Treatment of Idiopathic Pulmonary Fibrosis

  • Steven D Nathan
  • A Whitney Brown
  • Christopher S King
Chapter

Abstract

The treatment of idiopathic pulmonary fibrosis (IPF) can be broadly divided into pharmacologic therapies and non-pharmacologic interventions (Chap.  8). There are two agents that have been shown to slow the rate of deterioration in lung function in IPF. These two drugs, pirfenidone and nintedanib, have been variably approved in countries around the world.

Keywords

Obstructive Sleep Apnea Pulmonary Hypertension Lung Function Idiopathic Pulmonary Fibrosis Forced Vital Capacity 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Azuma A, Nukiwa T, Tsuboi E, et al. Double-blind, placebo-controlled trial of pirfenidone in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2005;171:1040–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Taniguchi H, Ebina M, Kondoh Y, et al. Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J. 2010;35:821–9.CrossRefPubMedGoogle Scholar
  3. 3.
    Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377:1760–9.CrossRefPubMedGoogle Scholar
  4. 4.
    King Jr TE, Bradford WZ, Castro-Bernardini S, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2083–92.CrossRefPubMedGoogle Scholar
  5. 5.
    King CS, Nathan SD. Practical considerations in the pharmacologic treatment of idiopathic pulmonary fibrosis. Curr Opin Pulm Med. 2015;21:479–89.CrossRefPubMedGoogle Scholar
  6. 6.
    Potts J, Yogaratnam D. Pirfenidone: a novel agent for the treatment of idiopathic pulmonary fibrosis. Ann Pharmacother. 2013;47:361–7.CrossRefPubMedGoogle Scholar
  7. 7.
    Richeldi L, du Bois RM, Raghu G, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2071–82.CrossRefPubMedGoogle Scholar
  8. 8.
    Richeldi L, Costabel U, Selman M, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365:1079–87.CrossRefPubMedGoogle Scholar
  9. 9.
    Cottin V, Maher T. Long-term clinical and real-world experience with pirfenidone in the treatment of idiopathic pulmonary fibrosis. Eur Respir Rev. 2015;24:58–64.CrossRefPubMedGoogle Scholar
  10. 10.
    Richeldi L, Costabel U, Selman M, et al. Efficacy and safety of nintedanib in patients with IPF beyond week 52: data from the Phase II TOMORROW trial. Am J Respir Crit Care Med. 2015;191:A1019.Google Scholar
  11. 11.
    Nathan SD, Albera C, Bradford WZ, et al. Effect of continued treatment with pirfenidone following a clinically meaningful decline in percent predicted forced vital capacity in patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2015;191:A1016.Google Scholar
  12. 12.
    Raghu G, Collard HR, Egan JJ, 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.CrossRefPubMedGoogle Scholar
  13. 13.
    Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G, Anstrom KJ, King Jr TE, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366:1968–77.CrossRefGoogle Scholar
  14. 14.
    Noth I, Anstrom KJ, Calvert SB, et al. A placebo-controlled randomized trial of warfarin in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2012;186:88–95.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Raghu G, Behr J, Brown KK, Egan JJ, et al. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial. Ann Intern Med. 2013;158:641–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Tzouvelekis A, Bonella F, Spagnolo P. Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag. 2015;11:359–70.PubMedPubMedCentralGoogle Scholar
  17. 17.
    Sherner J, Collen J, King CS, Nathan SD. Pulmonary hypertension in idiopathic pulmonary fibrosis: epidemiology, diagnosis, and therapeutic implications. Curr Respir Care Rep. 2012;1:233–42.CrossRefGoogle Scholar
  18. 18.
    Nathan SD, Shlobin OA, Ahmad S, Urbanek S, Barnett SD. Pulmonary hypertension and pulmonary function testing in idiopathic pulmonary fibrosis. Chest. 2007;131:657–63.CrossRefPubMedGoogle Scholar
  19. 19.
    Raghu G, Nathan SD, Behr J, et al. Pulmonary hypertension in idiopathic pulmonary fibrosis with mild-to-moderate restriction. Eur Respir J. 2015;46:1370–7.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Steven D Nathan
    • 1
  • A Whitney Brown
    • 1
  • Christopher S King
    • 1
  1. 1.Inova Fairfax Medical CampusFalls ChurchUSA

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