Skip to main content
Top
Published in: Respiratory Research 1/2020

01-12-2020 | Idiopathic Pulmonary Fibrosis | Review

Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme

Authors: Claudia Valenzuela, Sebastiano Emanuele Torrisi, Nicolas Kahn, Manuel Quaresma, Susanne Stowasser, Michael Kreuter

Published in: Respiratory Research | Issue 1/2020

Login to get access

Abstract

The approvals of nintedanib and pirfenidone changed the treatment paradigm in idiopathic pulmonary fibrosis (IPF), and increased our understanding of the underlying disease mechanisms. Nonetheless, many challenges and unmet needs remain in the management of patients with IPF and other progressive fibrosing interstitial lung diseases.
This review describes how the nintedanib clinical programme has helped to address some of these challenges. Data from this programme have informed changes to the IPF diagnostic guidelines, the timing of treatment initiation, and the assessment of disease progression. The use of nintedanib to treat patients with advanced lung function impairment, concomitant emphysema, patients awaiting lung transplantation and patients with IPF and lung cancer is discussed. The long-term use of nintedanib and an up-to-date summary of nintedanib in clinical practice are discussed. Directions for future research, namely emerging therapeutic options, precision medicine and other progressive fibrosing interstitial lung diseases, are described.
Further developments in these areas should continue to improve patient outcomes.
Literature
2.
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. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–82.PubMedCrossRef 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. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–82.PubMedCrossRef
3.
go back to reference Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, King TE Jr, Lancaster L, Sahn SA, Szwarcberg J, Valeyre D, du Bois RM. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. The Lancet. 2011;377:1760–9.CrossRef Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, King TE Jr, Lancaster L, Sahn SA, Szwarcberg J, Valeyre D, du Bois RM. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. The Lancet. 2011;377:1760–9.CrossRef
4.
go back to reference King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083–92.PubMedCrossRef King TE, Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, Gorina E, Hopkins PM, Kardatzke D, Lancaster L, Lederer DJ, Nathan SD, Pereira CA, Sahn SA, Sussman R, Swigris JJ, Noble PW. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083–92.PubMedCrossRef
5.
go back to reference Rivera-Ortega P, Hayton C, Blaikley J, Leonard C, Chaudhuri N. Nintedanib in the management of idiopathic pulmonary fibrosis: clinical trial evidence and real-world experience. Ther Adv Respir Dis. 2018;12:1753466618800618.PubMedPubMedCentralCrossRef Rivera-Ortega P, Hayton C, Blaikley J, Leonard C, Chaudhuri N. Nintedanib in the management of idiopathic pulmonary fibrosis: clinical trial evidence and real-world experience. Ther Adv Respir Dis. 2018;12:1753466618800618.PubMedPubMedCentralCrossRef
6.
go back to reference Crestani B, Huggins JT, Kaye M, Costabel U, Glaspole I, Ogura T, Song JW, Stansen W, Quaresma M, Stowasser S, Kreuter M. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2019;7:60–8.PubMedCrossRef Crestani B, Huggins JT, Kaye M, Costabel U, Glaspole I, Ogura T, Song JW, Stansen W, Quaresma M, Stowasser S, Kreuter M. Long-term safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis: results from the open-label extension study, INPULSIS-ON. Lancet Respir Med. 2019;7:60–8.PubMedCrossRef
7.
go back to reference Goldin J, Kim GH, Trampisch M, Homik L, Hotchkin DL, Ilowite J, Kaye M, Mogulkoc N, Ryerson CJ, Lancaster L, Conoscenti CS. Effect of nintedanib on quantitative lung fibrosis score in patients with idiopathic pulmonary fibrosis (IPF). Pacific Grove: International Colloquium On Lung And Airway Fibrosis; 19 July 2018; 2018. Goldin J, Kim GH, Trampisch M, Homik L, Hotchkin DL, Ilowite J, Kaye M, Mogulkoc N, Ryerson CJ, Lancaster L, Conoscenti CS. Effect of nintedanib on quantitative lung fibrosis score in patients with idiopathic pulmonary fibrosis (IPF). Pacific Grove: International Colloquium On Lung And Airway Fibrosis; 19 July 2018; 2018.
8.
go back to reference Vancheri C, Kreuter M, Richeldi L, Ryerson CJ, Valeyre D, Grutters JC, Wiebe S, Stansen W, Quaresma M, Stowasser S, Wuyts WA. Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis. Results of the INJOURNEY trial. Am J Respir Crit Care Med. 2018;197:356–63.PubMedCrossRef Vancheri C, Kreuter M, Richeldi L, Ryerson CJ, Valeyre D, Grutters JC, Wiebe S, Stansen W, Quaresma M, Stowasser S, Wuyts WA. Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis. Results of the INJOURNEY trial. Am J Respir Crit Care Med. 2018;197:356–63.PubMedCrossRef
9.
go back to reference Distler O, Brown KK, Distler JHW, Assassi S, Maher TM, Cottin V, Varga J, Coeck C, Gahlemann M, Sauter W, Schmidt H, Highland KB. Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS). Clin Exp Rheumatol. 2017;35(Suppl 106):75–81.PubMed Distler O, Brown KK, Distler JHW, Assassi S, Maher TM, Cottin V, Varga J, Coeck C, Gahlemann M, Sauter W, Schmidt H, Highland KB. Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS). Clin Exp Rheumatol. 2017;35(Suppl 106):75–81.PubMed
10.
go back to reference Richeldi L, Fletcher S, Adamali H, Chaudhuri N, Wiebe S, Wind S, Hohl K, Baker A, Schlenker-Herceg R, Stowasser S, Maher TM. No relevant pharmacokinetic drug–drug interaction between nintedanib and pirfenidone. Eur Respir J. 2019;53:1801060.PubMedCrossRef Richeldi L, Fletcher S, Adamali H, Chaudhuri N, Wiebe S, Wind S, Hohl K, Baker A, Schlenker-Herceg R, Stowasser S, Maher TM. No relevant pharmacokinetic drug–drug interaction between nintedanib and pirfenidone. Eur Respir J. 2019;53:1801060.PubMedCrossRef
11.
go back to reference Kolb M, Raghu G, Wells AU, Behr J, Richeldi L, Schinzel B, Quaresma M, Stowasser S, Martinez FJ. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2018;379:1722–31.PubMedCrossRef Kolb M, Raghu G, Wells AU, Behr J, Richeldi L, Schinzel B, Quaresma M, Stowasser S, Martinez FJ. Nintedanib plus sildenafil in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2018;379:1722–31.PubMedCrossRef
12.
go back to reference Maher TM, Stowasser S, Nishioka Y, White ES, Cottin V, Noth I, Selman M, Blahova Z, Wachtlin D, Diefenbach C, Jenkins RG. Investigating the effects of nintedanib on biomarkers of extracellular matrix turnover in patients with IPF: design of the randomised placebo-controlled INMARK® trial. BMJ Open Respir Res. 2018;5:e000325.PubMedPubMedCentralCrossRef Maher TM, Stowasser S, Nishioka Y, White ES, Cottin V, Noth I, Selman M, Blahova Z, Wachtlin D, Diefenbach C, Jenkins RG. Investigating the effects of nintedanib on biomarkers of extracellular matrix turnover in patients with IPF: design of the randomised placebo-controlled INMARK® trial. BMJ Open Respir Res. 2018;5:e000325.PubMedPubMedCentralCrossRef
13.
go back to reference Flaherty KR, Brown KK, Wells AU, Clerisme-Beaty E, Collard HR, Cottin V, Devaraj A, Inoue Y, Le Maulf F, Richeldi L, Schmidt H, Walsh S, Mezzanotte W, Schlenker-Herceg R. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4:e000212.PubMedPubMedCentralCrossRef Flaherty KR, Brown KK, Wells AU, Clerisme-Beaty E, Collard HR, Cottin V, Devaraj A, Inoue Y, Le Maulf F, Richeldi L, Schmidt H, Walsh S, Mezzanotte W, Schlenker-Herceg R. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017;4:e000212.PubMedPubMedCentralCrossRef
14.
go back to reference Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier J-F, 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, Talmadge E, King J, Kondoh Y, Myers J, Müller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schünemann HJ. An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.PubMedPubMedCentralCrossRef Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, Colby TV, Cordier J-F, 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, Talmadge E, King J, Kondoh Y, Myers J, Müller NL, Nicholson AG, Richeldi L, Selman M, Dudden RF, Griss BS, Protzko SL, Schünemann HJ. An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: Evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183:788–824.PubMedPubMedCentralCrossRef
15.
go back to reference Cottin V. Lung biopsy in interstitial lung disease: balancing the risk of surgery and diagnostic uncertainty. Eur Respir J. 2016;48:1274–7.PubMedCrossRef Cottin V. Lung biopsy in interstitial lung disease: balancing the risk of surgery and diagnostic uncertainty. Eur Respir J. 2016;48:1274–7.PubMedCrossRef
16.
go back to reference Hutchinson JP, Fogarty AW, McKeever TM, Hubbard RB. In-hospital mortality after surgical lung biopsy for interstitial lung disease in the United States. 2000 to 2011. Am J Respir Crit Care Med. 2016;193:1161–7.PubMedCrossRef Hutchinson JP, Fogarty AW, McKeever TM, Hubbard RB. In-hospital mortality after surgical lung biopsy for interstitial lung disease in the United States. 2000 to 2011. Am J Respir Crit Care Med. 2016;193:1161–7.PubMedCrossRef
17.
go back to reference Hutchinson JP, McKeever TM, Fogarty AW, Navaratnam V, Hubbard RB. Surgical lung biopsy for the diagnosis of interstitial lung disease in England: 1997–2008. Eur Respir J. 2016;48:1453–61.PubMedCrossRef Hutchinson JP, McKeever TM, Fogarty AW, Navaratnam V, Hubbard RB. Surgical lung biopsy for the diagnosis of interstitial lung disease in England: 1997–2008. Eur Respir J. 2016;48:1453–61.PubMedCrossRef
18.
