#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Idiopatic pulmonary fibrosis – news in multidisciplinary diagnostic and therapeutic approaches


Authors: Martina Vašáková 1;  Radoslav Matěj 2,3
Authors‘ workplace: Pneumologická klinika 1. LF UK, Thomayerova nemocnice, Praha 1;  Oddělení patologie a molekulární medicíny, Thomayerova nemocnice, Praha 2;  Ústav patologie 1. LF UK a VFN, Praha 3
Published in: Čes.-slov. Patol., 52, 2016, No. 2, p. 85-92
Category:

Idiopatická plicní fibróza (IPF) je primárně fibrotizujícím plicním procesem a je vzhledem k refrakternosti na dosud známou léčbu a špatné prognóze nejzávažnějším reprezentantem idiopatických intersticiálních pneumonií (IIP). Etiologie a patogeneze onemocnění není zatím plně objasněna, ale předpokládá se, že příčinou fibroproliferace jako odpovědi na neznámý inzult může být nerovnováha reparačních a imunitních dějů u geneticky disponovaného jedince středního a staršího věku.

Overview

Idiopathic pulmonary fibrosis (IPF) is a primary fibrosing pulmonary process. Due to the ineffectiveness of current therapeutic strategies and unfavorable prognosis, IPF is the most serious example of idiopathic interstitial lung diseases (ILD). Etiology and pathogenesis of this disorder have not been fully clarified yet; but it is anticipated, that the fibroproliferation is caused by the imbalance of reparative and immunologic processes in the genetically predisposed patients. Radiologically and histopathologically, IPF is characterized by specific pattern called usual interstitial pneumonia (UIP), however, this pattern is not fully typical in all cases, and, moreover, it could be seen in other ILD´s, e.g. chronic hypersensitivity pneumonitis, asbestosis, autoimmune connective tissue diseases and many others as well. The final diagnosis of IPF is a consensual result of multidisciplinary team composed of pulmologist, pathologist and radiologist. IPF was an incurable disease with prognosis worse than cancer till the year 2011, when antifibrotic drugs decelerating a progression of this disease have been introduced. Earlier and correct diagnosis of IPF is the most important issue for the patients because they could be effectively treated and thus, prolonging their survival as much as possible.

Keywords:
fibrosis – pulmonary – histopathology – diagnosis


Sources

1. Olson J, Colby TV, Elliott CG. Hamman-Rich syndrome revisited. Mayo Clin Proc. 1990; 65: 1538–1548.

2. Maffessanti M, Dalpiaz G. Diffuse Lung Diseases. Clinical Features, Pathology, HRCT. Springer, 2006.

3. Nalysnyk L, Cid-Ruzafa J, Rotella P, Esser D. Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature. Eur Respir Rev 2012; (21)126: 355-361.

4. Spagnolo P, Sverzellati N, Rossi G et al. Idiopathic pulmonary fibrosis: an update. Ann Med 2015; 47(1): 15-27.

5. Helling BA, Yang IV. Epigenetics in lung fibrosis: from pathobiology to treatment perspective. Curr Opin Pulm Med 2015; 21(5): 454-462.

6. Molyneaux PL, Maher TM. The role of infection in the pathogenesis of idiopathic pulmonary fibrosis. Eur Respir Rev 2013; 22(129): 376-381.

7. Molyneaux PL, Cox MJ, Willis-Owen SA et al. The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2014; 190(8): 906-913.

8. Folcik VA, Garofalo M, Coleman J et al. Idiopathic pulmonary fibrosis is strongly associated with productive infection by herpesvirus saimiri. Mod Pathol 2014; 27(6): 851-862.

9. Spagnolo P, Rossi G, Cavazza A et al. Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications. Expert Rev Clin Immunol 2014; 10(8): 1005-1017.

10. Borensztajn K, Crestani B, Kolb M. Idiopathic pulmonary fibrosis: from epithelial injury to biomarkers--insights from the bench side. Respiration 2013; 86(6): 441-452.

11. Wuyts WA, Agostini C, Antoniou KM et al. The pathogenesis of pulmonary fibrosis: a moving target. Eur Respir J 2013; 41: 1207-1218.

12. 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 Med 2013; 1(4): 309-317.

13. Putman RK, Rosas IO, Hunninghake GM. Genetics and early detection in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2014; 189(7): 770-778.

14. Hambly N Shimbori C, Kolb M. Molecular classification of idiopathic pulmonary fibrosis: personalized medicine, genetics and biomarkers. Respirology 2015; 20(7): 1010-1022.

15. Habiel DM, Hogaboam C. Heterogeneity in fibroblast proliferation and survival in idiopathic pulmonary fibrosis. Front Pharmacol 2014; 5: 2.

16. Ley B. Brown KK, Collard HR. Molecular biomarkers in idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 2014; 307(9): 681-691.

17. Fernandez IE, Eickelberg O. The impact of TGF-β on lung fibrosis: from targeting to biomarkers. Proc Am Thorac Soc 2012; 9(3): 111-116.

