The treatment of idiopathic pulmonary fibrosis

F1000Prime Reports

Volumn 6, Issue , 2014, Pages

  Woodcock, Hannah V ^a,b   Maher, Toby M ^a,c  

^a ROYAL BROMPTON HOSPITAL   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c IMPERIAL COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; AMBRISENTAN; AZATHIOPRINE; BOSENTAN; COTRIMOXAZOLE; ETANERCEPT; EVEROLIMUS; GAMMA1B INTERFERON; IMATINIB; LEBRIKIZUMAB; MACITENTAN; MONOCLONAL ANTIBODY; NINTEDANIB; PIRFENIDONE; SILDENAFIL; SIMTUZUMAB; STX 100; UNCLASSIFIED DRUG; WARFARIN;

ARTICLE; CLINICAL TRIAL (TOPIC); CORTICOSTEROID THERAPY; FIBROSING ALVEOLITIS; HUMAN; INTERSTITIAL PNEUMONIA; PATHOGENESIS; RANDOMIZED CONTROLLED TRIAL (TOPIC); SCAR FORMATION; SURVIVAL TIME;

EID: 84897499528     PISSN: None     EISSN: 20517599     Source Type: Journal    
DOI: 10.12703/P6-16     Document Type: Article

References (55)

1. Maher TM, Wells AU, Laurent GJ: Idiopathic pulmonary fibrosis: multiple causes and multiple mechanisms? Eur Respir J 2007, 30:835-9.
2. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G: Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006, 174:810-6.
3. Navaratnam V, Fleming KM, West J, Smith CJP, Jenkins RG, Fogarty A, Hubbard RB: The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax 2011, 66:462-7.
4. King TE, Pardo A, Selman M: Idiopathic pulmonary fibrosis. Lancet 2011, 378:1949-61.
5. Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, King TE: A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med 2004, 350:125-33.
6. 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 2001. Am J Respir Crit Care Med 2002, 165:277-304.
7. Luppi F, Spagnolo P, Cerri S, Richeldi L: The big clinical trials in idiopathic pulmonary fibrosis. Curr Opin Pulm Med 2012, 18:428-32.
8. Maher TM: Pirfenidone in idiopathic pulmonary fibrosis. Drugs Today 2010, 46:473-82.
9. Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM, MacNee W, Thomeer M, Wallaert B, Laurent F, Nicholson AG, Verbeken EK, Verschakelen J, Flower CDR, Capron F, Petruzzelli S, Vuyst P de, van den Bosch JMM, Rodriguez-Becerra E, Corvasce G, Lankhorst I, Sardina M, Montanari M: High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005, 353:2229-42.
10. Borok Z, Buhl R, Grimes GJ, Bokser AD, Hubbard RC, Holroyd KJ, Roum JH, Czerski DB, Cantin AM, Crystal RG: Effect of glutathione aerosol on oxidant-antioxidant imbalance in idiopathic pulmonary fibrosis. Lancet 1991, 338:215-6.
11. Kinnula VL, Fattman CL, Tan RJ, Oury TD: Oxidative stress in pulmonary fibrosis: a possible role for redox modulatory therapy. Am J Respir Crit Care Med 2005, 172:417-22.
12. Cantin AM, Hubbard RC, Crystal RG: Glutathione deficiency in the epithelial lining fluid of the lower respiratory tract in idiopathic pulmonary fibrosis. Am Rev Respir Dis 1989, 139:370-2.
13. Behr J, Degenkolb B, Krombach F, Vogelmeier C: Intracellular glutathione and bronchoalveolar cells in fibrosing alveolitis: effects of N-acetylcysteine. Eur Respir J 2002, 19:906-11.
14. Behr J, Maier K, Degenkolb B, Krombach F, Vogelmeier C: Antioxidative and clinical effects of high-dose N-acetylcysteine in fibrosing alveolitis. Adjunctive therapy to maintenance immunosuppression. Am J Respir Crit Care Med 1997, 156:1897-901.
15. Raghu G, Anstrom KJ, King TE, Lasky JA, Martinez FJ: Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012, 366:1968-77.
16. Nakayama S, Mukae H, Sakamoto N, Kakugawa T, Yoshioka S, Soda H, Oku H, Urata Y, Kondo T, Kubota H, Nagata K, Kohno S: Pirfenidone inhibits the expression of HSP47 in TGF-beta1-stimulated human lung fibroblasts. Life Sci 2008, 82:210-7.
17. Oku H, Shimizu T, Kawabata T, Nagira M, Hikita I, Ueyama A, Matsushima S, Torii M, Arimura A: Antifibrotic action of pirfenidone and prednisolone: different effects on pulmonary cytokines and growth factors in bleomycin-induced murine pulmonary fibrosis. Eur J Pharmacol 2008, 590:400-8.
