Idiopathic pulmonary fibrosis – changes in dia­gnostics and treatment

Authors: Vašáková M.
Authors‘ workplace: Pneumologická klinika 1. LF UK a Thomayerovy nemocnice, Praha
Published in: Kardiol Rev Int Med 2019, 21(3): 135-141


Idiopathic pulmonary fibrosis (IPF) belongs to the most difficult-to-treat and most serious of lung diseases, with prognosis similar to lung cancer. Epidemiologic data are highly probably underestimated in many countries since IPF used to be frequently misdia­gnosed for other dia­gnoses or not dia­gnosed at all. In the last century, when described for the first time, IPF was an extremely rare dia­gnosis. Nowadays, the incidence of IPF is undoubtedly rising, which is also sup­ported by improved dia­gnostic opportunities. Previously, IPF was considered an untreatable disease; however, this has changed in the last eight years as antifibrotic therapy has emerged. Due to this fact, IPF patients have a better chance of survival, although the disease is still uncurable.


idiopathic pulmonary fibrosis – dia­gnosis


1. American Thoracic Society, European Respiratory SocietyAmerican Thoracic Society. Idiopathic pulmonary fibrosis: dia­gnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000; 161(2 Pt 1): 646–664. doi: 10.1164/ ajrccm.161.2.ats3-00.

2. Raghu G, Collard HR, Egan JJ et al. ATS/ ERS/ JRS/ ALAT Committee on Idiopathic Pulmonary Fibrosis. 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(6): 788–824. doi: 10.1164/ rccm.2009-040GL.

3. Demedts M, Behr J, Buhl R et al. IFIGENIA Study Group. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005; 353(21): 2229–2242. doi: 10.1056/ NEJMoa042976.

4. McGrath EE, Millar AB. Hot off the breath: triple therapy for idiopathic pulmonary fibrosis-hear the PANTHER roar. Thorax 2012; 67(2): 97–98. doi: 10.1136/ thoraxjnl-2011-201398.

5. Noble PW, Albera C, Bradford WZ et al. CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377(9779): 1760–1769. doi: 10.1016/ S0140-6736(11)60405-4.

6. King TE Jr, Bradforf WB, Castro­Bernardini S et al. ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370(22): 2083–2092. doi: 10.1056/ NEJMoa1402582.

7. Raghu G, Rochwerg B, Zhang Y et al. American Thoracic Society; European Respiratory society; Japanese Respiratory Society; Latin American Thoracic Association. 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(2): e3–e19. doi: 10.1164/ rccm.201506-1063ST.

8. Lynch DA, Sverzellati N, Travis WD et al. Dia­gnostic criteria for idiopathic pulmonary fibrosis: a Fleisch­ner Society White Paper. Lancet Respir Med 2018; 6(2): 138–153. doi: 10.1016/ S2213-2600(17)30433-2.

9. Raghu G, Remy-Jardin M, Myers JL et al. American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Dia­gnosis of idiopathic pulmonary fibrosis. An Official ATS/ ERS/ JRS/ ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2018; 198(5): e44–e68. doi: 10.1164/ rccm.201807-1255ST.

10. Raghu G, Chen SY, Hou Q et al. Incidence and prevalence of idiopathic pulmonary fibrosis in US adults 18–64 years old. Eur Respir J 2016; 48(1): 179–186. doi: 10.1183/ 13993003.01653-2015.

11. Homolka J, Altmann V, Votava V. Increasing prevalence of idiopathic pulmonary fibrosis in the Czech Republic. Chest 1999; 116 (Suppl 2): 155.

12. European MultiPartner IPF Registry. Available at: http:/ / index-en.php.

13. Ravaglia C, Tomassetti S, Gurioli C et al. Features and outcome of familial idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2014; 31(1): 28–36.

14. Dove EP, Olson AL, Glassberg MK. Trends in IPF-related mortality in the United States: 2000–2017. Am J Respir Crit Care Med 2019. doi: 10.1164/ rccm.201905-0958LE.

15. Kropski JA, Blackwell TS, Loyd JE. The genetic basis of idiopathic pulmonary fibrosis. Eur Respir J 2015; 45(6): 1717–1727. doi: 10.1183/ 09031936.00163814.

16. Peljto AL, Zhang Y, Fingerlin TE et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA 2013; 309(21): 2232–2239. doi: 10.1001/ jama.2013.5827.

17. Wolters PJ, Blackwell TS, Eickelberg O et al. Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? Lancet Respir Med 2018; 6(2): 154–160. doi: 10.1016/ S2213-2600(18)30007-9.

18. Doporučené postupy. Dostupné na:

19. Lentz RJ, Argento AC, Colby TV et al. Trans­bronchial cryobio­psy for diffuse parenchymal lung disease: a state-of-the-art review of procedural techniques, current evidence, and future chal­lenges. J Thorac Dis 2017; 9(7): 2186–2203. doi: 10.21037/ jtd.2017.06.96.

20. Iftikhar IH, Alghothani L, Sardi A et al. Transbronchial lung cryobio­psy and video-assisted thoracoscopic lung bio­psy in the dia­gnosis of diffuse parenchymal lung disease. A Meta-analysis of Dia­gnostic Test Accuracy. Ann Am Thorac Soc 2017; 14(7): 1197–1211. doi: 10.1513/ AnnalsATS.201701-086SR.

21. Richeldi L, du Bois RM, Raghu G et al. INPULSIS Trial Investigators. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370(22): 2071–2082. doi: 10.1056/ NEJMoa1402584.

22. Oldham JM, Ma SF, Martinez FJ et al. IPFnet Investigators.TOLLIP, MUC5B, and the Response to N-Acetylcysteine among Individuals with Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2015; 192(12): 1475–1482. doi: 10.1164/ rccm.201505-1010OC.

23. Swigris JJ, Brown KK, Make BJ et al. Pulmonary rehabilitation in idiopathic pulmonary fibrosis: a call for continued investigation. Respir Med 2008; 102(12): 1675–1680. doi: 10.1016/ j.rmed.2008.08.014.

24. Standard Akutní exacerbace IPP v Sekci pro intenzivní pneumologii ČPFS. Dostupné na: http: / / stranka/ 58/ sekce-pro-intenzivni-pneumologii.

25. Tomassetti S, Ryu JH, Poletti V. Staging systems and disease severity assessment in interstitial lung diseases. Curr Opin Pulm Med 2015; 21(5): 463–439. doi: 10.1097/ MCP.0000000000000198.

26. Organ LA, Duggan AR, Oballa E et al. Biomarkers of collagen synthesis predict progression in the PROFILE idiopathic pulmonary fibrosis cohort. Respir Res 2019; 20(1): 148. doi: 10.1186/ s12931-019-1118-7.

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