Skip to main content

Advertisement

SpringerLink
Log in

Update on New Treatments for Idiopathic Pulmonary Fibrosis

  • COPD (J. Fanikos, Section Editor)
  • Published:
Current Emergency and Hospital Medicine Reports Aims and scope Submit manuscript

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic disorder that results in significant declines in respiratory function and a high mortality rate only a few years after diagnosis. Medical management of IPF has been attempted with various types of medications, such as immunosuppressants, anticoagulants, endothelin receptor antagonists, and anti-inflammatory drugs with less than conclusive results. However, with the approval of nintedanib and pirfenidone following the IMPULSIS-1, IMPULSIS-2, and ASCEND data, there is hope that medical therapy may be able to slow the progression of IPF and decrease the rate of acute exacerbations in this patient population.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  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(6):788–824.

    Article  PubMed  Google Scholar 

  2. 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):355–61.

    Article  PubMed  Google Scholar 

  3. King TE Jr, Schwarz MI, Brown K, Tooze JA, Colby TV, Waldron JA Jr, et al. Idiopathic pulmonary fibrosis: relationship between histopathologic features and mortality. Am J Respir Crit Care Med. 2001;164(6):1025–32.

    Article  PubMed  Google Scholar 

  4. Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I, Roberts RS, et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1(5):369–76.

    Article  PubMed Central  PubMed  Google Scholar 

  5. Lee JS, Ryu JH, Elicker BM, Lydell CP, Jones KD, Wolters PJ, et al. Gastroesophageal reflux therapy is associated with longer survival in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;184(12):1390–4.

    Article  PubMed Central  PubMed  Google Scholar 

  6. King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011;378(9807):1949–61.

    Article  PubMed  Google Scholar 

  7. Bjoraker JA, Ryu JH, Edwin MK, Myers JL, Tazelaar HD, Schroeder DR, et al. Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1998;157(1):199–203.

    Article  CAS  PubMed  Google Scholar 

  8. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–40.

    Article  PubMed  Google Scholar 

  9. Tzouvelekis A, Bonella F, Spagnolo P. Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag. 2015;11:359–70.

    PubMed Central  PubMed  Google Scholar 

  10. Collard HR, Moore BB, Flaherty KR, Brown KK, Kaner RJ, King TE Jr, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2007;176(7):636–43.

    Article  PubMed Central  PubMed  Google Scholar 

  11. Antoniou KM, Wells AU. Acute exacerbations of idiopathic pulmonary fibrosis. Respiration. 2013;86(4):265–74.

    Article  CAS  PubMed  Google Scholar 

  12. Ambrosini V, Cancellieri A, Chilosi M, Zompatori M, Trisolini R, Saragoni L, et al. Acute exacerbation of idiopathic pulmonary fibrosis: report of a series. Eur Respir J. 2003;22(5):821–6.

    Article  CAS  PubMed  Google Scholar 

  13. Parambil JG, Myers JL, Ryu JH. Histopathologic features and outcome of patients with acute exacerbation of idiopathic pulmonary fibrosis undergoing surgical lung biopsy. Chest. 2005;128(5):3310–5.

    Article  PubMed  Google Scholar 

  14. 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(6):604–10.

    Article  CAS  PubMed  Google Scholar 

  15. King TE Jr, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, 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(9685):222–8.

    Article  CAS  PubMed  Google Scholar 

  16. King TE Jr, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, et al. Build-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;177(1):75–81.

    Article  CAS  PubMed  Google Scholar 

  17. Kubo H, Nakayama K, Yanai M, Suzuki T, Yamaya M, Watanabe M, et al. Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest. 2005;128(3):1475–82.

    Article  CAS  PubMed  Google Scholar 

  18. 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(1):88–95.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  19. Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, et al. A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2004;350(2):125–33.

    Article  CAS  PubMed  Google Scholar 

  20. Raghu G, Brown KK, Costabel U, Cottin V, du Bois RM, Lasky JA, et al. Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med. 2008;178(9):948–55.

    Article  CAS  PubMed  Google Scholar 

  21. 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(7):620–8.

