Skip to main content
Top
Published in: Virchows Archiv 1/2021

01-01-2021 | Idiopathic Pulmonary Fibrosis | Review and Perspectives

Molecular approach to the classification of chronic fibrosing lung disease—there and back again

Authors: Stijn E. Verleden, Peter Braubach, Mark Kuehnel, Nicolas Dickgreber, Emily Brouwer, Pauline Tittmann, Florian Laenger, Danny Jonigk

Published in: Virchows Archiv | Issue 1/2021

Login to get access

Abstract

Chronic diffuse parenchymal lung disease (DPLD) is an umbrella term for a very heterogeneous group of lung diseases. Over the last decades, clinical, radiological and histopathological criteria have been established to define and separate these entities. More recently the clinical utility of this approach has been challenged as a unifying concept of pathophysiological mechanisms and a shared response to therapy across the disease spectrum have been described. In this review, we discuss molecular motifs for subtyping and the prediction of prognosis focusing on genetics and markers found in the blood, lavage and tissue. As a purely molecular classification so far lacks sufficient sensitivity and specificity for subtyping, it is not routinely used and not implemented in international guidelines. However, a better molecular characterization of lung disease with a more precise identification of patients with, for example, a risk for rapid disease progression would facilitate more accurate treatment decisions and hopefully contribute to better patients’ outcomes.
Literature
1.
go back to reference Länger F, Stark H, Braubach P, Ackermann M, Hussein K, Teiken K et al (2018) Injury patterns in interstitial lung diseases. Pathol. 39(Suppl 2):262–271 Länger F, Stark H, Braubach P, Ackermann M, Hussein K, Teiken K et al (2018) Injury patterns in interstitial lung diseases. Pathol. 39(Suppl 2):262–271
2.
go back to reference Noble PW, Homer RJ (2005) Back to the future: historical perspective on the pathogenesis of idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol. 33(2):113–120PubMed Noble PW, Homer RJ (2005) Back to the future: historical perspective on the pathogenesis of idiopathic pulmonary fibrosis. Am J Respir Cell Mol Biol. 33(2):113–120PubMed
3.
go back to reference (2000) American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 161(2 Pt 1):646–664 (2000) American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 161(2 Pt 1):646–664
4.
go back to reference American Thoracic Society, European Respiratory Society (2002) American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 165(2):277–304 American Thoracic Society, European Respiratory Society (2002) American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001. Am J Respir Crit Care Med. 165(2):277–304
5.
go back to reference Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King te Jr, Kondoh Y, Myers J, Müller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schünemann HJ, ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis (2011) An Official ATS/ERS/JRS/ALAT Statement: Idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 183(6):788–824PubMedPubMedCentral Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier JF, Flaherty KR, Lasky JA, Lynch DA, Ryu JH, Swigris JJ, Wells AU, Ancochea J, Bouros D, Carvalho C, Costabel U, Ebina M, Hansell DM, Johkoh T, Kim DS, King te Jr, Kondoh Y, Myers J, Müller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schünemann HJ, ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis (2011) An Official ATS/ERS/JRS/ALAT Statement: Idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 183(6):788–824PubMedPubMedCentral
6.
go back to reference Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, Pipavath SNJ, Buendía-Roldán I, Selman M, Travis WD, Walsh S, Wilson KC, American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society (2018) Diagnosis of idiopathic pulmonary fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 198(5):e44–e68PubMed Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, Flaherty KR, Wells A, Martinez FJ, Azuma A, Bice TJ, Bouros D, Brown KK, Collard HR, Duggal A, Galvin L, Inoue Y, Jenkins RG, Johkoh T, Kazerooni EA, Kitaichi M, Knight SL, Mansour G, Nicholson AG, Pipavath SNJ, Buendía-Roldán I, Selman M, Travis WD, Walsh S, Wilson KC, American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society (2018) Diagnosis of idiopathic pulmonary fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 198(5):e44–e68PubMed
7.
go back to reference De Sadeleer LJ, Meert C, Yserbyt J, Slabbynck H, Verschakelen JA, Verbeken EK et al (2018) Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: a retrospective observational study of 938 cases. Chest. 153(6):1416–1423PubMed De Sadeleer LJ, Meert C, Yserbyt J, Slabbynck H, Verschakelen JA, Verbeken EK et al (2018) Diagnostic ability of a dynamic multidisciplinary discussion in interstitial lung diseases: a retrospective observational study of 938 cases. Chest. 153(6):1416–1423PubMed
8.
go back to reference Noble PW, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Lancaster L, Lederer DJ, Leff JA, Nathan SD, Pereira CA, Swigris JJ, Valeyre D, King TE Jr (2016) Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 47(1):243–253PubMedPubMedCentral Noble PW, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Lancaster L, Lederer DJ, Leff JA, Nathan SD, Pereira CA, Swigris JJ, Valeyre D, King TE Jr (2016) Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials. Eur Respir J. 47(1):243–253PubMedPubMedCentral
9.
go back to reference King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK et al (2014) A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 370(22):2083–2092PubMed King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK et al (2014) A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 370(22):2083–2092PubMed
10.
go back to reference Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, Cottin V, Flaherty KR, Hansell DM, Inoue Y, Kim DS, Kolb M, Nicholson AG, Noble PW, Selman M, Taniguchi H, Brun M, le Maulf F, Girard M, Stowasser S, Schlenker-Herceg R, Disse B, Collard HR (2014) Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 370(22):2071–2082PubMed Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, Cottin V, Flaherty KR, Hansell DM, Inoue Y, Kim DS, Kolb M, Nicholson AG, Noble PW, Selman M, Taniguchi H, Brun M, le Maulf F, Girard M, Stowasser S, Schlenker-Herceg R, Disse B, Collard HR (2014) Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 370(22):2071–2082PubMed
11.
