Tuberc Respir Dis.  2013 Jan;74(1):1-6.

Recent Advances in Idiopathic Pulmonary Fibrosis

• Park S
• Lee EJ

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.

Keyword

Idiopathic Pulmonary Fibrosis; Etiology; Clinical Trial

MeSH Terms

Acetylcysteine
Asia
Europe
Hand
Idiopathic Pulmonary Fibrosis
Inflammation
Licensure
Pyridones
Sulfonamides
Warfarin
Acetylcysteine
Pyridones
Sulfonamides
Warfarin

Reference

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. PMID: 21471066.

2. King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011; 378:1949–1961. PMID: 21719092.

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. PMID: 21668038.

4. Fernandez IE, Eickelberg O. New cellular and molecular mechanisms of lung injury and fibrosis in idiopathic pulmonary fibrosis. Lancet. 2012; 380:680–688. PMID: 22901889.

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; 366:1968–1977. PMID: 22607134.

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; 17:467–477. PMID: 22257422.

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. PMID: 22986377.

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. PMID: 22561965.

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; 184:92–99. PMID: 21474646.

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; 377:1760–1769. PMID: 21571362.

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. PMID: 22035508.

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; 365:1079–1087. PMID: 21992121.

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; 190:523–527. PMID: 22810758.

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. PMID: 22017817.