Hilberg O, Hoffmann-Vold AM, Smith V, Bouros D, Kilpelainen M, Guiot J, et al. Epidemiology of interstitial lung diseases and their progressive-fibrosing behaviour in six European countries. ERJ Open Res. 2022;8(1):00597–2021.

Article  PubMed  PubMed Central  Google Scholar 

Podolanczuk AJ, Thomson CC, Remy-Jardin M, Richeldi L, Martinez FJ, Kolb M, et al. Idiopathic pulmonary fibrosis: state of the art for 2023. Eur Respir J. 2023;61(4):2200957.

Article  PubMed  Google Scholar 

Guenther A, Krauss E, Tello S, Wagner J, Paul B, Kuhn S, et al. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res. 2018;19(1):141.

Article  PubMed  PubMed Central  Google Scholar 

Jegal Y, Park JS, Kim SY, Yoo H, Jeong SH, Song JW, et al. Clinical features, diagnosis, management, and outcomes of idiopathic pulmonary fibrosis in Korea: analysis of the Korea IPF cohort (KICO) registry. Tubercul Respir Dis. 2022;85(2):185–94.

Article  Google Scholar 

Homma S, Suda T, Hongo Y, Yoshida M, Hiroi S, Iwasaki K, et al. Incidence and changes in treatment of acute exacerbation of idiopathic pulmonary fibrosis in Japan: a claims-based retrospective study. Respir Investig. 2022;60(6):798–805.

Article  PubMed  Google Scholar 

Zaman T, Lee JS. Risk factors for the development of idiopathic pulmonary fibrosis: a review. Curr Pulmonol Rep. 2018;7(4):118–25.

Article  PubMed  PubMed Central  Google Scholar 

Spagnolo P, Cottin V. Genetics of idiopathic pulmonary fibrosis: from mechanistic pathways to personalised medicine. J Med Genet. 2017;54(2):93–9.

Article  PubMed  Google Scholar 

Kropski JA, Blackwell TS. Progress in understanding and treating idiopathic pulmonary fibrosis. Annu Rev Med. 2019;70:211–24.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Fernandez IE, Eickelberg O. The impact of TGF-beta on lung fibrosis: From targeting to biomarkers. Proc Am Thorac Soc. 2012;9(3):111–6.

Article  CAS  PubMed  Google Scholar 

Munger JS, Huang X, Kawakatsu H, Griffiths MJ, Dalton SL, Wu J, et al. The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis. Cell. 1999;96(3):319–28.

Article  CAS  PubMed  Google Scholar 

John AE, Graves RH, Pun KT, Vitulli G, Forty EJ, Mercer PF, et al. Translational pharmacology of an inhaled small molecule alphavbeta6 integrin inhibitor for idiopathic pulmonary fibrosis. Nat Commun. 2020;11(1):4659.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Mora AL, Rojas M, Pardo A, Selman M. Emerging therapies for idiopathic pulmonary fibrosis, a progressive age-related disease. Nat Rev Drug Discov. 2017;16(11):755–72.

Article  CAS  PubMed  Google Scholar 

Borok Z, Horie M, Flodby P, Wang H, Liu Y, Ganesh S, et al. Grp78 loss in epithelial progenitors reveals an age-linked role for endoplasmic reticulum stress in pulmonary fibrosis. Am J Respir Crit Care Med. 2020;201(2):198–211.

Article  PubMed  PubMed Central  Google Scholar 

Yue YL, Zhang MY, Liu JY, Fang LJ, Qu YQ. The role of autophagy in idiopathic pulmonary fibrosis: from mechanisms to therapies. Ther Adv Respir Dis. 2022;16:17534666221140972.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Pardo A, Cabrera S, Maldonado M, Selman M. Role of matrix metalloproteinases in the pathogenesis of idiopathic pulmonary fibrosis. Respir Res. 2016;17:23.

Article  PubMed  PubMed Central  Google Scholar 

Flaherty KR, Colby TV, Travis WD, Toews GB, Mumford J, Murray S, et al. Fibroblastic foci in usual interstitial pneumonia: idiopathic versus collagen vascular disease. Am J Respir Crit Care Med. 2003;167(10):1410–5.

Article  PubMed  Google Scholar 

Vuga LJ, Ben-Yehudah A, Kovkarova-Naumovski E, Oriss T, Gibson KF, Feghali-Bostwick C, et al. WNT5A is a regulator of fibroblast proliferation and resistance to apoptosis. Am J Respir Cell Mol Biol. 2009;41(5):583–9.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Zhou Y, Huang X, Hecker L, Kurundkar D, Kurundkar A, Liu H, et al. Inhibition of mechanosensitive signaling in myofibroblasts ameliorates experimental pulmonary fibrosis. J Clin Invest. 2013;123(3):1096–108.