go back to reference Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V (2020) Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med. 8(2):147–157PubMed Maher TM, Corte TJ, Fischer A, Kreuter M, Lederer DJ, Molina-Molina M, Axmann J, Kirchgaessler KU, Samara K, Gilberg F, Cottin V (2020) Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial. Lancet Respir Med. 8(2):147–157PubMed
12.
go back to reference Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y et al (2019) Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med 381(18):1718–1727PubMed Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y et al (2019) Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med 381(18):1718–1727PubMed
13.
go back to reference Wells AU, Flaherty KR, Brown KK, Inoue Y, Devaraj A, Richeldi L et al (2020) Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med 8(5):453–460 Wells AU, Flaherty KR, Brown KK, Inoue Y, Devaraj A, Richeldi L et al (2020) Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med 8(5):453–460
14.
go back to reference Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M et al (2018) Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev Off J Eur Respir Soc. 31:27(150) Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M et al (2018) Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev Off J Eur Respir Soc. 31:27(150)
15.
go back to reference Wijsenbeek M, Cottin V (2020) Spectrum of Fibrotic Lung Diseases. N Engl J Med 383(10):958–968PubMed Wijsenbeek M, Cottin V (2020) Spectrum of Fibrotic Lung Diseases. N Engl J Med 383(10):958–968PubMed
16.
go back to reference Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ, IPF Consensus Working Group et al (2019) Idiopathic interstitial pneumonia or idiopathic interstitial pneumonitis: what’s in a name? Eur Respir J 53(2):1801939 Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ, IPF Consensus Working Group et al (2019) Idiopathic interstitial pneumonia or idiopathic interstitial pneumonitis: what’s in a name? Eur Respir J 53(2):1801939
17.
go back to reference Brown KK, Martinez FJ, Walsh SLF, Thannickal VJ, Prasse A, Schlenker-Herceg R et al (2020) The natural history of progressive fibrosing interstitial lung diseases. Eur Respir J. 55(6):2000085PubMedPubMedCentral Brown KK, Martinez FJ, Walsh SLF, Thannickal VJ, Prasse A, Schlenker-Herceg R et al (2020) The natural history of progressive fibrosing interstitial lung diseases. Eur Respir J. 55(6):2000085PubMedPubMedCentral
18.
go back to reference Selman M, Pardo A, Barrera L, Estrada A, Watson SR, Wilson K, Aziz N, Kaminski N, Zlotnik A (2006) Gene expression profiles distinguish idiopathic pulmonary fibrosis from hypersensitivity pneumonitis. Am J Respir Crit Care Med. 173(2):188–198PubMed Selman M, Pardo A, Barrera L, Estrada A, Watson SR, Wilson K, Aziz N, Kaminski N, Zlotnik A (2006) Gene expression profiles distinguish idiopathic pulmonary fibrosis from hypersensitivity pneumonitis. Am J Respir Crit Care Med. 173(2):188–198PubMed
19.
go back to reference Pankratz DG, Choi Y, Imtiaz U, Fedorowicz GM, Anderson JD, Colby TV, Myers JL, Lynch DA, Brown KK, Flaherty KR, Steele MP, Groshong SD, Raghu G, Barth NM, Walsh PS, Huang J, Kennedy GC, Martinez FJ (2017) Usual interstitial pneumonia can be detected in transbronchial biopsies using machine learning. Ann Am Thorac Soc. 14(11):1646–1654PubMed Pankratz DG, Choi Y, Imtiaz U, Fedorowicz GM, Anderson JD, Colby TV, Myers JL, Lynch DA, Brown KK, Flaherty KR, Steele MP, Groshong SD, Raghu G, Barth NM, Walsh PS, Huang J, Kennedy GC, Martinez FJ (2017) Usual interstitial pneumonia can be detected in transbronchial biopsies using machine learning. Ann Am Thorac Soc. 14(11):1646–1654PubMed
20.
go back to reference Raghu G, Flaherty KR, Lederer DJ, Lynch DA, Colby TV, Myers JL, Groshong SD, Larsen BT, Chung JH, Steele MP, Benzaquen S, Calero K, Case AH, Criner GJ, Nathan SD, Rai NS, Ramaswamy M, Hagmeyer L, Davis JR, Gauhar UA, Pankratz DG, Choi Y, Huang J, Walsh PS, Neville H, Lofaro LR, Barth NM, Kennedy GC, Brown KK, Martinez FJ (2019) Use of a molecular classifier to identify usual interstitial pneumonia in conventional transbronchial lung biopsy samples: a prospective validation study. Lancet Respir Med. 7(6):487–496PubMed Raghu G, Flaherty KR, Lederer DJ, Lynch DA, Colby TV, Myers JL, Groshong SD, Larsen BT, Chung JH, Steele MP, Benzaquen S, Calero K, Case AH, Criner GJ, Nathan SD, Rai NS, Ramaswamy M, Hagmeyer L, Davis JR, Gauhar UA, Pankratz DG, Choi Y, Huang J, Walsh PS, Neville H, Lofaro LR, Barth NM, Kennedy GC, Brown KK, Martinez FJ (2019) Use of a molecular classifier to identify usual interstitial pneumonia in conventional transbronchial lung biopsy samples: a prospective validation study. Lancet Respir Med. 7(6):487–496PubMed
21.
go back to reference Jonigk D, Stark H, Braubach P, Neubert L, Shin H, Izykowski N, Welte T, Janciauskiene S, Warnecke G, Haverich A, Kuehnel M, Laenger F (2019) Morphological and molecular motifs of fibrosing pulmonary injury patterns. J Pathol Clin Res. 5(4):256–271PubMedPubMedCentral Jonigk D, Stark H, Braubach P, Neubert L, Shin H, Izykowski N, Welte T, Janciauskiene S, Warnecke G, Haverich A, Kuehnel M, Laenger F (2019) Morphological and molecular motifs of fibrosing pulmonary injury patterns. J Pathol Clin Res. 5(4):256–271PubMedPubMedCentral
22.