18. Raghu G, Johnson WC, Lockhart D, Mageto Y: Treatment of idiopathic pulmonary fibrosis with a new antifibrotic agent, pirfenidone: results of a prospective, open-label Phase II study. Am J Respir Crit Care Med 1999, 159:1061-9.
19. Nagai S, Hamada K, Shigematsu M, Taniyama M, Yamauchi S, Izumi T: Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis. Intern Med 2002, 41:1118-23.
20. Gahl WA, Brantly M, Troendle J, Avila NA, Padua A, Montalvo C, Cardona H, Calis KA, Gochuico B: Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome. Mol Genet Metab 2002, 76:234-42.
21. Azuma A, Nukiwa T, Tsuboi E, Suga M, Abe S, Nakata K, Taguchi Y, Nagai S, Itoh H, Ohi M, Sato A, Kudoh S: Doubleblind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005, 171:1040-7.
22. Taniguchi H, Ebina M, Kondoh Y, Ogura T, Azuma A, Suga M, Taguchi Y, Takahashi H, Nakata K, Sato A, Takeuchi M, Raghu G, Kudoh S, Nukiwa T: Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010, 35:821-9.
23. Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, King TE, Lancaster L, Sahn SA, Szwarcberg J, Valeyre D, Du Bois RM: Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011, 377:1760-9.
24. Spagnolo P, Del Giovane C, Luppi F, Cerri S, Balduzzi S, Walters EH, D'Amico R, Richeldi L: Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2010:CD003134.
25. Adamali HI, Maher TM: Current and novel drug therapies for idiopathic pulmonary fibrosis. Drug Des Devel Ther 2012, 6:261-72.
26. Woodcock HV, Molyneaux PL, Maher TM: Reducing lung function decline in patients with idiopathic pulmonary fibrosis: potential of nintedanib. Drug Des Devel Ther 2013, 7:503-10.
27. Chaudhary NI, Roth GJ, Hilberg F, Müller-Quernheim J, Prasse A, Zissel G, Schnapp A, Park JE: Inhibition of PDGF, VEGF and FGF signalling attenuates fibrosis. Eur Respir J 2007, 29:976-85.
28. Richeldi L, Costabel U, Selman M, Kim DS, Hansell DM, Nicholson AG, Brown KK, Flaherty KR, Noble PW, Raghu G, Brun M, Gupta A, Juhel N, Klüglich M, Du Bois RM: Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011, 365:1079-87.
29. Maher TM: Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment. Clin Chest Med 2012, 33:69-83.
30. Barry-Hamilton V, Spangler R, Marshall D, McCauley S, Rodriguez HM, Oyasu M, Mikels A, Vaysberg M, Ghermazien H, Wai C, Garcia CA, Velayo AC, Jorgensen B, Biermann D, Tsai D, Green J, Zaffryar-Eilot S, Holzer A, Ogg S, Thai D, Neufeld G, van Vlasselaer P, Smith V: Allosteric inhibition of lysyl oxidase-like-2 impedes the development of a pathologic microenvironment. Nat Med 2010, 16:1009-17.
31. Chien JW, Richards TJ, Gibson KF, Zhang Y, Lindell KO, Shao L, Lyman SK, Adamkewicz JI, Smith V, Kaminski N, O'Riordan T: Serum lysyl oxidase like-2 levels and idiopathic pulmonary fibrosis disease progression. Eur Respir J 2013.
32. Akiri G, Sabo E, Dafni H, Vadasz Z, Kartvelishvily Y, Gan N, Kessler O, Cohen T, Resnick M, Neeman M, Neufeld G: Lysyl oxidase-related protein-1 promotes tumor fibrosis and tumor progression in vivo. Cancer Res 2003, 63:1657-66.
33. Hancock A, Armstrong L, Gama R, Millar A: Production of interleukin 13 by alveolar macrophages from normal and fibrotic lung. Am J Respir Cell Mol Biol 1998, 18:60-5.
34. Belperio JA, Dy M, Burdick MD, Xue YY, Li K, Elias JA, Keane MP: Interaction of IL-13 and C10 in the pathogenesis of bleomycin-induced pulmonary fibrosis. Am J Respir Cell Mol Biol 2002, 27:419-27.
35. Corren J, Lemanske RF, Hanania NA, Korenblat PE, Parsey MV, Arron JR, Harris JM, Scheerens H, Wu LC, Su Z, Mosesova S, Eisner MD, Bohen SP, Matthews JG: Lebrikizumab treatment in adults with asthma. N Engl J Med 2011, 365:1088-98.
36. Akhurst RJ, Hata A: Targeting the TGFb signalling pathway in disease. Nat Rev Drug Discov 2012, 11:790-811.