go back to reference Raj R, Raparia K, Lynch DA, Brown KK. Surgical lung biopsy for interstitial lung diseases. Chest. 2017;151:1131–40.PubMedCrossRef Raj R, Raparia K, Lynch DA, Brown KK. Surgical lung biopsy for interstitial lung diseases. Chest. 2017;151:1131–40.PubMedCrossRef
19.
go back to reference Jo HE, Glaspole I, Goh N, Hopkins PMA, Moodley Y, Reynolds PN, Chapman S, Walters EH, Zappala C, Allan H, Macansh S, Grainge C, Keir GJ, Hayen A, Henderson D, Klebe S, Heinze SB, Miller A, Rouse HC, Duhig E, Cooper WA, Mahar AM, Ellis S, McCormack SR, Ng B, Godbolt DB, Corte TJ. Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Respirology. 2019;24:361–8.PubMedCrossRef Jo HE, Glaspole I, Goh N, Hopkins PMA, Moodley Y, Reynolds PN, Chapman S, Walters EH, Zappala C, Allan H, Macansh S, Grainge C, Keir GJ, Hayen A, Henderson D, Klebe S, Heinze SB, Miller A, Rouse HC, Duhig E, Cooper WA, Mahar AM, Ellis S, McCormack SR, Ng B, Godbolt DB, Corte TJ. Implications of the diagnostic criteria of idiopathic pulmonary fibrosis in clinical practice: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Respirology. 2019;24:361–8.PubMedCrossRef
20.
go back to reference Mortimer K, Hartmann N, Chan C, Norman H, Wallace L, Enger C. Characterizing idiopathic pulmonary fibrosis patients using US Medicare-advantage health plan claims data. BMC Pulm Med. 2019;19:11.PubMedPubMedCentralCrossRef Mortimer K, Hartmann N, Chan C, Norman H, Wallace L, Enger C. Characterizing idiopathic pulmonary fibrosis patients using US Medicare-advantage health plan claims data. BMC Pulm Med. 2019;19:11.PubMedPubMedCentralCrossRef
21.
go back to reference Fernández-Fabrellas E, Molina-Molina M, Soriano JB, Portal JAR, Ancochea J, Valenzuela C, Xaubet A. Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry. Respir Res. 2019;20:127.PubMedPubMedCentralCrossRef Fernández-Fabrellas E, Molina-Molina M, Soriano JB, Portal JAR, Ancochea J, Valenzuela C, Xaubet A. Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry. Respir Res. 2019;20:127.PubMedPubMedCentralCrossRef
22.
go back to reference Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D, Andreas S, Claussen M, Grohé C, Wilkens H, Randerath W, Skowasch D, Meyer FJ, Kirschner J, Gläser S, Herth FJF, Welte T, Huber RM, Neurohr C, Schwaiblmair M, Kohlhäufl M, Höffken G, Held M, Koch A, Bahmer T, Pittrow D. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46:186–96.PubMedPubMedCentralCrossRef Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D, Andreas S, Claussen M, Grohé C, Wilkens H, Randerath W, Skowasch D, Meyer FJ, Kirschner J, Gläser S, Herth FJF, Welte T, Huber RM, Neurohr C, Schwaiblmair M, Kohlhäufl M, Höffken G, Held M, Koch A, Bahmer T, Pittrow D. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46:186–96.PubMedPubMedCentralCrossRef
23.
go back to reference Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, Maurer O, Heinemann S, Costabel U, Barbero MAN, Müller V, Bonniaud P, Vancheri C, Wells A, Vasakova M, Pesci A, Sofia M, Klepetko W, Seeger W, Drakopanagiotakis F, Crestani B. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19:141.PubMedPubMedCentralCrossRef Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, Maurer O, Heinemann S, Costabel U, Barbero MAN, Müller V, Bonniaud P, Vancheri C, Wells A, Vasakova M, Pesci A, Sofia M, Klepetko W, Seeger W, Drakopanagiotakis F, Crestani B. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19:141.PubMedPubMedCentralCrossRef
24.
go back to reference Chung JH, Chawla A, Peljto AL, Cool CD, Groshong SD, Talbert JL, McKean DF, Brown KK, Fingerlin TE, Schwarz MI, Schwartz DA, Lynch DA. CT scan findings of probable usual interstitial pneumonitis have a high predictive value for histologic usual interstitial pneumonitis. Chest. 2015;147:450–9.PubMedCrossRef Chung JH, Chawla A, Peljto AL, Cool CD, Groshong SD, Talbert JL, McKean DF, Brown KK, Fingerlin TE, Schwarz MI, Schwartz DA, Lynch DA. CT scan findings of probable usual interstitial pneumonitis have a high predictive value for histologic usual interstitial pneumonitis. Chest. 2015;147:450–9.PubMedCrossRef
25.
go back to reference Raghu G, Wells AU, Nicholson AG, Richeldi L, Flaherty KR, Maulf FL, Stowasser S, Schlenker-Herceg R, Hansell DM. Effect of nintedanib in subgroups of idiopathic pulmonary fibrosis by diagnostic criteria. Am J Respir Crit Care Med. 2017;195:78–85.PubMedPubMedCentralCrossRef Raghu G, Wells AU, Nicholson AG, Richeldi L, Flaherty KR, Maulf FL, Stowasser S, Schlenker-Herceg R, Hansell DM. Effect of nintedanib in subgroups of idiopathic pulmonary fibrosis by diagnostic criteria. Am J Respir Crit Care Med. 2017;195:78–85.PubMedPubMedCentralCrossRef
26.
go back to reference Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV, Galvin JR, Goldin JG, Hansell DM, Inoue Y, Johkoh T, Nicholson AG, Knight SL, Raoof S, Richeldi L, Ryerson CJ, Ryu JH, Wells AU. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6:138–53.PubMedCrossRef Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV, Galvin JR, Goldin JG, Hansell DM, Inoue Y, Johkoh T, Nicholson AG, Knight SL, Raoof S, Richeldi L, Ryerson CJ, Ryu JH, Wells AU. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6:138–53.PubMedCrossRef
27.
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, SNJ P, Buendía-Roldán I, Selman M, Travis WD, SLF W, Wilson KC. Diagnosis of Idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198:e44–68.PubMedCrossRef 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, SNJ P, Buendía-Roldán I, Selman M, Travis WD, SLF W, Wilson KC. Diagnosis of Idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198:e44–68.PubMedCrossRef
28.
go back to reference Raghu G, Remy-Jardin M, Myers J, Richeldi L, Wilson KC. The 2018 diagnosis of IPF guidelines: Surgical lung biopsy in probable UIP is not mandatory. Am J Respir Crit Care Med. 2019;200:1089–92.PubMedPubMedCentralCrossRef Raghu G, Remy-Jardin M, Myers J, Richeldi L, Wilson KC. The 2018 diagnosis of IPF guidelines: Surgical lung biopsy in probable UIP is not mandatory. Am J Respir Crit Care Med. 2019;200:1089–92.PubMedPubMedCentralCrossRef
29.
go back to reference Hetzel J, Maldonado F, Ravaglia C, Wells AU, Colby TV, Tomassetti S, Ryu JH, Fruchter O, Piciucchi S, Dubini A, Cavazza A, Chilosi M, Sverzellati N, Valeyre D, Leduc D, SLF W, Gasparini S, Hetzel M, Hagmeyer L, Haentschel M, Eberhardt R, Darwiche K, Yarmus LB, Torrego A, Krishna G, Shah PL, Annema JT, FJF H, Poletti V. Transbronchial cryobiopsies for the diagnosis of diffuse parenchymal lung diseases: expert statement from the cryobiopsy working group on safety and utility and a call for standardization of the procedure. Respiration. 2018;95:188–200.PubMedCrossRef Hetzel J, Maldonado F, Ravaglia C, Wells AU, Colby TV, Tomassetti S, Ryu JH, Fruchter O, Piciucchi S, Dubini A, Cavazza A, Chilosi M, Sverzellati N, Valeyre D, Leduc D, SLF W, Gasparini S, Hetzel M, Hagmeyer L, Haentschel M, Eberhardt R, Darwiche K, Yarmus LB, Torrego A, Krishna G, Shah PL, Annema JT, FJF H, Poletti V. Transbronchial cryobiopsies for the diagnosis of diffuse parenchymal lung diseases: expert statement from the cryobiopsy working group on safety and utility and a call for standardization of the procedure. Respiration. 2018;95:188–200.PubMedCrossRef
30.
go back to reference Tomassetti S, Wells AU, Costabel U, Cavazza A, Colby TV, Rossi G, Sverzellati N, Carloni A, Carretta E, Buccioli M, Tantalocco P, Ravaglia C, Gurioli C, Dubini A, Piciucchi S, Ryu JH, Poletti V. Bronchoscopic lung cryobiopsy increases diagnostic confidence in the multidisciplinary diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;193:745–52.PubMedCrossRef Tomassetti S, Wells AU, Costabel U, Cavazza A, Colby TV, Rossi G, Sverzellati N, Carloni A, Carretta E, Buccioli M, Tantalocco P, Ravaglia C, Gurioli C, Dubini A, Piciucchi S, Ryu JH, Poletti V. Bronchoscopic lung cryobiopsy increases diagnostic confidence in the multidisciplinary diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2016;193:745–52.PubMedCrossRef
31.
go back to reference Wälscher J, Groß B, Eberhardt R, Heussel CP, Eichinger M, Warth A, Lasitschka F, Herth FJF, Kreuter M. Transbronchial cryobiopsies for diagnosing interstitial lung disease: real-life experience from a tertiary referral center for interstitial lung disease. Respiration. 2019;97:348–54.PubMedCrossRef Wälscher J, Groß B, Eberhardt R, Heussel CP, Eichinger M, Warth A, Lasitschka F, Herth FJF, Kreuter M. Transbronchial cryobiopsies for diagnosing interstitial lung disease: real-life experience from a tertiary referral center for interstitial lung disease. Respiration. 2019;97:348–54.PubMedCrossRef
32.
go back to reference Troy LK, Grainge C, Corte TJ, Williamson JP, Vallely MP, Cooper WA, Mahar A, Myers JL, Lai S, Mulyadi E, Torzillo PJ, Phillips MJ, Jo HE, Webster SE, Lin QT, Rhodes JE, Salamonsen M, Wrobel JP, Harris B, Don G, PJC W, Ng BJ, Oldmeadow C, Raghu G, EMT L, Arnold D, Cao C, Cashmore A, Cleary S, Evans T-J, French B, Geis M, Glenn L, Hibbert M, Ing A, James A, Meredith G, Merry C, Pudipeddi A, Saghaie T, Thomas R, Thomson C, Twaddell S. Diagnostic accuracy of transbronchial lung cryobiopsy for interstitial lung disease diagnosis (COLDICE): a prospective, comparative study. Lancet Respir Med. 2019. Troy LK, Grainge C, Corte TJ, Williamson JP, Vallely MP, Cooper WA, Mahar A, Myers JL, Lai S, Mulyadi E, Torzillo PJ, Phillips MJ, Jo HE, Webster SE, Lin QT, Rhodes JE, Salamonsen M, Wrobel JP, Harris B, Don G, PJC W, Ng BJ, Oldmeadow C, Raghu G, EMT L, Arnold D, Cao C, Cashmore A, Cleary S, Evans T-J, French B, Geis M, Glenn L, Hibbert M, Ing A, James A, Meredith G, Merry C, Pudipeddi A, Saghaie T, Thomas R, Thomson C, Twaddell S. Diagnostic accuracy of transbronchial lung cryobiopsy for interstitial lung disease diagnosis (COLDICE): a prospective, comparative study. Lancet Respir Med. 2019.