go back to reference Boon K, Bailey NW, Yang J, Steel MP, Groshong S, Kervitsky D, Brown KK, Schwarz MI, Schwartz DA (2009) Molecular phenotypes distinguish patients with relatively stable from progressive idiopathic pulmonary fibrosis (IPF). PloS One. 4(4):e5134PubMedPubMedCentral Boon K, Bailey NW, Yang J, Steel MP, Groshong S, Kervitsky D, Brown KK, Schwarz MI, Schwartz DA (2009) Molecular phenotypes distinguish patients with relatively stable from progressive idiopathic pulmonary fibrosis (IPF). PloS One. 4(4):e5134PubMedPubMedCentral
23.
go back to reference Planas-Cerezales L, Arias-Salgado EG, Buendia-Roldán I, Montes-Worboys A, López CE, Vicens-Zygmunt V, Hernaiz PL, Sanuy RL, Leiro-Fernandez V, Vilarnau EB, Llinás ES, Sargatal JD, Abellón RP, Selman M, Molina-Molina M (2019) Predictive factors and prognostic effect of telomere shortening in pulmonary fibrosis. Respirol Carlton Vic. 24(2):146–153 Planas-Cerezales L, Arias-Salgado EG, Buendia-Roldán I, Montes-Worboys A, López CE, Vicens-Zygmunt V, Hernaiz PL, Sanuy RL, Leiro-Fernandez V, Vilarnau EB, Llinás ES, Sargatal JD, Abellón RP, Selman M, Molina-Molina M (2019) Predictive factors and prognostic effect of telomere shortening in pulmonary fibrosis. Respirol Carlton Vic. 24(2):146–153
24.
go back to reference Rosas IO, Richards TJ, Konishi K, Zhang Y, Gibson K, Lokshin AE, Lindell KO, Cisneros J, Macdonald SD, Pardo A, Sciurba F, Dauber J, Selman M, Gochuico BR, Kaminski N (2008) MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med. 5(4):e93PubMedPubMedCentral Rosas IO, Richards TJ, Konishi K, Zhang Y, Gibson K, Lokshin AE, Lindell KO, Cisneros J, Macdonald SD, Pardo A, Sciurba F, Dauber J, Selman M, Gochuico BR, Kaminski N (2008) MMP1 and MMP7 as potential peripheral blood biomarkers in idiopathic pulmonary fibrosis. PLoS Med. 5(4):e93PubMedPubMedCentral
25.
go back to reference White ES, Xia M, Murray S, Dyal R, Flaherty CM, Flaherty KR et al (2016) Plasma surfactant protein-D, matrix metalloproteinase-7, and osteopontin index distinguishes idiopathic pulmonary fibrosis from other idiopathic interstitial pneumonias. Am J Respir Crit Care Med 194(10):1242–1251PubMedPubMedCentral White ES, Xia M, Murray S, Dyal R, Flaherty CM, Flaherty KR et al (2016) Plasma surfactant protein-D, matrix metalloproteinase-7, and osteopontin index distinguishes idiopathic pulmonary fibrosis from other idiopathic interstitial pneumonias. Am J Respir Crit Care Med 194(10):1242–1251PubMedPubMedCentral
26.
go back to reference Richards TJ, Kaminski N, Baribaud F, Flavin S, Brodmerkel C, Horowitz D, Li K, Choi J, Vuga LJ, Lindell KO, Klesen M, Zhang Y, Gibson KF (2012) Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 185(1):67–76PubMedPubMedCentral Richards TJ, Kaminski N, Baribaud F, Flavin S, Brodmerkel C, Horowitz D, Li K, Choi J, Vuga LJ, Lindell KO, Klesen M, Zhang Y, Gibson KF (2012) Peripheral blood proteins predict mortality in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 185(1):67–76PubMedPubMedCentral
27.
go back to reference Song JW, Do KH, Jang SJ, Colby TV, Han S, Kim DS (2013) Blood biomarkers MMP-7 and SP-A: predictors of outcome in idiopathic pulmonary fibrosis. Chest. 143(5):1422–1429PubMed Song JW, Do KH, Jang SJ, Colby TV, Han S, Kim DS (2013) Blood biomarkers MMP-7 and SP-A: predictors of outcome in idiopathic pulmonary fibrosis. Chest. 143(5):1422–1429PubMed
28.
go back to reference Inoue Y, Kaner RJ, Guiot J, Maher TM, Tomassetti S, Moiseev S et al (2020) Diagnostic and prognostic biomarkers for chronic fibrosing interstitial lung diseases with a progressive phenotype. Chest 158(2):646–659 Inoue Y, Kaner RJ, Guiot J, Maher TM, Tomassetti S, Moiseev S et al (2020) Diagnostic and prognostic biomarkers for chronic fibrosing interstitial lung diseases with a progressive phenotype. Chest 158(2):646–659
29.
go back to reference Hamai K, Iwamoto H, Ishikawa N, Horimasu Y, Masuda T, Miyamoto S, Nakashima T, Ohshimo S, Fujitaka K, Hamada H, Hattori N, Kohno N (2016) Comparative study of circulating MMP-7, CCL18, KL-6, SP-A, and SP-D as disease markers of idiopathic pulmonary fibrosis. Dis Markers. 2016:1–8 Hamai K, Iwamoto H, Ishikawa N, Horimasu Y, Masuda T, Miyamoto S, Nakashima T, Ohshimo S, Fujitaka K, Hamada H, Hattori N, Kohno N (2016) Comparative study of circulating MMP-7, CCL18, KL-6, SP-A, and SP-D as disease markers of idiopathic pulmonary fibrosis. Dis Markers. 2016:1–8
30.