18. Gu H, Mickler EA, Cummings OW, et al. Crosstalk between TGF-β1 and complement activation augments epithelial injury in pulmonary fibrosis. FASEB J 2014; 28(10): 4223-4234.

19. Vasakova M, Sterclova M, Matej R et al. IL-4 polymorphisms, HRCT score and lung tissue markers in idiopathic pulmonary fibrosis. Hum Immunol 2013; 74(10): 1346-1351.

20. Nishioka Y, Azuma M, Kishi M, Aono Y. Targeting platelet-derived growth factor as a therapeutic approach in pulmonary fibrosis. J Med Invest 2013; 60(3-4): 175-183.

21. Thannickal VJ, Henke CA, Horowitz JC et al. Matrix biology of idiopathic pulmonary fibrosis: a workshop report of the national heart, lung, and blood institute. Am J Pathol 2014; 184(6): 1643-1651.

22. Redente EF, Keith RC, Janssen W et al. Tumor necrosis factor-α accelerates the resolution of established pulmonary fibrosis in mice by targeting profibrotic lung macrophages. Am J Respir Cell Mol Biol 2014; 50(4): 825-837.

23. Jakubzick C, Choi ES, Carpenter KJ et al. Human pulmonary fibroblasts exhibit altered interleukin-4 and interleukin-13 receptor subunit expression in idiopathic interstitial pneumonia. Am J Pathol 2004; 164(6): 1989-2001.

24. Estany S, Vicens-Zygmunt V, Llatjós R et al. Lung fibrotic tenascin-C upregulation is associated with other extracellular matrix proteins and induced by TGFβ1. BMC Pulm Med 2014; 14: 120.

25. Oruqaj G, Karnati S, Vijayan V et al. Compromised peroxisomes in idiopathic pulmonary fibrosis, a vicious cycle inducing a higher fibrotic response via TGF-β signaling. Proc Natl Acad Sci U S A 2015; 112(16): 2048-2057.

26. Marchand-Adam S, Fabre A, Mailleux AA et al. Defect of pro-hepatocyte growth factor activation by fibroblasts in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174(1): 58-66.

27. Hanumegowda C, Farkas L, Kolb M. Angiogenesis in pulmonary fibrosis: too much or not enough? Chest 2012; 142(1): 200-207.

28. Montes E, Ruiz V, Checa M et al. Renin is an angiotensin-independent profibrotic mediator: role in pulmonary fibrosis. Eur Respir J 2012; 39(1): 141-148.

29. Ajayi IO, Sisson TH, Higgins PD et al. X-linked inhibitor of apoptosis regulates lung fibroblast resistance to Fas-mediated apoptosis. Am J Respir Cell Mol Biol 2013; 49(1): 86-95.

30. 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.

31. Leung J, Cho Y, Lockey RF, Kolliputi N. The Role of Aging in Idiopathic Pulmonary Fibrosis. Lung 2015; 193(4): 605-610.

32. Yanai H, Shteinberg A, Porat Z et al. Cellular senescence-like features of lung fibroblasts derived from idiopathic pulmonary fibrosis patients. Aging (Albany NY) 2015; 7(9): 664-672.

33. 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(6): 788-824.

34. Travis WD, Costabel U, Hansell DM et al. 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

35. Raghu G, Rochwerg B, Zhang Y et al. 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): e3-19.

36. Gotway MB, Freemer MM, King TE Jr. Challenges in pulmonary fibrosis. The use of high resolution CT scanning of the lung for the evaluation of patients with idiopathic interstitial pneumonias. Thorax 2007; 62: 546–553.

37. Stern EJ, Swensen JS, Kanne PJ. High-Resolution CT of the Chest. Lippincott Williams, Wilkins, 2009.

38. Tomassetti S, Wells AU, Costabel U et al. Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med 2015; Nov 12. In press.

39. Katzenstein AL, Myers JL. Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med 1998; 157: 1301–1315.

40. Leslie KO. Historical perspective. A pathologic approach to the classification of idiopathic interstitial pneumonias. Chest 2005; 128(5): 513–519.

41. McGrath EE, Millar AB. Hot off the breath: triple therapy for idiopathic pulmonary fibrosis-hear the PANTHER roar. Thorax 2012; 67(2): 97-98.

42. Maher TM. Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment. Clin Chest Med 2012; 33(1): 69-83.

43. Ferreira A, Garvey C, Connors GL, et al. Pulmonary rehabilitation in interstitial lung disease: benefits and predictors of response. Chest 2009; 135: 442–447.

44. Mura M, Porretta MA, Bargagli E, et al. Predicting survival in newly diagnosed idiopathic pulmonary fibrosis: a 3-year prospective study. Eur Respir J 2012; 40: 101-109.

45. Du Bois, RM. Albera C, Bradford WZ et al. 6-minute walk distance is an independent predictor of mortality in patients with idiopathic pulmonary fibrosis. Eur Respir J 2014; 43: 1421-1429.

Labels
Anatomical pathology Pneumology and ftiseology General practitioner for adults Radiodiagnostics Forensic medical examiner Toxicology
Topics Journals
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#