Idiopathic pulmonary fibrosis, diagnosis, Pulmonologist, University, business.industry, MORTALITY, Original Articles, medicine.disease, respiratory tract diseases, Diagnostic Techniques, IPF Project Consortium, 030228 respiratory system, Differential, INTEROBSERVER AGREEMENT, UPDATE, COOPERAÇÃO INTERNACIONAL, Differential diagnosis, business
Abstract

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53–0.72, p20 years of experience (C-index=0.72, IQR 0.0–0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70–0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72–0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts., Academic status, access to MDT meetings and clinician experience predict accuracy of a clinical diagnosis of IPF http://ow.ly/k43W30cTMg1

Published
2017

39. Gene Expression Profiling in Patients with Idiopathic Pulmonary Fibrosis (IPF) in the INMARK Trial

Author

Selman, M., primary, Jenkins, R.G.G., additional, White, E.S., additional, Cottin, V., additional, Nishioka, Y., additional, Noth, I., additional, Prasse, A., additional, Song, J.W., additional, Strobel, B., additional, Leparc, G., additional, Ittrich, C., additional, Diefenbach, C., additional, Rohr, K., additional, Stowasser, S., additional, and Maher, T.M., additional

Published
2020
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41. Association of Circulating Proteins with Death or Lung Transplant in the IPF-PRO Registry Cohort

Author

Todd, J.L., primary, Neely, M.L., additional, Overton, R., additional, Mulder, H., additional, Roman, J., additional, Lasky, J.A., additional, De Andrade, J., additional, Gulati, M., additional, Huang, H., additional, Leonard, T.B., additional, Hesslinger, C., additional, Noth, I., additional, Belperio, J.A., additional, Flaherty, K.R., additional, and Palmer, S.M., additional

Published
2020
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48. Effect of nintedanib on biomarkers of extracellular matrix (ECM) turnover and FVC decline in patients with IPF: results from the INMARK study*

Author

Pfeifer, M, additional, Maher, TM, additional, Stowasser, S, additional, Nishioka, Y, additional, White, ES, additional, Cottin, V, additional, Noth, I, additional, Selman, M, additional, Rohr, KB, additional, Wachtlin, D, additional, Ittrich, C, additional, Diefenbach, C, additional, and Jenkins, RG, additional

Published
2020
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49. Cardiovascular safety of nintedanib in subgroups by cardiovascular risk at baseline in the TOMORROW and INPULSIS trials

Author

Noth, I. (Imre), Wijsenbeek-Lourens, M.S. (Marlies), Kolb, M. (Martin), Bonella, F. (Francesco), Moros, L. (Lizette), Wachtlin, D. (Daniel), Corte, T.J. (Tamera J.), Noth, I. (Imre), Wijsenbeek-Lourens, M.S. (Marlies), Kolb, M. (Martin), Bonella, F. (Francesco), Moros, L. (Lizette), Wachtlin, D. (Daniel), and Corte, T.J. (Tamera J.)

Abstract

Nintedanib is a tyrosine kinase inhibitor used to treat idiopathic pulmonary fibrosis (IPF). We investigated the cardiovascular safety of nintedanib using pooled data from the TOMORROW and INPULSIS trials.Cardiovascular events were assessed post hoc in patients with a history of atherosclerotic cardiovascular disease (CVD) and/or one or more cardiovascular risk factors at baseline ("higher cardiovascular risk") and patients with no history of atherosclerotic CVD and no cardiovascular risk factors at baseline ("lower cardiovascular risk").Incidence rates were calculated for 1231 patients (n=723 nintedanib and n=508 placebo), of whom 89.9% had higher cardiovascular risk. Incidence rates of major adverse cardiovascular events were similar in the nintedanib and placebo groups in patients with higher cardiovascular risk (3.88 (95% CI 2.58-5.84) and 3.49 (95% CI 2.10-5.79) per 100 patient-years, respectively) and lower cardiovascular risk (4.78 (95% CI 1.54-14.82) and 5.37 (95% CI 1.73-16.65) per 100 patient-years, respectively). Incidence rates of myocardial infarction in the nintedanib and placebo groups, respectively, were 3.03 (95% CI 1.91-4.81) and 1.16 (95% CI 0.48-2.79) per 100 patient-years in patients with higher cardiovascular risk and 1.59 (95% CI 0.22-11.29) and 1.78 (95% CI 0.25-12.64) per 100 patient-years in patients with lower cardiovascular risk. Incidence rates of other ischaemic heart disease in the nintedanib and placebo groups, respectively, were 1.85 (95% CI 1.02-3.34) and 3.28 (95% CI 1.94-5.54) per 100 patient-years in patients with higher cardiovascular risk and 0 and 1.80 (95% CI 0.25-12.78) per 100 patient-years in patients with lower cardiovascular risk.These data help to establish the cardiovascular safety profile of nintedanib in IPF.