go back to reference Ohshimo S, Ishikawa N, Horimasu Y, Hattori N, Hirohashi N, Tanigawa K, Kohno N, Bonella F, Guzman J, Costabel U (2014) Baseline KL-6 predicts increased risk for acute exacerbation of idiopathic pulmonary fibrosis. Respir Med. 108(7):1031–1039PubMed Ohshimo S, Ishikawa N, Horimasu Y, Hattori N, Hirohashi N, Tanigawa K, Kohno N, Bonella F, Guzman J, Costabel U (2014) Baseline KL-6 predicts increased risk for acute exacerbation of idiopathic pulmonary fibrosis. Respir Med. 108(7):1031–1039PubMed
31.
go back to reference Maher TM, Oballa E, Simpson JK, Porte J, Habgood A, Fahy WA, Flynn A, Molyneaux PL, Braybrooke R, Divyateja H, Parfrey H, Rassl D, Russell AM, Saini G, Renzoni EA, Duggan AM, Hubbard R, Wells AU, Lukey PT, Marshall RP, Jenkins RG (2017) An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study. Lancet Respir Med. 5(12):946–955PubMed Maher TM, Oballa E, Simpson JK, Porte J, Habgood A, Fahy WA, Flynn A, Molyneaux PL, Braybrooke R, Divyateja H, Parfrey H, Rassl D, Russell AM, Saini G, Renzoni EA, Duggan AM, Hubbard R, Wells AU, Lukey PT, Marshall RP, Jenkins RG (2017) An epithelial biomarker signature for idiopathic pulmonary fibrosis: an analysis from the multicentre PROFILE cohort study. Lancet Respir Med. 5(12):946–955PubMed
32.
go back to reference Neighbors M, Cabanski CR, Ramalingam TR, Sheng XR, Tew GW, Gu C, Jia G, Peng K, Ray JM, Ley B, Wolters PJ, Collard HR, Arron JR (2018) Prognostic and predictive biomarkers for patients with idiopathic pulmonary fibrosis treated with pirfenidone: post-hoc assessment of the CAPACITY and ASCEND trials. Lancet Respir Med. 6(8):615–626PubMed Neighbors M, Cabanski CR, Ramalingam TR, Sheng XR, Tew GW, Gu C, Jia G, Peng K, Ray JM, Ley B, Wolters PJ, Collard HR, Arron JR (2018) Prognostic and predictive biomarkers for patients with idiopathic pulmonary fibrosis treated with pirfenidone: post-hoc assessment of the CAPACITY and ASCEND trials. Lancet Respir Med. 6(8):615–626PubMed
33.
go back to reference Prasse A, Probst C, Bargagli E, Zissel G, Toews GB, Flaherty KR et al (2009) Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 179(8):717–723PubMed Prasse A, Probst C, Bargagli E, Zissel G, Toews GB, Flaherty KR et al (2009) Serum CC-chemokine ligand 18 concentration predicts outcome in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 179(8):717–723PubMed
34.
go back to reference Cai M, Bonella F, He X, Sixt SU, Sarria R, Guzman J, Costabel U (2013) CCL18 in serum, BAL fluid and alveolar macrophage culture supernatant in interstitial lung diseases. Respir Med. 107(9):1444–1452PubMed Cai M, Bonella F, He X, Sixt SU, Sarria R, Guzman J, Costabel U (2013) CCL18 in serum, BAL fluid and alveolar macrophage culture supernatant in interstitial lung diseases. Respir Med. 107(9):1444–1452PubMed
35.
go back to reference Invernizzi R, Barnett J, Rawal B, Nair A, Ghai P, Kingston S et al (2020) Bacterial burden in the lower airways predicts disease progression in idiopathic pulmonary fibrosis and is independent of radiological disease extent. Eur Respir J 55(4):1901519 Invernizzi R, Barnett J, Rawal B, Nair A, Ghai P, Kingston S et al (2020) Bacterial burden in the lower airways predicts disease progression in idiopathic pulmonary fibrosis and is independent of radiological disease extent. Eur Respir J 55(4):1901519
36.
go back to reference Molyneaux PL, Cox MJ, Wells AU, Kim HC, Ji W, Cookson WOC et al (2017) Changes in the respiratory microbiome during acute exacerbations of idiopathic pulmonary fibrosis. Respir Res 18(1):29PubMedPubMedCentral Molyneaux PL, Cox MJ, Wells AU, Kim HC, Ji W, Cookson WOC et al (2017) Changes in the respiratory microbiome during acute exacerbations of idiopathic pulmonary fibrosis. Respir Res 18(1):29PubMedPubMedCentral
37.
go back to reference Prasse A, Binder H, Schupp JC, Kayser G, Bargagli E, Jaeger B, Hess M, Rittinghausen S, Vuga L, Lynn H, Violette S, Jung B, Quast K, Vanaudenaerde B, Xu Y, Hohlfeld JM, Krug N, Herazo-Maya JD, Rottoli P, Wuyts WA, Kaminski N (2019) BAL Cell gene expression is indicative of outcome and airway basal cell involvement in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 199(5):622–630PubMedPubMedCentral Prasse A, Binder H, Schupp JC, Kayser G, Bargagli E, Jaeger B, Hess M, Rittinghausen S, Vuga L, Lynn H, Violette S, Jung B, Quast K, Vanaudenaerde B, Xu Y, Hohlfeld JM, Krug N, Herazo-Maya JD, Rottoli P, Wuyts WA, Kaminski N (2019) BAL Cell gene expression is indicative of outcome and airway basal cell involvement in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 199(5):622–630PubMedPubMedCentral
38.
go back to reference Walsh SLF (2018) Imaging biomarkers and staging in IPF. Curr Opin Pulm Med. 24(5):445–452PubMed Walsh SLF (2018) Imaging biomarkers and staging in IPF. Curr Opin Pulm Med. 24(5):445–452PubMed
39.
go back to reference Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH, Burch LH, Wahidi MM, Phillips JA 3rd, Sporn TA, McAdams H, Schwarz MI, Schwartz DA (2005) Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 172(9):1146–1152PubMedPubMedCentral Steele MP, Speer MC, Loyd JE, Brown KK, Herron A, Slifer SH, Burch LH, Wahidi MM, Phillips JA 3rd, Sporn TA, McAdams H, Schwarz MI, Schwartz DA (2005) Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med. 172(9):1146–1152PubMedPubMedCentral
40.
