4.7 Review

Idiopathic pulmonary fibrosis: Recent advances on pharmacological therapy

Journal

PHARMACOLOGY & THERAPEUTICS
Volume 152, Issue -, Pages 18-27

Publisher

PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.pharmthera.2015.04.005

Keywords

Clinical trials; Idiopathic pulmonary fibrosis; Nintedanib; Pirfenidone; Therapy; Treatment

Funding

  1. InterMune
  2. Boehringer Ingelheim
  3. GSK
  4. UCB
  5. Novartis
  6. Bayer
  7. Biogen
  8. ProMedior
  9. Lanthio
  10. Roche
  11. Takeda
  12. Sanofi-Aventis
  13. AstraZeneca
  14. Gilead
  15. Celgene
  16. Shionogi
  17. Biogen Idec
  18. MedImmune
  19. ImmuneWorks

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Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias with an estimated 5-year survival of approximately 20%. In the last two decades our understanding of disease pathogenesis has substantially evolved and novel compounds have been developed consequent to the increasing knowledge of the mechanisms underlying disease pathobiology. The disease appears to be driven - following chronic injury - by abnormal/dysfunctional alveolar epithelial cells that promote fibroblast recruitment and proliferation, resulting in scarring of the lung and irreversible loss of function. With very few exceptions, clinical trials evaluating novel potential therapies have provided disappointing results. More recently, pirfenidone-and nintedanib, two compounds with pleiotropic mechanisms of action, have proven effective in slowing functional decline and disease progression in IPF patients with mild to moderate functional impairment, highlighting the importance of timely diagnosis and administration of treatment in early stages of disease. However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and risks. This article provides an overview of the most recent clinical trials in IPF and discusses how their results are going to change the clinical and clinical research landscape in IPF. A number of agents with high potential are currently being tested and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF. (C) 2015 Elsevier Inc. All rights reserved.

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