    Article  PubMed  Google Scholar 

  22. Alagha K, Secq V, Pahus L, Sofalvi T, Palot A, Bourdin A, et al. We should prohibit warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2015;191(8):958–60.

    Article  CAS  PubMed  Google Scholar 

  23. Peikert T, Daniels CE, Beebe TJ, Meyer KC, Ryu JH. Assessment of current practice in the diagnosis and therapy of idiopathic pulmonary fibrosis. Respir Med. 2008;102(9):1342–8.

    Article  CAS  PubMed  Google Scholar 

  24. Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM, et al. High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2005;353(21):2229–42.

    Article  CAS  PubMed  Google Scholar 

  25. 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(21):1968–77.

    Article  CAS  PubMed  Google Scholar 

  26. Martinez FJ, de Andrade JA, Anstrom KJ, King TE Jr, Raghu G. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2093–101.

    Article  PubMed Central  PubMed  Google Scholar 

  27. Azuma A, Nukiwa T, Tsuboi E, Suga M, Abe S, Nakata K, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2005;171(9):1040–7.

    Article  PubMed  Google Scholar 

  28. 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(2 Pt 1):L311–8.

    CAS  PubMed  Google Scholar 

  29. Misra HP, Rabideau C. Pirfenidone inhibits NADPH-dependent microsomal lipid peroxidation and scavenges hydroxyl radicals. Mol Cell Biochem. 2000;204(1–2):119–26.

    Article  CAS  PubMed  Google Scholar 

  30. 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(4 Pt 1):1061–9.

    Article  CAS  PubMed  Google Scholar 

  31. Taniguchi H, Ebina M, Kondoh Y, Ogura T, Azuma A, Suga M, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35(4):821–9.

    Article  CAS  PubMed  Google Scholar 

  32. 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(9779):1760–9.

    Article  CAS  PubMed  Google Scholar 

  33. •• King TE, Jr., Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083–92. This study was a phase 3 international, multicenter study that evaluated the effects of pirfenidone vs. placebo for 52 weeks in patients with IPF. Patients who received perfenidone had significantly less decline in lung function. There also was improvement in exercise tolerance and progression-free survival.

  34. Hilberg F, Roth GJ, Krssak M, Kautschitsch S, Sommergruber W, Tontsch-Grunt U, et al. BIBF 1120: triple angiokinase inhibitor with sustained receptor blockade and good antitumor efficacy. Cancer Res. 2008;68(12):4774–82.

    Article  CAS  PubMed  Google Scholar 

  35. 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(12):1079–87.

    Article  CAS  PubMed  Google Scholar 

  36. •• Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071–82. The two IMPULSIS studies evaluated the safety and efficacy of nintedanib compared to placebo in patients with confirmed IPF. Both studies showed a beneficial effect of nintedanib on disease progression, and IMPULSIS-2 demonstrated a decrease in the rate of acute exacerbations.

  37. Hunninghake GM. A new hope for idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2142–3.

    Article  CAS  PubMed  Google Scholar 

  38. Sakamoto S, Muramatsu Y, Satoh K, Ishida F, Kikuchi N, Sano G, et al. Effectiveness of combined therapy with pirfenidone and inhaled n-acetylcysteine for advanced idiopathic pulmonary fibrosis: a case-control study. Respirology. 2015;20(3):445–52.

    Article  PubMed  Google Scholar 

  39. Moodley Y, Corte T, Richeldi L, King TE Jr. Do all patients with idiopathic pulmonary fibrosis warrant a trial of therapeutic intervention? A pro-con perspective. Respirology. 2015;20(3):389–94.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Brigham and Women’s Hospital, 75 Francis St, Boston, MA, 02115, USA

    Jeremy R. DeGrado & James F. Gilmore

Authors
  1. Jeremy R. DeGrado
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James F. Gilmore
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jeremy R. DeGrado.

Additional information

This article is part of the Topical Collection on COPD.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

DeGrado, J.R., Gilmore, J.F. Update on New Treatments for Idiopathic Pulmonary Fibrosis. Curr Emerg Hosp Med Rep 3, 134–138 (2015). https://doi.org/10.1007/s40138-015-0076-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40138-015-0076-8

Keywords

Search

Navigation