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Idiopathic pulmonary fibrosis: Does the time for change of diagnostic and therapeutic recommendations come?

Česká verzia

Authors: Martina Vašáková
Authors place of work: Pneumologická klinika 1. LF UK a Thomayerovy nemocnice, Praha
Published in the journal: Čas. Lék. čes. 2018; 157: 237-243
Category: Review Article

Summary

Idiopathic pulmonary fibrosis (IPF) belongs to the most severe and difficult-to-treat lung diseases, with median survival 2–3 years if not treated. The disease arises in genetically disposed individuals of middle and older age based on repeated alveolar injuries. Fibroproliferative healing of these lesions form the pathogenetic background of the disease, leading to the end-stage pulmonary fibrosis.

Our knowledge of pathogenesis, diagnosis and treatment of IPF increases dynamically since the beginning of new millennium. Progressive breathlessness, crepitus and clubbed fingers are typical for diagnosis of IPF. Radiologic picture of IPF in high resolution CT imaging is characterized by usual interstitial pneumonia, however nearly half of the patients do not have this typical pattern. The careful evaluation of clinical picture, radiologic findings and additional investigations as bronchoalveolar lavage differential cell count and immunologic results, by multidisciplinary team is essential in these cases. In minority of the patients lung biopsy, either surgical or cryobiopsy, is recommended.

Only last 7 years causal treatment of IPF with antifibrotic drugs is available. Nevertheless, antifibrotic treatment only slows down the disease progression. Minor part of the patients with end-stage-diseased is referred for lung transplant. Palliative and symptomatic care is an integral part of IPF treatment.

Key words:

idiopathic pulmonary fibrosis, typical and atypical radiologic findings, cryobiopsy, antifibrotic treatment, lung transplantation, palliative care

Zdroje
  1. Raghu G, Collard HR, Egan JJ et al., on behalf of 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.
  2. Raghu G, Rochwerg B, Zhang Y et al., on behalf of ATS, ERS, JRS and ALAT. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis: executive summary. Am J Respir Crit Care Med 2015; 192(2): e3–e19.
  3. Fernández Pérez ER, Daniels CE, Schroeder DR et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest 2010; 137(1): 129–137.
  4. Hopkins RB, Burke N, Fell C et al. Epidemiology and survival of idiopathic pulmonary fibrosis from national data in Canada. Eur Respir J 2016; 48(1): 187–195.
  5. Olson AL, Swigris JJ. Idiopathic pulmonary fibrosis: diagnosis and epidemiology. Clin Chest Med 2012; 33(1): 41–50.
  6. Agabiti N, Porretta MA, Bauleo L et al. Idiopathic pulmonary fibrosis (IPF) incidence and prevalence in Italy. Sarcoidosis Vasc Diffuse Lung Dis 2014; 31(3): 191–197.
  7. Selman M, Pardo A. Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis. An integral model. Am J Respir Crit Care Med 2014; 189: 1161–1172.
  8. Savarino E, Carbone R, Marabotto E et al. Gastro-oesophageal reflux and gastric aspiration in idiopathic pulmonary fibrosis patients. Eur Respir J 2013; 42(5): 1322–1331.
  9. 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.
  10. Vašáková M. Idiopatická plicní fibróza. In Vašáková M., Polák J., Matěj R. Intersticiální plicní procesy (2., rozšířené vyd.). Maxdorf, Praha, 2016.
  11. Travis WD, Costabel U, Hansell DM et al.; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013; 188(6): 733–748.
  12. 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.
  13. Lynch DA, Godwin JD, Safrin S et al.; Idiopathic Pulmonary Fibrosis Study Group. High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med 2005; 172(4): 488–493.
  14. Vašáková M., Šterclová M, Sekce IPP ČPFS. Kapitola 6.1. Idiopatická plicní fibróza (Doporučený postup pro diagnózu léčbu a sledování). In: Kolek V a kol. Doporučené postupy v pneumologii. Maxdorf, Praha, 2017.
  15. Ohshimo S, Bonella F, Cui A et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179(11): 1043–1047.
  16. Chung JH, Lynch DA. The value of a multidisciplinary approach to the diagnosis of usual interstitial pneumonitis and idiopathic pulmonary fibrosis: radiology, pathology, and clinical correlation. AJR Am J Roentgenol 2016; 206(3): 463–471.
  17. Wells AU. Managing diagnostic procedures in idiopathic pulmonary fibrosis. Eur Respir Rev 2013; 22(128): 158–162.
  18. Xaubet A, Behr J, Bendstrup E et al. Review of IPF diagnosis and management recommendations in Europe. Sarcoidosis Vasc Diffuse Lung Dis 2013; 30(4): 249–261.
  19. Casoni GL, Tomassetti S, Cavazza A et al. Transbronchial lung cryobiopsy in the diagnosis of fibrotic interstitial lung diseases. PLoS One 2014; 9(2): e86716.
  20. 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.
  21. King TE Jr, Bradford WZ, Castro-Bernardini S et al.; ASCEND Study Group. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 22: 2083–2092.
  22. Nathan SD, Albera C, Bradford WZ et al. Effect of pirfenidone on mortality: pooled analyses and meta-analyses of clinical trials in idiopathic pulmonary fibrosis. Lancet Respir Med 2017; 5(1): 33–41.
  23. Hilberg O, Simonsen U, du Bois R et al. Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis. Clin Respir J 2012; 6(3): 131–143.
  24. Richeldi L, du Bois RM, Raghu G et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl. J Med 2014; 370: 2071–2082.
  25. 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.
  26. Suissa S, Ernst P. The INPULSIS enigma: exacerbations in idiopathic pulmonary fibrosis. Thorax 2015; 70(5): 508–510.
  27. Kreuter M, Wuyts W, Renzoni E et al. Antacid therapy and disease outcomes in idiopathic pulmonary fibrosis: a pooled analysis. Lancet Respir Med 2016; 4(5): 381–389.
  28. Thabut G, Christie JD, Ravaud P et al. Survival after bilateral versus single-lung transplantation for idiopathic pulmonary fibrosis. Ann Intern Med 2009; 151(11): 767–774.
  29. Mooney JJ, Raimundo K, Chang E et al. Mechanical ventilation in idiopathic pulmonary fibrosis: a nationwide analysis of ventilator use, outcomes, and resource burden. BMC Pulm Med 2017; 17: 84.
  30. Nishiyama O, Kondoh Y, Kimura T et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology 2008; 13(3): 394–399.
  31. Maher TM, Whyte MK, Hoyles RK et al. Development of a consensus statement for the definition, diagnosis, and treatment of acute exacerbations of idiopathic pulmonary fibrosis using the Delphi technique. Adv Ther 2015; 32(10): 929–943.
  32. Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183(4): 431–440.
  33. Kreuter M, Wijsenbeek MS, Vašáková M et al. Unfavourable effects of medically indicated oral anticoagulants on survival in idiopathic pulmonary fibrosis. Eur Respir J 2016; 47(6): 1776–1784.
  34. Doubková M, Švancara J, Svoboda M et al. EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis. Clin Respir J 2018; 12(4): 1526–1535.
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Journal of Czech Physicians

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