go back to reference Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE et al (2011) A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. 364(16):1503–1512PubMedPubMedCentral Seibold MA, Wise AL, Speer MC, Steele MP, Brown KK, Loyd JE et al (2011) A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med. 364(16):1503–1512PubMedPubMedCentral
41.
go back to reference Borie R, Le Guen P, Ghanem M, Taillé C, Dupin C, Dieudé P et al (2019) The genetics of interstitial lung diseases. Eur Respir Rev Off J Eur Respir Soc. 30:28(153) Borie R, Le Guen P, Ghanem M, Taillé C, Dupin C, Dieudé P et al (2019) The genetics of interstitial lung diseases. Eur Respir Rev Off J Eur Respir Soc. 30:28(153)
42.
go back to reference Mathai SK, Pedersen BS, Smith K, Russell P, Schwarz MI, Brown KK et al (2016) Desmoplakin variants are associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 193(10):1151–1160PubMedPubMedCentral Mathai SK, Pedersen BS, Smith K, Russell P, Schwarz MI, Brown KK et al (2016) Desmoplakin variants are associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 193(10):1151–1160PubMedPubMedCentral
43.
go back to reference Allen RJ, Porte J, Braybrooke R, Flores C, Fingerlin TE, Oldham JM, Guillen-Guio B, Ma SF, Okamoto T, John AE, Obeidat M, Yang IV, Henry A, Hubbard RB, Navaratnam V, Saini G, Thompson N, Booth HL, Hart SP, Hill MR, Hirani N, Maher TM, McAnulty R, Millar AB, Molyneaux PL, Parfrey H, Rassl DM, Whyte MKB, Fahy WA, Marshall RP, Oballa E, Bossé Y, Nickle DC, Sin DD, Timens W, Shrine N, Sayers I, Hall IP, Noth I, Schwartz DA, Tobin MD, Wain LV, Jenkins RG (2017) Genetic variants associated with susceptibility to idiopathic pulmonary fibrosis in people of European ancestry: a genome-wide association study. Lancet Respir Med. 5(11):869–880PubMedPubMedCentral Allen RJ, Porte J, Braybrooke R, Flores C, Fingerlin TE, Oldham JM, Guillen-Guio B, Ma SF, Okamoto T, John AE, Obeidat M, Yang IV, Henry A, Hubbard RB, Navaratnam V, Saini G, Thompson N, Booth HL, Hart SP, Hill MR, Hirani N, Maher TM, McAnulty R, Millar AB, Molyneaux PL, Parfrey H, Rassl DM, Whyte MKB, Fahy WA, Marshall RP, Oballa E, Bossé Y, Nickle DC, Sin DD, Timens W, Shrine N, Sayers I, Hall IP, Noth I, Schwartz DA, Tobin MD, Wain LV, Jenkins RG (2017) Genetic variants associated with susceptibility to idiopathic pulmonary fibrosis in people of European ancestry: a genome-wide association study. Lancet Respir Med. 5(11):869–880PubMedPubMedCentral
44.
go back to reference Moore C, Blumhagen RZ, Yang IV, Walts A, Powers J, Walker T et al (2019) Resequencing study confirms that host defense and cell senescence gene variants contribute to the risk of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 200(2):199–208PubMedPubMedCentral Moore C, Blumhagen RZ, Yang IV, Walts A, Powers J, Walker T et al (2019) Resequencing study confirms that host defense and cell senescence gene variants contribute to the risk of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 200(2):199–208PubMedPubMedCentral
45.
go back to reference Allen RJ, Guillen-Guio B, Oldham JM, Ma S-F, Dressen A, Paynton ML et al (2020) Genome-wide association study of susceptibility to idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 201(5):564–574PubMedPubMedCentral Allen RJ, Guillen-Guio B, Oldham JM, Ma S-F, Dressen A, Paynton ML et al (2020) Genome-wide association study of susceptibility to idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 201(5):564–574PubMedPubMedCentral
46.
go back to reference Ley B, Newton CA, Arnould I, Elicker BM, Henry TS, Vittinghoff E, Golden JA, Jones KD, Batra K, Torrealba J, Garcia CK, Wolters PJ (2017) The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: an observational cohort-control study. Lancet Respir Med. 5(8):639–647PubMedPubMedCentral Ley B, Newton CA, Arnould I, Elicker BM, Henry TS, Vittinghoff E, Golden JA, Jones KD, Batra K, Torrealba J, Garcia CK, Wolters PJ (2017) The MUC5B promoter polymorphism and telomere length in patients with chronic hypersensitivity pneumonitis: an observational cohort-control study. Lancet Respir Med. 5(8):639–647PubMedPubMedCentral
47.
go back to reference Juge P-A, Lee JS, Ebstein E, Furukawa H, Dobrinskikh E, Gazal S et al (2018) MUC5B Promoter variant and rheumatoid arthritis with interstitial lung disease. N Engl J Med 379(23):2209–2219PubMedPubMedCentral Juge P-A, Lee JS, Ebstein E, Furukawa H, Dobrinskikh E, Gazal S et al (2018) MUC5B Promoter variant and rheumatoid arthritis with interstitial lung disease. N Engl J Med 379(23):2209–2219PubMedPubMedCentral
48.