33.
go back to reference Troy LK, Grainge C, Corte T, Williamson JP, Vallely MP, Cooper W, Mahar AM, Lai S, Mulyadi E, Torzillo PJ, Salamonsen M, Don G, Myers J, Raghu G, Lau EMT. Cryobiopsy versus open lung biopsy in the diagnosis of interstitial lung disease (COLDICE): protocol of a multicentre study. BMJ Open Respir Res. 2019;6:e000443.PubMedPubMedCentral Troy LK, Grainge C, Corte T, Williamson JP, Vallely MP, Cooper W, Mahar AM, Lai S, Mulyadi E, Torzillo PJ, Salamonsen M, Don G, Myers J, Raghu G, Lau EMT. Cryobiopsy versus open lung biopsy in the diagnosis of interstitial lung disease (COLDICE): protocol of a multicentre study. BMJ Open Respir Res. 2019;6:e000443.PubMedPubMedCentral
34.
go back to reference Romagnoli M, Colby TV, Berthet J-P, Gamez AS, Mallet J-P, Serre I, Cancellieri A, Cavazza A, Solovei L, Dell’Amore A, Dolci G, Guerrieri A, Reynaud P, Bommart S, Zompatori M, Dalpiaz G, Nava S, Trisolini R, Suehs CM, Vachier I, Molinari N, Bourdin A. Poor concordance between sequential transbronchial lung cryobiopsy and surgical lung biopsy in the diagnosis of diffuse interstitial lung diseases. Am J Respir Crit Care Med. 2019;199:1249–56.PubMedCrossRef Romagnoli M, Colby TV, Berthet J-P, Gamez AS, Mallet J-P, Serre I, Cancellieri A, Cavazza A, Solovei L, Dell’Amore A, Dolci G, Guerrieri A, Reynaud P, Bommart S, Zompatori M, Dalpiaz G, Nava S, Trisolini R, Suehs CM, Vachier I, Molinari N, Bourdin A. Poor concordance between sequential transbronchial lung cryobiopsy and surgical lung biopsy in the diagnosis of diffuse interstitial lung diseases. Am J Respir Crit Care Med. 2019;199:1249–56.PubMedCrossRef
35.
go back to reference Raghu G, Rochwerg B, Zhang Y, Garcia CAC, Azuma A, Behr J, Brozek JL, Collard HR, Cunningham W, Homma S, Johkoh T, Martinez FJ, Myers J, Protzko SL, Richeldi L, Rind D, Selman M, Theodore A, Wells AU, Hoogsteden H, Schünemann HJ. An official ATS/ERS/JRS/ALAT clinical practice guideline: Treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192:e3–e19.PubMedCrossRef Raghu G, Rochwerg B, Zhang Y, Garcia CAC, Azuma A, Behr J, Brozek JL, Collard HR, Cunningham W, Homma S, Johkoh T, Martinez FJ, Myers J, Protzko SL, Richeldi L, Rind D, Selman M, Theodore A, Wells AU, Hoogsteden H, Schünemann HJ. An official ATS/ERS/JRS/ALAT clinical practice guideline: Treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192:e3–e19.PubMedCrossRef
36.
go back to reference Maher TM, Molina-Molina M, Russell A-M, Bonella F, Jouneau S, Ripamonti E, Axmann J, Vancheri C. Unmet needs in the treatment of idiopathic pulmonary fibrosis―insights from patient chart review in five European countries. BMC Pulm Med. 2017;17:124.PubMedPubMedCentralCrossRef Maher TM, Molina-Molina M, Russell A-M, Bonella F, Jouneau S, Ripamonti E, Axmann J, Vancheri C. Unmet needs in the treatment of idiopathic pulmonary fibrosis―insights from patient chart review in five European countries. BMC Pulm Med. 2017;17:124.PubMedPubMedCentralCrossRef
37.
go back to reference Pesonen I, Carlson L, Murgia N, Kaarteenaho R, Sköld CM, Myllärniemi M, Ferrara G. Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries. Multidiscip Respir Med. 2018;13:14.PubMedPubMedCentralCrossRef Pesonen I, Carlson L, Murgia N, Kaarteenaho R, Sköld CM, Myllärniemi M, Ferrara G. Delay and inequalities in the treatment of idiopathic pulmonary fibrosis: the case of two Nordic countries. Multidiscip Respir Med. 2018;13:14.PubMedPubMedCentralCrossRef
38.
go back to reference Maher TM, Swigris JJ, Kreuter M, Wijsenbeek M, Cassidy N, Ireland L, Axmann J, Nathan SD. Identifying barriers to idiopathic pulmonary fibrosis treatment: A survey of patient and physician views. Respiration. 2018;96:514–24.PubMedCrossRef Maher TM, Swigris JJ, Kreuter M, Wijsenbeek M, Cassidy N, Ireland L, Axmann J, Nathan SD. Identifying barriers to idiopathic pulmonary fibrosis treatment: A survey of patient and physician views. Respiration. 2018;96:514–24.PubMedCrossRef
39.
go back to reference Fletcher SV, Jones MG, Renzoni EA, Parfrey H, Hoyles RK, Spinks K, Kokosi M, Kwok A, Warburton C, Titmuss V, Thillai M, Simler N, Maher TM, Brereton CJ, Chua F, Wells AU, Richeldi L, Spencer LG. Safety and tolerability of nintedanib for the treatment of idiopathic pulmonary fibrosis in routine UK clinical practice. ERJ Open Res. 2018;4:00049–2018.PubMedPubMedCentralCrossRef Fletcher SV, Jones MG, Renzoni EA, Parfrey H, Hoyles RK, Spinks K, Kokosi M, Kwok A, Warburton C, Titmuss V, Thillai M, Simler N, Maher TM, Brereton CJ, Chua F, Wells AU, Richeldi L, Spencer LG. Safety and tolerability of nintedanib for the treatment of idiopathic pulmonary fibrosis in routine UK clinical practice. ERJ Open Res. 2018;4:00049–2018.PubMedPubMedCentralCrossRef
40.
go back to reference Costabel U, Inoue Y, Richeldi L, Collard HR, Tschoepe I, Stowasser S, Azuma A. Efficacy of nintedanib in idiopathic pulmonary fibrosis across prespecified subgroups in INPULSIS. Am J Respir Crit Care Med. 2016;193:178–85.PubMedCrossRef Costabel U, Inoue Y, Richeldi L, Collard HR, Tschoepe I, Stowasser S, Azuma A. Efficacy of nintedanib in idiopathic pulmonary fibrosis across prespecified subgroups in INPULSIS. Am J Respir Crit Care Med. 2016;193:178–85.PubMedCrossRef
41.
go back to reference Brown KK, Flaherty KR, Cottin V, Raghu G, Inoue Y, Azuma A, Huggins JT, Richeldi L, Stowasser S, Stansen W, Schlenker-Herceg R, Maher TM, Wells AU. Lung function outcomes in the INPULSIS trials of nintedanib in idiopathic pulmonary fibrosis. Respir Med. 2019;146:42–8.PubMedCrossRef Brown KK, Flaherty KR, Cottin V, Raghu G, Inoue Y, Azuma A, Huggins JT, Richeldi L, Stowasser S, Stansen W, Schlenker-Herceg R, Maher TM, Wells AU. Lung function outcomes in the INPULSIS trials of nintedanib in idiopathic pulmonary fibrosis. Respir Med. 2019;146:42–8.PubMedCrossRef
42.
go back to reference Kolb M, Richeldi L, Behr J, Maher TM, Tang W, Stowasser S, Hallmann C, du Bois RM. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017;72:340–6.PubMedCrossRef Kolb M, Richeldi L, Behr J, Maher TM, Tang W, Stowasser S, Hallmann C, du Bois RM. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017;72:340–6.PubMedCrossRef
43.
go back to reference Maher TM, Stowasser S, Nishioka Y, White ES, Cottin V, Noth I, Selman M, Rohr KB, Michael A, Ittrich C, Diefenbach C, Jenkins RG, on behalf of the INMARK trial investigators. Biomarkers of extracellular matrix turnover in patients with idiopathic pulmonary fibrosis given nintedanib (INMARK study): a randomised, placebo-controlled study. Lancet Respir Med. 2019;7(9):771–9.PubMedCrossRef Maher TM, Stowasser S, Nishioka Y, White ES, Cottin V, Noth I, Selman M, Rohr KB, Michael A, Ittrich C, Diefenbach C, Jenkins RG, on behalf of the INMARK trial investigators. Biomarkers of extracellular matrix turnover in patients with idiopathic pulmonary fibrosis given nintedanib (INMARK study): a randomised, placebo-controlled study. Lancet Respir Med. 2019;7(9):771–9.PubMedCrossRef
44.
go back to reference Song JW, Hong S-B, Lim C-M, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37:356–63.PubMedCrossRef Song JW, Hong S-B, Lim C-M, Koh Y, Kim DS. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37:356–63.PubMedCrossRef
45.
go back to reference Collard HR, Richeldi L, Kim DS, Taniguchi H, Tschoepe I, Luisetti M, Roman J, Tino G, Schlenker-Herceg R, Hallmann C, du Bois RM. Acute exacerbations in the INPULSIS trials of nintedanib in idiopathic pulmonary fibrosis. Eur Respir J. 2017;49:1601339.PubMedCrossRef Collard HR, Richeldi L, Kim DS, Taniguchi H, Tschoepe I, Luisetti M, Roman J, Tino G, Schlenker-Herceg R, Hallmann C, du Bois RM. Acute exacerbations in the INPULSIS trials of nintedanib in idiopathic pulmonary fibrosis. Eur Respir J. 2017;49:1601339.PubMedCrossRef
46.
go back to reference Collard HR, Yow E, Richeldi L, Anstrom KJ, Glazer C, investigators ftI. Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials. Respir Res. 2013;14:73.PubMedPubMedCentralCrossRef Collard HR, Yow E, Richeldi L, Anstrom KJ, Glazer C, investigators ftI. Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials. Respir Res. 2013;14:73.PubMedPubMedCentralCrossRef
47.
go back to reference Kreuter M, Koegler H, Trampisch M, Geier S, Richeldi L. Differing severities of acute exacerbations of idiopathic pulmonary fibrosis (IPF): insights from the INPULSIS® trials. Respir Res. 2019;20:71.PubMedPubMedCentralCrossRef Kreuter M, Koegler H, Trampisch M, Geier S, Richeldi L. Differing severities of acute exacerbations of idiopathic pulmonary fibrosis (IPF): insights from the INPULSIS® trials. Respir Res. 2019;20:71.PubMedPubMedCentralCrossRef
48.
go back to reference Behr J, Günther A, Bonella F, Geißler K, Koschel D, Kreuter M, Prasse A, Schönfeld N, Sitter H, Müller-Quernheim J, Costabel U. S2k-Leitlinie Idiopathische Lungenfibrose – Update zur medikamentösen Therapie 2017. Pneumologie. 2017;71:460–74.PubMedCrossRef Behr J, Günther A, Bonella F, Geißler K, Koschel D, Kreuter M, Prasse A, Schönfeld N, Sitter H, Müller-Quernheim J, Costabel U. S2k-Leitlinie Idiopathische Lungenfibrose – Update zur medikamentösen Therapie 2017. Pneumologie. 2017;71:460–74.PubMedCrossRef
49.
go back to reference Funke-Chambour M, Azzola A, Adler D, Barazzone-Argiroffo C, Benden C, Boehler A, Bridevaux PO, Brutsche M, Clarenbach CF, Hostettler K, Kleiner-Finger R, Nicod LP, Soccal PM, Tamm M, Geiser T, Lazor R. Idiopathic pulmonary fibrosis in Switzerland: Diagnosis and treatment. Respiration. 2017;93:363–78.PubMedCrossRef Funke-Chambour M, Azzola A, Adler D, Barazzone-Argiroffo C, Benden C, Boehler A, Bridevaux PO, Brutsche M, Clarenbach CF, Hostettler K, Kleiner-Finger R, Nicod LP, Soccal PM, Tamm M, Geiser T, Lazor R. Idiopathic pulmonary fibrosis in Switzerland: Diagnosis and treatment. Respiration. 2017;93:363–78.PubMedCrossRef
