Emerging potential treatments: New hope for idiopathic pulmonary fibrosis patients?

European Respiratory Review

Volumn 20, Issue 121, 2011, Pages 201-207

  Costabel, U ^a  

^a RUHRLANDKLINIK   (Germany)

Author keywords

Idiopathic pulmonary fibrosis; Meta analysis; N acetylcysteine; Pirfenidone; Progression free survival; Vital capacity

Indexed keywords

ACETYLCYSTEINE; AZATHIOPRINE; BOSENTAN; ETANERCEPT; GAMMA1B INTERFERON; IMATINIB; PIRFENIDONE; PLACEBO; PREDNISONE; SILDENAFIL;

6 MINUTE WALK TEST; ANTIINFLAMMATORY ACTIVITY; DIARRHEA; DISEASE COURSE; DOSE RESPONSE; DRUG DOSE COMPARISON; DRUG EFFICACY; DRUG MEGADOSE; DRUG SAFETY; DRUG TOLERABILITY; DRUG WITHDRAWAL; EXERCISE TEST; FIBROSING ALVEOLITIS; FORCED VITAL CAPACITY; GASTROINTESTINAL SYMPTOM; HUMAN; LOW DRUG DOSE; LUNG FUNCTION; META ANALYSIS (TOPIC); MULTICENTER STUDY (TOPIC); NAUSEA; NONHUMAN; PHASE 2 CLINICAL TRIAL (TOPIC); PHASE 3 CLINICAL TRIAL (TOPIC); PHOTOSENSITIVITY; PROGNOSIS; PROGRESSION FREE SURVIVAL; RANDOMIZED CONTROLLED TRIAL (TOPIC); RASH; REVIEW;

ANIMALS; CLINICAL TRIALS AS TOPIC; EVIDENCE-BASED MEDICINE; HUMANS; IDIOPATHIC PULMONARY FIBROSIS; LUNG; PYRIDONES; RESPIRATORY SYSTEM AGENTS; TREATMENT OUTCOME;

EID: 80052536198     PISSN: 09059180     EISSN: 16000617     Source Type: Journal    
DOI: 10.1183/09059180.00002011     Document Type: Review

References (37)

1. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 2000; 161: 646-664.
2. Hodgson U, Laitinen T, Tukiainen P. Nationwide prevalence of sporadic and tamilial idiopathic pulmonary fibrosis: evidence of founder effect among multiplex families in Finland. Thorax 2002; 57: 388.
3. Orphanet. Orphanet Report Series Rare Diseases Collection. Prevalence of rare diseases: bibliographic data. May 2011 Number 1. www.orphanet.net
4. American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002; 165: 277-304.
5. Vancheri C, Failla M, Crimi N, et al. Idiopathic pulmonary fibrosis: a disease with similarities and links to cancer biology. Eur Respir J 2010; 35: 496-504.
6. Olson AL, Swigris JJ, Lezotte DC, et al. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med 2007; 176: 277-284.
7. Harari S, Caminati A. Update on diffuse parenchymal lung disease. Eur Respir Rev 2010; 19: 97-108.
8. Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63: Suppl. 5, v1-v58.
9. Raghu G, Collard HR, Egan J, et al. An Official ATS/ERS/JRS/ALAY Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management. Am J Respir Crit Care Med 2011; 183: 788-824.
10. du Bois RM. Strategies for treating idiopathic pulmonary fibrosis. Nat Rev Drug Discov 2010; 9: 129-140.
11. Meltzer EB, Noble PW. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008; 3: 8.
12. Selman M, King TE, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med 2001; 134: 136-151.
13. King TE, Behr J, Brown KK, et al. BUILD-1: a randomized placebocontrolled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2008; 177: 75-81.
14. Daniels CE, Lasky JA, Limper AH. Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med 2010; 181: 604-610.
15. Zisman DA, Schwarz M, Anstrom KJ, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010; 363: 620-628.
16. Raghu G, Brown KK, Costabel U, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebocontrolled trial. Am J Respir Crit Care Med 2008; 178: 948-955.
17. King TE, Albera C, Bradford WZ, et al. Effect of interferon gamma- 1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 2009; 374: 222-228.
18. Demedts M, Behr J Buhl R, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 2005; 353: 2229-2242.
19. Behr J, Demedts M, Buhl R, et al. Lung function in idiopathic pulmonary fibrosis - extended analyses of the IFIGENIA trial. Respir Res 2009; 10: 101-109.
20. 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.
21. ClinicalTrials.gov. Evaluating the effectiveness of prednisone, azathioprine, and N-acetylcysteine in people with idiopathic pulmonary fibrosis (PANTHER-IPF). Identifier NCT00650091. http://clinicaltrials.gov/ct2/home
22. Iyer SN, Gurujeyalakshmi G, Giri SN. Effects of pirfenidone on transforming growth factor-beta gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. J Pharmacol Exp Ther 1999; 291: 367-373.
23. Gurujeyalakshmi G, Hollinger MA, Giri SN. Pirfenidone inhibits PDGF isoforms in bleomycin hamster model of lung fibrosis at the translational level. Am J Physiol 1999; 276: L311-L318.
24. Iyer SN, Gurujeyalakshmi G, Giri SN. Effects of pirfenidone on procollagen gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. J Pharmacol Exp Ther 1999; 289: 211-218.
25. Oku H, Shimizu T, Kawabata T, et al. 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: 400-408.
26. Raghu G, Johnson WC, Lockhart D, et al. 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: 1061-1069.
27. Nagai S, Hamada K, Shigematsu M, et al. Open-label compassionate use one year-treatment with pirfenidone to patients with chronic pulmonary fibrosis. Intern Med 2002; 41: 1118-1123.
28. Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebocontrolled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2005; 171: 1040-1047.
29. Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 2010; 35: 821-829.
30. Noble PW, Albera C, Bradford W, et al. The CAPACITY (CAP) trials: randomized, double-blind, placebo-controlled, phase III trials of pirfenidone (PFD) in patients with idiopathic pulmonary fibrosis (IPF). 2009 American Thoracic Society International Conference (San Diego, CA, USA). Am J Respir Crit Care Med 2009; 179: A1129.
31. du Bois R, Albera C, Bradford W, et al. Pirfenidone treatment for idiopathic pulmonary fibrosis (IPF): a comprehensive analysis of safety. Eur Respir J 2009; 34: Suppl. 53, 494s.
32. Costabel U, Albera C, Bradford W, et al. Pirfenidone dose-response in patients with idiopathic pulmonary fibrosis (IPF). A comprehensive analysis of outcomes in CAPACITY 2. Eur Respir J 2010; 36: Suppl. 54, 41s.
33. Albera C, Bradford W, Costabel U, et al. The magnitude of pirfenidone treatment effect in patients with idiopathic pulmonary fibrosis (IPF): a pooled analysis of outcomes in the CAPACITY (CAP) studies. Eur Respir J 2010; 36: Suppl. 54, 41s.
34. Karimi-Shah BA. Pulmonary-Allergy Drugs Advisory Committee (PADAC): Pirfenidone Capsules NDA 22-535, S-000. U.S. Food and Drug Administration. www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs/Pulmonary-AllergyDrugsAdvisoryCommittee/UCM206398.pdf
35. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY: two randomised trials). Lancet 2011; 377: 1760-1769.
36. Spagnolo P, Del Giovane C, Luppi F, et al. Non-steroid agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev 2010; 9: CD003134.
37. du Bois R, Albera C, Bradford W, et al. Predictors of mortality in patients with idiopathic pulmonary fibrosis (IPF). 2009 American Thoracic Society International Conference (San Diego, CA, USA). Am J Respir Crit Care Med 2009; 179: A1114.