Article  CAS  PubMed  PubMed Central  Google Scholar 

LeBleu VS, Neilson EG. Origin and functional heterogeneity of fibroblasts. FASEB J. 2020;34(3):3519–36.

Article  CAS  PubMed  Google Scholar 

Martinez FJ, Collard HR, Pardo A, Raghu G, Richeldi L, Selman M, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.

Article  PubMed  Google Scholar 

Ikenaga N, Peng ZW, Vaid KA, Liu SB, Yoshida S, Sverdlov DY, et al. Selective targeting of lysyl oxidase-like 2 (LOXL2) suppresses hepatic fibrosis progression and accelerates its reversal. Gut. 2017;66(9):1697–708.

Article  CAS  PubMed  Google Scholar 

Barry-Hamilton V, Spangler R, Marshall D, McCauley S, Rodriguez HM, Oyasu M, et al. Allosteric inhibition of lysyl oxidase-like-2 impedes the development of a pathologic microenvironment. Nat Med. 2010;16(9):1009–17.

Article  CAS  PubMed  Google Scholar 

Aumiller V, Strobel B, Romeike M, Schuler M, Stierstorfer BE, Kreuz S. Comparative analysis of lysyl oxidase (like) family members in pulmonary fibrosis. Sci Rep. 2017;7(1):149.

Article  PubMed  PubMed Central  Google Scholar 

Chien JW, Richards TJ, Gibson KF, Zhang Y, Lindell KO, Shao L, et al. Serum lysyl oxidase-like 2 levels and idiopathic pulmonary fibrosis disease progression. Eur Respir J. 2014;43(5):1430–8.

Article  CAS  PubMed  Google Scholar 

Raghu G, Brown KK, Collard HR, Cottin V, Gibson KF, Kaner RJ, et al. Efficacy of simtuzumab versus placebo in patients with idiopathic pulmonary fibrosis: a randomised, double-blind, controlled, phase 2 trial. Lancet Respir Med. 2017;5(1):22–32.

Article  CAS  PubMed  Google Scholar 

Xiong L, Xiong L, Ye H, Ma WL. Animal models of rheumatoid arthritis-associated interstitial lung disease. Immun Inflamm Dis. 2021;9(1):37–47.

Article  CAS  PubMed  Google Scholar 

Duerr J, Leitz DHW, Szczygiel M, Dvornikov D, Fraumann SG, Kreutz C, et al. Conditional deletion of Nedd4-2 in lung epithelial cells causes progressive pulmonary fibrosis in adult mice. Nat Commun. 2020;11(1):2012.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Moore BB, Lawson WE, Oury TD, Sisson TH, Raghavendran K, Hogaboam CM. Animal models of fibrotic lung disease. Am J Respir Cell Mol Biol. 2013;49(2):167–79.

Article  CAS  Google Scholar 

Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–40.

Article  PubMed  Google Scholar 

Brown KK, Flaherty KR, Cottin V, Raghu G, Inoue Y, Azuma A, et al. Lung function outcomes in the INPULSIS((R)) trials of nintedanib in idiopathic pulmonary fibrosis. Respir Med. 2019;146:42–8.

Article  PubMed  Google Scholar 

Fainberg HP, Oldham JM, Molyneau PL, Allen RJ, Kraven LM, Fahy WA, et al. Forced vital capacity trajectories in patients with idiopathic pulmonary fibrosis: a secondary analysis of a multicentre, prospective, observational cohort. Lancet Digit Health. 2022;4(12):e862–72.

Article  CAS  PubMed  Google Scholar 

Herrmann FE, Hesslinger C, Wollin L, Nickolaus P. BI 1015550 is a PDE4B inhibitor and a clinical drug candidate for the oral treatment of idiopathic pulmonary fibrosis. Front Pharmacol. 2022;13:838449.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Spagnolo P, Bonella F. Trial of a phosphodiesterase 4 inhibitor for idiopathic pulmonary fibrosis. N Engl J Med. 2022;387(8):761–2.

Article  PubMed  Google Scholar 

Nolan CM, Polgar O, Schofield SJ, Patel S, Barker RE, Walsh JA, et al. Pulmonary rehabilitation in idiopathic pulmonary fibrosis and COPD: a propensity-matched real-world study. Chest. 2022;161(3):728–37.

Article  PubMed  Google Scholar 

Richeldi L, Kolb M, Jouneau S, Wuyts WA, Schinzel B, Stowasser S, et al. Efficacy and safety of nintedanib in patients with advanced idiopathic pulmonary fibrosis. BMC Pulm Med. 2020;20(1):3.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Costabel U, Albera C, Glassberg MK, Lancaster LH, Wuyts WA, Petzinger U, et al. Effect of pirfenidone in patients with more advanced idiopathic pulmonary fibrosis. Respir Res. 2019;20(1):55.

Article  PubMed  PubMed Central