50.
go back to reference Cottin V, Crestani B, Cadranel J, Cordier JF, Marchand-Adam S, Prevot G, Wallaert B, Bergot E, Camus P, Dalphin JC, Dromer C, Gomez E, Israel-Biet D, Jouneau S, Kessler R, Marquette CH, Reynaud-Gaubert M, Aguilaniu B, Bonnet D, Carre P, Danel C, Faivre JB, Ferretti G, Just N, Lebargy F, Philippe B, Terrioux P, Thivolet-Bejui F, Trumbic B, Valeyre D. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2017 update. Rev Mal Respir. 2017;34:900–68.PubMedCrossRef Cottin V, Crestani B, Cadranel J, Cordier JF, Marchand-Adam S, Prevot G, Wallaert B, Bergot E, Camus P, Dalphin JC, Dromer C, Gomez E, Israel-Biet D, Jouneau S, Kessler R, Marquette CH, Reynaud-Gaubert M, Aguilaniu B, Bonnet D, Carre P, Danel C, Faivre JB, Ferretti G, Just N, Lebargy F, Philippe B, Terrioux P, Thivolet-Bejui F, Trumbic B, Valeyre D. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2017 update. Rev Mal Respir. 2017;34:900–68.PubMedCrossRef
51.
go back to reference Robbie H, Daccord C, Chua F, Devaraj A. Evaluating disease severity in idiopathic pulmonary fibrosis. Eur Respir Rev. 2017;26:170051.PubMedCrossRef Robbie H, Daccord C, Chua F, Devaraj A. Evaluating disease severity in idiopathic pulmonary fibrosis. Eur Respir Rev. 2017;26:170051.PubMedCrossRef
52.
go back to reference Richeldi L, Crestani B, Azuma A, Kolb M, Selman M, Stansen W, Quaresma M, Stowasser S, Cottin V. Outcomes following decline in forced vital capacity in patients with idiopathic pulmonary fibrosis: Results from the INPULSIS and INPULSIS-ON trials of nintedanib. Respir Med. 2019;156:20–5.PubMedCrossRef Richeldi L, Crestani B, Azuma A, Kolb M, Selman M, Stansen W, Quaresma M, Stowasser S, Cottin V. Outcomes following decline in forced vital capacity in patients with idiopathic pulmonary fibrosis: Results from the INPULSIS and INPULSIS-ON trials of nintedanib. Respir Med. 2019;156:20–5.PubMedCrossRef
53.
go back to reference Nathan SD, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Glasscock KF, King TE, Lancaster L, Lederer DJ, Lin Z, Pereira CA, Swigris JJ, Valeyre D, Noble PW, Wells AU. Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis. Thorax. 2016;71:429–35.PubMedCrossRef Nathan SD, Albera C, Bradford WZ, Costabel U, du Bois RM, Fagan EA, Fishman RS, Glaspole I, Glassberg MK, Glasscock KF, King TE, Lancaster L, Lederer DJ, Lin Z, Pereira CA, Swigris JJ, Valeyre D, Noble PW, Wells AU. Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis. Thorax. 2016;71:429–35.PubMedCrossRef
54.
go back to reference Weatherley ND, Eaden JA, Stewart NJ, Bartholmai BJ, Swift AJ, Bianchi SM, Wild JM. Experimental and quantitative imaging techniques in interstitial lung disease. Thorax. 2019;74:611–9.PubMedCrossRef Weatherley ND, Eaden JA, Stewart NJ, Bartholmai BJ, Swift AJ, Bianchi SM, Wild JM. Experimental and quantitative imaging techniques in interstitial lung disease. Thorax. 2019;74:611–9.PubMedCrossRef
55.
go back to reference Kim HG, Tashkin DP, Clements PJ, Li G, Brown MS, Elashoff R, Gjertson DW, Abtin F, Lynch DA, Strollo DC, Goldin JG. A computer-aided diagnosis system for quantitative scoring of extent of lung fibrosis in scleroderma patients. Clin Exp Rheumatol. 2010;28:S26–35.PubMedPubMedCentral Kim HG, Tashkin DP, Clements PJ, Li G, Brown MS, Elashoff R, Gjertson DW, Abtin F, Lynch DA, Strollo DC, Goldin JG. A computer-aided diagnosis system for quantitative scoring of extent of lung fibrosis in scleroderma patients. Clin Exp Rheumatol. 2010;28:S26–35.PubMedPubMedCentral
56.
go back to reference Kim HJ, Brown MS, Chong D, Gjertson DW, Lu P, Kim HJ, Coy H, Goldin JG. Comparison of the quantitative CT imaging biomarkers of idiopathic pulmonary fibrosis at baseline and early change with an interval of 7 months. Acad Radiol. 2015;22:70–80.PubMedCrossRef Kim HJ, Brown MS, Chong D, Gjertson DW, Lu P, Kim HJ, Coy H, Goldin JG. Comparison of the quantitative CT imaging biomarkers of idiopathic pulmonary fibrosis at baseline and early change with an interval of 7 months. Acad Radiol. 2015;22:70–80.PubMedCrossRef
57.
go back to reference Sellarés J, Hernández-González F, Lucena CM, Paradela M, Brito-Zerón P, Prieto-González S, Benegas M, Cuerpo S, Espinosa G, Ramírez J, Sánchez M, Xaubet A. Auscultation of velcro crackles is associated with usual interstitial pneumonia. Medicine. 2016;95:e2573.PubMedPubMedCentralCrossRef Sellarés J, Hernández-González F, Lucena CM, Paradela M, Brito-Zerón P, Prieto-González S, Benegas M, Cuerpo S, Espinosa G, Ramírez J, Sánchez M, Xaubet A. Auscultation of velcro crackles is associated with usual interstitial pneumonia. Medicine. 2016;95:e2573.PubMedPubMedCentralCrossRef
58.
go back to reference Sgalla G, Larici AR, Sverzellati N, Bartholmai B, Walsh SLF, Nikolic D, Barney A, Fletcher S, Jones M, Davies DD, Richeldi L. Quantitative analysis of lung sounds for monitoring idiopathic pulmonary fibrosis: a prospective pilot study. Eur Respir J. 2019;53:1802093.PubMedCrossRef Sgalla G, Larici AR, Sverzellati N, Bartholmai B, Walsh SLF, Nikolic D, Barney A, Fletcher S, Jones M, Davies DD, Richeldi L. Quantitative analysis of lung sounds for monitoring idiopathic pulmonary fibrosis: a prospective pilot study. Eur Respir J. 2019;53:1802093.PubMedCrossRef
59.
go back to reference Swigris JJ, Brown KK, Behr J, du Bois RM, King TE, Raghu G, Wamboldt FS. The SF-36 and SGRQ: Validity and first look at minimum important differences in IPF. Respir Med. 2010;104:296–304.PubMedCrossRef Swigris JJ, Brown KK, Behr J, du Bois RM, King TE, Raghu G, Wamboldt FS. The SF-36 and SGRQ: Validity and first look at minimum important differences in IPF. Respir Med. 2010;104:296–304.PubMedCrossRef
60.
go back to reference Swigris JJ, Wilson H, Esser D, Conoscenti CS, Stansen W, Kline Leidy N, Brown KK. Psychometric properties of the St George’s Respiratory Questionnaire in patients with idiopathic pulmonary fibrosis: Insights from the INPULSIS trials. BMJ Open Respir Res. 2018;5:e000278.PubMedPubMedCentralCrossRef Swigris JJ, Wilson H, Esser D, Conoscenti CS, Stansen W, Kline Leidy N, Brown KK. Psychometric properties of the St George’s Respiratory Questionnaire in patients with idiopathic pulmonary fibrosis: Insights from the INPULSIS trials. BMJ Open Respir Res. 2018;5:e000278.PubMedPubMedCentralCrossRef
61.
go back to reference Jenkins RG, Simpson JK, Saini G, Bentley JH, Russell A-M, Braybrooke R, Molyneaux PL, McKeever TM, Wells AU, Flynn A, Hubbard RB, Leeming DJ, Marshall RP, Karsdal MA, Lukey PT, Maher TM. Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study. Lancet Respir Med. 2015;3:462–72.PubMedCrossRef Jenkins RG, Simpson JK, Saini G, Bentley JH, Russell A-M, Braybrooke R, Molyneaux PL, McKeever TM, Wells AU, Flynn A, Hubbard RB, Leeming DJ, Marshall RP, Karsdal MA, Lukey PT, Maher TM. Longitudinal change in collagen degradation biomarkers in idiopathic pulmonary fibrosis: an analysis from the prospective, multicentre PROFILE study. Lancet Respir Med. 2015;3:462–72.PubMedCrossRef
62.
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. 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. 2018;6:615–26.PubMedCrossRef 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. 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. 2018;6:615–26.PubMedCrossRef
63.
go back to reference Richeldi L, Costabel U, Selman M, Kim DS, Hansell DM, Nicholson AG, Brown KK, Flaherty KR, Noble PW, Raghu G, Brun M, Gupta A, Juhel N, Klüglich M, du Bois RM. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365:1079–87.PubMedCrossRef Richeldi L, Costabel U, Selman M, Kim DS, Hansell DM, Nicholson AG, Brown KK, Flaherty KR, Noble PW, Raghu G, Brun M, Gupta A, Juhel N, Klüglich M, du Bois RM. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365:1079–87.PubMedCrossRef
64.
go back to reference Behr J, Kolb M, Song JW, Luppi F, Schinzel B, Stowasser S, Quaresma M, Martinez FJ, on behalf of the INSTAGE trial investigators. Nintedanib and sildenafil in patients with idiopathic pulmonary fibrosis and right heart dysfunction (INSTAGE): A pre-specified sub-group analysis of a double-blind, randomized clinical trial. Am J Respir Crit Care Med. 2019;200:1505–12.PubMedCrossRefPubMedCentral Behr J, Kolb M, Song JW, Luppi F, Schinzel B, Stowasser S, Quaresma M, Martinez FJ, on behalf of the INSTAGE trial investigators. Nintedanib and sildenafil in patients with idiopathic pulmonary fibrosis and right heart dysfunction (INSTAGE): A pre-specified sub-group analysis of a double-blind, randomized clinical trial. Am J Respir Crit Care Med. 2019;200:1505–12.PubMedCrossRefPubMedCentral
65.
go back to reference Wuyts WA, Kolb M, Stowasser S, Stansen W, Huggins JT, Raghu G. First data on efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis and forced vital capacity of ≤50 % of predicted value. Lung. 2016;194:739–43.PubMedPubMedCentralCrossRef Wuyts WA, Kolb M, Stowasser S, Stansen W, Huggins JT, Raghu G. First data on efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis and forced vital capacity of ≤50 % of predicted value. Lung. 2016;194:739–43.PubMedPubMedCentralCrossRef
66.
go back to reference Galli JA, Pandya A, Vega-Olivo M, Dass C, Zhao H, Criner GJ. Pirfenidone and nintedanib for pulmonary fibrosis in clinical practice: Tolerability and adverse drug reactions. Respirology. 2017;22:1171–8.PubMedCrossRef Galli JA, Pandya A, Vega-Olivo M, Dass C, Zhao H, Criner GJ. Pirfenidone and nintedanib for pulmonary fibrosis in clinical practice: Tolerability and adverse drug reactions. Respirology. 2017;22:1171–8.PubMedCrossRef
67.