go back to reference Peljto AL, Zhang Y, Fingerlin TE, Ma S-F, Garcia JGN, Richards TJ, Silveira LJ, Lindell KO, Steele MP, Loyd JE, Gibson KF, Seibold MA, Brown KK, Talbert JL, Markin C, Kossen K, Seiwert SD, Murphy E, Noth I, Schwarz MI, Kaminski N, Schwartz DA (2013) Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA. 309(21):2232–2239PubMedPubMedCentral Peljto AL, Zhang Y, Fingerlin TE, Ma S-F, Garcia JGN, Richards TJ, Silveira LJ, Lindell KO, Steele MP, Loyd JE, Gibson KF, Seibold MA, Brown KK, Talbert JL, Markin C, Kossen K, Seiwert SD, Murphy E, Noth I, Schwarz MI, Kaminski N, Schwartz DA (2013) Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA. 309(21):2232–2239PubMedPubMedCentral
49.
go back to reference Oldham JM, Ma S-F, Martinez FJ, Anstrom KJ, Raghu G, Schwartz DA, Valenzi E, Witt L, Lee C, Vij R, Huang Y, Strek ME, Noth I, IPFnet Investigators (2015) TOLLIP, MUC5B, and the response to N-acetylcysteine among individuals with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 192(12):1475–1482PubMedPubMedCentral Oldham JM, Ma S-F, Martinez FJ, Anstrom KJ, Raghu G, Schwartz DA, Valenzi E, Witt L, Lee C, Vij R, Huang Y, Strek ME, Noth I, IPFnet Investigators (2015) TOLLIP, MUC5B, and the response to N-acetylcysteine among individuals with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 192(12):1475–1482PubMedPubMedCentral
50.
go back to reference Armanios M, Alder JK, Parry EM, Karim B, Strong MA, Greider CW (2009) Short telomeres are sufficient to cause the degenerative defects associated with aging. Am J Hum Genet. 85(6):823–832PubMedPubMedCentral Armanios M, Alder JK, Parry EM, Karim B, Strong MA, Greider CW (2009) Short telomeres are sufficient to cause the degenerative defects associated with aging. Am J Hum Genet. 85(6):823–832PubMedPubMedCentral
51.
go back to reference Dressen A, Abbas AR, Cabanski C, Reeder J, Ramalingam TR, Neighbors M, Bhangale TR, Brauer MJ, Hunkapiller J, Reeder J, Mukhyala K, Cuenco K, Tom J, Cowgill A, Vogel J, Forrest WF, Collard HR, Wolters PJ, Kropski JA, Lancaster LH, Blackwell TS, Arron JR, Yaspan BL (2018) Analysis of protein-altering variants in telomerase genes and their association with MUC5B common variant status in patients with idiopathic pulmonary fibrosis: a candidate gene sequencing study. Lancet Respir Med. 6(8):603–614PubMedPubMedCentral Dressen A, Abbas AR, Cabanski C, Reeder J, Ramalingam TR, Neighbors M, Bhangale TR, Brauer MJ, Hunkapiller J, Reeder J, Mukhyala K, Cuenco K, Tom J, Cowgill A, Vogel J, Forrest WF, Collard HR, Wolters PJ, Kropski JA, Lancaster LH, Blackwell TS, Arron JR, Yaspan BL (2018) Analysis of protein-altering variants in telomerase genes and their association with MUC5B common variant status in patients with idiopathic pulmonary fibrosis: a candidate gene sequencing study. Lancet Respir Med. 6(8):603–614PubMedPubMedCentral
52.
go back to reference Swaminathan AC, Neely ML, Frankel CW, Kelly FL, Petrovski S, Durheim MT, Bush E, Snyder L, Goldstein DB, Todd JL, Palmer SM (2019) Lung transplant outcomes in patients with pulmonary fibrosis with telomere-related gene variants. Chest. 156(3):477–485PubMed Swaminathan AC, Neely ML, Frankel CW, Kelly FL, Petrovski S, Durheim MT, Bush E, Snyder L, Goldstein DB, Todd JL, Palmer SM (2019) Lung transplant outcomes in patients with pulmonary fibrosis with telomere-related gene variants. Chest. 156(3):477–485PubMed
53.
go back to reference Ley B, Torgerson DG, Oldham JM, Adegunsoye A, Liu S, Li J et al (2019) Rare protein-altering telomere-related gene variants in patients with chronic hypersensitivity pneumonitis. Am J Respir Crit Care Med 200(9):1154–1163PubMedPubMedCentral Ley B, Torgerson DG, Oldham JM, Adegunsoye A, Liu S, Li J et al (2019) Rare protein-altering telomere-related gene variants in patients with chronic hypersensitivity pneumonitis. Am J Respir Crit Care Med 200(9):1154–1163PubMedPubMedCentral
54.
go back to reference Juge P-A, Borie R, Kannengiesser C, Gazal S, Revy P, Wemeau-Stervinou L et al (2017) Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis. Eur Respir J 49(5):1602314 Juge P-A, Borie R, Kannengiesser C, Gazal S, Revy P, Wemeau-Stervinou L et al (2017) Shared genetic predisposition in rheumatoid arthritis-interstitial lung disease and familial pulmonary fibrosis. Eur Respir J 49(5):1602314
55.
go back to reference Yang IV, Burch LH, Steele MP, Savov JD, Hollingsworth JW, McElvania-Tekippe E, Berman KG, Speer MC, Sporn TA, Brown KK, Schwarz MI, Schwartz DA (2007) Gene expression profiling of familial and sporadic interstitial pneumonia. Am J Respir Crit Care Med. 175(1):45–54PubMed Yang IV, Burch LH, Steele MP, Savov JD, Hollingsworth JW, McElvania-Tekippe E, Berman KG, Speer MC, Sporn TA, Brown KK, Schwarz MI, Schwartz DA (2007) Gene expression profiling of familial and sporadic interstitial pneumonia. Am J Respir Crit Care Med. 175(1):45–54PubMed
56.
