Idiopathic pulmonary fibrosis prognostic factors – analysis of the Czech registry
Authors:
Martina Doubková 1; Michal Uher 2; Vladimír Bartoš 3; Martina Šterclová 4; Ladislav Lacina 5; Vladimíra Lošťáková 6; Ilona Binková 1; Martina Plačková 7; Monika Žurková 6; Radka Bittenglová 8; Jana Pšikalová 9; Lenka Šišková 10; Pavlína Lisá 11; František Petřík 11; Jaroslav Polák 12; Vladimír Řihák 10; Jana Skřičková 1; Martina Vašáková 4
Authors‘ workplace:
Klinika nemocí plicních a TBC LF MU a FN Brno
1; Institut biostatistiky a analýz LF MU Brno
2; Plicní klinika LF UK a FN Hradec Králové
3; Pneumologická klinika 1. LF UK a Thomayerovy nemocnice v Praze
4; Klinika pneumologie a hrudní chirurgie Nemocnice Na Bulovce v Praze
5; Klinika plicních nemocí a tuberkulózy LF UP a FN Olomouc
6; Klinika plicních nemocí a tuberkulózy LF OU a FN Ostrava
7; Klinika pneumologie a ftizeologie LF UK a FN Plzeň
8; Pneumologicko-alergologické oddělení Kroměřížské nemocnice
9; Plicní oddělení Krajské nemocnice T. Bati Zlín
10; Pneumologická klinika 2. LF UK a FN Motol v Praze
11; Dopravní zdravotnictví a. s., Nemocnice s poliklinikou Praha Italská
12
Published in:
Čas. Lék. čes. 2016; 155: 188-194
Category:
Original Article
Overview
Idiopathic pulmonary fibrosis (IPF) is a rare, progressive and usually fatal form of idiopathic interstitial pneumonia. IPF is characterized by failure of alveolar re-epithelization, persistence of fibroblasts, deposition of extracellular matrix, and distortion of lung architecture, which ultimately results in respiratory failure.
We analysed 202 consecutive patients with IPF diagnosed at the Departments of Pulmonary Diseases and Tuberculosis in the Czech Republic, who they were included in the nationwide Czech IPF registry. Our aim was to determine prognostic factors of IPF and outcome of the disease.
There were 129 males and 73 females who were the median age 67 years. IPF was biopsy-proven in 66 (33 %) of patients. Median time from the first symptom to diagnosis was 12 months. Diagnosis was made in 57 patients (28.3 %) within 6 months from the onset of respiratory symptoms. 8 (4 %) patients had an acute exacerbation during the course of the disease.
In uniparametric (univariate) analysis as prognostic factors associated with poorer survival were found: higher age, higher degree dyspnea scores, clubbing fingers, comorbidities (arterial hypertension, osteoporosis), patients without histology biopsy, and bronchoalveolar increased neutrophil count. We found these positive prognostic factors: higher levels of VC (vital capacity), TLC (total lung capacity) and DLCO (diffusing capacity for carbon monooxide).
In multiparametric (multivariate) analysis as prognostic factors associated with mortality were found: higher age, higher degree of dyspnoe score. Increased lymphocytes in bronchoalveolar fluid, higher level of VC a DLCO were associated with better survival. There was no difference in survival of patients by sex, by smoking status. No significant difference in survival rates was found between IPF with and without emphysema, between the extent of fibrosis on HRCT (high resolution computed tomography) of thorax and mortality. Median survival was 51.6 months. 58 (28.7 %) patients died. The most frequent reason of dead was IPF progression with respiratory failure.
Key words:
Idiopathic pulmonary fibrosis; prognosis; treatment
Sources
1. Raghu G, Collard HR, Egan JJ et al. 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.
2. American Thoracic Society;European Respiratory Society. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2011. Am J Respir Crit Care Med 2002; 165: 277–304.
3. Vašáková M, Šterclová M. Idiopatická plicní fibróza – doporučený postup pro diagnózu, léčbu a sledovní (2. aktualizace). http://www.pneumologie.cz/guidelines
4. Fernández Pérez ER, Daniels CE, Schroeder DR et al. Incidence, prevalence and clinical course of idiopathic pulmonary fibrosis. Chest 2010; 137: 129–137.