go back to reference Barczi E, Starobinski L, Kolonics-Farkas A, Eszes N, Bohacs A, Vasakova M, Hejduk K, Müller V. Long-term effects and adverse events of nintedanib therapy in idiopathic pulmonary fibrosis patients with functionally advanced disease. Adv Ther. 2019;36(5):1221–32.PubMedCrossRef Barczi E, Starobinski L, Kolonics-Farkas A, Eszes N, Bohacs A, Vasakova M, Hejduk K, Müller V. Long-term effects and adverse events of nintedanib therapy in idiopathic pulmonary fibrosis patients with functionally advanced disease. Adv Ther. 2019;36(5):1221–32.PubMedCrossRef
68.
go back to reference Nakamura M, Okamoto M, Fujimoto K, Ebata T, Tominaga M, Nouno T, Zaizen Y, Kaieda S, Tsuda T, Kawayama T, Hoshino T. A retrospective study of the tolerability of nintedanib for severe idiopathic pulmonary fibrosis in the real world. Ann Transl Med. 2019;7:262.PubMedPubMedCentralCrossRef Nakamura M, Okamoto M, Fujimoto K, Ebata T, Tominaga M, Nouno T, Zaizen Y, Kaieda S, Tsuda T, Kawayama T, Hoshino T. A retrospective study of the tolerability of nintedanib for severe idiopathic pulmonary fibrosis in the real world. Ann Transl Med. 2019;7:262.PubMedPubMedCentralCrossRef
70.
go back to reference Cottin V, Hansell DM, Sverzellati N, Weycker D, Antoniou KM, Atwood M, Oster G, Kirchgaessler K-U, Collard HR, Wells AU. Effect of emphysema extent on serial lung function in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2017;196:1162–71.PubMedCrossRef Cottin V, Hansell DM, Sverzellati N, Weycker D, Antoniou KM, Atwood M, Oster G, Kirchgaessler K-U, Collard HR, Wells AU. Effect of emphysema extent on serial lung function in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2017;196:1162–71.PubMedCrossRef
71.
go back to reference Akagi T, Matsumoto T, Harada T, Tanaka M, Kuraki T, Fujita M, Watanabe K. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med. 2009;103:1209–15.PubMedCrossRef Akagi T, Matsumoto T, Harada T, Tanaka M, Kuraki T, Fujita M, Watanabe K. Coexistent emphysema delays the decrease of vital capacity in idiopathic pulmonary fibrosis. Respir Med. 2009;103:1209–15.PubMedCrossRef
72.
go back to reference Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs S, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Vachiéry J-L. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013;62:D109–D16.PubMedCrossRef Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs S, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Vachiéry J-L. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013;62:D109–D16.PubMedCrossRef
73.
go back to reference Jacob J, Bartholmai BJ, Rajagopalan S, Kokosi M, Maher TM, Nair A, Karwoski R, Renzoni E, Walsh SLF, Hansell DM, Wells AU. Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis. Eur Respir J. 2017;50:1700379.PubMedCrossRef Jacob J, Bartholmai BJ, Rajagopalan S, Kokosi M, Maher TM, Nair A, Karwoski R, Renzoni E, Walsh SLF, Hansell DM, Wells AU. Functional and prognostic effects when emphysema complicates idiopathic pulmonary fibrosis. Eur Respir J. 2017;50:1700379.PubMedCrossRef
74.
go back to reference Cottin V, Azuma A, Raghu G, Stansen W, Stowasser S, Schlenker-Herceg R, Kolb M. Therapeutic effects of nintedanib are not influenced by emphysema in the INPULSIS trials. Eur Respir J. 2019;53:1801655.CrossRef Cottin V, Azuma A, Raghu G, Stansen W, Stowasser S, Schlenker-Herceg R, Kolb M. Therapeutic effects of nintedanib are not influenced by emphysema in the INPULSIS trials. Eur Respir J. 2019;53:1801655.CrossRef
75.
go back to reference Laporta Hernandez R, Aguilar Perez M, Lázaro Carrasco MT, Ussetti GP. Lung transplantation in idiopathic pulmonary fibrosis. Med Sci. 2018;6:68. Laporta Hernandez R, Aguilar Perez M, Lázaro Carrasco MT, Ussetti GP. Lung transplantation in idiopathic pulmonary fibrosis. Med Sci. 2018;6:68.
76.
go back to reference Weill D, Benden C, Corris PA, Dark JH, Davis RD, Keshavjee S, Lederer DJ, Mulligan MJ, Patterson GA, Singer LG, Snell GI, Verleden GM, Zamora MR, Glanville AR. A consensus document for the selection of lung transplant candidates: 2014–An update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34:1–15.PubMedCrossRef Weill D, Benden C, Corris PA, Dark JH, Davis RD, Keshavjee S, Lederer DJ, Mulligan MJ, Patterson GA, Singer LG, Snell GI, Verleden GM, Zamora MR, Glanville AR. A consensus document for the selection of lung transplant candidates: 2014–An update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34:1–15.PubMedCrossRef
77.
go back to reference Roth GJ, Binder R, Colbatzky F, Dallinger C, Schlenker-Herceg R, Hilberg F, Wollin S-L, Kaiser R. Nintedanib: From discovery to the clinic. J Med Chem. 2015;58:1053–63.PubMedCrossRef Roth GJ, Binder R, Colbatzky F, Dallinger C, Schlenker-Herceg R, Hilberg F, Wollin S-L, Kaiser R. Nintedanib: From discovery to the clinic. J Med Chem. 2015;58:1053–63.PubMedCrossRef
78.
go back to reference Roodhart JM, Langenberg MH, Witteveen E, Voest EE. The molecular basis of class side effects due to treatment with inhibitors of the VEGF/VEGFR pathway. Curr Clin Pharmacol. 2008;3:132–43.PubMedCrossRef Roodhart JM, Langenberg MH, Witteveen E, Voest EE. The molecular basis of class side effects due to treatment with inhibitors of the VEGF/VEGFR pathway. Curr Clin Pharmacol. 2008;3:132–43.PubMedCrossRef
79.
go back to reference Shah RR, Morganroth J. Update on cardiovascular safety of tyrosine kinase inhibitors: With a special focus on qt interval, left ventricular dysfunction and overall risk/benefit. Drug Saf. 2015;38:693–710.PubMedCrossRef Shah RR, Morganroth J. Update on cardiovascular safety of tyrosine kinase inhibitors: With a special focus on qt interval, left ventricular dysfunction and overall risk/benefit. Drug Saf. 2015;38:693–710.PubMedCrossRef
80.
go back to reference van Cruijsen H, van der Veldt A, Hoekman K. Tyrosine kinase inhibitors of VEGF receptors: clinical issues and remaining questions. Front Biosci. 2009;14:2248–68.CrossRef van Cruijsen H, van der Veldt A, Hoekman K. Tyrosine kinase inhibitors of VEGF receptors: clinical issues and remaining questions. Front Biosci. 2009;14:2248–68.CrossRef
81.
go back to reference Shah DR, Dholakia S, Shah RR. Effect of tyrosine kinase inhibitors on wound healing and tissue repair: implications for surgery in cancer patients. Drug Saf. 2014;37:135–49.PubMedCrossRef Shah DR, Dholakia S, Shah RR. Effect of tyrosine kinase inhibitors on wound healing and tissue repair: implications for surgery in cancer patients. Drug Saf. 2014;37:135–49.PubMedCrossRef
82.
go back to reference Powers CJ, McLeskey SW, Wellstein A. Fibroblast growth factors, their receptors and signaling. Endocr Relat Cancer. 2000;7:165.PubMedCrossRef Powers CJ, McLeskey SW, Wellstein A. Fibroblast growth factors, their receptors and signaling. Endocr Relat Cancer. 2000;7:165.PubMedCrossRef
83.
go back to reference Noth I, Oelberg D, Kaul M, Conoscenti CS, Raghu G. Safety and tolerability of nintedanib in patients with IPF in the United States. Eur Respir J. 2018;52:1702106.CrossRef Noth I, Oelberg D, Kaul M, Conoscenti CS, Raghu G. Safety and tolerability of nintedanib in patients with IPF in the United States. Eur Respir J. 2018;52:1702106.CrossRef
84.
go back to reference Hughes G, Toellner H, Morris H, Leonard C, Chaudhuri N. Real world experiences: Pirfenidone and nintedanib are effective and well tolerated treatments for idiopathic pulmonary fibrosis. J Clin Med. 2016;5:78.PubMedCentralCrossRef Hughes G, Toellner H, Morris H, Leonard C, Chaudhuri N. Real world experiences: Pirfenidone and nintedanib are effective and well tolerated treatments for idiopathic pulmonary fibrosis. J Clin Med. 2016;5:78.PubMedCentralCrossRef
85.
go back to reference Bonella F, Kreuter M, Hagmeyer L, Neurohr C, Keller C, Kohlhaeufl MJ, Müller-Quernheim J, Milger K, Prasse A. Insights from the German compassionate use program of nintedanib for the treatment of idiopathic pulmonary fibrosis. Respiration. 2016;92:98–106.PubMedCrossRef Bonella F, Kreuter M, Hagmeyer L, Neurohr C, Keller C, Kohlhaeufl MJ, Müller-Quernheim J, Milger K, Prasse A. Insights from the German compassionate use program of nintedanib for the treatment of idiopathic pulmonary fibrosis. Respiration. 2016;92:98–106.PubMedCrossRef
86.
go back to reference Tzouvelekis A, Karampitsakos T, Kontou M, Granitsas A, Malliou I, Anagnostopoulos A, Ntolios P, Tzilas V, Bouros E, Steiropoulos P, Chrysikos S, Dimakou K, Koulouris N, Bouros D. Safety and efficacy of nintedanib in idiopathic pulmonary fibrosis: A real-life observational study in Greece. Pulm Pharmacol Ther. 2018;49:61–6.PubMedCrossRef Tzouvelekis A, Karampitsakos T, Kontou M, Granitsas A, Malliou I, Anagnostopoulos A, Ntolios P, Tzilas V, Bouros E, Steiropoulos P, Chrysikos S, Dimakou K, Koulouris N, Bouros D. Safety and efficacy of nintedanib in idiopathic pulmonary fibrosis: A real-life observational study in Greece. Pulm Pharmacol Ther. 2018;49:61–6.PubMedCrossRef
87.
go back to reference Barratt SL, Mulholland S, Al Jbour K, Steer H, Gutsche M, Foley N, Srivastava R, Sharp C, Adamali HI. South–west of England’s experience of the safety and tolerability pirfenidone and nintedanib for the treatment of idiopathic pulmonary fibrosis (IPF). Front Pharmacol. 2018;9:1480.PubMedPubMedCentralCrossRef Barratt SL, Mulholland S, Al Jbour K, Steer H, Gutsche M, Foley N, Srivastava R, Sharp C, Adamali HI. South–west of England’s experience of the safety and tolerability pirfenidone and nintedanib for the treatment of idiopathic pulmonary fibrosis (IPF). Front Pharmacol. 2018;9:1480.PubMedPubMedCentralCrossRef
88.