37. Horan GS, Wood S, Ona V, Li DJ, Lukashev ME, Weinreb PH, Simon KJ, Hahm K, Allaire NE, Rinaldi NJ, Goyal J, Feghali-Bostwick CA, Matteson EL, O'Hara C, Lafyatis R, Davis GS, Huang X, Sheppard D, Violette SM: Partial inhibition of integrin alpha(v)beta6 prevents pulmonary fibrosis without exacerbating inflammation. Am J Respir Crit Care Med 2008, 177:56-65.
38. Raghu G, Collard HR, Anstrom KJ, Flaherty KR, Fleming TR, King TE, Martinez FJ, Brown KK: Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. Am J Respir Crit Care Med 2012, 185:1044-8.
39. Vancheri C, Du Bois RM: A progression-free end-point for idiopathic pulmonary fibrosis trials: lessons from cancer. Eur Respir J 2013, 41:262-9.
40. Du Bois RM, Nathan SD, Richeldi L, Schwarz MI, Noble PW: Idiopathic pulmonary fibrosis: lung function is a clinically meaningful endpoint for phase III trials. Am J Respir Crit Care Med 2012, 186:712-5.
41. Spagnolo P, Luppi F, Maher TM, Wuyts WA, Grutters JC: Primary endpoints in phase 3 clinical trials in idiopathic pulmonary fibrosis: one step at a time. AmJ Respir Crit CareMed 2013, 187:1271-2.
42. Wells AU, Behr J, Costabel U, Cottin V, Poletti V, Richeldi L: Hot of the breath: mortality as a primary end-point in IPF treatment trials: the best is the enemy of the good. Thorax 2012, 67:938-40.
43. Du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, Lancaster L, Noble PW, Raghu G, Sahn SA, Szwarcberg J, Thomeer M, Valeyre D, King TE: Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011, 184:459-66.
44. Maher TM: PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery. Eur Respir Rev 2013, 22:148-52.
45. Noth I, Anstrom KJ, Calvert SB, Andrade J de, Flaherty KR, Glazer C, Kaner RJ, Olman MA: A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2012, 186:88-95.
46. Malouf MA, Hopkins P, Snell G, Glanville AR: An investigatordriven study of everolimus in surgical lung biopsy confirmed idiopathic pulmonary fibrosis. Respirology 2011, 16:776-83.
47. King TE, Behr J, Brown KK, Du Bois RM, Lancaster L, Andrade JA de, Stahler G, Leconte I, Roux S, Raghu G: BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008, 177:75-81.
48. King TE, Brown KK, Raghu G, Du Bois RM, Lynch DA, Martinez F, Valeyre D, Leconte I, Morganti A, Roux S, Behr J: BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2011, 184:92-9.
49. Raghu G, Behr J, Brown KK, Egan JJ, Kawut SM, Flaherty KR, Martinez FJ, Nathan SD, Wells AU, Collard HR, Costabel U, Richeldi L, Andrade J de, Khalil N, Morrison LD, Lederer DJ, Shao L, Li X, Pedersen PS, Montgomery AB, Chien JW, O'Riordan TG: Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med 2013, 158:641-9.
50. Raghu G, Million-Rousseau R, Morganti A, Perchenet L, Behr J: Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J 2013, 42:1622-32.
51. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, Hunninghake GW: A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010, 363:620-8.
52. King TE, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, Noble PW, Sahn SA, Szwarcberg J, Thomeer M, Valeyre D, Du Bois RM: Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009, 374:222-8.
53. Raghu G, Brown KK, Costabel U, Cottin V, Du Bois RM, Lasky JA, Thomeer M, Utz JP, Khandker RK, McDermott L, Fatenejad S: Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med 2008, 178:948-55.
54. Daniels CE, Lasky JA, Limper AH, Mieras K, Gabor E, Schroeder DR: Imatinib treatment for idiopathic pulmonary fibrosis: Randomized placebo-controlled trial results. Am J Respir Crit Care Med 2010, 181:604-10.
55. Shulgina L, Cahn AP, Chilvers ER, Parfrey H, Clark AB, Wilson ECF, Twentyman OP, Davison AG, Curtin JJ, Crawford MB, Wilson AM: Treating idiopathic pulmonary fibrosis with the addition of co-trimoxazole: a randomised controlled trial. Thorax 2013, 68:155-62.