Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
권호 표지
DOI QR코드

DOI QR Code

Recent Advances in Idiopathic Pulmonary Fibrosis

  • Park, Sanghoon (Department of Internal Medicine, KEPCO Medical Center) ;
  • Lee, Eun Joo (Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University College of Medicine)
  • Received : 2012.12.21
  • Accepted : 2012.12.28
  • Published : 2013.01.30
Download PDF
⟨ Previous Next ⟩

Abstract

The concept on idiopathic pulmonary fibrosis (IPF) pathogenesis has progressed from chronic inflammation to aberrant wounding healing and even more to the current paradigms of a multifactorial and heterogeneous disease process. Despite the growth of clinical trials for IPF, most of the results, including N-acetylcysteine combination, warfarin, and bosentan, were disappointing. On the other hand, there have been a number of important developments; the foremost is the licensing of pirfenidone in Europe and Asia. In this article, we briefly review the recent knowledge of pathogenesis of IPF. We also summarize the recent clinical trials regarding the management of IPF.

Keywords

References

  1. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, 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. https://doi.org/10.1164/rccm.2009-040GL
  2. King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet 2011;378:1949-61. https://doi.org/10.1016/S0140-6736(11)60052-4
  3. Ding Q, Luckhardt T, Hecker L, Zhou Y, Liu G, Antony VB, et al. New insights into the pathogenesis and treatment of idiopathic pulmonary fibrosis. Drugs 2011;71: 981-1001. https://doi.org/10.2165/11591490-000000000-00000
  4. Fernandez IE, Eickelberg O. New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis. Lancet 2012;380:680-8. https://doi.org/10.1016/S0140-6736(12)61144-1
  5. Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012;366: 1968-77. https://doi.org/10.1056/NEJMoa1113354
  6. Homma S, Azuma A, Taniguchi H, Ogura T, Mochiduki Y, Sugiyama Y, et al. Efficacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic pulmonary fibrosis. Respirology 2012;17:467-77. https://doi.org/10.1111/j.1440-1843.2012.02132.x
  7. Horton MR, Santopietro V, Mathew L, Horton KM, Polito AJ, Liu MC, et al. Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: a randomized trial. Ann Intern Med 2012;157:398-406. https://doi.org/10.7326/0003-4819-157-6-201209180-00003
  8. Noth I, Anstrom KJ, Calvert SB, de Andrade J, Flaherty KR, Glazer C, et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012;186:88-95. https://doi.org/10.1164/rccm.201202-0314OC
  9. King TE Jr, Brown KK, Raghu G, du Bois RM, Lynch DA, Martinez F, et al. BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011;184:92-9. https://doi.org/10.1164/rccm.201011-1874OC
  10. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011;377:1760-9. https://doi.org/10.1016/S0140-6736(11)60405-4
  11. Azuma A, Taguchi Y, Ogura T, Ebina M, Taniguchi H, Kondoh Y, et al. Exploratory analysis of a phase III trial of pirfenidone identifies a subpopulation of patients with idiopathic pulmonary fibrosis as benefiting from treatment. Respir Res 2011;12:143. https://doi.org/10.1186/1465-9921-12-143
  12. Richeldi L, Costabel U, Selman M, Kim DS, Hansell DM, Nicholson AG, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011;365:1079-87. https://doi.org/10.1056/NEJMoa1103690
  13. Couluris M, Kinder BW, Xu P, Gross-King M, Krischer J, Panos RJ. Treatment of idiopathic pulmonary fibrosis with losartan: a pilot project. Lung 2012;190:523-7. https://doi.org/10.1007/s00408-012-9410-z
  14. Tzouvelekis A, Koliakos G, Ntolios P, Baira I, Bouros E, Oikonomou A, et al. Stem cell therapy for idiopathic pulmonary fibrosis: a protocol proposal. J Transl Med 2011;9:182. https://doi.org/10.1186/1479-5876-9-182

Cited by

  1. The LIM-Only Protein FHL2 Attenuates Lung Inflammation during Bleomycin-Induced Fibrosis vol.8, pp.11, 2013, https://doi.org/10.1371/journal.pone.0081356
  2. CCN5 overexpression inhibits profibrotic phenotypes via the PI3K/Akt signaling pathway in lung fibroblasts isolated from patients with idiopathic pulmonary fibrosis and in an in vivo model of lung fib vol.33, pp.2, 2013, https://doi.org/10.3892/ijmm.2013.1565
  3. Pharmacodynamic and pharmacokinetic assessment of pulmonary rehabilitation mixture for the treatment of pulmonary fibrosis vol.7, pp.None, 2013, https://doi.org/10.1038/s41598-017-02774-1
  4. Overexpression of OSM and IL-6 impacts the polarization of pro-fibrotic macrophages and the development of bleomycin-induced lung fibrosis vol.7, pp.None, 2013, https://doi.org/10.1038/s41598-017-13511-z
  5. Histone Deacetylases Promote ER Stress Induced Epithelial Mesenchymal Transition in Human Lung Epithelial Cells vol.46, pp.5, 2013, https://doi.org/10.1159/000489367
  6. Comparison of Hibiscus sabdariffa L. Extract and Enalapril with Regard to Their Effect on Lung Fibrosis in a Bleomycin-induced Rat Model of Lung Fibrosis vol.13, pp.2, 2013, https://doi.org/10.17795/jjnpp-38798
  7. Evaluation of potential anti-fibrotic effect of oleuropein on bleomycin-induced pulmonary fibrosis in rat vol.39, pp.2, 2013, https://doi.org/10.1080/15569543.2018.1488733
  8. Treatment of chronic fibrosing interstitial lung diseases vol.64, pp.4, 2021, https://doi.org/10.5124/jkma.2021.64.4.277