go back to reference Bendstrup E, Wuyts W, Alfaro T, Chaudhuri N, Cornelissen R, Kreuter M, Melgaard Nielsen K, Münster AMB, Myllärniemi M, Ravaglia C, Vanuytsel T, Wijsenbeek M. Nintedanib in idiopathic pulmonary fibrosis: Practical management recommendations for potential adverse events. Respiration. 2019;97:173–84.PubMedCrossRef Bendstrup E, Wuyts W, Alfaro T, Chaudhuri N, Cornelissen R, Kreuter M, Melgaard Nielsen K, Münster AMB, Myllärniemi M, Ravaglia C, Vanuytsel T, Wijsenbeek M. Nintedanib in idiopathic pulmonary fibrosis: Practical management recommendations for potential adverse events. Respiration. 2019;97:173–84.PubMedCrossRef
89.
go back to reference Delanote I, Wuyts WA, Yserbyt J, Verbeken EK, Verleden GM, Vos R. Safety and efficacy of bridging to lung transplantation with antifibrotic drugs in idiopathic pulmonary fibrosis: a case series. BMC Pulm Med. 2016;16:156.PubMedPubMedCentralCrossRef Delanote I, Wuyts WA, Yserbyt J, Verbeken EK, Verleden GM, Vos R. Safety and efficacy of bridging to lung transplantation with antifibrotic drugs in idiopathic pulmonary fibrosis: a case series. BMC Pulm Med. 2016;16:156.PubMedPubMedCentralCrossRef
90.
go back to reference Leuschner G, Stocker F, Veit T, Kneidinger N, Winter H, Schramm R, Weig T, Matthes S, Ceelen F, Arnold P, Munker D, Klenner F, Hatz R, Frankenberger M, Behr J, Neurohr C. Outcome of lung transplantation in idiopathic pulmonary fibrosis with previous anti-fibrotic therapy. J Heart Lung Transplant. 2018;37:268–74.CrossRef Leuschner G, Stocker F, Veit T, Kneidinger N, Winter H, Schramm R, Weig T, Matthes S, Ceelen F, Arnold P, Munker D, Klenner F, Hatz R, Frankenberger M, Behr J, Neurohr C. Outcome of lung transplantation in idiopathic pulmonary fibrosis with previous anti-fibrotic therapy. J Heart Lung Transplant. 2018;37:268–74.CrossRef
91.
go back to reference Lambers C, Boehm PM, Lee S, Ius F, Jaksch P, Klepetko W, Tudorache I, Ristl R, Welte T, Gottlieb J. Effect of antifibrotics on short-term outcome after bilateral lung transplantation: a multicentre analysis. Eur Respir J. 2018;51:1800503.PubMedCrossRef Lambers C, Boehm PM, Lee S, Ius F, Jaksch P, Klepetko W, Tudorache I, Ristl R, Welte T, Gottlieb J. Effect of antifibrotics on short-term outcome after bilateral lung transplantation: a multicentre analysis. Eur Respir J. 2018;51:1800503.PubMedCrossRef
93.
go back to reference Yoon JH, Nouraie M, Chen X, Zou RH, Sellares J, Veraldi KL, Chiarchiaro J, Lindell K, Wilson DO, Kaminski N, Burns T, Trejo Bittar H, Yousem S, Gibson K, Kass DJ. Characteristics of lung cancer among patients with idiopathic pulmonary fibrosis and interstitial lung disease – analysis of institutional and population data. Respir Res. 2018;19:195.PubMedPubMedCentralCrossRef Yoon JH, Nouraie M, Chen X, Zou RH, Sellares J, Veraldi KL, Chiarchiaro J, Lindell K, Wilson DO, Kaminski N, Burns T, Trejo Bittar H, Yousem S, Gibson K, Kass DJ. Characteristics of lung cancer among patients with idiopathic pulmonary fibrosis and interstitial lung disease – analysis of institutional and population data. Respir Res. 2018;19:195.PubMedPubMedCentralCrossRef
94.
go back to reference Karampitsakos T, Tzilas V, Tringidou R, Steiropoulos P, Aidinis V, Papiris SA, Bouros D, Tzouvelekis A. Lung cancer in patients with idiopathic pulmonary fibrosis. Pulm Pharmacol Ther. 2017;45:1–10.PubMedCrossRef Karampitsakos T, Tzilas V, Tringidou R, Steiropoulos P, Aidinis V, Papiris SA, Bouros D, Tzouvelekis A. Lung cancer in patients with idiopathic pulmonary fibrosis. Pulm Pharmacol Ther. 2017;45:1–10.PubMedCrossRef
95.
go back to reference Raghu G, Amatto VC, Behr J, Stowasser S. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review. Eur Respir J. 2015;46:1113–30.PubMedCrossRef Raghu G, Amatto VC, Behr J, Stowasser S. Comorbidities in idiopathic pulmonary fibrosis patients: a systematic literature review. Eur Respir J. 2015;46:1113–30.PubMedCrossRef
96.
go back to reference Kreuter M, Ehlers-Tenenbaum S, Schaaf M, Oltmanns U, Palmowski K, Hoffmann H, Schnabel PA, Heussel CP, Puderbach M, Herth FJ, Warth A. Treatment and outcome of lung cancer in idiopathic interstitial pneumonias. Sarcoidosis Vasc Diffuse Lung Dis. 2015;31:266–74.PubMed Kreuter M, Ehlers-Tenenbaum S, Schaaf M, Oltmanns U, Palmowski K, Hoffmann H, Schnabel PA, Heussel CP, Puderbach M, Herth FJ, Warth A. Treatment and outcome of lung cancer in idiopathic interstitial pneumonias. Sarcoidosis Vasc Diffuse Lung Dis. 2015;31:266–74.PubMed
98.
go back to reference Dhillon S. Nintedanib: A review of its use as second-line treatment in adults with advanced non-small cell lung cancer of adenocarcinoma histology. Target Oncol. 2015;10:303–10.PubMedCrossRef Dhillon S. Nintedanib: A review of its use as second-line treatment in adults with advanced non-small cell lung cancer of adenocarcinoma histology. Target Oncol. 2015;10:303–10.PubMedCrossRef
99.
go back to reference Oldham JM, Collard HR. Comorbid conditions in idiopathic pulmonary fibrosis: recognition and management. Front Med. 2017;4:123.CrossRef Oldham JM, Collard HR. Comorbid conditions in idiopathic pulmonary fibrosis: recognition and management. Front Med. 2017;4:123.CrossRef
100.
go back to reference Fukunaga K, Yokoe S, Kawashima S, Uchida Y, Nakagawa H, Nakano Y. Nintedanib prevented fibrosis progression and lung cancer growth in idiopathic pulmonary fibrosis. Respirol Case Rep. 2018;6:e00363.PubMedPubMedCentral Fukunaga K, Yokoe S, Kawashima S, Uchida Y, Nakagawa H, Nakano Y. Nintedanib prevented fibrosis progression and lung cancer growth in idiopathic pulmonary fibrosis. Respirol Case Rep. 2018;6:e00363.PubMedPubMedCentral
101.
go back to reference Otsubo K, Kishimoto J, Kenmotsu H, Minegishi Y, Ichihara E, Shiraki A, Kato T, Atagi S, Horinouchi H, Ando M, Kondoh Y, Kusumoto M, Ichikado K, Yamamoto N, Nakanishi Y, Okamoto I. Treatment rationale and design for J-SONIC: A randomized study of carboplatin plus nab-paclitaxel with or without nintedanib for advanced non-small-cell lung cancer with idiopathic pulmonary fibrosis. Clin Lung Cancer. 2018;19:e5–9.PubMedCrossRef Otsubo K, Kishimoto J, Kenmotsu H, Minegishi Y, Ichihara E, Shiraki A, Kato T, Atagi S, Horinouchi H, Ando M, Kondoh Y, Kusumoto M, Ichikado K, Yamamoto N, Nakanishi Y, Okamoto I. Treatment rationale and design for J-SONIC: A randomized study of carboplatin plus nab-paclitaxel with or without nintedanib for advanced non-small-cell lung cancer with idiopathic pulmonary fibrosis. Clin Lung Cancer. 2018;19:e5–9.PubMedCrossRef
102.
go back to reference Richeldi L, Kreuter M, Selman M, Crestani B, Kirsten A-M, Wuyts WA, Xu Z, Bernois K, Stowasser S, Quaresma M, Costabel U. Long-term treatment of patients with idiopathic pulmonary fibrosis with nintedanib: results from the TOMORROW trial and its open-label extension. Thorax. 2018;73:581–3.PubMedCrossRef Richeldi L, Kreuter M, Selman M, Crestani B, Kirsten A-M, Wuyts WA, Xu Z, Bernois K, Stowasser S, Quaresma M, Costabel U. Long-term treatment of patients with idiopathic pulmonary fibrosis with nintedanib: results from the TOMORROW trial and its open-label extension. Thorax. 2018;73:581–3.PubMedCrossRef
103.
go back to reference Corte T, Bonella F, Crestani B, Demedts MG, Richeldi L, Coeck C, Pelling K, Quaresma M, Lasky JA. Safety, tolerability and appropriate use of nintedanib in idiopathic pulmonary fibrosis. Respir Res. 2015;16:116.PubMedPubMedCentralCrossRef Corte T, Bonella F, Crestani B, Demedts MG, Richeldi L, Coeck C, Pelling K, Quaresma M, Lasky JA. Safety, tolerability and appropriate use of nintedanib in idiopathic pulmonary fibrosis. Respir Res. 2015;16:116.PubMedPubMedCentralCrossRef
104.
go back to reference Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM. Nintedanib for systemic sclerosis–associated interstitial lung disease. N Engl J Med. 2019;381(16):1596–7.PubMed Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, Raghu G, Sauter W, Girard M, Alves M, Clerisme-Beaty E, Stowasser S, Tetzlaff K, Kuwana M, Maher TM. Nintedanib for systemic sclerosis–associated interstitial lung disease. N Engl J Med. 2019;381(16):1596–7.PubMed
105.
go back to reference Flaherty KR, Wells AU, Clerisme-Beaty E, Cottin V, Devaraj A, Inoue Y, Richeldi L, Walsh S, Goeldner R-G, Schlenker-Herceg R, Brown KK, investigators obotIt. Characteristics of patients with progressive fibrosing interstitial lung diseases (ILDs) in the INBUILD trial of nintedanib. Am J Respir Crit Care Med. 2019;199:A5627. Flaherty KR, Wells AU, Clerisme-Beaty E, Cottin V, Devaraj A, Inoue Y, Richeldi L, Walsh S, Goeldner R-G, Schlenker-Herceg R, Brown KK, investigators obotIt. Characteristics of patients with progressive fibrosing interstitial lung diseases (ILDs) in the INBUILD trial of nintedanib. Am J Respir Crit Care Med. 2019;199:A5627.
107.
go back to reference Harari S, Caminati A, Poletti V, Confalonieri M, Gasparini S, Lacedonia D, Luppi F, Pesci A, Sebastiani A, Spagnolo P, Vancheri C, Balestro E, Bonifazi M, Cerri S, De Giacomi F, Della Porta R, Foschino Barbaro MP, Fui A, Pasquinelli P, Rosso R, Tomassetti S, Specchia C, Rottoli P. A real-life multicenter national study on nintedanib in severe idiopathic pulmonary fibrosis. Respiration. 2018;95:433–40.PubMedCrossRef Harari S, Caminati A, Poletti V, Confalonieri M, Gasparini S, Lacedonia D, Luppi F, Pesci A, Sebastiani A, Spagnolo P, Vancheri C, Balestro E, Bonifazi M, Cerri S, De Giacomi F, Della Porta R, Foschino Barbaro MP, Fui A, Pasquinelli P, Rosso R, Tomassetti S, Specchia C, Rottoli P. A real-life multicenter national study on nintedanib in severe idiopathic pulmonary fibrosis. Respiration. 2018;95:433–40.PubMedCrossRef
108.
go back to reference Bargagli E, Piccioli C, Rosi E, Torricelli E, Turi L, Piccioli E, Pistolesi M, Ferrari K, Voltolini L. Pirfenidone and nintedanib in idiopathic pulmonary fibrosis: Real-life experience in an Italian referral centre. Pulmonology. 2019;25:149–53.PubMedCrossRef Bargagli E, Piccioli C, Rosi E, Torricelli E, Turi L, Piccioli E, Pistolesi M, Ferrari K, Voltolini L. Pirfenidone and nintedanib in idiopathic pulmonary fibrosis: Real-life experience in an Italian referral centre. Pulmonology. 2019;25:149–53.PubMedCrossRef