5. Kolek V. Epidemiology of cryptogenic fibrosing alveolitis in Moravia and Silesia. Acta Palacki Olomuc Fac Med 1994; 137: 49–50.
6. Levinský L. Chronická difuzní intersticiální plicní fibróza (idiopatická). 1. sdělení. Čas Lék Čes 1953; 92: 976–981.
7. Miller MR, Hankinson J, Brusasco V et al. Standardisation of spirometry. Eur Respir J 2005; 26: 319–338.
8. Wanger J, Clausen JL, Coates A et al. Standardisation of the measurement of lung volumes. Eur Respir J 2005; 26: 511–522.
9. Macintyre N, Crapo JE, Viegi G et al. Standardisation of the single-breath determination of carbon monoxide uptáme in the lung. Eur Respir J 2005; 26: 720–735.
10. Gay SE, Kazerooni EA, Toews GB et al. Idiopathic pulmonary fibrosis – predicting response to therapy and survival. Am J Respir Crit Care Med 1998; 157: 1063–1072.
11. Šterclová M, Vašáková M. Kvantifikace rozsahu postižení u nemocných s fibrotizujícími intersticiálními procesy. Čes Radiol 2013; 67: 204–208.
12. Skřičková J, Kolaříková R. Standardní postup při provádění bronchoalveolární laváže (BAL) a vyšetřování bronchoalveolární tekutiny (BAT). http://www.pneumologie.cz/soubory/BAL_Standard_Studia_po_recenzi_8_2004.pdf
13. Raghu G, Weycker D, Edelsberg J et al. Incidence a prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 810–816.
14. Baumgartner KB, Samet JM, Stidley CA et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 1997; 155: 242–248.
15. Antoniou KM, Hansell DM, Rubens MB et al. Idiopathic pulmonary fibrosis outcome in relation to smoking status. Am J Respir Crit Care Med 2008; 177: 190–194.
16. Kim DS, Collard HR, King TE jr. et al. Classification and natural history of idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006; 3: 285–292.
17. Toovey OT, Eisenhauer HJ et al. A new hypothesis on the mechanism of digital clubbing secondary to pulmonary pathologies. Med Hypothese 2010; 75: 511–513.
18. Gribbin J, Hubbard R, Smith C et al. Role of diabetes mellitus and gastro-oesophageal reflux in the aetiology of idiopathic pulmonary fibrosis. Respir Med 2009; 103: 927–931.
19. Mura M, Poretta M, Bargagli E et al. Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study. Eur Respir J 2012; 40: 101–109.
20. Selman M, Carrillo G, Estrada A et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One 2007; 2: e482.
21. Noth I, Zhang Y, Ma SF et al. Genetic variants associated with idiopathic pulmonary fibrosis susceptibility and mortality: a genome-wide association study. Lancet Respir Med2013; 1: 309–317.
22. Barlo NP, van Moorsel Ch, van den Bosch et al. Predicting prognosis in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2010; 27: 85–95.
23. Martinez FJ, Safrin S, Weycker D et al. The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med 2005; 142: 963–967.
24. King TE jr., Tooze JA, Schwarz MI et al. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med 2001; 164: 1171–1181.
25. Carbone R, Balleari J, Grosso M et al. Predictors of mortality of idiopathic pulmonary fibrosis. Eur Rev Med Pharmacol Sci 2008; 12: 97–104.
26. Lee SH, Shim HS, Cho SH et al. Prognostic factors for idiopathic pulmonary fibrosis: clinical, physiologic, pathologic and molecular aspects. Sarcoidosis Vasc Diffuse Lung Dis 2011; 28: 102–112.
27. Doubková M, Binková I, Jančíková J et al. Jak včasná je diagnostika idiopatické plicní fibrózy a jak úspěšná je její terapie? Stud Pneumol Phtiseol 2007: 67: 113–119.
28. Nadrous HF, Myers JL, Decker PA et al. Idiopathic pulmonary fibrosis in patients younger than 50 years. Mayo Clin Proc 2005; 80: 37–40.
29. Collared HR, King TE JR, Bartelson BB et al. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2003; 168: 538–542.
30. Jegal Y, Kim DS, Shim TS et al. Physiology is a stronger predictor of survival than patology in fibrotic interstitial pneumonia. Am J Respir Crit Care Med 2005; 171: 639–644.