go back to reference Kim SY, Diggans J, Pankratz D, Huang J, Pagan M, Sindy N, Tom E, Anderson J, Choi Y, Lynch DA, Steele MP, Flaherty KR, Brown KK, Farah H, Bukstein MJ, Pardo A, Selman M, Wolters PJ, Nathan SD, Colby TV, Myers JL, Katzenstein ALA, Raghu G, Kennedy GC (2015) Classification of usual interstitial pneumonia in patients with interstitial lung disease: assessment of a machine learning approach using high-dimensional transcriptional data. Lancet Respir Med. 3(6):473–482PubMed Kim SY, Diggans J, Pankratz D, Huang J, Pagan M, Sindy N, Tom E, Anderson J, Choi Y, Lynch DA, Steele MP, Flaherty KR, Brown KK, Farah H, Bukstein MJ, Pardo A, Selman M, Wolters PJ, Nathan SD, Colby TV, Myers JL, Katzenstein ALA, Raghu G, Kennedy GC (2015) Classification of usual interstitial pneumonia in patients with interstitial lung disease: assessment of a machine learning approach using high-dimensional transcriptional data. Lancet Respir Med. 3(6):473–482PubMed
57.
go back to reference Sivakumar P, Thompson JR, Ammar R, Porteous M, McCoubrey C, Cantu E et al (2019) RNA sequencing of transplant-stage idiopathic pulmonary fibrosis lung reveals unique pathway regulation. ERJ Open Res 5(3):00117–2019 Sivakumar P, Thompson JR, Ammar R, Porteous M, McCoubrey C, Cantu E et al (2019) RNA sequencing of transplant-stage idiopathic pulmonary fibrosis lung reveals unique pathway regulation. ERJ Open Res 5(3):00117–2019
58.
go back to reference McDonough JE, Ahangari F, Li Q, Jain S, Verleden SE, Herazo-Maya J et al (2019) Transcriptional regulatory model of fibrosis progression in the human lung. JCI Insight. 14:4(22) McDonough JE, Ahangari F, Li Q, Jain S, Verleden SE, Herazo-Maya J et al (2019) Transcriptional regulatory model of fibrosis progression in the human lung. JCI Insight. 14:4(22)
59.
go back to reference Yang IV, Coldren CD, Leach SM, Seibold MA, Murphy E, Lin J, Rosen R, Neidermyer AJ, McKean DF, Groshong SD, Cool C, Cosgrove GP, Lynch DA, Brown KK, Schwarz MI, Fingerlin TE, Schwartz DA (2013) Expression of cilium-associated genes defines novel molecular subtypes of idiopathic pulmonary fibrosis. Thorax. 68(12):1114–1121PubMedPubMedCentral Yang IV, Coldren CD, Leach SM, Seibold MA, Murphy E, Lin J, Rosen R, Neidermyer AJ, McKean DF, Groshong SD, Cool C, Cosgrove GP, Lynch DA, Brown KK, Schwarz MI, Fingerlin TE, Schwartz DA (2013) Expression of cilium-associated genes defines novel molecular subtypes of idiopathic pulmonary fibrosis. Thorax. 68(12):1114–1121PubMedPubMedCentral
60.
go back to reference Patel NM, Kawut SM, Jelic S, Arcasoy SM, Lederer DJ, Borczuk AC (2013) Pulmonary arteriole gene expression signature in idiopathic pulmonary fibrosis. Eur Respir J. 41(6):1324–1330PubMedPubMedCentral Patel NM, Kawut SM, Jelic S, Arcasoy SM, Lederer DJ, Borczuk AC (2013) Pulmonary arteriole gene expression signature in idiopathic pulmonary fibrosis. Eur Respir J. 41(6):1324–1330PubMedPubMedCentral
61.
go back to reference Ackermann M, Stark H, Neubert L, Schubert S, Borchert P, Linz F et al (2020) Morphomolecular motifs of pulmonary neoangiogenesis in interstitial lung diseases. Eur Respir J 55(3):1900933 Ackermann M, Stark H, Neubert L, Schubert S, Borchert P, Linz F et al (2020) Morphomolecular motifs of pulmonary neoangiogenesis in interstitial lung diseases. Eur Respir J 55(3):1900933
62.
go back to reference Vukmirovic M, Kaminski N (2018) Impact of transcriptomics on our understanding of pulmonary fibrosis. Front Med. 5:87 Vukmirovic M, Kaminski N (2018) Impact of transcriptomics on our understanding of pulmonary fibrosis. Front Med. 5:87
63.
go back to reference Morse C, Tabib T, Sembrat J, Buschur KL, Bittar HT, Valenzi E et al (2019) Proliferating SPP1/MERTK-expressing macrophages in idiopathic pulmonary fibrosis. Eur Respir J 54(2):1802441 Morse C, Tabib T, Sembrat J, Buschur KL, Bittar HT, Valenzi E et al (2019) Proliferating SPP1/MERTK-expressing macrophages in idiopathic pulmonary fibrosis. Eur Respir J 54(2):1802441
64.
go back to reference Selman M, Carrillo G, Estrada A, Mejia M, Becerril C, Cisneros J, Gaxiola M, Pérez-Padilla R, Navarro C, Richards T, Dauber J, King TE, Pardo A, Kaminski N (2007) Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PloS One. 2(5):e482PubMedPubMedCentral Selman M, Carrillo G, Estrada A, Mejia M, Becerril C, Cisneros J, Gaxiola M, Pérez-Padilla R, Navarro C, Richards T, Dauber J, King TE, Pardo A, Kaminski N (2007) Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PloS One. 2(5):e482PubMedPubMedCentral
65.
go back to reference Tomassetti S, Ravaglia C, Wells AU, Cavazza A, Colby TV, Rossi G et al (2020) Prognostic value of transbronchial lung cryobiopsy for the multidisciplinary diagnosis of idiopathic pulmonary fibrosis: a retrospective validation study. Lancet Respir Med. 8(8):786–794PubMed Tomassetti S, Ravaglia C, Wells AU, Cavazza A, Colby TV, Rossi G et al (2020) Prognostic value of transbronchial lung cryobiopsy for the multidisciplinary diagnosis of idiopathic pulmonary fibrosis: a retrospective validation study. Lancet Respir Med. 8(8):786–794PubMed
66.