109.
go back to reference Toellner H, Hughes G, Beswick W, Crooks MG, Donaldson C, Forrest I, Hart SP, Leonard C, Major M, Simpson AJ, Chaudhuri N. Early clinical experiences with nintedanib in three UK tertiary interstitial lung disease centres. Clin Transl Med. 2017;6:41.PubMedPubMedCentralCrossRef Toellner H, Hughes G, Beswick W, Crooks MG, Donaldson C, Forrest I, Hart SP, Leonard C, Major M, Simpson AJ, Chaudhuri N. Early clinical experiences with nintedanib in three UK tertiary interstitial lung disease centres. Clin Transl Med. 2017;6:41.PubMedPubMedCentralCrossRef
110.
go back to reference Brunnemer E, Wälscher J, Tenenbaum S, Hausmanns J, Schulze K, Seiter M, Heussel CP, Warth A, Herth FJF, Kreuter M. Real-world experience with nintedanib in patients with idiopathic pulmonary fibrosis. Respiration. 2018;95:301–9.PubMedCrossRef Brunnemer E, Wälscher J, Tenenbaum S, Hausmanns J, Schulze K, Seiter M, Heussel CP, Warth A, Herth FJF, Kreuter M. Real-world experience with nintedanib in patients with idiopathic pulmonary fibrosis. Respiration. 2018;95:301–9.PubMedCrossRef
111.
go back to reference Maher T, Noth I, Allinger A, Kaul M, Conoscenti C, Oelberg D. P168 Safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis (IPF): One-year data from post-marketing surveillance in the United States. Thorax. 2016;71:A175. Maher T, Noth I, Allinger A, Kaul M, Conoscenti C, Oelberg D. P168 Safety and tolerability of nintedanib in patients with idiopathic pulmonary fibrosis (IPF): One-year data from post-marketing surveillance in the United States. Thorax. 2016;71:A175.
112.
go back to reference Kreuter M, Ehlers-Tenenbaum S, Palmowski K, Bruhwyler J, Oltmanns U, Muley T, Heussel CP, Warth A, Kolb M, Herth FJF. Impact of comorbidities on mortality in patients with idiopathic pulmonary fibrosis. PLoS One. 2016;11:e0151425.PubMedPubMedCentralCrossRef Kreuter M, Ehlers-Tenenbaum S, Palmowski K, Bruhwyler J, Oltmanns U, Muley T, Heussel CP, Warth A, Kolb M, Herth FJF. Impact of comorbidities on mortality in patients with idiopathic pulmonary fibrosis. PLoS One. 2016;11:e0151425.PubMedPubMedCentralCrossRef
113.
go back to reference Kreuter M, Swigris J, Pittrow D, Geier S, Klotsche J, Prasse A, Wirtz H, Koschel D, Andreas S, Claussen M, Grohé C, Wilkens H, Hagmeyer L, Skowasch D, Meyer JF, Kirschner J, Gläser S, Herth FJF, Welte T, Neurohr C, Schwaiblmair M, Held M, Bahmer T, Frankenberger M, Behr J. Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry. Respir Res. 2017;18:139.PubMedPubMedCentralCrossRef Kreuter M, Swigris J, Pittrow D, Geier S, Klotsche J, Prasse A, Wirtz H, Koschel D, Andreas S, Claussen M, Grohé C, Wilkens H, Hagmeyer L, Skowasch D, Meyer JF, Kirschner J, Gläser S, Herth FJF, Welte T, Neurohr C, Schwaiblmair M, Held M, Bahmer T, Frankenberger M, Behr J. Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry. Respir Res. 2017;18:139.PubMedPubMedCentralCrossRef
114.
go back to reference Tomioka H, Takada H. Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis. Respirol Case Rep. 2017;5:e00215.PubMedPubMedCentral Tomioka H, Takada H. Treatment with nintedanib for acute exacerbation of idiopathic pulmonary fibrosis. Respirol Case Rep. 2017;5:e00215.PubMedPubMedCentral
115.
go back to reference Ito Y, Tazaki G, Kondo Y, Takahashi G, Sakamaki F. Therapeutic effect of nintedanib on acute exacerbation of interstitial lung diseases. Respir Med Case Rep. 2019;26:317–20.PubMedPubMedCentral Ito Y, Tazaki G, Kondo Y, Takahashi G, Sakamaki F. Therapeutic effect of nintedanib on acute exacerbation of interstitial lung diseases. Respir Med Case Rep. 2019;26:317–20.PubMedPubMedCentral
116.
go back to reference Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y, Reynolds PN, Chapman S, Walters EH, Zappala C, Allan H, Keir GJ, Hayen A, Cooper WA, Mahar AM, Ellis S, Macansh S, Corte TJ. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017;49:1601592.PubMedCrossRef Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y, Reynolds PN, Chapman S, Walters EH, Zappala C, Allan H, Keir GJ, Hayen A, Cooper WA, Mahar AM, Ellis S, Macansh S, Corte TJ. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 2017;49:1601592.PubMedCrossRef
117.
go back to reference Behr J, Wirtz H, Pittrow D, Prasse A, Koschel D, Geier S, Klotsche J, Andreas S, Claussen M, Grohé C, Wilkens H, Hagmeyer L, Skowasch D, Meyer JF, Kirschner J, Gläser S, Kahn N, Welte T, Neurohr C, Schwaiblmair M, Held M, Bahmer T, Oqueka T, Frankenberger M, Kreuter M. Survival and course of lung function in patients with idiopathic pulmonary fibrosis with or without antifibrotic treatment: long-term results of the INSIGHTS-IPF registry. Madrid: Oral presentation at: ERS International Congress; 2019. Behr J, Wirtz H, Pittrow D, Prasse A, Koschel D, Geier S, Klotsche J, Andreas S, Claussen M, Grohé C, Wilkens H, Hagmeyer L, Skowasch D, Meyer JF, Kirschner J, Gläser S, Kahn N, Welte T, Neurohr C, Schwaiblmair M, Held M, Bahmer T, Oqueka T, Frankenberger M, Kreuter M. Survival and course of lung function in patients with idiopathic pulmonary fibrosis with or without antifibrotic treatment: long-term results of the INSIGHTS-IPF registry. Madrid: Oral presentation at: ERS International Congress; 2019.
118.
go back to reference Ogura T, Taniguchi H, Azuma A, Inoue Y, Kondoh Y, Hasegawa Y, Bando M, Abe S, Mochizuki Y, Chida K, Klüglich M, Fujimoto T, Okazaki K, Tadayasu Y, Sakamoto W, Sugiyama Y. Safety and pharmacokinetics of nintedanib and pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2015;45:1382–92.PubMedCrossRef Ogura T, Taniguchi H, Azuma A, Inoue Y, Kondoh Y, Hasegawa Y, Bando M, Abe S, Mochizuki Y, Chida K, Klüglich M, Fujimoto T, Okazaki K, Tadayasu Y, Sakamoto W, Sugiyama Y. Safety and pharmacokinetics of nintedanib and pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2015;45:1382–92.PubMedCrossRef
119.
go back to reference Flaherty KR, Fell CD, Huggins JT, Nunes H, Sussman R, Valenzuela C, Petzinger U, Stauffer JL, Gilberg F, Bengus M, Wijsenbeek M. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. Eur Respir J. 2018;52:1800230.PubMedPubMedCentralCrossRef Flaherty KR, Fell CD, Huggins JT, Nunes H, Sussman R, Valenzuela C, Petzinger U, Stauffer JL, Gilberg F, Bengus M, Wijsenbeek M. Safety of nintedanib added to pirfenidone treatment for idiopathic pulmonary fibrosis. Eur Respir J. 2018;52:1800230.PubMedPubMedCentralCrossRef
120.
go back to reference Milger K, Kneidinger N, Neurohr C, Reichenberger F, Behr J. Switching to nintedanib after discontinuation of pirfenidone due to adverse events in IPF. Eur Respir J. 2015;46:1217–21.PubMedCrossRef Milger K, Kneidinger N, Neurohr C, Reichenberger F, Behr J. Switching to nintedanib after discontinuation of pirfenidone due to adverse events in IPF. Eur Respir J. 2015;46:1217–21.PubMedCrossRef
121.
go back to reference Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I, Roberts RS, Yow E, Raghu G. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1:369–76.PubMedPubMedCentralCrossRef Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I, Roberts RS, Yow E, Raghu G. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1:369–76.PubMedPubMedCentralCrossRef
122.
go back to reference Kreuter M, Wuyts W, Renzoni E, Koschel D, Maher TM, Kolb M, Weycker D, Spagnolo P, Kirchgaessler K-U, Herth FJF, Costabel U. Antacid therapy and disease outcomes in idiopathic pulmonary fibrosis: a pooled analysis. Lancet Respir Med. 2016;4:381–9.PubMedCrossRef Kreuter M, Wuyts W, Renzoni E, Koschel D, Maher TM, Kolb M, Weycker D, Spagnolo P, Kirchgaessler K-U, Herth FJF, Costabel U. Antacid therapy and disease outcomes in idiopathic pulmonary fibrosis: a pooled analysis. Lancet Respir Med. 2016;4:381–9.PubMedCrossRef
123.
go back to reference Costabel U, Behr J, Crestani B, Stansen W, Schlenker-Herceg R, Stowasser S, Raghu G. Anti-acid therapy in idiopathic pulmonary fibrosis: insights from the INPULSIS® trials. Respir Res. 2018;19:167.PubMedPubMedCentralCrossRef Costabel U, Behr J, Crestani B, Stansen W, Schlenker-Herceg R, Stowasser S, Raghu G. Anti-acid therapy in idiopathic pulmonary fibrosis: insights from the INPULSIS® trials. Respir Res. 2018;19:167.PubMedPubMedCentralCrossRef
124.
go back to reference Bavishi C, DuPont HL. Systematic review: The use of proton pump inhibitors and increased susceptibility to enteric infection. Aliment Pharmacol Ther. 2011;34:1269–81.PubMedCrossRef Bavishi C, DuPont HL. Systematic review: The use of proton pump inhibitors and increased susceptibility to enteric infection. Aliment Pharmacol Ther. 2011;34:1269–81.PubMedCrossRef
125.
go back to reference Somogyi V, Chaudhuri N, Torrisi SE, Kahn N, Müller V, Kreuter M. The therapy of idiopathic pulmonary fibrosis: what is next? Eur Respir Rev. 2019;28:190021.PubMedCrossRef Somogyi V, Chaudhuri N, Torrisi SE, Kahn N, Müller V, Kreuter M. The therapy of idiopathic pulmonary fibrosis: what is next? Eur Respir Rev. 2019;28:190021.PubMedCrossRef
126.
go back to reference Maher TM, Kreuter M, Lederer DJ, Brown KK, Wuyts W, Verbruggen N, Stutvoet S, Fieuw A, Ford P, Abi-Saab W, Wijsenbeek M. Rationale, design and objectives of two phase III, randomised, placebo-controlled studies of GLPG1690, a novel autotaxin inhibitor, in idiopathic pulmonary fibrosis (ISABELA 1 and 2). BMJ Open Respir Res. 2019;6:e000422.PubMedPubMedCentralCrossRef Maher TM, Kreuter M, Lederer DJ, Brown KK, Wuyts W, Verbruggen N, Stutvoet S, Fieuw A, Ford P, Abi-Saab W, Wijsenbeek M. Rationale, design and objectives of two phase III, randomised, placebo-controlled studies of GLPG1690, a novel autotaxin inhibitor, in idiopathic pulmonary fibrosis (ISABELA 1 and 2). BMJ Open Respir Res. 2019;6:e000422.PubMedPubMedCentralCrossRef