Published
2019
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50. Resequencing study confirms that host defense and cell senescence gene variants contribute to the risk of idiopathic pulmonary fibrosis

Author

Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., Schwartz, D.A., Moore, C., Blumhagen, R.Z., Yang, I.V., Walts, A., Powers, J., Walker, T., Bishop, M., Russell, P., Vestal, B., Cardwell, J., Markin, C.R., Mathai, S.K., Schwarz, M.I., Steele, M.P., Lee, J., Brown, K.K., Loyd, J.E., Crapo, J.D., Silverman, E.K., Cho, M.H., James, J.A., Guthridge, J.M., Cogan, J.D., Kropski, J.A., Swigris, J.J., Bair, C., Kim, D.S., Ji, W., Kim, H., Song, J.W., Maier, L.A., Pacheco, K.A., Hirani, N., Poon, A.S., Li, F., Jenkins, R.G., Braybrooke, R., Saini, G., Maher, T.M., Molyneaux, P.L., Saunders, P., Zhang, Y., Gibson, K.F., Kass, D.J., Rojas, M., Sembrat, J., Wolters, P.J., Collard, H.R., Sundy, J.S., O’Riordan, T., Strek, M.E., Noth, I., Ma, S-F, Porteous, M.K., Kreider, M.E., Patel, N.B., Inoue, Y., Hirose, M., Arai, T., Akagawa, S., Eickelberg, O., Fernandez, I.E., Behr, J., Mogulkoc, N., Corte, T.J., Glaspole, I., Tomassetti, S., Ravaglia, C., Poletti, V., Crestani, B., Borie, R., Kannengiesser, C., Parfrey, H., Fiddler, C., Rassl, D., Molina-Molina, M., Machahua, C., Worboys, A.M., Gudmundsson, G., Isaksson, H.J., Lederer, D.J., Podolanczuk, A.J., Montesi, S.B., Bendstrup, E., Danchel, V., Selman, M., Pardo, A., Henry, M.T., Keane, M.P., Doran, P., Vašáková, M., Sterclova, M., Ryerson, C.J., Wilcox, P.G., Okamoto, T., Furusawa, H., Miyazaki, Y., Laurent, G., Baltic, S., Prêle, C., Moodley, Y., Shea, B.S., Ohta, K., Suzukawa, M., Narumoto, O., Nathan, S.D., Venuto, D.C., Woldehanna, M.L., Kokturk, N., de Andrade, J.A., Luckhardt, T., Kulkarni, T., Bonella, F., Donnelly, S.C., McElroy, A., Armstong, M.E., Aranda, A., Carbone, R.G., Puppo, F., Beckman, K.B., Nickerson, D.A., Fingerlin, T.E., and Schwartz, D.A.

Abstract

Rationale: Several common and rare genetic variants have been associated with idiopathic pulmonary fibrosis, a progressive fibrotic condition that is localized to the lung. Objectives: To develop an integrated understanding of the rare and common variants located in multiple loci that have been reported to contribute to the risk of disease. Methods: We performed deep targeted resequencing (3.69 Mb of DNA) in cases (n = 3,624) and control subjects (n = 4,442) across genes and regions previously associated with disease. We tested for associations between disease and 1) individual common variants via logistic regression and 2) groups of rare variants via sequence kernel association tests. Measurements and Main Results: Statistically significant common variant association signals occurred in all 10 of the regions chosen based on genome-wide association studies. The strongest risk variant is the MUC5B promoter variant rs35705950, with an odds ratio of 5.45 (95% confidence interval, 4.91–6.06) for one copy of the risk allele and 18.68 (95% confidence interval, 13.34–26.17) for two copies of the risk allele (P = 9.60 × 10−295). In addition to identifying for the first time that rare variation in FAM13A is associated with disease, we confirmed the role of rare variation in the TERT and RTEL1 gene regions in the risk of IPF, and found that the FAM13A and TERT regions have independent common and rare variant signals. Conclusions: A limited number of common and rare variants contribute to the risk of idiopathic pulmonary fibrosis in each of the resequencing regions, and these genetic variants focus on biological mechanisms of host defense and cell senescence.

Published
2019

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