31. Stephan S, de Castro Pereira, Coletta EM et al. Oxygen desaturation during a 4-minute step test: predicting survival in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 2007; 24: 70–76.
32. Miki K, Maekura R, Hiraga T et al. Prognosis of idiopathic pulmonary fibrosis. Respir Med 2003; 97: 482–490.
33. Flaherty KR, Andrei AC, Murray S et al. Idiopathic pulmonary fibrosis: prognostic value of changes in physiology and six-minute-walk test. Am J Respir Crit Care Med 2006; 174: 803–809.
34. Kinder BW, Brown KK, Schwarz MI et al. Baseline BAL neutrophilia predicts early mortality in idiopathic pulmonary fibrosis. Chest 2008; 133: 226–232.
35. Tabuena RP, Nagai S, Tsutsumi T et al. Cell profiles of bronchoalveolar lavage fluid as prognosticators of idiopathic pulmonary fibrosis/usual interstitial pneumonia among Japanese patients. Respiration 2005; 72: 490–498.
36. Veeraraghavan S, Latsi PI, Wells AU et al. BAL findings in idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia. Eur Respir J 2003; 22: 239–244.
37. Lettieri CJ, Nathan SD, Barnett SD et al. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 129; 746–752.
38. Alakhras M, Decker PA, Nadrou HF et al. Body mass index and mortality in patients with idiopathic pulmonary fibrosis. Chest 2007; 131: 1448–1453.
39. Wells AU, Desai SR, Rubens MB et al. Idiopathic pulmonary fibrosis. A Composite Physiologic Index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med 2003; 167: 962–969.
40. Cottin V, Nunes H, Brillet PY et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J 2005; 26: 586–593.
41. Kurashima K, Takayanagi N, Tsuchiya N et al. The effect of emphysema on lung function and survival in patients with idiopathic pulmonary fibrosis. Respirology 2010; 15: 843–848.
42. Mejía M, Carillo G, Rojas-Serrano J et al. Idiopathic pulmonary fibrosis and emphysema: decreased survival associated with severe pulmonary arterial hypertension. Chest 2009; 136: 10–15.
43. Sugino K, Ischida F, Kikuchi N et al. Comparison of clinical characteristics and prognostic factors of combined pulmonary fibrosis and emphysema versus idiopathic pulmonary fibrosis alone. Respirology 2014; 19: 239–245.
44. Lazor R, Bonetti A, Nicod LP et al. Acute exacerbations of idiopathic pulmonary fibrosis. Rev Med Suisse 2010; 6: 2228–2230, 2232.
45. Song JW, Hong SB, Lim CM et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir 2011; 37: 356–363.
46. Hyzy R, Huang S, Myers J et al. Acute exacerbation of idiopathic pulmonary fibrosis. Chest 2007; 132: 1652–1658.
47. Raghu G, Anstrom KJ, King TE et al. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012; 366: 1968–1977.
48. Martinez FJ, de Andrade JA, Anstrom KJ et al. Randomized trial of acetylcystein in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2093–2101.
49. King TE jr., Bradford JZ, Catro-Bernardini S et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2083–2092.
50. Ley B, Collard HR, King TE JR et al. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011; 183: 431–440.
51. Saydain G, Islam A, Afessa B et al. Outcome of patients with idiopathic pulmonary fibrosis admitted to the intensive care unit. Am J Respir Crit Care Med 2002; 166: 839–842.
52. Panos R, Mortenson RL, Nicolli SA. Clinical deterioration in patients with idiopathic pulmonary fibrosis: cause and assessment. Am J Med 1990; 88: 396–404.
Labels
Addictology Allergology and clinical immunology Angiology Audiology Clinical biochemistry Dermatology & STDs Paediatric gastroenterology Paediatric surgery Paediatric cardiology Paediatric neurology Paediatric ENT Paediatric psychiatry Paediatric rheumatology Diabetology Pharmacy Vascular surgery Pain management Dental HygienistArticle was published in
Journal of Czech Physicians
Most read in this issue
- The importance of HPV vaccination in men
- Dermatomyositis
- Functional assessment of coronary stenosis by fractional flow reserve
- Matrix Gla protein as natural inhibitor of vascular calcification and potential treatment target