127.
go back to reference Raghu G, van den Blink B, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS, Vasakova M, Pesci A, Antin-Ozerkis DE, Meyer KC, Kreuter M, Santin-Janin H, Mulder G-J, Bartholmai B, Gupta R, Richeldi L. Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis: A randomized clinical trialrecombinant human pentraxin 2 vs placebo and lung function in idiopathic pulmonary fibrosisrecombinant human pentraxin 2 vs placebo and lung function in idiopathic pulmonary fibrosis. J Am Med Assoc. 2018;319:2299–307.CrossRef Raghu G, van den Blink B, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS, Vasakova M, Pesci A, Antin-Ozerkis DE, Meyer KC, Kreuter M, Santin-Janin H, Mulder G-J, Bartholmai B, Gupta R, Richeldi L. Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis: A randomized clinical trialrecombinant human pentraxin 2 vs placebo and lung function in idiopathic pulmonary fibrosisrecombinant human pentraxin 2 vs placebo and lung function in idiopathic pulmonary fibrosis. J Am Med Assoc. 2018;319:2299–307.CrossRef
128.
go back to reference Khalil N, Manganas H, Ryerson CJ, Shapera S, Cantin AM, Hernandez P, Turcotte EE, Parker JM, Moran JE, Albert GR, Sawtell R, Hagerimana A, Laurin P, Gagnon L, Cesari F, Kolb M. Phase 2 clinical trial of PBI-4050 in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2018;53:1800663.CrossRef Khalil N, Manganas H, Ryerson CJ, Shapera S, Cantin AM, Hernandez P, Turcotte EE, Parker JM, Moran JE, Albert GR, Sawtell R, Hagerimana A, Laurin P, Gagnon L, Cesari F, Kolb M. Phase 2 clinical trial of PBI-4050 in patients with idiopathic pulmonary fibrosis. Eur Respir J. 2018;53:1800663.CrossRef
129.
go back to reference Gorina E, Richeldi L, Raghu G, Fernandez Perez E, Costabel U, Albera C, Lederer D, Flaherty K, Ettinger N, Bercz P, Singh B, Perez R, Goldin J, Kouchakji E, Porter S. PRAISE, a randomized, placebo-controlled, double-blind Phase 2 clinical trial of pamrevlumab (FG-3019) in IPF patients. Eur Respir J. 2017;50:OA3400. Gorina E, Richeldi L, Raghu G, Fernandez Perez E, Costabel U, Albera C, Lederer D, Flaherty K, Ettinger N, Bercz P, Singh B, Perez R, Goldin J, Kouchakji E, Porter S. PRAISE, a randomized, placebo-controlled, double-blind Phase 2 clinical trial of pamrevlumab (FG-3019) in IPF patients. Eur Respir J. 2017;50:OA3400.
130.
go back to reference Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;(10):CD006322. Dowman L, Hill CJ, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2014;(10):CD006322.
132.
go back to reference White ES, Borok Z, Brown KK, Eickelberg O, Guenther A, Jenkins RG, Kolb M, Martinez FJ, Roman J, Sime P, American Thoracic Society Respiratory C, Molecular Biology Assembly Working Group on Pulmonary F. An American Thoracic Society official research statement: Future directions in lung fibrosis research. Am J Respir Crit Care Med. 2016;193:792–800.PubMedPubMedCentralCrossRef White ES, Borok Z, Brown KK, Eickelberg O, Guenther A, Jenkins RG, Kolb M, Martinez FJ, Roman J, Sime P, American Thoracic Society Respiratory C, Molecular Biology Assembly Working Group on Pulmonary F. An American Thoracic Society official research statement: Future directions in lung fibrosis research. Am J Respir Crit Care Med. 2016;193:792–800.PubMedPubMedCentralCrossRef
133.
go back to reference Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ. What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51:1800692.PubMedCrossRef Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ. What's in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51:1800692.PubMedCrossRef
134.
go back to reference Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M, Skowasch D, Park JS, Poonyagariyagorn HK, Wuyts W, Wells AU. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180076.PubMedCrossRef Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M, Skowasch D, Park JS, Poonyagariyagorn HK, Wuyts W, Wells AU. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180076.PubMedCrossRef
135.
go back to reference Richeldi L, Varone F, Bergna M, de Andrade J, Falk J, Hallowell R, Jouneau S, Kondoh Y, Morrow L, Randerath W, Strek M, Tabaj G. Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence. Eur Respir Rev. 2018;27:180074.PubMedCrossRef Richeldi L, Varone F, Bergna M, de Andrade J, Falk J, Hallowell R, Jouneau S, Kondoh Y, Morrow L, Randerath W, Strek M, Tabaj G. Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence. Eur Respir Rev. 2018;27:180074.PubMedCrossRef
136.
go back to reference Cottin V. Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases. Eur Respir Rev. 2019;28:190109.PubMedCrossRef Cottin V. Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases. Eur Respir Rev. 2019;28:190109.PubMedCrossRef
137.
go back to reference Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Štork J, Morovic-Vergles J, Walker UA. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15.PubMedCrossRef Tyndall AJ, Bannert B, Vonk M, Airò P, Cozzi F, Carreira PE, Bancel DF, Allanore Y, Müller-Ladner U, Distler O, Iannone F, Pellerito R, Pileckyte M, Miniati I, Ananieva L, Gurman AB, Damjanov N, Mueller A, Valentini G, Riemekasten G, Tikly M, Hummers L, Henriques MJ, Caramaschi P, Scheja A, Rozman B, Ton E, Kumánovics G, Coleiro B, Feierl E, Szucs G, Von Mühlen CA, Riccieri V, Novak S, Chizzolini C, Kotulska A, Denton C, Coelho PC, Kötter I, Simsek I, de la Pena Lefebvre PG, Hachulla E, Seibold JR, Rednic S, Štork J, Morovic-Vergles J, Walker UA. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15.PubMedCrossRef
138.
go back to reference Bergamasco A, Hartmann N, Wallace L, Verpillat P. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol. 2019;11:257–73.PubMedPubMedCentralCrossRef Bergamasco A, Hartmann N, Wallace L, Verpillat P. Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease. Clin Epidemiol. 2019;11:257–73.PubMedPubMedCentralCrossRef
140.
go back to reference Goh NS, Hoyles RK, Denton CP, Hansell DM, Renzoni EA, Maher TM, Nicholson AG, Wells AU. Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol. 2017;69:1670–8.PubMedCrossRef Goh NS, Hoyles RK, Denton CP, Hansell DM, Renzoni EA, Maher TM, Nicholson AG, Wells AU. Short-term pulmonary function trends are predictive of mortality in interstitial lung disease associated with systemic sclerosis. Arthritis Rheumatol. 2017;69:1670–8.PubMedCrossRef
141.
go back to reference Man A, Davidyock T, Ferguson LT, Ieong M, Zhang Y, Simms RW. Changes in forced vital capacity over time in systemic sclerosis: application of group-based trajectory modelling. Rheumatology (Oxford). 2015;54:1464–71.CrossRef Man A, Davidyock T, Ferguson LT, Ieong M, Zhang Y, Simms RW. Changes in forced vital capacity over time in systemic sclerosis: application of group-based trajectory modelling. Rheumatology (Oxford). 2015;54:1464–71.CrossRef
142.
go back to reference Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner R-G, Schlenker-Herceg R, Brown KK. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381:1718–27.CrossRef Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner R-G, Schlenker-Herceg R, Brown KK. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381:1718–27.CrossRef
143.
go back to reference Goldberg HJ. Understanding progressive fibrosing interstitial lung disease through therapeutic trials. N Engl J Med. 2019;381:1775–7.CrossRef Goldberg HJ. Understanding progressive fibrosing interstitial lung disease through therapeutic trials. N Engl J Med. 2019;381:1775–7.CrossRef
144.
go back to reference Torrisi SE, Kahn N, Wälscher J, Sarmand N, Polke M, Lars K, Eichinger M, Heussel CP, Palmucci S, Sambataro FM, Sambataro G, Sambataro D, Vancheri C, Kreuter M. Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases. BMC Pulm Med. 2019;19:213.PubMedPubMedCentralCrossRef Torrisi SE, Kahn N, Wälscher J, Sarmand N, Polke M, Lars K, Eichinger M, Heussel CP, Palmucci S, Sambataro FM, Sambataro G, Sambataro D, Vancheri C, Kreuter M. Possible value of antifibrotic drugs in patients with progressive fibrosing non-IPF interstitial lung diseases. BMC Pulm Med. 2019;19:213.PubMedPubMedCentralCrossRef
Metadata
Title
Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme
Authors
Claudia Valenzuela
Sebastiano Emanuele Torrisi
Nicolas Kahn
Manuel Quaresma
Susanne Stowasser
Michael Kreuter
Publication date
01-12-2020
Publisher
BioMed Central
Published in
Respiratory Research / Issue 1/2020
Electronic ISSN: 1465-993X
DOI
https://doi.org/10.1186/s12931-019-1269-6

Other articles of this Issue 1/2020

Respiratory Research 1/2020 Go to the issue
Obesity Clinical Trial Summary

At a glance: The STEP trials

A round-up of the STEP phase 3 clinical trials evaluating semaglutide for weight loss in people with overweight or obesity.

Developed by: Springer Medicine

Highlights from the ACC 2024 Congress

Year in Review: Pediatric cardiology

Watch Dr. Anne Marie Valente present the last year's highlights in pediatric and congenital heart disease in the official ACC.24 Year in Review session.

Year in Review: Pulmonary vascular disease

The last year's highlights in pulmonary vascular disease are presented by Dr. Jane Leopold in this official video from ACC.24.

Year in Review: Valvular heart disease

Watch Prof. William Zoghbi present the last year's highlights in valvular heart disease from the official ACC.24 Year in Review session.

Year in Review: Heart failure and cardiomyopathies

Watch this official video from ACC.24. Dr. Biykem Bozkurt discuss last year's major advances in heart failure and cardiomyopathies.