go back to reference Courtwright AM, El-Chemaly S (2019) Telomeres in interstitial lung disease: the short and the long of it. Ann Am Thorac Soc. 16(2):175–181PubMedPubMedCentral Courtwright AM, El-Chemaly S (2019) Telomeres in interstitial lung disease: the short and the long of it. Ann Am Thorac Soc. 16(2):175–181PubMedPubMedCentral
67.
go back to reference Wiertz IA, Moll SA, Seeliger B, Barlo NP, van der Vis JJ, Korthagen NM et al (2020) Genetic variation in CCL18 gene influences CCL18 expression and correlates with survival in idiopathic pulmonary fibrosis: Part A. J Clin Med. 21:9(6) Wiertz IA, Moll SA, Seeliger B, Barlo NP, van der Vis JJ, Korthagen NM et al (2020) Genetic variation in CCL18 gene influences CCL18 expression and correlates with survival in idiopathic pulmonary fibrosis: Part A. J Clin Med. 21:9(6)
68.
go back to reference Raghu G, Richeldi L, Jagerschmidt A, Martin V, Subramaniam A, Ozoux M-L et al (2018) Idiopathic pulmonary fibrosis: prospective, case-controlled study of natural history and circulating biomarkers. Chest. 154(6):1359–1370PubMed Raghu G, Richeldi L, Jagerschmidt A, Martin V, Subramaniam A, Ozoux M-L et al (2018) Idiopathic pulmonary fibrosis: prospective, case-controlled study of natural history and circulating biomarkers. Chest. 154(6):1359–1370PubMed
69.
go back to reference Todd JL, Neely ML, Overton R, Durham K, Gulati M, Huang H et al (2019) Peripheral blood proteomic profiling of idiopathic pulmonary fibrosis biomarkers in the multicentre IPF-PRO Registry. Respir Res. 20(1):227PubMedPubMedCentral Todd JL, Neely ML, Overton R, Durham K, Gulati M, Huang H et al (2019) Peripheral blood proteomic profiling of idiopathic pulmonary fibrosis biomarkers in the multicentre IPF-PRO Registry. Respir Res. 20(1):227PubMedPubMedCentral
70.
go back to reference Meyer KC, Raghu G (2011) Bronchoalveolar lavage for the evaluation of interstitial lung disease: is it clinically useful? Eur Respir J. 38(4):761–769PubMed Meyer KC, Raghu G (2011) Bronchoalveolar lavage for the evaluation of interstitial lung disease: is it clinically useful? Eur Respir J. 38(4):761–769PubMed
71.
go back to reference Adderley N, Humphreys CJ, Barnes H, Ley B, Premji ZA, Johannson KA (2020) Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis. Eur Respir J 56(2):2000206 Adderley N, Humphreys CJ, Barnes H, Ley B, Premji ZA, Johannson KA (2020) Bronchoalveolar lavage fluid lymphocytosis in chronic hypersensitivity pneumonitis: a systematic review and meta-analysis. Eur Respir J 56(2):2000206
72.
go back to reference Molyneaux PL, Cox MJ, Willis-Owen SAG, Mallia P, Russell KE, Russell A-M, Murphy E, Johnston SL, Schwartz DA, Wells AU, Cookson WO, Maher TM, Moffatt MF (2014) The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 190(8):906–913PubMedPubMedCentral Molyneaux PL, Cox MJ, Willis-Owen SAG, Mallia P, Russell KE, Russell A-M, Murphy E, Johnston SL, Schwartz DA, Wells AU, Cookson WO, Maher TM, Moffatt MF (2014) The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 190(8):906–913PubMedPubMedCentral
73.
go back to reference Kitsios GD, Rojas M, Kass DJ, Fitch A, Sembrat JC, Qin S et al (2018) Microbiome in lung explants of idiopathic pulmonary fibrosis: a case-control study in patients with end-stage fibrosis. Thorax. 73(5):481–484PubMed Kitsios GD, Rojas M, Kass DJ, Fitch A, Sembrat JC, Qin S et al (2018) Microbiome in lung explants of idiopathic pulmonary fibrosis: a case-control study in patients with end-stage fibrosis. Thorax. 73(5):481–484PubMed
74.
go back to reference Becker A, Vella G, Galata V, Rentz K, Beisswenger C, Herr C et al (2019) The composition of the pulmonary microbiota in sarcoidosis - an observational study. Respir Res. 20(1):46PubMedPubMedCentral Becker A, Vella G, Galata V, Rentz K, Beisswenger C, Herr C et al (2019) The composition of the pulmonary microbiota in sarcoidosis - an observational study. Respir Res. 20(1):46PubMedPubMedCentral
75.
go back to reference Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ (2018) What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 51(5):1800692PubMed Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ (2018) What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 51(5):1800692PubMed
76.
go back to reference De Sadeleer LJ, Goos T, Yserbyt J, Wuyts WA (2020) Towards the essence of progressiveness: bringing progressive fibrosing interstitial lung disease (PF-ILD) to the next stage. J Clin Med. 9(6):1722PubMedCentral De Sadeleer LJ, Goos T, Yserbyt J, Wuyts WA (2020) Towards the essence of progressiveness: bringing progressive fibrosing interstitial lung disease (PF-ILD) to the next stage. J Clin Med. 9(6):1722PubMedCentral
Metadata
Title
Molecular approach to the classification of chronic fibrosing lung disease—there and back again
Authors
Stijn E. Verleden
Peter Braubach
Mark Kuehnel
Nicolas Dickgreber
Emily Brouwer
Pauline Tittmann
Florian Laenger
Danny Jonigk
Publication date
01-01-2021
Publisher
Springer Berlin Heidelberg
Published in
Virchows Archiv / Issue 1/2021
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI
https://doi.org/10.1007/s00428-020-02964-9

Other articles of this Issue 1/2021

Virchows Archiv 1/2021 Go to the issue